# BIGPATS_DOC_STORE

CONTENT:
Loocie Sanders, M.D.          RE: Maxine Warren
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/02/91

Dear Dr. Sanders:

We had the pleasure of seeing Maxine in the Renal Transplant Program on Tuesday, September 3, 1996.  She has chronic renal failure secondary to glomerular cystic disease.  She had a renal transplant on 9/3/92.

Maxine's parents offer no complaints today.  She continues to have nocturnal enuresis.  On review it appears that she goes to the bathroom every hour when she is awake.  She is able to hold the urine for an extended period of time during long trips.

Her current medications include:
     IMURAN 25 mg po q.d.
     SANDIMMUNE 60 mg po t.i.d.
     PREDNISONE 10 mg q.o.d.


Maxine's physical exam was notable for:
     BP 106/63   P 114   R 16   HT 108.3cm   WT 19.1kg   
     General: well developed, well nourished in no distress
     Skin: no lesions
     HEENT: within normal limits
     Neck: supple without lymphadenopathy
     Chest: clear to auscultation
     Heart: regular rate and rhythm, no murmur
     Abdomen: soft without tenderness nor bruits at graft site
     Extremities: no edema
     Neurological: no lateralizing signs

Laboratory studies at this time include:
          K           4.5  MMOL/L       MCHC       34.9    G/DL    
          CA          9.8   MG/DL       RDW        15.2       %    
          CR          1.0   MG/DL (H)   HDW        2.83    G/DL    
          CYA         105   MCG/L       MPV         7.3      FL    
          GLU          77   MG/DL       PLT UNC     443   K/MM3 (H)
          PHOS        3.9   MG/DL (L)   NEUT AB    5.50   K/MM3    
          BUN          41   MG/DL (H)   LYMPH A    3.74   K/MM3    
          NA          140  MMOL/L       EOS ABS     .11   K/MM3    
          CL          106  MMOL/L       NEUT-A     54.9       %    
          CO 2         24   MEQ/L       LYMPH-A    37.3       %    
          WBC       10.02   K/MM3       MONO-A      4.5       %    
          RBC        4.07   M/MM3       EOS-A       1.1       %    
          HGB        11.3    G/DL       BASO-A       .7       %    
          HCT.       32.4       % (L)   ATYP LY     1.5       %    
          MCV        79.5      FL       MICRO-A       +    
          MCH        27.8      PG       LEFT SH  ABSENT    

Maxine has stable renal function.  Her nocturnal enuresis may be related to voiding habits because she does have a normal bladder capacity and is able to hold her urine for extended periods of time when desired.  We will discuss with them that she should learn to hold herself from voiding during weekends and try to prolong the intervals between urination.  We will try to avoid giving medications as of now and try behavioral modification.

Thank you for the opportunity to participate in Maxine's care.  We would like to review her in three months. In the interim, we would be happy to discuss or review Maxine should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Loocie Sanders, M.D.          RE: Maxine Warren
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/02/91

Dear Dr. Sanders:

We had the pleasure of seeing Maxine in the Renal Transplant Program on Tuesday, December 3, 1996.  She has chronic renal failure secondary to glomerular cystic disease.  She had a renal transplant on 9/3/92.

Maxine continues to do well and offers no complaints today.  She continues to have nocturnal bedwetting, however, remains dry during the day.

Her current medications include:
     IMURAN 25 mg po q.d.
     SANDIMMUNE 60 mg po t.i.d.
     PREDNISONE 10 mg q.o.d.


Maxine's physical exam was notable for:
     BP 127/51   P 114   HT 110.3cm   WT 19.9kg   
     General: well appearing, in no distress
     Skin: no lesions
     HEENT: within normal limits
     Neck: supple without lymphadenopathy
     Chest: clear to auscultation
     Heart: regular rate and rhythm, no murmur
     Abdomen: soft without tenderness nor bruits at graft site
     Extremities: no edema
     Neurological: no lateralizing signs

A urinalysis revealed:
     SPEC GR UA  1.011           WBC/HPF     2-3        
     WBC ENZ UA  N               RBC/HPF     N          
     PH UA       5.0             WBC CAST    0          
     PROTEIN UA  N               RBC CAST    0          
     BLOOD UA    N               GRAN CAST   0          
     GLUCOSE UA  N               WAXY CAST   0          
     BACTERIA    N               SQUAM EPI   few   
     MICROSCOPC             
0-1 hyaline cast

Laboratory studies at this time include:
          K           5.1  MMOL/L (H)   MCHC       34.9    G/DL    
          CA          9.7   MG/DL       RDW        14.9       %    
          CR          0.8   MG/DL (H)   HDW        2.93    G/DL    
          CYA          98   MCG/L       MPV         9.0      FL    
          GLU          72   MG/DL       PLT UNC     415   K/MM3 (H)
          PHOS        3.5   MG/DL (L)   NEUT AB    7.10   K/MM3    
          BUN          30   MG/DL (H)   LYMPH A    1.00   K/MM3    
          NA          135  MMOL/L       EOS ABS     .08   K/MM3    
          CL          105  MMOL/L       NEUT-A     81.2       % (H)
          CO 2         22   MEQ/L       LYMPH-A    11.5       % (L)
          WBC        8.74   K/MM3       MONO-A      4.1       %    
          RBC        4.16   M/MM3       EOS-A        .9       %    
          HGB        11.5    G/DL       BASO-A       .2       %    
          HCT.       32.9       % (L)   ATYP LY     2.0       %    
          MCV        79.1      FL       MICRO-A       +    
          MCH        27.6      PG       LEFT SH  ABSENT    

Maxine continues to do well with stable renal function.

Thank you for the opportunity to participate in Maxine's care.  We would like to review her in three months. In the interim, we would be happy to discuss or review Maxine should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

We had the pleasure of seeing Hobart in the Endocrine Program on 11/17/94 for .

History: We had the pleasure of seeing Hobart in Endocrine Clinic on November 17, 1994 for evaluation of "growth problem" well defined by the growth data you so kindly forwarded at the time of referral.

The patient's father reported that he initially had grown normally beginning to show decreased growth velocity in height at about 4 years of age.  A bone age done here at that time was read as 4.5 years.   The patient has continued to do well but is now the smallest in his class and, thus, has been referred for further evaluation.  Otherwise, Hobart is doing well developmentally; he is in preschool, knows his colors, age and counts well.   Nutritionally, the patient's father reports he is not a big eater but he does eat vegetables and all four food groups. There is a history of occasional constipation treated by increased juice which is currently not a problem.

Current medications: none.

Past medical history:  The patient was born by emergency C-section secondary to failure to progress with Brom for 20 hours.  Sepsis work up was negative. The patient was home in 5-7 days with his mother.  Birth weight was 8 pounds 3 ounces.  No history of infectious diseases including no episodes of otitis media.  The patient did have toxic synovitis diagnosed at 18 months.  He has had two episodes necessitating ER visits here; a 1.5 cm laceration to the forehead treated on 5/20/93 with no signs of internal head trauma and an episode of leg pain with negative exam on 5/2/91.  No other history of injuries.  Review of systems is otherwise negative.

Family history:  Positive for two brothers ages 4 months and 2.5 years who are both well.  Dad has a history of allergies and has a history of delayed onset of growth spurt at 18 years old.  There is also a history of Paternal grandmother has a history of a "thyroid problem" requiring surgery.   Mother's height is 5'0", dad's height is 5'8".

Physical exam:  
Height: 96.3 cm         Weight: 16.9 kg         BP: 80/60               
Pulse: 80               R. testis: 1.9 x 1 cm   L. testis: 2 x 1 cm     
Phallus length: 9.3 cm  Mid-shaft diam.: 1.4 cm 
Height: 96.3 cm.   Weight 16.9 kg.  Blood pressure: 80/60   Pulse 80  Right Testicle: 2x1 cm  Left Testicle: 1.9x1 cm Penis: stretch length 9.3 cm,  mid-shaft diameter 1.4 cm
 
General: Appears well but is short for age with weight 2.4 kg[16%]> average for height.  No dysmorphic features.  Skin: clear.  HEENT: Negative including fundi.  Neck: Supple.  Thyroid not felt.  No GGE.  Chest: Clear to auscultation.  Cardiac: Regular rhythm without murmur.  Abdomen: Soft, nontender, nondistended, positive bowel sounds.  No hepatosplenomegaly or masses. GU: Normal immature male.  See measurements above. Extremities: Free range of motion.  No deformities.  Neurologic: Nonfocal.  Reflexes physiological.

Laboratory studies:  Bone Age: 4.5 years at chronological age of 4 years.

Scheduled for neuroendocrine function tests (insulin-glucagon-TRH) 12/14/94;  Head MRI 12/5/94;  X-ray hand and wrist 12/5/94.

Impression: Short stature with decreased growth velocity probably due to growth hormone deficiency.  Etiology undefined at present.

Disposition: The above opinion was discussed in detail with Mr. Browne.  Above studies will be carried out as schedule.  Will contact you and the family when results are available to discuss further management.
Thank you for letting us share in the care of Hobart.

Sincerely,


Gleide Swartzenegger, MD              Mitsuko Simpson, MD             
                             Chief Emeritus               


Problem list to date:
SHORT-STATURE                                12/01/94  
HYPOPITUITARISM                              12/01/94  
GH-DEFICIENCY                                12/01/94  
Transcribed by Sullivan_ma


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

We had the pleasure of seeing Hobart in the Endocrine Program on 12/14/94 for .

History: Enclosed please find the complete report of the insulin-glucagon-TRH tests carried out on Hobart on 12/14/94.  By these tests, there is no evidence of pituitary dysfunction which would explain his very significant decreased growth velocity over the last 18 months as defined by your growth data.

As you know, an MRI scan of the brain and hypothalamic-pituitary area on 12/5/94 was reported to show a "small pituitary gland" but otherwise was normal.  An x-ray of the hand and wrist on the same day was reported to show a bone age of 4 3/12 years but when I reviewed this and the x-ray taken 12/93 at your request (read by Mitsuko Kirkpatrick as showing a bone age of 4 6/12 years and commenting on the less mature carpal and ulna-radius epiphysis), I also noted the spread between centers with phalanges and metacarpals near 5 years and the others < 3 years.  The point of all this is that either something interrupted maturation at a critical time for the carpal, ulna-radius centers or there is some anomaly in the development of epiphysis which accounts for this scatter.  Usually with growth hormone deficiency, bone maturation is delayed whereas in Hobart, it is comparable to his chronological age, a finding more often observed with primary skeletal disorders resulting in short stature.

In any event, I do not have a good explanation for the decreased growth velocity of the last 18 months or so in Hobart.  When we saw him in November, I recognized no dsymorphic features such as might be associated with primary skeletal disorders such as chondrodystrophies and we did not measure body proportions at that time.  When he had lower extremities radiologically examined around 18 months of age because of fever with left hip pain, no abnormalities of his skeleton were noted by anyone and are not evident on review now.  Nevertheless, in light of his subsequent course, further films of his skeleton (especially, vertebrae and lower extremities) maybe warranted in an effort to explain the change in growth velocity.  Hobart appears to be such a well adjusted child that I find it difficult to believe that he experienced a functional decrease in growth hormone production such as seen in psychosocial growth retardation.  But, then again, maybe such a functional change occurred for some other reason that we did not discern in our history.  If this is the case and the cause is gone, he should show improved growth velocity in the future and return to his genetic potential for height.

For the immediate future, I should recommend only follow-up in 3 months at which time we can determine whether there has been an improvement in growth velocity.  If it does not improve, further evaluations depending upon his findings at the time will be in order.  I have shared the above thoughts with Mr. Browne.

Physical exam:  




Thank you for letting us share in the care of Hobart.

Sincerely,


Mitsuko Simpson, MD             
Chief Emeritus


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

We had the pleasure of seeing Hobart in the Endocrine Program on 3/9/95 for .

History: I saw Hobart and his parents in the Endocrine Program on 3/9/95 for further evaluation of his growth and development and discussion of the laboratory studies obtained in December 1994.

Interval History:  Aside from a minor sledding accident in mid-February where he jammed his leg agaionst a rock, his interim history is negative.  An x-ray and orthopedic evaluation in the CHER revealed no significant injury to the leg and he, subsequently, has recovered function completely.  Measurement of the parents at this visit revealed that Mr. Browne was 172.9 cm (68.1") and Mrs Browne 150.4 cm (59.2") which gives a midparental height (target height) of 168.15 cm (66.2").

Current medications: None.

Physical exam:  
Height: 97.6 cm         Weight: 17 kg           BP: 98/64               
Pulse: 100              U/L ratio:  1.17        Arm span: 97            
Breast: Tanner I        Pubic hair: Tanner I    R. testis: 2 x 1 cm     
L. testis: 2 x 1 cm     Phallus length: 5.3 cm  Mid-shaft diam.: 1.4 cm 
Height 97.6 cm  Length 98.4 cm  Weight 17 kg P 100  BP 98/64  LS 45 cm  U/L 1.17  Arm Span 97 cm  Breast and sexual hair Tanner I  Penis 5.3 x 1.4 cm (SL x MSD)  Testes: R & L 2 x 1 cm
General: Appears well with a GV of 4.2 cm since last visit. Weight appropriate for height.  Skin: normal.  EENT negative including fundi.  Tonsils moderately enlarged.  Neck: thyroid not felt.  No GGE. Chest: resp regular. Lungs: clear.  Heart: normal.  Abdomen: No organomegaly or masses.  Genitals: normal immature male.  Extremities: no deformities. Neurological: reflexes physiological.

Laboratory studies:  None.

Impression: Familial short stature with a significantly slower growth velocity since 3 years.  Perhaps, increasing slightly since last visit.

Disposition: Reviewed findings (clinical and laboratory) to date with parents.  If patient establishes a normal growth velocity for current height in the future, no further studies or therapeutic intervention will be needed.  If decreased GV persist, other studies including overnight monitoring of neuroendocrine hormone production may be desireable to define function prior to considering any intervention with hormonal therapy.  In light of the findings to date, I suspect in the future he may very well follow a growth pattern consistent with his genetic potential but we shall have to wait to find this out.  Since I shall be retiring on 4/30/95, I took the liberty of introducing the Brownes to Dr. Sally Radovick who will be happy to follow Hobart in the future if it is agreeable to you and the Brownes.  I believe we should continue to follow him at 4 month intervals until his subsequent growth pattern is clearly defined.
Thank you for letting us share in the care of Hobart.

Sincerely,


Mitsuko Simpson, MD             
Chief Emeritus


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

Hobart is a 5 and 6/12 year old who was seen 7/3/95 for short stature.  He has been seen previously by Dr. Crigler.  After 3 years, he fell from ~ 50th percentile to now where he is growing at a delayed pace.  A full pituitary growth evaluation was performed and was normal.  Concern was raised on the last visit when Hobart had grown ~ 1 cm. over 3 months.  However, if you consider his target height as 66 cm. (based on mid-parentals), he is growing along the appropriate curve.

Physical exam:  
Height: 99.5 cm         Weight: 17 kg           Pubic hair: Tanner I    
General:  Alert and cooperative child.
Skin:  Normal turgor and texture. 
HEENT:  PERRLA, fundi normal.
Neck:  Thyroid normal size and consistency.
Chest: Lungs clear. 
Heart:  Regular rate and rhythm.
Abdomen:  Soft, nontender, no hepatosplenomegaly, no masses.
Genitourinary:  Prepubertal. 
Neurological:  DTR 2+/4+ equal bilaterally.

Impression: Hobart has grown 4 cm. over the last six months which is excellent.  After discussing many of the points that Dr. Crigler has previously made, we will continue to follow Hobart clinically.

Disposition: I will see Hobart in six months and obtain a bone age at that time.

Thank you for letting mr share in the care of Hobart.


Sincerely,




Ariel Adler, MD           
Attending in Endocrinology


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

Hobart is a 5 and 11/12 year old who is being seen today for short stature.   Previous testing for physiologic etiology has been entirely negative.   In the interval history since I saw him in July of 1995, he has had no  problems.  He is doing well in school and actually beginning to read.   Hobart is having problems with both fine motor and gross motor skills as  evaluated by the school, and some remedial programs have been suggested.   Some of this may, again, be due to his short stature, and I have asked  the parents to investigate this further.  He is on no medications.

Physical exam:
Height: 101.9 cm    Weight: 18.1 cm         Bone age: 5 years
BP: 96/66           Pulse: 90               
Breast: Tanner I        Pubic hair: Tanner I    
He is an alert  and cooperative child.  Skin: slightly dry on the arms.  HEENT: pupils are equal and equally reactive to light and  accommodation.  Extraocular movements are intact and fundi are normal.   Neck: thyroid has a normal size and consistency.  Lungs are clear.  Abdomen: soft and non-tender.  He has no hepatosplenomegaly and no  masses.  GU: he is  prepubertal.  Testicular volume is 2 cc bilaterally.  Neurologic: deep tendon reflexes are  2+/4+ and equal bilaterally.

Impression: Hobart's growth velocity is about 5.6 cm over the last year,  thus he is paralleling the delayed growth curve.  His weight has  increased 1 kg over the last six months after not having changed much  over the previous six to eight months.  There were no studies ordered  today.

Disposition: Since Hobart is showing a normal growth velocity and a good weight  gain, we would do no further testing, but continue to follow him, and we  have asked that he return to clinic in six months.   

Again, thank you for allowing us to share in the care of Hobart.


Sincerely,




Ariel Adler, MD           
Attending in Endocrinology


CONTENT:
Hooan Domingo, M.D.          RE: Hobart Browne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/30/89

Dear Dr. Domingo:

I saw Hobart in the Endocrine Program on 6/3/96 for short stature.

History: As you know, Hobart is now a 6 and 5/12 year old being seen for short stature.  Mom has noted that Hobart has had an increased appetite over the last six months.  He is healthy, except for occasional Strep throat.  Physically he is very active.  He is currently in adaptive gym to help correct some coordination difficulties he has had.  He is doing very well in kindergarten.  In addition, his eyesight has stabilized according to his Ophthalmologist.  Mom does note that she has had to buy Hobart new shoes recently and does think he has been doing some growing.

Current medications: None.

Physical exam:
Height: 105.2 cm        Weight: 20.1 kg
Pubic hair: Tanner I    R. testis: 2 x 2 cm     L. testis: 2 x 2 cm     
General appearance:  He is alert and cooperative.  His skin has normal turgor and texture.  HEENT:  Pupils are equal and equally reactive to light and accommodation.  Extraocular movements are intact.  Fundi are normal.  His abdomen is soft, nontender, no hepatosplenomegaly, no masses.  GU:  Normal prepubertal male.  Neurologic:  Deep tendon reflexes are 2+/4+ and equal bilaterally.

Laboratory studies:  IGF-I  73   ng/mL (80-470), IGF BP3 2 mg/L (1.5-3.4), T4 6.6 ug/dL, TBGI 1.10, TSH 1.80  uU/ML

Impression: Hobart has grown 3.3 cm. over the last six months.  This gives him a growth velocity of about 7 cm. over the last year, which is at the lower end of a normal growth velocity for a prepubertal child.  In addition, we have never had an explanation for his fall off in growth between the ages of 2-3 years of age and 4-5 years of age.  Therefore, today I would like to do some screening studies to look for growth hormone deficiency including an IGF1 and an IGF BP3.  I will get back with you and Mom and Dad as soon as I obtain the results of these studies.  

Thank you for allowing us to share in the care of Hobart.



Sincerely,




Ariel Adler, MD           
Attending in Endocrinology   


Addendum: Since the laboratory values are normal (the IGF-1 being just sligtly below normal) and unchanged from December 1994, and since Hobart has a normal growth velocity, I suggest continuing close clinical monitoiring of his growth at this time.


cc:  Mrs. Julie Browne   20 Lincoln Drive Apt   Acton , MA  01720


CONTENT:
Pluto, M.D.          RE: Owen Insull
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/25/81

Dear Dr. Pluto:

We had the pleasure of seeing Owen in the Endocrine Program on 08/29/91 for evaluation of premature adrenarche.

History: As you know, Owen is now a 9 and 11/12 year old boy here for follow-up of premature adrenarche.  Cortrosyn stimulation test was performed on June 13, 1991 with elevated stimulated 17-Hydroxypregnatolone to 17-Hydroxyprogesterone and DHEA to androstine dion ratios.  He also had an elevated baseline DHEA level.  All the values are consistent with a mild 3-Beta Hydroxy Steroid Dehydrogenase Deficiency.  Since his last visit, his grandmother reports no change in pubic or axillary hair and his activity level has remained good.

Current medications: Owen continues on no medications.

Physical exam:  
Height: 138.6 cm        Weight: 27.8 kg         BP: 102/70              
Pulse: 84               Pubic hair Tanner: 3    Penile str. l.: 6 cm    
Mid-shaft diam.: 1.6 cm 
Owen was a healthy appearing boy in no acute distress.  Examination of his skin was still notable for mild to moderate facial acne and oiliness.  Examination of the neck revealed a minimally palpable thyroid.  HEENT exam revealed a moist orapharynx without hyperpigmentation.  Extraocular movements were intact.  Chest was clear to auscultation.  There was no gynecomastia.  Abdomen was soft and without hepatosplenomegaly.  GU exam revealed testes measuring 1.0 x 2.0cm bilaterally.  There were five strands of axillary hair bilaterally.

In summary: Since his last visit, Owen has had perhaps a mild acceleration in his growth, though no progression in his secondary sexual characteristics.  Because of his bone age being consistent with his height age and his normal cortisol response to cortrosine, despite his mild androgen excess, because of his 3-Beta Steroid Dehydrogenase block, we have elected not to treat Owen at this time but merely follow his height and bone age progression to ensure he reaches a normal adult height.  Should he have substantially worse facial acne or an accelerating bone age, we would consider intervention with hydrocortisone replacement at that time.  We hope to see him back in six months.

Thank you for letting us share in the care of Owen.

Sincerely,


Maureen Shields, MD                      Kendell Simpson, MD
Fellow in Endocrinology               Clinical Director

LM/SN/smp


CONTENT:
Pluto, M.D.          RE: Owen Insull
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/25/81

Dear Dr. Pluto:

We had the pleasure of seeing Owen in the Endocrine Program on 08/29/91 for evaluation of premature adrenarche.

History: As you know, Owen is now a 9 and 11/12 year old boy here for follow-up of premature adrenarche. A Cortrosyn stimulation test was performed on June 13, 1991 with elevated stimulated 17-Hydroxypregnenolone to 17-Hydroxyprogesterone and DHEA to androsterone ratios. He also had an elevated baseline DHEA level.  All the values are consistent with a mild 3-Beta Hydroxsteroid Dehydrogenase Deficiency.  Since his last visit, his grandmother reports no change in pubic or axillary hair and his activity level has remained good.

Current medications: Owen continues on no medications.

Physical exam:  
Height: 138.6 cm        Weight: 27.8 kg         BP: 102/70              
Pulse: 84               Pubic hair Tanner: 3    Penile str. l.: 6 cm    
Mid-shaft diam.: 1.6 cm 
Owen was a healthy appearing boy in no acute distress.  Examination of his skin was still notable for mild to moderate facial acne and oiliness.  Examination of the neck revealed a minimally palpable thyroid.  HEENT exam revealed a moist orapharynx without hyperpigmentation.  Extraocular movements were intact.  Chest was clear to auscultation.  There was no gynecomastia.  Abdomen was soft and without hepatosplenomegaly.  GU exam revealed testes measuring 1.0 x 2.0cm bilaterally.  There were five strands of axillary hair bilaterally.

In summary: Since his last visit, Owen has had perhaps a mild acceleration in his growth, though no progression in his secondary sexual characteristics.  Because of his bone age being consistent with his chronologic age and his normal cortisol response to Cortrosyn, we have elected not to treat Owen at this time but merely follow his height and bone age progression to ensure he reaches a normal adult height.  Should he have substantially worse facial acne or an accelerating bone age, we would consider intervention with hydrocortisone replacement at that time.  We hope to see him back in six months.

Disposition: 

Thank you for letting us share in the care of Owen.

Sincerely,


Maureen Shields, MD                      Kendell Simpson, MD


CONTENT:
Bleck Pavarotti, M.D.          RE: Owen Insull
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/25/81

Dear Dr. Pavarotti:

We had the pleasure of seeing Justin in the Endocrine Program on 08/29/91 for evaluation of precocious puberty.

History: As you know, Justin is a 10 and 4/12 year old boy with congenital blindness and a suprasellar tumor here for follow-up.  Because of his accelerating bone age and continuing pubertal development, he was begun on intramuscular Lupron at the end of April 1991.  Three days prior to his third dose of Lupron a GnRH stimulation test was performed which showed appropriately suppressed LH/FSH and testosterone.  Of note at this time, a repeat SmC was obtained which was still high at 4.08U/ml.  Justin denies recent headache, dizziness, or lethargy.  His mother reports he does appear to be growing normally, though is having somewhat increased appetite.

Current medications: Justin's only current medication is Luprone 7.5mg im q month.

Physical exam:  
Height: 140.3 cm        Weight: 48 kg           BP: 120/80              
Pulse: 92               Pubic hair Tanner: 4    Penile str. l.: 7.8 cm  
Mid-shaft diam.: 2.5 cm 
Justin was a slightly obese white male.  Skin: good turgor noand cutaneous lesions.  Neck examination revealed a minimally palpable thyroid.  HEENT exam showed unchanged rotatory and pendular nystagmus.  Chest exam was notable for increased subcutaneous fat, though no gynecomastia.  Cardiac and abdominal exams were unremarkable.  GU exam revealed testes measuring 2.0 x 4.0 bilaterally.   There was no axillary hair.  Neurologic exam was notable for 2+ and equal deep tendon reflexes with normal relaxation and unchanged diminished visual acuity.

In summary: Justin has had an appropriate halt to his pubertal progression on his current dose of IM Lupron.  Over the last four months, he has also exhibited an acceptable growth velocity despite his rather low growth hormone values on provocative testing.  We would like to continue to follow Justin to ensure his growth velocity remains normal and that his pubertal suppression continues at its current level.  To do this, we will repeat a bone age and GnRH stimulation testin approximately four months, at which time we will also re-measure him and reassess his pubertal status.

Disposition: 

Thank you for letting us share in the care of Justin.

Sincerely,


Maureen Shields, MD                      Rahul Ibsen, MD


CONTENT:
Pluto, M.D.          RE: Owen Insull
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/25/81

Dear Dr. Pluto:

We had the pleasure of seeing Owen in the Endocrine Program on 08/29/91 for evaluation of premature adrenarche.

History: As you know, Owen is now a 9 and 11/12 year old boy here for follow-up of premature adrenarche. A cortrosyn stimulation test was performed on June 13, 1991 with elevated stimulated 17-Hydroxypregnenolone to 17-Hydroxyprogesterone and DHEA to androstenione ratios.  He also had an elevated baseline DHEA level.  All the values are consistent with a mild 3-Beta Hydroxysteroid Dehydrogenase Deficiency.  Since his last visit, his grandmother reports no change in pubic or axillary hair and his activity level has remained good.

Current medications: Owen continues on no medications.

Physical exam:  
Height: 138.6 cm        Weight: 27.8 kg         BP: 102/70              
Pulse: 84               Pubic hair Tanner: 3    Penile str. l.: 6 cm    
Mid-shaft diam.: 1.6 cm 
Owen was a healthy appearing boy in no acute distress.  Examination of his skin was still notable for mild to moderate facial acne and oiliness.  Examination of the neck revealed a minimally palpable thyroid.  HEENT exam revealed a moist orapharynx without hyperpigmentation.  Extraocular movements were intact.  Chest was clear to auscultation.  There was no gynecomastia.  Abdomen was soft and without hepatosplenomegaly.  GU exam revealed testes measuring 1.0 x 2.0cm bilaterally.  There were five strands of axillary hair bilaterally.

In summary: Since his last visit, Owen has had perhaps a mild acceleration in his growth, though no progression in his secondary sexual characteristics.  Because of his bone age being consistent with his chronologic age and his normal cortisol response to cortisyn, we have elected not to treat Owen at this time but merely follow his height and bone age progression to ensure he reaches a normal adult height.  Should he have substantially worse facial acne or an accelerating bone age, we would consider intervention with hydrocortisone replacement at that time.  We hope to see him back in six months.

Disposition: 

Thank you for letting us share in the care of Owen.

Sincerely,


Maureen Shields, MD                      Kendell Simpson, MD


CONTENT:
Hagen, M.D.          RE: Owen Insull
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/25/81

Dear Dr. Hagen:

We had the pleasure of seeing Owen in the Endocrine Program on 3/9/92 for  follow-up mild beta 3-beta hydroxysteroid dehydrogenase deficiency.

History: Owen is currently 10 and 6/12 year old boy who since his last visit has had no significant medical problems. Other than infrequent stomach aches, he denies complaints of headache, diarrhea and constipation.  He is currently on no medications.  Owen claims his acne has remained unchanged on his forehead and he continues to use deodorant.  Owen feels his voice may be deepening but has had no change in axillary or pubic hair.  Intermittently, Owen feels somewhat short of breath usually while exercising though not entirely limited to exercising.

Physical exam:  
Height: 140.3 cm        Weight: 32.3 kg         BP: 96/70               
Pulse: 88               R. testis: 1.5 x 0.8 cm L. testis: 1.5 x 0.8 cm 
Phallus length: 6 cm    Mid-shaft diam.: 1.5 cm 
General: Healthy appearing and in no distress.
Skin: Mild acne on the forehead.  Turgor was good.  
HEENT: Flat optic discs and intact extraocular movements.  
Neck: There was no thyromegaly.
Chest: There was no gynecomastia.  Lungs were clear to auscultation.
Heart: Regular rate and rhythm without murmr.  Pulses were 2+ and equal.
Abdomen: Soft and without hepatosplenomegaly.
Genitourinary: Tanner pubic hair stage III-IV.
Extremities: Without clubbing, cyanosis or edema.
Neurological: 2+ and equal deep tendon reflexes.  Muscle bulk and tone was normal.

Impression: Our impression is that since his last visit, Owen has exhibited no progression in androgen affects.  He has had no acceleration in growth or excessive weight gain either.  Owen brought with him a 24 hour urine collection which we will send fro pregnanetriol.  We also obtained a bone age today to determine if it has progressed in excess of his chronologic age since his last study one year ago.  Owen's intermittent complaints of shortness of breath are of some concern to him and we referred him to follow-up with you for further work-up.  We hope to see Owen again in one years.


Thank you for letting us share in the care of Owen.

Sincerely,


Maureen Shields, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director            


Problem list to date:
CONG.-ADRENOCORTICAL-HYPERPLAStart: 03/09/92  End:          Invalid: 
3-BETA-HYDROXYSTEROID-DEFICIEStart: 03/09/92  End:          Invalid: 

Transcribed by Sullivan


CONTENT:
Kosmo DeNiro, M.D.          RE: Cesar Abbott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/91

Dear Dr. DeNiro:

     We had the pleasure of seeing Cesar in the Renal Program on 7/13/95 for presumptive ARPKD.
     Cesar is a 4 year old with hepatic fibrosis and echogenic  kidneys, who had previously been evaluated by Lisa Guay Woodford.   Cesar presented with splenomegaly as an infant.  Liver biopsy at the  time revealed hepatic fibrosis.  In the interval since his last visit in  1992, he has been well.  He has had no upper or lower GI bleeding,  hypertension or hospitalizations.  He is followed yearly by Harland  Winter, MD.  His most recent labs on June 2, 1995 included a BUN of 15 mg/d,  creatinine of 0.4 and a hematocrit of 36%.

His current medications include:


Cesar's physical exam was notable for:
     BP 112/59   P 98   R 16   T    HT 102.9cm   WT 18.5kg
     Cesar was a charming, well appearing boy.   
     HEENT: No evidence of infection, fundi benign.  
     Chest: Lungs were clear with good aeration. 
     Heart: Regular rhythm without murmur.
     Abdomen: Spleen palpable below left costal margin; liver palpable below right costal margin; kidneys not palpable. 
A urinalysis revealed:
     SPEC GR UA  1.019           RBC/HPF     0          
     PH UA       5               WBC/HPF     0          
     BLOOD UA    Neg.            SQUAM EPI   0          
     PROTEIN UA  Trace           BACTERIA    Neg.  
     GLUCOSE UA  Neg.       
     
Abdominal Ultrasound:
Spleen is enlarged, measuring 12.6 cm.  The liver is slightly enlarged with focal areas of dilatation of hepatic bile ducts consistent with hepatic fibrosis.  

His kidneys were both narrow in  the cross-sectional plane with the left measuring 9.6 cm and the right  8.4 cm.  They were mildly echogenic.

Laboratory studies at this time include:


     Overall, Cesar continues to do well.  His kidneys are  slightly enlarged.  Although they are long in the longitudinal plane,  both of them are narrow, presumably due to compression from his other  abdominal viscera.  He continues to have normal blood pressure and renal  function. 

In the interval since Cesar's last visit further work has been done in localizing the gene for ARPKD.  Lisa Guay Woodford and her collaborators  have found that it is on chromosome 6.  At this time, 100 children with ARPKD and varying degrees of severity of renal and hepatic involvement have been  associated with the defect in this location.  She has also used this for  prenatal diagnosis.  The abnormal protein has not yet been  identified.  I discussed with Mr. Abbott the role of genetic testing for  Cesar.  We would need to have blood from Cesar and both of his  parents to determine if he indeed does have this defect.  Since the  diagnosis of ARPKD has not been definitively made, this might be  helpful.  Mr. Abbott is going to consider the possibility of testing in  conversations with Cesar's mother.  If we do decide to do this testing  it can be done in Westwood.   I would continue to follow  Cesar's blood pressure yearly.  If it becomes elevated we would consider  treatment.

     Thank you for the opportunity to participate in Cesar's care.  We would like to review him in Two to three years.  In the interim, we would be happy to discuss or review Cesar should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Mitsuko Goldwater, M.D.          RE: Phil Ogden
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/08/89

Dear Dr. Goldwater:

We had the pleasure of seeing Phil in the Endocrine Program on 1/23/92 for  failure to thrive.

History: Phil is a 26 1/2 month boy.  He is the former 6 lb 9 oz product of a full-term pregnancy that was uncomplicated.  Labor and delivery were normal.  He was initially thriving with a weight in approximately the 30th percentile on breast milk and Isomil.  Solids were introduced at 5 months and he continued to do well until 10 months when he began to fall off his growth curve predominently with a fall in his weight percentile from 35th percentile to less than the 5th percentile.  By age 1 1/2 his height had started to fall off his growth curve as well.  As you know, he has had an extensive work-up which is most notable for an abnormal pancreatic stimulation test with a low amylase but normal trypsin.  This result was felt to be consistent with a partial pancreatic insufficiency.  His sweat test was normal.  Endoscopy and small bowel biopsy was negative for Celiac disease.  In addition, he has had normal electrolytes, normal total protein and albumin and a CBC that was remarkable for a hematocrit of 40, platelet count of 401, and a WBC equal to 10 but with only 13% polys.  Stool culture and three stools for ova and parasites were normal.  A chest x-ray was normal.  Urinalysis in the past has been normal with a pH of 5-6, specific gravity of 1.029 and no evidence of white blood cells or reducing substances.  An SPA showed only a slight increase in his IGM level, serum urine, organic and amino acids  were negative.  Serum T4 and T3 were normal.  A bone age approximately 6 months ago was reported to be equal to his chronological age.  By his mother's report, calorie counts in the past have showed that his caloric intake was adequate.  The diet history is consistent with this as he seems to have a good appetite with a typical day's intake including cereal, yogurt, juice, macaroni and cheese, cake, green beans and meat.  Over the last several months, he has been on caloric supplements including frappes and Carnation milk with no weight gain.  In addition, he has had a 6 week trial of Creon due to his partial pancreatic insufficiency with no weight gain.  He has no history of vomiting, or headaches.  He has 3-4 formed stools a day that are not described a foul smelling or large and have no visible blood in them.

Current medications: Creon 1 half capsule TID.

Past medical history:  Significant for otitis media twice.  He has had no surgeries.

Family history:  He has an 11 year old sibling who has hypogammaglobulinemia and a milk protein allergy.  In addition, he has a maternal uncle who has Celiac disease, and a paternal uncle with diabetes.  His father's height is 5'10" tall, mother's height is 5'3" tall.  

Developmentally he appears to be normal.  He is using 4-5 word sentences.  He climbs stairs, chairs and tables easily.  He walked at 12 months.  He is putting puzzles together and is able to color within the lines.

Physical exam:  
Height: 82.6 cm         Weight: 10.3 kg         
(on the 5th percentile).
(less than the 5th percentile and an average weight for a 9 month old).

Skin: No lesions.
HEENT: Head circumference: 48 cm which is in the 10th percentile but has fallen off from the 50th percentile.  Round, reactive pupils.  Tympanic membranes were clear.  Orophyarnx was benign.  He did have shoddy, cervical adenopathy.  
Neck: No thyromegaly.  
Chest: Clear.
Heart: Regular rate and rhythm with no murmur.
Abdomen: Soft and nontender.  The liver was 2.5 cm below the costal margin and he had no splenomegaly or other masses.
Genitourinary: Normal prepubertal male.  Testes were descended bilaterally.
Extremities: No clubbing, cyanosis or edema.
Neurological: Deep tendon reflexes were 2+ and symmetric.  He had normal strength and tone.

Laboratory studies:  Na 140, K 4.8, Cl 105, CO2 20, BUN 10, Cr 0.4, glucose 95, Ca 10.0, Phos 5.9, Magnesium 2.3, Total protein 7.3, albumin 4.4, Amylase 80, AST 25, ALT 25. Venous pH 7.34, pCO2 44, bicarbonate 24.  Iron 94, TIBC 428.  Urine specific gravity 1.015, pH 6, negative otherwise.  

Alkaline phosphatase 872 MU/ml, LDH 142 MU/ml, Amonia 81 mcg/dl.  

Growth hormone 11.0 ng/ml. IGF1-BP3: 2.2 mg/L (normal 1.4-3.0).  IGF-1           u/ml.

T4: 10.6 mcg/dl, TBGI 0.89, TSH 3.80 mcU/ml.

WBC 11.3, RBC 4.74, Hb 12.6, Ht 37.4, MCV 79.0, MCH 26.6, MCHC 33.7, Platelets 428.

ESR 18 mm/hour.

Anti-endomysial and antigliadin antibodies: negative.

In summary: Phil does not have an endocrine cause for his failure to thrive.  Of the multitude tests performed only the Alkaline phosphatase was abnormal, LDH and NH were slightly increased.  It will be of interest to fractionate the alkaline phosphatase should it persist to be elevated.


Thank you for letting us share in the care of Phil.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Neptune, M.D.          RE: Brenda Petrovich
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/05/91

Dear Dr. Neptune:

We had the pleasure of seeing Brenda in the Endocrine Program on 2/10/94 for follow up of nutritional rickets.

History: Brenda is now 2 years and 7 months old.  She has been on Ergocalciferol for about 7 months.  Her walking has improved, she is strong, and she is running well.  Her diet consists of milk and cereal in the morning, juices as a snack, lunch of spaghetti or rice, vegetables an juice, a snack of milk in the afternoon, and a dinner of rice, cheese, yogurt and juice.

Medications:  Ergocalciferol 8000 u/day.

Physical exam:  
Height: 89 cm           Weight: 12.7 kg         
(25th percentile)  (25 percentile).
General: Well developed, well nourished, no apparent distress.
Skin: Normal.
HEENT: Normocephalic, atraumatic.  PERRL.  Ears were normal.  Teeth were noraml.
Neck: 
Chest:   Clear.
Heart: Regular rate and rhythm without murmurs.
Abdomen: No hepatosplenomegaly.
Genitourinary: 
Extremities: Slightly widen epiphyses but no bowing of the legs.
Neurological:

Laboratory studies:  PTH=21 pg/ml, Ca=9.8 mg/dl, phosphorus-4.8 mg/dl, alkaline phosphatase=286 U/L.  1,25-dihydroxy vitamin D=82 pg/ml, 25-hydroxy vitamin D=22 ng/ml.  Left knee x-rays: resolution of rickets changes seen May 1993.

Impression: Brenda is doing quite well.  Her vitamin D levels are in the normal range, and we will decrease her to 400 units of vitamin D a day.

Disposition: Return to clinic in six months.
Thank you for letting us share in the care of Brenda.

Sincerely,


Penelope Hagen, MD              
Attending in Endocrinology


CONTENT:
Neptune, M.D.          RE: Brenda Petrovich
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/05/91

Dear Dr. Neptune:

We had the pleasure of seeing Brenda in the Endocrine Program on 5/24/93 for evalatuion of nutritional rickets.

History: Brenda is a 22 month old female.  She was seen at CCHP at about 1 year of age because she was late in walking.  She was referred to the Neurology Clinic but started to walk at 15 months of age so this appointment was canceled.  However, mom has noted that Brenda is unstable on her feet and has problems with running, balance and gait.  She was taken to CCHP about two months ago for this problem, and was referred to physical therapy.  The physical therapist found that her joints and bones were normal.   At that time, she was having some wheezing, so a chest x-ray was performed and showed some evidence of rickets.  She has had no other x-rays performed.

Overall, Brenda has been a healthy infant.  She was breast fed and is currently weaning.  She was not supplemented with formula, however she was started on cow's milk at 12 months of age and is now drinking about 8 oz of cow's milk a day.  She started on solids foods at about 10 months of age with Cream of Wheat.  At 12 months other solids were started.  She now has a varied diet.

Current medications: None.

Past medical history:  Former 38 week product born at the Beth Israel Hospital after an uncomplicated pregnancy, labor and delivery.  Birth length was 20", weight was 7 lbs.  Mother was a G4P3SAB1 33 years old woman at the time of the delivery.  growth and development have been as above.   The only operation was an adenoidectomy due to some difficulty with breathing.

Review of systems:  Negative.

Family history:  Mother has adult onset diabetes that she developed during the pregnancy.  She is 35 years old and is 5'3 1/2" tall.  Dad is 36 years old and 6'4" tall.  There are two other boys, ages 4 and 8 years old without medical problems.

Physical exam:  
Height: 79.7 cm         Weight: 9.52 kg         Pulse: 120              
Arm span: 83.5          Breast: Tanner I        Pubic hair: Tanner I    
General: Well developed, well nourished, no apparent distress. Skin: Normal. HEENT: Normocephalic/atraumatic.  There was no fontanelle.   Neck: No thyromegaly. Chest: Clear. Heart: Regular rate and rhythm without murmur. Abdomen: Benign. Genitourinary: Prepubertal  Extremities: No bowing of the upper or lower extremities.  She had very prominent knees and prominent epiphyses. Neurological: Normal.

Laboratory studies:  May 10, 1993: 25dihydroxy vitamin D 9, 1,25 dihydroxy vitamin D and PTH are pending.  Urine calcium=7.8 mg/dL, urine creatinine 84.4 mg/dL, urine calcium/creatinine ratio: 0.09, urine phosphate 91.5 mg/dL, alk phos 568 mU/mL, calcium 9.9, phosphorous 5.3.

Impression: Brenda most likely has nutritional rickets as evident by her low vitamin D level although we do not have the 1,25 dihydroxy vitamin D  or PTH results at this time.  In the meantime, however, we will start Brenda on Ergocalcifecol and obtain knee, hand and wrist x-rays to evaluate the state of her rickets.

Disposition: Ergocalcificol 8000U/day.  Return to the Endocrine Clinic in two months.

Laboratory studies pending at this time include: X-rays of her left hand and wrist for bone age,and knees x-rays.
Thank you for letting us share in the care of Brenda.

Sincerely,


Penelope Hagen, MD              
Attending in Endocrinology


CONTENT:
Cavinder Plato, M.D.          RE: Bess Tobin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/90

Dear Dr. Plato:

     We had the pleasure of seeing Bess in the Renal Program on 7/14/94 for ARPKD.
     Bess was seen in follow-up for ARPKD.  She previously was followed by Dr. Marc Lande.  In the interval since her last visit in March, Bess has been feeling well.  Her blood pressures at home have averaged 110/55 by dinamap.  She has had no unexplained fevers or abdominal pain.

Current medications include:
     VASOTEC 3.75 mg b.i.d.
     LASIX .5 mg b.i.d.

Physical exam:
     BP 100/56   P 106   R 20   HT 98.2cm   WT 16kg
     General: Well appearing, active toddler with weight at 50th% and height at 25th%
     Skin: Echymoses on shins
     HEENT: Scant nasal discharge, otherwise no evidence of infection, fundi benign
     Chest: Lungs clear with good aeration
     Heart: Regular rhythm, without murmur
     Abdomen: Protuberant, soft and non-tender with liver palpable approximately 4 cm below the right costal margin and spleen palpable 3cm below the left costal margin
     Extremities: Normal form, no edema
     Neurological:Non focal exam

Urinalysis:
     SPEC GR UA  1.010           PH UA       5          
     PROTEIN UA  N               BLOOD UA    N          
     GLUCOSE UA  N               WBC/HPF     10-15      
     RBC/HPF     N               SQUAM EPI   N     
     BACTERIA    1+              CASTS       N

Laboratory studies at this time include:
          NA  138        K  4.4        CL  102       CO2  27
          HCT  30.7      WBC  6.08     RBC  4.12     PLT  152
          HGB  10.3      MCV  74.5     BUN  19       CR  0.6
          PT  12.0       PTT  26.9     AST  27       ALT  9
          ALK PH  224
          URINE CULTURE:  No growth or less than 1,000 organisms/ML
          
          ABDOMINAL ULTRASOUND:  Kidneys continue to be echogenic and enlarged (approximately 11 cm bilaterally).  The liver continues to show coarsened texture with normal direction to blood flow and her spleen remains enlarged.  These studies are essentially unchanged from her previous exam.

     Bess continues to have stable renal function and good metaboic control.  Her blood pressure is well controlled on her current regimen.  The decrease in her hematocrit may reflect hypersplenism or given her MCV iron deficiency.

Since Bess is doing so well I recommended that she come back in six months, at the same time as her follow-up visit with Dr. Jonas.  I suggested that she see Dr. Plato in six months to review her status and blood pressure measurements.  Ms. Tattan will call if her blood pressure becomes elevated.

     We have asked Bess to return in six months.  Thank you for allowing us to share in Bess's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Cavinder Plato, M.D.          RE: Bess Tobin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/90

Dear Dr. Plato:

     We had the pleasure of seeing Bess in the Renal Program on 2/9/95 for ARPKD.
     Bess has generally been feeling well since the time of her last visit in July of 1994.  She has developed enuresis over the last six weeks.  This time interval coincides with some changes in the family living arrangements.  Prior to this, Bess had been dry for several years.  On review of Bess's drinking and voiding habits, it is clear that she drinks extensive amounts of fluid.  In addition, we have had her on Lasix for a number of years.  It is not clear at this point whether it is necessary for her to drink fluids due to concentrating defect or whether this is a habit that has been encouraged.

Current medications include:
     VASOTEC 3.75 mg b.i.d.
     LASIX 5 mg q.d.


Physical exam:
     BP 108/49   P 101   R 16   T    HT 102.2cm   WT 17.7kg
     General: Well appearing, very active toddler.
     Skin: No lesions.
     HEENT: No evidence of infection.
     Lungs:  Clear with good aeration.
     Heart: Regular rate and rhythm without murmur.
     Abdomen: Protuberant with liver and spleen edges both palpable approximately 4cm below the costal margin.
     Extremities: Normal form and no edema.
     Neurological: Non-focal exam.
Urinalysis:
     SPEC GR UA  1.003           PH UA       5          
     PROTEIN UA  trace           BLOOD UA    1+         
     GLUCOSE UA  N               WBC/HPF     0          
     RBC/HPF     0               SQUAM EPI   0     


Laboratory studies at this time include:
          ALKP        222     U/L       CO 2         23   MEQ/L    
          CR          0.8   MG/DL (H)   FE           47  MCG/DL (L)
          AST          29     U/L       ALT           9     U/L    
          BUN          34   MG/DL (H)   NA          137  MMOL/L    
          K           4.4  MMOL/L       CL           99  MMOL/L    
          TIBC        252  MCG/DL       PLT         110   K/MM3 (L)
          WBC        5.56   K/MM3 (L)   RBC        4.06   M/MM3    
          HGB         9.8    G/DL (L)   HCT.       31.5       % (L)
          MCV        77.6      FL       MCH        24.1      PG (L)
          MCHC       31.1    G/DL       RDW        14.4       %    
          HDW        2.87    G/DL       PLT UNC     110   K/MM3 (L)

          CASTS       N

     Bess continues to do well.  Her blood pressure is well controlled and her renal function is minimally depressed.  Her estimated GFR is 70ml/1.73m2.  Her hematologic status is stable.  I noted Bess's low MCV and requested iron studies.  She continues to have borderline iron stored.  I suggested to Mrs. Tobin that I would leave it to Dr. Plato to determine if supplementation would be appropriate.  Although I think that Bess's enuresis is likely due to changes in the family living arrangements, I discussed several approaches to try to decrease its frequency.  Of note, Miss Tattam is not particularly concerned with this.  It seems that Bess has frequency in the daytime as well.  She also wakes up to void several times during the night.  This is due likely to the large amount of fluid that she consumes.  I suggested that we discontinue to evening Lasix dose.  It is not clear to me that she needs any Lasix.  Miss Tattam was concerned about discontinuing because she thought it was important for her hypertension so I have discontinued one dose.  I expect to stop the next morning dose at the next visit.  The Enalopril should be sufficient for blood pressure control.  I also suggested that she try not to have Bess drink anything after dinner.  It is not clear that these will succeed as Bess is a very determined young lady.  I have made no other changes in Bess's regimen.

     We have asked Bess to return in .

     Thank you for allowing us to share in Bess's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Cavinder Plato, M.D.          RE: Bess Tobin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/90

Dear Dr. Plato:

     We had the pleasure of seeing Bess in the Renal Program on 9/7/95 for ARPKD.
     Bess has generally been well since the time of her last visit.  The enuresis that she had developed prior to her visit in February has resolved.  Her mother associates this with discontinuation of the evening Lasix dose.  Bess was seen by Maureen  Jonas in Gastroenterology on July 26.  Laboratory evaluation at that time included a BUN of 19, creatinine of 0.8, normal electrolytes, hematocrit of 30%, and an estimated GFR of 72.  Dr. Jonas also was concerned about a change in her cardiac exam and wondered about the merits of cardiology evaluation.

Her current medications include:
     VASOTEC 3.75 mg b.i.d.
     LASIX 5 mg q.d.


Bess's physical exam was notable for:
     BP 104/48   P 90   R 18   T    HT 105.2cm   WT 18.7kg
     General: Bess was active and well-appearing with a weight in approximately the 50th percentile and height in the 25th percentile.  
     HEENT:  No evidence of infection.  
     Lungs:  Clear with good aeration.  
     Heart:  Regular rate with a I-II/VI systolic murmur best heard in the left upper sternal border.  The first heart sound is slightly muffled which may reflect a diastolic component to the murmur.  
     Abdomen:  Positive bowel sounds, soft, nontender.  
     Liver:  Palpable, approximately 3 cm. below the right costal margin.  Spleen tip palpable approximately 4 cm. below the left costal margin.  
     Extremities:  No edema.  
     
     Abdominal Ultrasound: The liver and spleen size are increased from previous examination in July of 1994.  The spleen now measures 11.3 cm.  There is increased flow in the hepatic artery without reversal of flow in the portal system.  Both kidneys are enlarged, measuring 9.8 cm.  They are diffusely echogenic.
A urinalysis revealed:
     SPEC GR UA  1.005           GLUCOSE UA  N     
     PH UA       N               RBC/HPF     N     
     BLOOD UA    n               WBC/HPF     N     
     PROTEIN UA  N          

Laboratory studies at this time include:


     Bess has stable renal function with estimated GFR of 72 ml/minute/1.73 m2.  Her blood pressure is well controlled on her current regimen. 

I discussed again with Ms. Tattan the merit of scheduling her follow-up visit with Dr. Jonas and myself at the same time.  She will try to arrange that.

     Thank you for the opportunity to participate in Bess's care.  We would like to review her in six months.  In the interim, we would be happy to discuss or review Bess should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Cavinder Plato, M.D.          RE: Bess Tobin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/90

Dear Dr. Plato:

     We had the pleasure of seeing Bess in the Renal Program on 1/18/96 for ARPKD.
     Bess has been doing well since the time of her last visit.  Her blood  pressures at home have been approximately 120/60.  She is in  kindergarten where she is doing well.

Her current medications include:
     LASIX 5 mg q.d.
     ENALOPRIL 3.75 mg b.i.d.

Bess's physical exam was notable for:
     BP 108/62   P 114   R 18   T    HT 107.7cm   WT 19.3kg
     In general, Bess is a pale but well and active appearing child with a weight at the 75th percentile and a height at the 25th percentile.  
     HEENT:  No evidence of infection.  
     Lungs:  clear with good aeration.  
     Heart:  Regular rate with a Grade II/VI systolic murmur best heard at the left upper sternal border.  
     Abdomen:  Protuberant but soft with liver palpable 3 cm below the right costal margin and the spleen tip palpable 4 cm below the left costal margin.  Extremities:  No  edema.
A urinalysis revealed:
     SPEC GR UA  1.010           WBC/HPF     0          
     PH UA       5               RBC CAST    0          
     BLOOD UA    n               WBC CAST    0          
     PROTEIN UA  n               GRAN CAST   0     
     GLUCOSE UA  n               WAXY CAST   0     
     RBC/HPF     0               BACTERIA    n     
     8-10 TUBULAR  EPITHELIAL CELLS

Laboratory studies at this time include:
          AST          34     U/L       BILI TO     0.9   MG/DL    
          ALKP        205     U/L       BILI DI     0.1   MG/DL    
          K           4.6  MMOL/L (H)   WBC        6.23   K/MM3    
          CA          9.3   MG/DL       RBC        3.84   M/MM3    
          CR          0.9   MG/DL (H)   HGB         9.5    G/DL (L)
          LDH         156     U/L       HCT.       28.5       % (L)
          PHOS        4.5   MG/DL       MCV        74.2      FL (L)
          ALT           8     U/L       MCH        24.7      PG (L)
          BUN          19   MG/DL (H)   MCHC       33.3    G/DL    
          NA          140  MMOL/L       RDW        15.1       %    
          CL          106  MMOL/L       HDW        3.15    G/DL    
          CO 2         23   MEQ/L       PLT UNC     115   K/MM3 (L)

     Bess is generally doing well.  Her creatinine of 0.9 is consistent  with an estimated GFR of 66 mL/min/1.73 M2.  Her electrolytes are all  within normal limits.  She continues to be anemic with a low MCV. This may reflect iron deficienc.  It would be reasonable to obtain iron studies with her next phlebotomy. 

It is not clear whether Bess still needs to take the Lasix.   Concentrating defects are common in ARPKD and enalapril does not require a diuretic as concomitant treatment.  I, therefore, suggested to Ms. Patton that she discontinue the  Lasix.  I would like her to monitor her blood pressures at  different times during the day.  If her blood pressure does become more  elevated I would like to increase her enalapril.  It is likely to be  more elevated during the day, in which case we would increase her  enalapril to 5 mg in the morning and 3.75 mg in the afternoon.

Bess is seeing Dr. Jonas  on a different date.  I suggested again to  Ms. Patton that she try to arrange a visit on the same day, as it's a long trip.

     Thank you for the opportunity to participate in Bess's care.  We would like to review her in six months.  In the interim, we would be happy to discuss or review Bess should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Cavinder Plato, M.D.          RE: Bess Tobin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/90

Dear Dr. Plato:

     We had the pleasure of seeing Bess in the Renal Program on 6/12/96 for ARPKD.
     Bess comes to us for follow up.  Briefly, she is a 6 year old diagnosed to have autosomal recessive polycystic kidney disease and congenital hepatic fibrosis when she presented at 8 months of age with enlarged kidneys.  Liver and kidney  biopsies done in November of 1991 confirmed the diagnosis.  Her problems  include hypertension that is currently controlled and portal  hypertension with splenomegaly secondary to hepatic fibrosis.  Her last  GI visit with Dr. Jonas revealed normal transaminases and portal  hypertension without complications.  She was previously followed by Dr.  Jabs with stable mild renal insufficiency.    Her last renal ultrasound in September of 1995 revealed a  liver and spleen which are increased in size compared to previous study,  enlarged kidneys, and diffusely echogenic kidneys.  During her last  visit with us, she was also noted to have a new onset murmur and mild  anemia.  In the interim, Bess has been asymptomatic, without any  complaints.

Her current medications include:
     VASOTEC 3.75 mg b.i.d.

Bess's physical exam was notable for:
     BP 118/56   P 86   R 12   T    HT 110.5cm   WT 20.5kg
     Bess is comfortable and in no distress.  
     HEENT: within normal limits.  
     Neck: supple without lymphadenopathy.  
     Chest  clear.  
     Heart: Grade I/VI systolic ejection murmur, non-radiating,  heard best at the left lower sternal border.  
     Abdomen: enlarged spleen palpated 5 cm below the left costal margin and liver tip  2 to 3 cm below the right costal margin.  
     Extremities: without edema.   
     Skin: warm and well-perfused.  
     Neurologic: no lateralizing signs.
A urinalysis revealed:
     SPEC GR UA  1.009           WBC/HPF     0-3        
     PH UA       5               SQUAM EPI   few        
     WBC ENZ UA  tr              RBC CAST    none       
     BLOOD UA    -               WBC CAST    none       
     PROTEIN UA  -               GRAN CAST   none       
     GLUCOSE UA  -               WAXY CAST   none       
     RBC/HPF     0-1             BACTERIA    -     

Laboratory studies at this time include:
          AST          28     U/L       RDW        14.8       %    
          ALKP        236     U/L       HDW        2.89    G/DL    
          GGTP         10     U/L       MPV         9.8      FL    
          CR          0.9   MG/DL (H)   PLT UNC     111   K/MM3 (L)
          FE           23  MCG/DL (L)   NEUT AB    2.05   K/MM3    
          LDH         190     U/L       LYMPH A    1.43   K/MM3    
          ALT           4     U/L       EOS ABS     .07   K/MM3    
          TRF         240   MG/DL       NEUT-A     52.1       %    
          BUN          23   MG/DL (H)   LYMPH-A    36.3       %    
          TIBC        248  MCG/DL (L)   MONO-A      6.0       %    
          WBC        3.93   K/MM3 (L)   EOS-A       1.8       %    
          RBC        4.19   M/MM3       BASO-A       .6       %    
          HGB         9.8    G/DL (L)   ATYP LY     3.1       %    
          HCT.       30.3       % (L)   MICRO-A      ++ (*)
          MCV        72.2      FL (L)   HYPO-A        +    
          MCH        23.4      PG (L)   LEFT SH  ABSENT    
          MCHC       32.4    G/DL       FER        22.4   NG/ML    

     In summary, this 6 year old girl with the autosomal recessive inform of polycystic kidney disease and congenital  hepatic fibrosis is generally doing well.  It appears that her renal  function has remained relatively stable with mild renal insufficiency.   Her blood pressure  remains controlled and no changes are necessary with her Enalapril.  She  is also growing at a good rate.  Her anemia appears to be secondary to iron deficiency as demonstrated by above studies.  We would like her to initiate oral iron replacement therapy.

     Thank you for the opportunity to participate in Bess's care.  We would like to review her in 6 months.  In the interim, we would be happy to discuss or review Bess should you or the family wish it.

               Sincerely,


               Joakuim Chef, MD           Antonio Khan, MD
               Fellow, Pediatric Nephrology Attending, Pediatric Nephrology


CONTENT:
Matlock, M.D.          RE: Clarissa Eldon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/27/91

Dear Dr. Matlock:

We had the pleasure of seeing Clarissa in the Endocrine Program on 9/21/92 for evaluation of precocious puberty.

History: Clarissa is a 15 month old black female referred for recent new onset of breast development. 

Clarissa was born to a 42 year old woman with multiple sclerosis diagnosed in 1985. Pregnancy was further complicated by extensive use of cocaine resulting in a positive urine screen in the infant and severe withdrawal necessitating a two day ICU  admission and two week hospital stay. Clarissa was also noted to have hydrocephalus at birth. She was placed from birth in the custody of her 22 year old half sister, Sheila Walker, who brought her here today. 

Over the first few years of life, Clarissa's hydrocephalus resolved spontaneously. A CT scan done in September 1991 was normal. She was also noted to have some hypertonicity which has been followed at the neurology clinic at Children's Hospital and has since resolved. 

Over the past few months Clarissa has grown well and has always been big for her age. She has a large appetite and in general has been in good health. About one month ago, Ms. Walker noted enlargement of the breast, left > right which has continued to increase over the last month. There has been no galactorrhea, axillary or pubic hair development.  Ms. Walker and Clarissa live with her biologic mother. The latter is in a wheelchair and has many problems. She allegedly continues to use drugs herself and with others in the house as well. The children are exposed to cocaine, and smoke and are deprived of their food and welfare funds by the mother's drug use. There is no known exposure to marijuana, birth control pills, or estrogen creams. Ms. Walker has reported this to the DSS several times.

Past medical history:  Clarissa had otitis media x 2 and gastroenteritis x 1.

Review of systems:  Development has been progressing. Currently, Clarissa is very alert and intelligent and understands language well.

Family history:  Clarissa's half sister, Ms. Walker, had early puberty with breast development at age 7 and menses at age 8. Clarissa's biologic mother and grandmother have thyroid problems. In general, the family members are tall.

Physical exam:  
Height: 82 cm           Weight: 11.3 kg         
Chest: Lungs clear. Examination of the breasts showed bilateral breast tissue. Left breast measuring 5 x 3 cm and right 4.5 x 2.5 cm. On the lower back there was a large pigmented area, probably a Mongolian spot. There were two other such spots higher up on the back.   Heart:  Regular rate and rhythm.   Abdomen: Benign.   Genitourinary:  Normal female genitalia. No pubic or axillary hair.  Vaginal mucosa was pink.   Neurological:  Normal. Fundi unremarkable.

Impression: Clarissa, at 14 months of age has had the appearance of breast development with bilateral breast tissue increasing over the last month, as well as other evidence of estrogenization. We must rule out  both ovarian and pituitary abnormality.

Disposition: Return to endocrine in approximately one month for further work-up if necessary. In addition, we will contact the DSS worker involved in Clarissa's case and report to her what Ms. Walker has shared with us during this visit, as we are concerned about the environment in which Clarissa lives.
Thank you for letting us share in the care of Clarissa.

Sincerely,


Tinisha Khan, MD       Bernardo Bird, MD         
                             Associate in Endocrinology   


Addendum: September 28, 1992:  Ultrasound showed bilateral small ovarian cysts. There was no evidence of adrenal abnormality. The uterus was at the limits of normal size. Estradiol level was <20 pg/mL, LH<1.2, FSH 1.4 mIU/mL.  Bone age was 2 years (SD=2.7 months) I contacted Ms. Walker with these results and also called DSS to discuss the social environment. Ms. Walker has left the home and will most likely enter a shelter and hopes to be placed in an apartment. She reported typical incidences of drug use in the home that precipitated her leaving the home. She will keep in touch with us to schedule appointments for Clarissa.


CONTENT:
Matlock, M.D.          RE: Clarissa Eldon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/27/91

Dear Dr. Matlock:

We had the pleasure of seeing Clarissa in the Endocrine Program on 11/2/92 for premature thelarche.

History: This is a follow-up visit for Clarissa Eldon, a 16 month old with early breast development.  She is one month s/p work-up for this condition.  She has been in good general health and active and has a good appetite.  She has no galactorrhea or vaginal discharge.  Clarissa has a disrupted social situation.  She is now with her biological mother instead of her older sister who has been the legal guardian.  DSS was contacted and they are followinng the situation and feel that Clarissa is in no danger at this time.  Ellen Goodman, LICSW, will be in contact with DSS in the future.

Family history:  As previously stated the family history is positive for early menarche in the mother(age 9) and Clarissa's sister (also age 9).

Physical exam:  
Height: 85 cm           Weight: 12.0 kg         
Her general physical exam is within normal limits.  Her breast development is unchanged.  Genital exam is also unchanged.

Laboratory studies:  Positive ultrasound for multiple ovarian cysts and an advanced bone age.

Impression: Our impression is that Clarissa exhibits an increased growth rate crossing percentiles along with an advanced bone age,  and ultrasound showing multiple ovarian cysts.  She is at risk for possible precocity and should be followed closely.  We would like to see her again in one month and continue to follow the growth rate.  Should this increase,  Clarissa will be started on Lupron.

Thank you for letting us share in the care of Clarissa.

Sincerely,


Tinisha Khan, MD       Mitsuko Simpson, MD             
                             Chief, Emeritus


CONTENT:
Matlock, M.D.          RE: Clarissa Eldon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/27/91

Dear Dr. Matlock:

We had the pleasure of seeing Clarissa in the Endocrine Program on 1/7/93 for follow-up of premature thelarche.

History: As you know, Clarissa is now an 18 month old who at the time of her last visit 2 months ago was noted to have slightly accelerated linear growth. This in combination with her unchanging breast exam and slightly advanced bone age prompted an early return visit to the endocrine program to document whether continued pubertal progression was occurring. Since her last visit, she has shown no acceleration in linear growth according to her mother and no noted change in the size of her breasts. She sleeps well at night and is quite active during the day. She has no new skin lesions.

Current medications: None.

Physical exam:  
BP: 102/66              Pulse: 96               
Head circumference: 45.25 cm
Skin: Turgor was good. No cutaneous lesions. 
HEENT:  Intact extraocular movements, equally responsive pupils and a moist oropharynx.  Neck: Without thyromegaly.  Chest: Left breast bud measured  4 x 4.5 cm and the right bud 4.5 x 4.0 cm. There was no discharge from the breast.  Abdomen:  Soft without hepatosplenomegaly or masses.  Genitourinary: No clitoromegaly and a red, vaginal mucosa. Extremities:  Deep tendon reflexes were 2+ and equal with normal relaxation phase.  Neurological: Nonfocal.

Impression: Since her last visit, Clarissa has exhibited a normal rate of linear growth as well as weight gain. She has had no progression in the size of her breasts and no appearance of pubic hair.  Though this still could be an intermittently present sexual precocity, her course is not inconsistent with premature thelarche as well. We will continue to follow Clarissa at four month intervals to document her continued linear growth as well as any changes in physical exam that may be occurring.  We ordered no studies today but would like to see Clarissa back in four months.

Thank you for letting us share in the care of Clarissa.

Sincerely,





Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

     We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, June 14, 1994.  He has chronic renal failure secondary to .  He had a Renal Transplant on .
     Frederic underwent cadaveric renal allograft transplantation in 11/92.  He is doing very well.  Recent serum creatinine values have been 0.7 and blood pressure has been well controlled.  He received a several week course of Acylovir after having been exposed to chicken pox.  He did not develop an infection and completed the course one week ago.  He is followed by Dr. Kaelin in Burlington Vermont.

Current medications include:
     CYCLOSPORINE 70 mg po t.i.d.
     IMURAN 50 mg po q.o.d.
     IMURAN 25 mg po q.o.d.
     PREDNISONE 8 mg po q.o.d.
     PROCARDIA XL 30 mg po q.d.
     LASIX 10 mg po q.d.


Physical exam:
     BP 104/50   P 80   R 16   T    HT 103.1cm   WT 18.6kg   HC cm
     General: Active healthy appearing boy who is hirsute
     HEENT: unremarkable without evidence of cataracts
     Chest: clear
     Heart: normal
     Abdomen: soft nontender with normal bowel sounds.  Allograft is nontender. 
     Extremities: no edema

Urinalysis:
     SPEC GR UA  1.015           WBC ENZ UA  0          
     PH UA       5               PROTEIN UA  N          
     BLOOD UA    N               GLUCOSE UA  N     mg/dl
     WBC/HPF     N     /HPF      RBC/HPF     N     /HPF 
     WBC CAST    N     /LPF      RBC CAST    N     /LPF 
     GRAN CAST   N     /LPF      WAXY CAST   N     /LPF 
     SQUAM EPI   N     /HPF      BACTERIA    N     


Laboratory studies at this time include:
          BUN 34     CR 0.6   NA 141  K 3.8   CL 105
          GLU 91      CA 9.3    PO4 4.4   CYA 170
          WBC 6.99 HCT 32.6  HGB 11.8  MCV 83.5  PLT 352
          N - 21.4   L - 64.7   B - .7   ABSN - 1.50

     Frederic continues to do very well.  He is displaying catch-up growth.  He has stable graft function on a triple immunosuppressant.  His blood pressure is well controlled.

     We have asked Frederic to return in .

     Thank you for allowing us share in Frederic's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Gleide Blue, MD


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

     We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, December 13, 1994.  He has chronic renal failure secondary to obstructive uropathy.  He had a Renal Transplant on November 21, 1992.
     Frederic has been well since his last visit.  He has had an occasional headache which is short lasting and central and is not associated with vomiting.  He also has had a series of ear infections and currently has a cold.

Current medications include:
     CYCLOSPORINE 80 mg t.i.d.
     PREDNISONE 9 mg q.o.d.
     IMURAN 50 mg/25 mg q.o.d.
     PROCARDIA XL 30 mg q.d.

Physical exam:
     BP 110/65   P 75   R 20    HT 107.3cm   WT 19.68kg   
     General: he appeared well, hirsute
     Skin: normal
     HEENT: bilateral otitis media  
     Neck: supple, no masses, lymph nodes not palpable
     Chest: clear
     Heart: 2 heart sounds, no murmurs
     Abdomen: soft, non-tender
     Genitourinary: Tanner 1
     Extremities: no edema
     Neurological: normal
Urinalysis:
     SPEC GR UA  1.020           WBC ENZ UA  0          
     PH UA       5               PROTEIN UA  N          
     BLOOD UA    N               GLUCOSE UA  N     mg/dl
     WBC/HPF     N               RBC/HPF     N          
     WBC CAST    N               RBC CAST    N          
     GRAN CAST   N               WAXY CAST   N          
     SQUAM EPI   N               BACTERIA    N     


Laboratory studies at this time include:
          CA          9.6   MG/DL       CR          0.6   MG/DL    
          CYA         126   MCG/L       GLU          79   MG/DL    
          PHOS        3.9   MG/DL (L)   BUN          18   MG/DL    
          NA          141  MMOL/L       K           3.3  MMOL/L    
          CL          103  MMOL/L       WBC        5.28   K/MM3 (L)
          RBC        4.24   M/MM3       HGB        12.3    G/DL    
          HCT.       35.5       %       MCV        83.7      FL    
          MCH        29.1      PG       MCHC       34.7    G/DL    
          RDW        11.7       %       HDW        2.74    G/DL    
          PLT UNC     306   K/MM3       NEUT         17       % (L)
          LYMPH        65       % (H)   MONOS         3       %    
          ATYP LY       8       % (H)   BAND          3       %    
          OTHER         4       %       CELLS C     100    
          ABS NEU    1.06   K/MM3       ABS LYM    3.43   K/MM3    
          ABS EOS     .00   K/MM3       MORPH       YES    
          ANISO        1+       


     Frederic continues to do well post transplant.  We have prescribed Amoxicillin 250 mg tid for ten days for his otitis media.

     We have asked Frederic to return in six months.

     Thank you for allowing us to share in Frederic's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

     We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, June 20, 1995.  He has chronic renal failure secondary to obstructive uropathy.  He had a renal transplant on 11/21/92.
     Frederic has been well since his last visit in December with the exception of intermittent migraine type headaches.  Because of the headaches his Procadia was discontinued and he was started on Propranolol 10 mg tid.  The headaches have subsided significantly since this change in medications.

His current medications include:
     CYCLOSPORINE 85 mg po t.i.d.
     PREDNISONE 10 mg po q.o.d.
     PROPRANOLOL HCL 10 mg po t.i.d.
     IMURAN  50 mg/25 mg po q.o.d.

Frederic's physical exam was notable for:
     BP 125/85    HT 111.8cm   WT 21.1kg   
     General: he appeared well, hirsute
     Skin: without rashes
     HEENT: benign
     Neck: supple, no masses, no lymph nodes 
     Chest: clear
     Heart: regular rate and rhythm without murmurs, gallops or rubs with a normal S1 and physiologic split S2
     Abdomen: soft, non-tender with well healed scars from bilateral nephrectomies, he has a palpable graft in his left lower quadrant which is non-tender
     Genitourinary: Tanner 1
     Extremities: no edema
     Neurological: non-focal
A urinalysis revealed:
     SPEC GR UA  1.014           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5               RBC CAST    N          
     PROTEIN UA  N               GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    none  

Laboratory studies at this time include:
          CA          9.7   MG/DL       CR          1.0   MG/DL (H)
          CYA         251   MCG/L       GLU         116   MG/DL (H)
          PHOS        4.9   MG/DL       BUN          34   MG/DL (H)
          NA          142  MMOL/L       K           4.0  MMOL/L    
          CL          108  MMOL/L       WBC        4.84   K/MM3    
          RBC        4.11   M/MM3       HGB        12.5    G/DL    
          HCT.       35.3       %       MCV        85.8      FL    
          MCH        30.4      PG       MCHC       35.4    G/DL    
          RDW        13.6       %       HDW        2.85    G/DL    
          PLT UNC     262   K/MM3       NEUT         27       % (L)
          LYMPH        60       % (H)   MONOS         6       %    
          EOS           2       %       BASO          2       %    
          ATYP LY       1       %       BAND          2       %    
          CELLS C     100               ABS NEU    1.40   K/MM3    
          ABS LYM    2.90   K/MM3       ABS EOS     .10   K/MM3    
          ANISO        1+       


     Frederic appears stable and is doing well post transplant.  His CYA level is a little high today and should be repeated in the near future.

     Thank you for the opportunity to participate in Frederic's care.  We would like to review him in six months.  In the interim, we would be happy to discuss or review Frederic should you or the family wish it.

               Sincerely,


               Gleide Blue, MD
               Attending, Pediatric Nephrology


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

     We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, December 12, 1995.  He has chronic renal failure secondary to obstructive uropathy.  He had a renal transplant on 11/21/92.

     Frederic reports persistent headaches associated with light sensitivity.  The headache is relieved with Tylenol and rest in a dark room.  Also, the father also reports hyperactivity and attention difficulties at school and that he was held back in school this year.

His current medications include:
     PREDNISONE 10 mg po q.o.d.
     PROPRANOLOL HCL 20 mg t.i.d.
     CYCLOSPORINE 85 mg t.i.d.
     IMURAN  50 mg/25 mg po q.o.d.

Frederic's physical exam was notable for:
     BP 130/88   P 60     HT 114.3cm   WT 22.7kg   
     General: well developed, well nourished male.
     Skin: warm.
     HEENT: normal cephalic atraumatic.  Pupils are equal and reactive to light.  Oropharynx is benign.
     Neck: supple, lymph nodes with negative adenopathy
     Chest: clear to auscultation.
     Heart: no murmurs.
     Abdomen: soft, non-tender, non-distended with positive bowel sounds.  Transplant kidney is in the left lower quadrant which is non-tender.
     Extremities: no edema.
     Neurological: non-focal.

A urinalysis revealed:
     SPEC GR UA  1.014           RBC/HPF     0-1        
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       6               RBC CAST    N          
     PROTEIN UA  N               GRAN CAST   N          
     BLOOD UA    trace           WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     0-1             BACTERIA    0     
     renal tubular epi cells 0-1

Laboratory studies at this time include:
          K           4.3  MMOL/L       RDW        13.7       %    
          CA          9.1   MG/DL       HDW        3.16    G/DL    
          CR          0.9   MG/DL (H)   PLT UNC     220   K/MM3    
          CYA         169   MCG/L       NEUT AB    4.26   K/MM3    
          GLU         121   MG/DL (H)   LYMPH A     .75   K/MM3    
          PHOS        3.8   MG/DL (L)   EOS ABS     .02   K/MM3    
          BUN          32   MG/DL (H)   NEUT-A     73.0       % (H)
          NA          136  MMOL/L       LYMPH-A    12.8       % (L)
          CL          107  MMOL/L       MONO-A     11.5       % (H)
          WBC        5.84   K/MM3       EOS-A        .4       %    
          RBC        3.74   M/MM3       BASO-A       .2       %    
          HGB        11.4    G/DL       ATYP LY     2.0       %    
          HCT.       31.3       % (L)   NF-2002  ABSENT    
          MCV        83.6      FL       MORPH      SAME    
          MCH        30.5      PG       MICRO        1+    
          MCHC       36.5    G/DL (H)   SPHERO     RARE (*)

     Frederic is status post renal transplant with persistent headaches.  His blood pressure today was slightly elevated today 130/88.  We have advised his father that you will reassess him regarding blood pressure monitoring.

     Thank you for the opportunity to participate in Frederic's care.  We would like to review him in six months.  In the interim, we would be happy to discuss or review Frederic should you or the family wish it.

               Sincerely,


               Gleide Blue, MD
               Attending, Pediatric Nephrology


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

     We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, June 11, 1996.  He has chronic renal failure secondary to obstructive uropathy.  He had a renal transplant on 11/21/92.

     Over the past few months Frederic has been complaining of headaches which have been decreasing in frequency according to his father.  He also had an otitis media episode 2-3 weeks ago that was treated with Amoxicillin.  In the interim he has been doing quite well.  Today he complains of vague abdominal pain and occasionally when somebody pushes over his bladder.  He has been afebrile and denies any dysuria.

His current medications include:
     PREDNISONE 10 mg po q.o.d.
     PROPRANOLOL HCL 20 mg t.i.d.
     SANDIMMUNE 85 mg t.i.d.
     PROCARDIA XL 30 mg q.d.
     IMURAN  50 mg/25 mg po q.o.d.

Frederic's physical exam was notable for:
     BP 115/70   P 86   R 18   HT 117.8cm   WT 23.8kg   
     General: alert, cooperative very active boy
     Skin: well healed surgical scars.
     HEENT: normal cephalic atraumatic.  Pupils are equal and reactive to light.  TM's are normal bilaterally with good mobility. Oropharynx with moist mucous membranes.  He is missing his two upper teeth and his two lower teeth are growing in.  
     Neck: supple with full range of motion, lymph nodes are normal.
     Chest: clear.
     Heart: regular rate and rhythm with no murmurs.
     Abdomen: soft, non-tender, non-distended there is no tenderness with palpation over the bladder.  His transplanted kidney is not tender.
     Extremities: warm and well perfused with no edema.
     Neurological: non-focal.

A urinalysis revealed:
     SPEC GR UA  1.011           RBC/HPF     0          
     WBC ENZ UA  0               WBC CAST    0          
     PH UA       5               RBC CAST    0          
     PROTEIN UA  0               GRAN CAST   0          
     BLOOD UA    trace           WAXY CAST   0          
     GLUCOSE UA  0     mg/dl     SQUAM EPI   0          
     WBC/HPF     0               BACTERIA    0     

Laboratory studies at this time include:
          K           4.0  MMOL/L       MCHC       34.7    G/DL    
          CA          9.1   MG/DL       RDW        13.4       %    
          CL          105  MMOL/L       HDW        3.06    G/DL    
          CR          0.8   MG/DL (H)   MPV         8.3      FL    
          CYA         522   MCG/L       PLT UNC     277   K/MM3    
          GLU         101   MG/DL       NEUT AB    6.00   K/MM3    
          PHOS        4.2   MG/DL (L)   LYMPH A    3.72   K/MM3    
          NA          139  MMOL/L       EOS ABS     .39   K/MM3    
          BUN          21   MG/DL (H)   NEUT-A     52.5       %    
          WBC       11.43   K/MM3       LYMPH-A    32.5       %    
          RBC        3.93   M/MM3       MONO-A      8.9       % (H)
          HGB        11.4    G/DL       EOS-A       3.4       %    
          HCT.       32.8       % (L)   BASO-A       .6       %    
          MCV        83.5      FL       ATYP LY     2.0       %    
          MCH        29.0      PG       LEFT SH  ABSENT    

     Frederic's complaints of lower abdominal pain without dysuria are suggestive of cystitis although the urinalysis and microscopic are negative at this time.  The urine culture was negative.  We will confirm when his CYA level was drawn in relation to his dose.  His renal function and growth are excellent.  We increased the Prednisone to 12 mg qod and Imuran to 50 mg qd because of his growth.

     Thank you for the opportunity to participate in Frederic's care.  We would like to review him in six months, with follow up in Vermont as scheduled.  In the interim, we would be happy to discuss or review Frederic should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Ludlow Kaelin, M.D.          RE: Frederic Ledbetter
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/04/89

Dear Dr. Kaelin:

We had the pleasure of seeing Frederic in the Renal Transplant Program on Tuesday, December 17, 1996.  He has chronic renal failure secondary to obstructive uropathy.  He had a renal transplant on 11/21/92.

Frederic offers no complaints today.  He is recovering from an upper respiratory tract infection.  He has had no fever or cough and is otherwise feeling well.

His current medications include:
     PROPRANOLOL HCL 20 mg t.i.d.
     PROCARDIA XL 30 mg q.d.
     PREDNISONE 12 mg q.o.d.
     SANDIMMUNE 95 mg t.i.d.
     IMURAN  50 mg q.d.

Frederic's physical exam was notable for:
     BP 114/62   P 64   R 15    HT 121.8cm   WT 25.7kg   
     General: well appearing and in no distress.
     HEENT: within normal limits.  Positive gingival hyperplasia.
     Neck: supple without lymphadenopathy.
     Chest: clear to auscultation.
     Heart: regular rate and rhythm.
     Abdomen: soft without bruits or tenderness at graft site.
     Extremities: warm and well perfused.
     Skin:  positive for hirsutism

A urinalysis revealed:
     SPEC GR UA  1.015           RBC/HPF     N          
     WBC ENZ UA  0               WBC CAST    0          
     PH UA       5               RBC CAST    0          
     PROTEIN UA  N               GRAN CAST   0          
     BLOOD UA    N               WAXY CAST   0          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   occ        
     WBC/HPF     2-3             BACTERIA    N     
0-1 fine granular casts

Laboratory studies at this time include:
          K           4.7  MMOL/L (H)   MCH        30.6      PG    
          CA          9.5   MG/DL       MCHC       36.4    G/DL (H)
          MG          1.9   MG/DL       RDW        13.5       %    
          CR          0.9   MG/DL (H)   HDW        2.91    G/DL    
          CYA         152   MCG/L       MPV         8.8      FL    
          GLU         111   MG/DL       PLT UNC     296   K/MM3    
          PHOS        4.5   MG/DL       NEUT         59       %    
          BUN          39   MG/DL (H)   LYMPH        30       %    
          NA          141  MMOL/L       MONOS         9       % (H)
          CL          105  MMOL/L       ATYP LY       2       %    
          CO 2         25   MEQ/L       CELLS C     100    
          WBC        9.65   K/MM3       ABS NEU    5.69   K/MM3    
          RBC        4.12   M/MM3       ABS LYM    2.90   K/MM3    
          HGB        12.6    G/DL       ABS EOS     .00   K/MM3    
          HCT.       34.6       %       ANISO        1+    
          MCV        83.9      FL       POLYCHRINCREASE (*)

Frederic continues to have stable renal function.  He is continuing to follow his growth curve.  We are going to discuss the issue of changing him from Sandimmune to Neoral.  While there is no clear indication for doing so, he may have trouble finding Sandimmune locally.

Thank you for the opportunity to participate in Frederic's care.  We would like to review him in six months. In the interim, we would be happy to discuss or review Frederic should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Cavinder Leary, M.D.          RE: Steward Chambers
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/12/85

Dear Dr. Leary:

We had the pleasure of seeing Steward in the Endocrine Program on 08/15/91 for evaluation of short stature.

History: Steward is a 5 and 11/12 year old formerly a 6lb. product of a full-term gestation complicated by toxemia.  Delivery was complicated by cephalo-pelvic disproportion resulting in an emergency C-Section.  There were no further complications.  Steward was breast fed for 9 months.  Solids were introduced at 5 months.  Cow's milk formula was added to his diet at 9 months of age.  At 18 months of age the parents noted a deceleration in growth.  At 4 years of age, because of a cousin's treatment of growth hormone, the parental concern regarding insufficient growth became sufficient to be brought to the attention of an Endocrinologist.  Steward has daily bowel movements.  He denies nocturia and does not report headaches or heat or cold intolerance.  He eats three meals per day and has two snacks.  He drinks three 8oz. glasses of skim milk per day and he is very active.  On July of 1990 he underwent an L-dopa and insulin growth hormone stimulation test and on 3/7/91 he was admitted for an overnight growth hormone study in Nebraska.

Current medications: None.

Past medical history:  Steward has had multiple episodes of otitis media but has only required treatment with antibiotics.  He walked at 12 months and said "Dada, Mama" at 5 months.

Family history:  Steward has one brother 3 and 1/2 years of age who is in the 3rd centile for height.  Paternal height is 6'4" and the maternal height is 5'2".  Maternal menarche was between 11 and 12 years of age.  There is a paternal uncle who is 6'1", a paternal grandmother who is 5'10", and a paternal grandfather who is 6'3".  Maternal uncles are 5'8" and 5'3" and maternal grandmother is 4'11" and the maternal grandfather is 5'8".  Steward has two maternal first cousins, both are on growth hormone therapy, one of whom was said to have a clear growth hormone deficiency and the other had a border line response to growth hormone testing.  The maternal grandmother was also noted to have had Meniere's Disease and hyperthyroidism.  There is no family history of diabetes mellitus.

Physical exam:  
Height: 106.9 cm       Weight: 17.8 kg        BP: 85/50
Pulse: 75              U/L ratio:  1.13       Pubic hair Tanner: 1
R. testis: 2.0 x 1.2 cm             L. testis: 2.0 x 1.1 cm 
Penile stretch length: 7.5 cm       Mid-shaft diam.: 1.6 cm 
General, comfortable male.  Skin: no lesions noted.  HEENT: fundi benign, oropharynx benign.  Neck: no thyromegaly.  Chest: clear to ausciltation.  Heart: regular rate and rhythm.  Abdomen: no hepatosplenomegaly.  GU: prepubertal.  Extremities: were without cyanosis, clubbing, or edema.  Neuro: unremarkable.

In summary: On review of Steward's insulin, and induced hypoglycemia L-dopa tests, as well as his overnight study, Steward does not appear to be growth hormone deficient.  However, these tests, by their nature, cannot rule out a partial growth hormone deficiency.  On review of his growth curve, Steward is following the delayed case on the longitudinal growth curve of Bayley and Bayer.  His his bone age at this time is delayed (B.A. = 4 years) this is further evidence that he is constitutionally delayed.  We did inform the parents that growth hormone sufficient children can be made to have a growth accelleration with pharmacological doses of growth hormone but, there is little evidence at this time to suggest that final adult heights are impacted.

Disposition: We informed Steward's parents of the results of the bone age study and suggested that it was congruent with constitutional delay.

Thank you for letting us share in the care of Steward.

Sincerely,


Lancelot Blue, MD                      Kendell Simpson, MD
Fellow in Endocrinology               Clinical Director

IK/SN/smp


CONTENT:
Saurabh Hammer, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Hammer:

We had the pleasure of seeing Evelyn in the Endocrine Program on 09/04/91 for evaluation of hypothyroidism.

History: Evelyn is a 3 month old female, former 780g product of a 25-26 week gestation, which was otherwise uncomplicated.  She was delivered vaginally to a 35 year old G6 Para 2-3, fetal demise times three, neonatal demise times one.  Neonatal history has been complicated by BPD, ROP, apnea and bradycardia, hyperbilirubinemia requiring photo therapy, patent ductus arteriousus treated with Indomethacin, and staph. epi sepsis.  A neonatal state screen reportedly obtained at two weeks of life revealed a T4 of < 1.0mcg/dl and TSH of > 200mcU/ml.  At the time of Evelyn's transfer to Winchester Hospital, a repeat set of thyroid function tests were obtained on 7/23/91, which revealed a T4 of 2.6mcg/dl, a T3 of 125ng/dl, T3U of 30% and a TSH > 100mcU/ml.  Urinary iodide was 1.12mcg/dl (normal 0.01 to 0.8), urinary creatinine was 13mg/dl.  At that time, Evelyn was started on 25mcg of Synthroid per day.  Repeat thyroid function tests on 8/7/91 revealed a T4 of 20.1mcg/dl, a T3 197ng/dl, TSH 0.7mcU/ml.  The dosage of Synthroid was then reduced to 20mcg per day.  On 8/16/91 the T4 was 17.9mg/dl, T3 164ng/dl, T3U 34%, and TSH was 0.2mcU/ml.  Subsequently, Evelyn has been on Synthroid 25mcg every day three out of four days.  Evelyn's discharge weight from Winchester Hospital on 8/23/91 was 2095g.  She breast feeds once a day and has the remainder of her intake as breast milk mixed with MCT oil to make 24 calories per oz.  She drinks 65-70cc of this formula every four hours.  She has bowel movements once or twice per day and has at least six episodes of urination per day.  She is reportedly starting to do fix and follow, and on her most recent ophthalmologic exam, she apparently did not have significant progression of her retinopathy.

Current medications: Synthroid 25mcg per day, three out of four days.  Poly-vi-sol 1cc per day.  Fer-in-sol 0.6cc q d.

Family history:  Father is 5'11" and the mother is 5'5".  Evelyn has a nine year old older sister who is also a former preemie and is doing well.  There is a history of at least transient hypothyroidism in the maternal grandmother and Evelyn's mother has been told that she has had lower limits of normal thyroid function tests.  There is a history of adult onset diabetes mellitus in a distant paternal relatives.

Physical exam:  
Height: 46.0 cm         Weight: 2.35 kg         Pulse: 110 
Breast Tanner: 1        Pubic hair Tanner: 1    
Active infant in no apparent distress.  Skin: slightly mottled.  HEENT: head circumference 34.0cm, anterior fontanelle open measuring 5 x 3cm.  Posterior fontanelle was also open, mucus membranes were moist.  Neck: no thyromegaly.  Chest: rhonchi were heard throughout.  Heart: regular rate and rhythm with no murmur.  Abdomen: liver down 1.5cm.  GU: anogenital ratio was 0.35.  Extremities: no deformities or cyanosis or edema.  Neuro: Unremarkable.

In summary: Evelyn is growing well and appears to be in good health.  Her hypothyroidism may either have been caused by a transient blockade by Iodine, presumably through exposure in the early neonatal period or permanent due to ectopic or absent thyroid gland.  Due to her history of prematurity and relatively low phosphorous and calcium intake, she is at some risk for Rickets.

Disposition: We were unable to obtain enough blood for TFT's at the time of this visit but have arranged for them to be obtained by Dr. Chen.  At the time of this visit, alkaline phosphatase was 412mU/ml, Ca 9.7mg/dl and phosphorous 5.3mg/dl, all of which are acceptable levels at this age.

Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD                      Kendell Simpson, MD
Fellow in Endocrinology               Clinical Director

IK/SN/smp


CONTENT:
Saurabh Hammer, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Hammer:

We had the pleasure of seeing Evelyn in the Endocrine Program on 11/14/91 for evaluation of congenital hypothyroidism.

History: Evelyn is a 5 month old former 25-26 week premature infant who was last seen on 9/4/91 for congenital hypothyroidism.  At that time, she was on 25 mcg every 3 out of 4 days which comes out to an average daily dose of .75 mcg day.  Her last T4 obtained in the 3rd week of October was 10.4 mcg /dl.  She has had a good appetite since her last visit drinking breast milk between 20 and 30 minutes 5 times a day.  She has bowel movements once or twice a day and her diaper is always wet when it is changed.  Her mother noticed that Evelyn is less mottled.

Current medications: Synthroid 25 mcg 3 out of 4 days.

Physical exam:  
Height: 53 cm           Weight: 4.05 kg         Pulse: 120              
Breast Tanner: 1        Pubic hair Tanner: 1    
General: Active infant in no apparent distress.
Skin: No mottling, nebus flammeus at the nape of the neck.
HEENT: Head circumference 39 cm. Anterior fontanel open and measuring 3 1/2 x 3 1/2 cm. Posterior fontanel closed. Extraocular movements full. 
Neck: No thyromegaly.
Chest: Clear to ausculation.
Heart: Regular rate and rhythm without murmur.
Abdomen: No hepatosplenomegaly.
Genitourinary: Prepubertal.
Extremities: No deformities, no cyanosis.
Neurological: Unremarkable.

In summary: Evelyn is growing well and gaining weight well.  She appears to be thriving and therefore her hypothyroidism appears well controlled as evidence by her recent T4 in the normal range.  As mentioned previously, it remains unclear whether her thyroidism was caused by iodine exposure or true congenital hypothyroidism.  In either case we will continue to treat her without interruption through at least one year of life.

Disposition: We obtained thyroid function tests at the time of this visit. T4 was 11.9 mcg/dl, TT3 155 ng/dl, TBGI 1.02 and TSH 0.3 mcU/ml. We would very much like to see Evelyn in 2 months.

Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Elmo Domingo, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Domingo:

We had the pleasure of seeing Evelyn in the Endocrine Program on 2/10/92 for  congenital hypothyroidism.

History: Evelyn is an 8 month old with congenital hypothyroidism who was last seen on November 14, 1991.  She has been treated with daily Synthroid.  She has been noted to have a brisker appetite and started on rice cereal last month.  She is also breast fed 4-5 times per day, each feeding lasting approximately 15 minutes.  She has bowel movements 1-2 times every other day.  She wets her diaper regularly and is starting to make sounds although not forming words.

Current medications: Synthroid 25 mcg QD.

Physical exam:  
Height: 60.1 cm         Weight: 5.77 kg         Breast Tanner: 1        
Pubic hair Tanner: 1    
General: Obviously dolicocephalic infant in no apparent distress.
Skin: 
HEENT: Anterior fontanel 4 x 3 cm.  Head circumference: 43.5 cm, pupils equal, round and reactive to light, extraocular movements intact.  
Neck: No thyromegaly.
Chest: Clear to auscultation.
Heart: Regular rate and rhythm and no murmur.
Abdomen: No hepatosplenomegaly.
Genitourinary: Prepubertal genitalia with unestrogenized vaginal mucosa.
Neurological: Very slightly hypertonic female infant.

In summary: Impression:  Evelyn is a former 25 week premature infant with a history of hypothyroidism who is now on 25 mcg Synthroid.  At this time she shows no evidence of hypothyroidism.

Disposition: WE have obtained another set of thyroid function tests at this time and would like to see Evelyn again 3 months.

Laboratory studies pending at this time include: Addendum:  T4 11.9 mcg/dl, TBGi 1.02, TT3 155 ng/dl, TSH 0.3 mcU/ml.

Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Saurabh Hammer, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Hammer:

We had the pleasure of seeing Evelyn in the Endocrine Program on 05/28/92 for follow-up evaluation of congenital hypothyroidism.

History: Evelyn is a 11 and 1/2 month old girl with congenital hypothyroidism who was last seen on 2/10/92 and whose latest TFT's on 3/9/92 included a T4 of 11.6 mcg/dL and a TSH of 1.4 mcU/mL.  Developmentally she is doing well, starting to  saying "Dada and Mama", sits unsupported and is vigorous.  Bowel movements occur every day to every other day.  Diapers are wet at changing time.  she is now eating "baby" fruits and vegetablesand she continues to be breast fed throughout the day and continues to drink juice.

Current medications: Synthroid 25 mcg/day; Poly-Vi-Sol with iron and fluoride.

Physical exam:  
Height: 67 cm           Weight: 7.04 kg         Pubic hair: Tanner I    
General: Active, babbling infant.
Skin: Warm, well-perfused.
HEENT: Anterior fontanelle 2.5 x 2.5 cm, edentulous, slightly depressed nasal bridge. 
Neck: No thyromegaly.
Chest: Clear to auscultation. 
Heart: Regular rate and rhythm. 
Abdomen: No hepatosplenomegaly.
Genitourinary: Unestrogenized vaginal mucosa.
Neurological: Non focal, developmentally appropriate; deep tendon reflexes normal.

Laboratory studies:  T4 10.0 pg/mL; TBGI 1.05; TSH 1.90 mcU/mL.

Impression: Evelyn is growing and developing well, especially considering that she is a former premature infant.  She appears to be euthyroid.

Disposition: Since her TFT's are normal, we will not change her dose at this time.  We would like to see Evelyn back in the Endocrinology Clinic in two months.
Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             Elmo Domingo, MD                 
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Elmo Domingo, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Domingo:

We had the pleasure of seeing Evelyn in the Endocrine Program on 7/20/92 for congenital hypothyroidism.

History: Evelyn is now 13 months old and has been doing well except for a recent upper respiratory infection.  She is now "cruising", says "Dada, Mama" and imitates animal sounds.  She sleeps through the night (12:00 to 8:00am).  She has bowel movements 1-2 times daily.  She eats four meals a day and also is breastfed.

Current medications: Synthroid 25 mcg/day.  Poly-Vi-Sol with iron and fluoride.

Physical exam:  
Height: 67.7 cm         Weight: 7.42 kg         
General: Dolicocephalic, well-appearing child.
Skin: 
HEENT: Anterior fontanelle 2.5 x 2 cm, single lower incisor.
Neck: No thyromegaly.
Chest: Clear to auscultation.
Heart: Regular rate and rhythm.
Abdomen: No hepatosplenomegaly.
Genitourinary: Unestrogenized vaginal mucosa.
Extremities: No edema.
Neurological: Deep tendon reflexes 2+.

Impression: Evelyn is doing well with adequate TFT's on her prior visit.

Disposition: We will adjust Evelyn's Synthroid dose only if her T4 is below age normals.

Laboratory studies pending at this time include: T4, TBGI, and TSH.
Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             
Fellow in Endocrinology


CONTENT:
Elmo Domingo, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Domingo:

We had the pleasure of seeing Evelyn in the Endocrine Program on 10/13/92 for congenital hypothyoridism.

History: Evelyn is a 16 month old baby girl who has been doing well except for an episode of strep throat on August 25th.  This was associated with a temperature of 104F.  Evelyn also suffered from an episode of measles after her MMR shot on the 15th of September.  This was followed by a runny nose last week and this was treated with Rynaten, 1/2 tsp on a prn basis.  Her appetite has been good.  The diet is adequate.  She is presently being weaned off breast milk and also consumes baby food about 3 to 4 times a day.  Her bowel movements are two per day, the stool being soft in consistency.  Her development is appropriate for age.  She talks and verbalizes.

Current medications: Synthroid 25 mcg/day, Poly-vi-sol with iron and fluoride and Rynaten 1/2 tsp once or twice per day on a prn basis.

Physical exam:  
Height: 73.1 cm         Weight: 8.15 kg         Pulse: 128              
HEENT: Marked frontal bossing.  Head circumference: 47.2 cm.    The head was otherwise atraumatic.  Pupils bilaterally equal and reactive to light.  She also has two lower incisors and one upper incisor.  The anterior fontanelle measured 2.0x2.1 cm.    Neck: No thyromegaly or lymphadenopathy.    Chest: Lungs were clear to auscultation.  Heart: Regular rate and rhythm.  S1 and S2 heard without murmur.  Abdomen: Unremarkable.  Genitourinary: Unremarkable.  Tanner stage I.  Neurological: Reflexes were intact and cranial nerves were normal.

Impression: Evelyn is a healthy 16 month old being maintained on Synthroid 25 mcg/day.  She is doing well, growing along the 3rd percentile for weight and height, which was her previous level.  The results of her past labs are also normal and hence, at this time, we wish to continue as before.

Disposition: Evelyn is to return to clinic in four months.

Laboratory studies pending at this time include: T4, TBGI and TSH.
Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology   


Addendum: Nadya Ajanee, MD
Harvard Medical Student - IV


CONTENT:
Elmo Domingo, M.D.          RE: Evelyn Muller
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/09/91

Dear Dr. Domingo:

We had the pleasure of seeing Evelyn in the Endocrine Program on 6/21/93 for follow-up of congenital hypothyroidism.

History: Evelyn is a 2 year old girl with congenital hypothyroidism. She has been biochemically euthyroid on 25 mcg a day of Synthroid without constipation, or developmental delay.

Her growth curve shows good linear growth.  She is now between the 3rd and 10th centile.

Current medications: Synthroid 25 mcg/day.

Physical exam:  
Height: 81 cm           Weight: 9.0 kg          Pulse: 90               
General: Dolicocephalic quiet child.  Skin: Warm.  HEENT: Anterior fontanelle closed, mucous membranes moist.  Neck: No thyromegaly.  Chest: Clear.  Heart: Regular rate and rhythm.  Abdomen: No hepatomegaly.  Genitourinary: Prepubertal.  Extremities: No edema.

Impression: Evelyn's course suggests neonatal hypothyroidism was likely due to iodine exposure. I have asked her parents to take her off of Synthroid during August and I have given mother lab slips to check thyroid function tests one month after stopping the Synthroid.

Disposition: To be arranged pending the thyroid function tests results.

Laboratory studies pending at this time include: T4, TSH, TBGI.
Thank you for letting us share in the care of Evelyn.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology


CONTENT:
Brett Kramer, M.D.          RE: Pam Landon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/25/75

Dear Dr. Kramer:

We had the pleasure of seeing Pam in the Endocrine Program on 9/19/91 for evaluation of question of cushin's disease.

History: History is via interpreter.  Patient is a resident of Puerto Rico.  Chief complaint: 16 1/2 year old female referred by neurologist for question of Cushin's Disease.  History of present illness:  She was born 7lb. 11oz., 21" long, was always tall during childhood.  She developed moderate obesity over the last year an a half.  She has had no change in shoe size or ring for two years. She has hypertension noted in 6/91. She has seizure disorder which first was a febrile seizure during infancy but had a catatonic seizure approximately a year ago.  At that time was treated with Dilantin which was switched in February of 1991 to Tegretol because gum hypertraphy acne and hirshitim.  By report, she shaves her armpits as well as the region between symp? pubic to umbilicis.  She had menarche at age 13 but has had irregular menses, with regular menses occurring during the last four months.  She was evaluated by a gynecologist in Puerto Rico which work up included an abdominal ultrasound which showed cystic ovaries.

Current medications: Tegretol.

Family history:  Father is 6'3", grandfather is 6'1", mother is 5'4", sister is age 17 and 5'11".  Mother had polycystic ovary disease requiring oophorectomy.

Physical exam:  
Height: 182.2 cm        Weight: 98.7 kg         BP: 140/80              
Pulse: 80               Breast Tanner: 5        Pubic hair Tanner: 5    
Arm span is 194 1/2cm.  Upper segment is 73cm, lower segment is 109cm.
Skin: notable for acne and moderate hirsitism on the face.
HEENT: normal.
Neck: without goiter
Chest: lungs clear
Cardiac: S1 S2 with a 1/6 systolic murmur
Abdomen: mildly obese with brown stria
Extremities: normal with no muscle atrophy, strength normal
G.U.: genitals Tanner 5

In summary: 16 1/2 year old female with hirshitism, hypertension and obesity abdominal stria.  We doubt that she is Cushin's Disease given the absence of all other stigmata.  However that it is likely that her hypertension hirshitism may secondary to polycystic ovary disease.  Plan is to check FSH, LH, testosterone, fetestosterone, DHEA, DHEAS, androstenedione, 17-hydroxyprogesterone, 24 hour urine, 24 hour ?cortisol.

Disposition: 

Thank you for letting us share in the care of Pam.

Sincerely,


Etta Kramer, MD                      Etta Kramer, MD


CONTENT:
Brett Kramer, M.D.          RE: Pam Landon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/25/75

Dear Dr. Kramer:

We had the pleasure of seeing Pam in the Endocrine Program on 9/19/91 for evaluation of question of cushin's disease.

History: Pam, who is a resident of Puerto Rico, was born by NSVD at 7lb 11oz.  She has febrile seizures as an infant.  She was tall throughout childhood, and developed marked weight gain over the past 1-2 years.  A seizure disorder developed 1 year ago, for which she was placed on Dilantin, changed to Tegretol last February because of hirsutism.  Menarche was at age 13 years, with irregular menses up until 4-6 months ago, with regular cycles since then.  A gyn evaluation in Puerto Rico revealed cystic ovaries on April 5.  Currently, Pam shaves her legs, axillae, and her linea albis.  Of note, Pam was noted to be hypertensive last June.

Current medications: Tegretol.

Family history:  Father is 6'3", grandfather is 6'1", mother is 5'4", sister is age 17 and 5'11".  Mother had polycystic ovary disease requiring oophorectomy.

Physical exam:  
Height: 182.2 cm        Weight: 98.7 kg         BP: 140/80              
Pulse: 80               Breast Tanner: 5        
Arm span is 194 1/2cm.  Upper segment is 73cm, lower segment is 109cm.
Skin: notable for acne and moderate hirsitism on the face.
HEENT: normal.
Neck: without goiter
Chest: lungs clear
Cardiac: S1 S2 with a 1/6 systolic murmur
Abdomen: mildly obese with brown stria
Extremities: normal with no muscle atrophy, strength normal
G.U.: Tanner 5 pubic hair.  No clitiromegaly.

In summary: Pam is a 16 1/2 year old female with hirsitism, borderline hypertension, and history of previous irregular menses, and obesity with abdominal stria.  We doubt that she has Cushing's Syndrome given the absence of other stigmata.  However that it is quite possible that she has polycystic ovary disease. Our plan is to check FSH, LH, testosterone, free testosterone, estradiol, DHEA, DHEAS, androstenedione,17-hydroxyprogesterone,  and 24 hour urine for KS and free cortisol

Disposition: 

Thank you for letting us share in the care of Pam.

Sincerely,


Etta Kramer, MD                      Etta Kramer, MD


CONTENT:
Pluto, M.D.          RE: Bart Buchman
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Pluto:

We had the pleasure of seeing Bart in the Endocrine Program on 10/21/91 for evaluation of short stature.

History: Bart is a 13 year old who was growing in height between the 10th and 25th percentile until age 12 when he began to fall off the NCHS growth curve.  His weight has also fallen off.  He has a good appetite, but was eating low calorie foods such as salads and yogurt until the past month.  Now he is on a regular diet with supplemental Carnation Instant Breakfast.

He denies abdominal pain, diarrhea, fatty or foul smelling stools, constipation, cold intolerance, dysuria, a history of urinary tract infections, headaches, blurred vision, coughing, or frequent upper respiratory tract infections.

Current medications: None

Past medical history:  Birth: He was a 9 pound 13 ounce product of a 42 week gestation, delivered by Cesarean section without complications.  He has attention deficit disorder and was treated with Ritalin from 1986 to December of 1990.  He had Giardia about three years ago which presented with intermittent abdominal pain and was treated with Quinacrine.  

He has had no hospitalizations or surgery.
Injuries: he fractured his right arm two years ago.

Review of Systems:  He has multiple episodes of epistaxis in the winter that resolve after two minutes.

Family history:  His ten year old brother is of short average height, and his six year old sister is the smallest in her class.  Maternal height 5 foot 1 inches, with onset of menses at 13 to 14 years old.  Paternal height 5 foot 10 inches.  Maternal grandmother about 5 foot 7 inches, maternal grandfather 5 foot 9 inches.
Paternal grandmother about 5 foot 3 inches, paternal grandfather reportedly tall.

Physical exam:  
Height: 142.8 cm        Weight: 34.6 kg         BP: 112/82              
Pulse: 50               Pubic hair Tanner: 1    R. testis: 1.4 x 1.1 cm 
L. testis: 1.4 x 1.2 cm Phallus length: 7.5 cm  Mid-shaft diam.: 2.5 cm 
General: 
Skin: no lesions, warm 
HEENT: pupils equal, round and reactive to light.  Extraocular movements intact. Discs benign. Oropharynx clear.
Neck: thyroid not enlarged
Chest: lungs clear
Cor: regular rate and rhythm with a split S2, no murmur. 
Heart: 
Abdomen: soft, no hepatosplenomegaly 
Genitourinary: no pubic hair
Extremities: warm and well perfused
Neurological: 2+ symmetrical deep tendon reflexes bilaterally

Laboratory studies:
T4 9.3 mcg/dl, TBGI 0.97, TT3 169 ng/dl, TSH 2.60 mU/ml -- within normal limits.  CBC and ESR were normal.  IGF-1 0.91 (nl), IGF-BP3 2.5 (nl 2.1 - 6.2).  Antiendomysial antibodies: negative.  Bone Age: 11 1/2 years, PAH 67 1/2".

In summary: Bart seems to be growing parallel to the delayed case on the Bayer-Bayley growth curve, which, along with the delayed bone age and delayed puberty, the normal CBC and ESR, the normal TFT SmC and IGF-BP3, and the negative antiendomysial antibodies, is consistent with Constitutional Delay.

Disposition: Bart is to return to Endocrine clinic in one year.  If he desires an earlier onset of puberty and growth spurt he could be started on testosterone in the future.

Thank you for letting us share in the care of Bart.

Sincerely,


Leif Pavarotti, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Antonio Khan, M.D.          RE: Harriot Logan
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/87

Dear Dr. Khan:

We had the pleasure of seeing Harriot in the Endocrine Program on 03/09/92 for to consider the use of growth hormone treatment.

History: Harriot is a 5 8/12 year old girl with Spondylo-epiphysial dysplasia.  She was born extremely short (14" in length) and carried for some time the diagnosis of hypochondroplasia.  She has been growing at a very slow rate and had surgery a year ago on her hips.  The parents wanted information about the possibility of using growth hormone to increase her rate of growth.

Physical exam:  
Height: 83.8 cm         Weight: 13.5 kg         U/L ratio:  1.3         
Arm span: 90            
Harriot was extremely short but pleasant and cooperative.  She has a waddling gait, a relatively large head, and infantile U/L ration for her age.  Besides the skeletal changes, her physical examination was essentially negative.

Impression: I discussed at length with the parents the unlikelihood that Harriot has growth hormone deficiency; we will however, obtain an IGF1 as a screening test to rule out growth hormone deficiency.  If she is growth hormone sufficient the likelihood of significantly altering her growth velocity may be possible only if high pharmacologic doses are used which will be more likely to be associated with risks of growth hormone excess.  Even then there is no assurance that she will be a taller adult.  The parents were of the opinion that if she has no evidence of growth hormone deficiency they will not pursue further the option of growth hormone administration.

Thank you for letting us share in the care of Harriot.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Giancarlo Red, M.D.          RE: Judith Asbury
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/16/91

Dear Dr. Red:

We had the pleasure of seeing Judith in the Endocrine Program on 10/26/92 for enlarged breast buds.

History: Judith is a 13 month old female who is felt to have always had breast buds but hey have increased in size since 6 months of age.  She has had no lactation, no vaginal bleeding or discharge.  She is on no medications and has had no access to parental medications.

Past medical history:  Notable for being a post-date Cesarean section for malposition.  Birth weight was 8 lb 10 oz.  She has a small number of otitides.

Review of systems:  History of a large hemangioma on the right parietal scalp and a smaller one in the nuchal region.  She also has a history of wheezing and bronchitis which is treated with a humidifier.

Family history:  Only remarkable  for maternal grandfather with diabetes.  The maternal pubertal history was normal.

Physical exam:  
Height: 73.4 cm         Weight: 9.39 kg         
10th to 25th percentile)      (25th to 50th percentile)
General: Well-nourished, well-developed white female. Skin: 5x5 cm hemangioma on her right parietal scalp and a 1 x 1cm. hemangioma on the nape of her neck.  There were no cafe-au-lait spots.   HEENT: Anterior fontanelle was open, soft but fibrous.   Neck: Supple. Chest: Lungs clear. Breasts:  Right and left Tanner stage III. The dimensions of the buds were 4 cm. and the areolae 1.2 cm. in diameter each.  Heart: Regular rate and rhythm without murmur. Abdomen: Soft, no masses, no hepatosplenomegaly.  Genitourinary: Vaginal mucosa was pink.   Extremities: Normal. Neurological: Nonfocal.

Impression: Judith is a 13 month old with premature thelarche.  While it is probably idiopathic, differential diagnosis includes ovarian cyst or tumor, or central precocious puberty. We have ordered a pelvic ultrasound in order to rule out ovarian pathology.

Disposition: We would like to see Judith back in clinic in six months for follow-up.
Thank you for letting us share in the care of Judith.

Sincerely,


Yale Blue, MD            Kendell Simpson, MD             
                             Clinical Director            


Addendum: The pelvic ultrasound revealed no cysts or tumor of the ovaries and thus was consistent with premature thelarche.


CONTENT:
Giancarlo Red, M.D.          RE: Judith Asbury
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/16/91

Dear Dr. Red:

We had the pleasure of seeing Judith in the Endocrine Program on 06/28/93 for follow up of her premature thelarche.

History: Since last seen on 10/26/92 Judith has been well.  Her parents think that the size of her breasts has regressed.  She had no significant intercurrent illness, however, on one occasion she had a trauma to the scalp hemangioma which bled some.  The review of systems was negative.  She had a normal development but she does not have a good appetite.

Physical exam:  
Height: 81.7 cm         Weight: 10.51 kg        Breast: Tanner III      
Pubic hair: Tanner I    
Judith was healthy looking.  Skin:  The hemangioma of the scalp has not changed in size but has become softer.  HEENT:  The anterior fontanelle closed.  She has 16 teeth.   Neck:  Thyroid not enlarged.  Lymph Nodes:  No lymphadenopathy.  Chest:  She still had enlarged breasts: each has a diameter of 3.0 cm with an areola of 1.5 cm.  Cor:  Normal S1 and S2, no murmurs.  Abdomen:  Soft, no hepatosplenomegaly.  GU:  Vaginal mucosa not estrogenized.  Extremities:  Normal.  Neurological:  Non-focal.

Laboratory studies:  [10/30/92]  Pelvic ultrasound: normal size ovaries, no tumors or cysts.

Impression: Premature Thelarche.  Although the breast tissues have not disappeared, they have not progressed and actually regressed some.  She did not have nay acceleration of her linear growth, and her vaginal mucosa was not estrogenized.  These finding suggest premature thelarche rather than precocious puberty having ruled out an ovarian cyst or tumor on her previous visit.

Disposition: Reassess in six months if the breasts have not regressed in size.
Thank you for letting us share in the care of Judith.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Matlock, M.D.          RE: Nellie Vinogradoff
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/11/89

Dear Dr. Matlock:

We had the pleasure of seeing Nellie in the Endocrine Program on 1/9/92 for  obesity.

History: Nellie is a 2.5 year old black female referred here for obesity.  She is a product of a  normal pregnancy and has had normal development to date.  She walked at age 10 months and has been talking since that age as well.  Her past medical history has essentially been unremarkable.  She has been overweight, according to mother, for most of her life, particularly this past year but is not particularly underactive.  She doesn't eat an incredibly large amount, nor does she have an overaggressive appetite.  She doesn't  have cold intolerance or constipation.

Past medical history:  Nellie was the product of a normal pregnancy and vaginal delivery.  Growth and development have been normal to date.   There have been no major infectious diseases, major injuries, hospitalizations or surgeries. 

Review of systems is essentially negative.

Family history:  Positive for obesity.  Mother is clearly obese as is Nellie's brother.  Mother has hypothyroidism and is on Synthroid.  Other than that there are no other major endocrine disorders in the family.

Physical exam:  
Height: 92.1 cm         Weight: 24.1 kg         BP: 90/60               
Pulse: 64               Pubic hair Tanner: 1    
General: Clearly obese young black female in no apparent distress.
Skin: No evidence of hypo or hyperpigmentation.  No obvious hair loss.  Normal temperature and texture.
HEENT: Normocephalic, atraumatic.  Extraocular movements were intact.  Pupils were equal, round and reactive to light. Conjuntiva were pink.  Throat was clear. 
Neck: Supple, without evidence of thyromegaly or adenopathy.
Chest: Clear.
Heart: Regular rate and rhythm without murmurs, rubs or gallops.
Abdomen: Obese without any striae, masses or hepatosplenomegaly.
Genitourinary: Normal, prepubertal xam.
Extremities: Without cyanosis, clubbing or edema.
Neurological: Alert, oriented X3, reflexes were normal and her strength was good.

Laboratory studies:  Outside labs that were sent from the Neighborhood Health Plan dated 12/12/91 revealed a free T4 of 1.4, with a normal range being 0.71-1.85, and a TSH of 0.9 normal range being 0.46-4.98.

In summary: Nellie is a 2.5 year old female with fairly severe obesity.  She is approximately 50% above her ideal weight for height.  She has had normal linear growth and development to date.  Therefore, we doubt that she has any of the usual endocrine causes for obesity including hypothyroidism, growth hormone deficiency, Cushing's syndrome or hypothalamic lesions.  We feel that the most likely explanation for her problem is a constitutional obesity given that both her mother and her brother are severely overweight themselves.  We have suggested that Nellie see a nutritionist for dietary counseling and that she increase her exercise level.  This will undoubtedly require a family-wide effort.


Thank you for letting us share in the care of Nellie.

Sincerely,


Rahul Ibsen, MD                
Attending in Endocrinology


CONTENT:
Aurimar Swartzenegger, M.D.          RE: Agatha Grandi
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/01/88

Dear Dr. Swartzenegger:

We had the pleasure of seeing Agatha in the Endocrine Program on 1/23/92 for  evaluation for short stature.

History: Agatha is a 3 1/2 year old white female referred here for short stature.  She has always been in good health without any significant past medical history except for several viral infections.  She has always been small for her age. She has no complaints of headahces, fatigue, constipation, dry skin or hair loss.  She is a poor eater, however, and according to her parents often leaves much of her meal.  Review of symptoms were unremarkable.

Past medical history:  Pregnancy was Caserean section and her neonatal course was unremarkable.  Her growth and dvelopment have been normal to date.  She has had no major infections other than chicken pox.

Family history:  Negative for any major endocrine disorder other than her grandfather who has Type II diabetes.

Physical exam:  
Height: 87.4 cm         Weight: 11.7 kg         BP: 80/50               
Pulse: 120              Pubic hair Tanner: 1    
General: Well-developed 3 1/2 year old female in no apparent distress.
Skin: Normal without hypo-or hyperpigmentation.  
HEENT: Normocephallic, atraumatic.  Extraocular muscles are intact.  Pupils are round and equally reactive to light.  Conjunctiva is pink.  Sclera is white and throat is clear.
Neck: No evidence of thyromegaly.
Chest: Clear.  Breasts:  Tanner stage I.
Heart: Regular without murmurs, rub or gallops.
Abdomen: Benign without hepatosplenomegaly or masses.
Genitourinary: Normal genitalia.  Tanner stage I.
Extremities: No cyanosis, clubbing or edema.
Neurological: Alert and oriented with a nonfocal exam and normal reflexes.

Laboratory studies:  CBC, ESR, electrolytes, TCO2, BUN, creatinine -- all normal.  TFT'S: T4 8.1 MG/DL, TBGI 1.04, TT3 144 NG/DL, TSH 1.0 mcU/ml -- all normal.  Endomysial antibodies, Somatomedin-C and bone age are pending.

In summary: Our impression is that Agatha is likely folloiwng a genetic pattern of growth.  However, given the marked degree of her short stature, we would like to see her again in 4-6 months to reassess her grwoth velocity.  Given the fact that Agatha is underweight for height (HA=2 years, WA=18 months), it might be prudent to have the family meet with a nutritionist to assess her caloric intake and make some specific recommendations.

Thank you for letting me participate in Agatha's care.  Please don't hesitate to call if you have any questions.


Thank you for letting us share in the care of Agatha.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology


CONTENT:
Aurimar Swartzenegger, M.D.          RE: Agatha Grandi
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/01/88

Dear Dr. Swartzenegger:

We had the pleasure of seeing Agatha in the Endocrine Program on 8/3/92 for follow-up of short stature.

History: Agatha is a 4 and 2/12 year old girl here to check her growth progression. She was last seen in February of 1992. The impression at that time was that she was most likely following a genetic pattern of growth. It was recommended at that time that mother meet with a nutritionist because Agatha was also underweight for her height. The family did not follow through on that and will be addressed at this time. A bone age was obtained at a chronologic age of 2 and 8/12 which was 3 years. Maternal concerns at this time is that Agatha does not eat enough. Her diet is limited to peanut butter and jelly sandwiches and pasta. She does take a vitamin supplement daily. She has had no intercurrent illnesses. She has two siblings who are appropriate for height. Agatha is the only child born after the mother was diagnosed with multiple sclerosis.

Past medical history:  Mother was a 26 year old G3PT2-3 who delivered Agatha with an uncomplicated caesarean section.

Review of systems:  She denies constipation, diarrhea, nausea, and vomiting.

Family history:  Positive for MS in mother. Mother's height is 65 inches. Father's height is 65-67".

Physical exam:  
Height: 88.9 cm         Weight: 12.6 kg         BP: 78/60               
Pulse: 120              U/L ratio:  1.14        Arm span: 84.5          
Breast Tanner: 1        Pubic hair Tanner: 1    

General: There has been a 1.5 cm increase in height in the past six months. Weight is up 0.9 kilos. She is an active girl, but is quiet. 
Skin: No rashes or lesions. 
HEENT: No arching of her palette. Pupils equal, round and reactive to light. 
Neck: No thyromegaly, no webbing of the neck. 
Chest: Lungs clear. 
Heart: Regular rate and rhythm without murmur. 
Abdomen: Soft, non tender. 
Genitourinary: Normal female genitalia. 
Extremities: Slight in toeing when she walks.

Laboratory studies:  1/92 electrolytes normal, IGF1 41 (normal, but low). T4 8.1, TBGI 1.04, Total TT3 144. TSH 1. Thyroid function tests were all normal. Endomesial antibodies negative.

Impression: Agatha most likely has constitutional short stature although, Turner's syndrome must be ruled out.

Disposition: We are helping her mother to arrange a meeting with a nutritionist. We would like to see her again in clinic in six months to chart progression of her growth and evaluate her for an insulin tolerance test.

Laboratory studies pending at this time include: IGF1, binding protein 3, and karyotyping.
Thank you for letting us share in the care of Agatha.

Sincerely,


Bernardo Red, MD           
                             


Addendum: Dr. Quinn

Problem list to date:
                             

Transcribed by Cassells


CONTENT:
Shahin Pulanski, M.D.          RE: Robert Polo
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/22/75

Dear Dr. Pulanski:

We had the pleasure of seeing Robert in the Endocrine Program on 2/10/94 for .

History: I most recently Robert in Endocrinology on February 10, 1994.   As you know, he is an 18 and a half year old young man whom I am following for Kallman Syndrome.   He is in the 12th grade, and works 25 hours a week in a grocery store.   

He is on Testosterone Enanthate therapy, receiving 100 mg every two weeks, which represents an adult replacement dose.  
His intercurrent history and review of systems are unremarkable.  He describes normal sexual function.

Physical exam:  
Height: 170.8 cm        Weight: 71.2 kg         BP: 110/70              
HEENT:  PERRL, Fundoscopic exam was benign.  
Neck:  Revealed edges which where palpable, with smooth and symmetric lobes.  GU:  Revealed Tanner V pubic hair.   Testes measuring 1.5x2.0 on the left and 1.5x1.7 cm on the right.   The phallus measured 2.5x12.0 cm.  
The remainder of the exam was unremarkable.

Impression: Robert continues to do well on his testosterone therapy.   I have sent him for the following hormone studies:  FSH, LH, DHEA sulfate, testosterone, thyroid functions and prolactin.

Disposition: I plan to see Robert again in six months time in order to follow his course.
Thank you for letting us share in the care of Robert.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology   


Addendum: FSH-0.2 mIU/ml, LH-<0.12 mIU/ml, DS-231 mcg/dl, T4-5.3 mcg/dl, TSH 1.8 mcU/ml, TT3-110 ng/dl, testosterone 357 ng/dl.

Problem list to date:
KALLMAN'S-SYNDROME                           04/06/93  
Transcribed by Sullivan_ma


CONTENT:
Shahin Pulanski, M.D.          RE: Robert Polo
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/22/75

Dear Dr. Pulanski:

We had the pleasure of seeing Robert in the Endocrine Program on 2/6/92 for  evaluation of delayed puberty.

History: Robert is a 16 year 8 month old boy who was the product of a full term pregnancy.  He was seen at New England Medical Center at age of 2 for possible small testes.  Work-up at that time revealed 46 XY karyotype Endocrine evaluation at that time revealed testes the size of 1.5 ml in volume bilaterally with penile length of 3.0-3.5 cm.  Since it was thought that the size of his genitals was within normal limits, no specific treatment was recommended.  Robert did not seem gain much development in his sexual organs over the years.  His penis, in fact, remains relatively small.  He also noticed that sometimes his testes would retract back to the inguinal canal.  He started using deodorant about 2 years ago, and he noticed axillary and pubic hair growth in the last 6 months.  He state that he cannot smell any odor, for example onion, hamburger, chocolate, etc.  He also wets his bed frequently.  His review of systems is otherwise unremarkable.

Past medical history:  Significant for nondiplaced parietal skull fracture without any sequalae.   He is not taking any medications and there are no known drug allergies.

Family history:  Father is 5'6", mother is 5'5".  There is an aunt with a thyroid problem.  There is also significant family history of diabetes Type II.  There is no family history of micropenis or delayed puberty.

Physical exam:  
Height: 158.4 cm        Weight: 65.6 kg         BP: 110/70              
Pulse: 80               R. testis: 1.6 x 1 cm   L. testis: 2 x 1 cm     
Phallus length: 4.5 cm  Mid-shaft diam.: 1.5 cm 
HEENT: Pupils equal, round and reactive to light.  Extraocular movements intact.  Fundus:  normal disc.  He was unable to smell coffee.
Neck: Supple.  No JVD.  No goiter.
Chest: Lungs clear.
Heart: Regular sinus rhythm.  Normal S1, S2, no murmur.
Abdomen: Soft, bowel sound present.  Nontender.  No mass. No hepatosplenomegaly.
Genitourinary: Tanner III pubic hair.  Sparse axillary hair noticed on both sides.  No gynecomastia.
Neurological: Cranial nerves 2-12 intact.

In summary: Robert's presentation is consistent with with Kalman's syndrome (hypogonadotropic hypogonadism).  We will check FSH, LH, testosterone, Somatomedin-C, thyroid function tests, cortisol, prolactin and DHEAS today.  We will also schedule a head MRI to rule out pituitary lesions and also schedule hand x-rays to determine his bone age.  Once these studies are back, we will schedule an LHRH stimulation test to assess his gonadotropin axis.  We also discussed several options of treatment for Kalman's syndrome with the family, including testosterone and HCG injections.  We will decide with the family on therapy after we obtain the results from the laboratory studies.


Thank you for letting us share in the care of Robert.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology


CONTENT:
Shahin Pulanski, M.D.          RE: Robert Polo
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/22/75

Dear Dr. Pulanski:

We had the pleasure of seeing Robert in the Endocrine Program on 7/23/92 for follow-up of kallman's syndrome.

History: As you know, Robert is a 17 year old boy whom I am following for Kallman's syndrome. Last April, he was started on testosterone enanthate, receiving 50 mg each month. His intercurrent history and review of systems are unremarkable. Robert does not note any body changes since starting the testosterone therapy.

Physical exam:  
Height: 162.5 cm        Weight: 67.3 kg         BP: 120/60              
Arm span: 165.5         Pubic hair Tanner: 3    R. testis: 2.2 x 1.0 cm 
L. testis: 2.0 x 1.0 cm Phallus length: 6 cm    Mid-shaft diam.: 2.0 cm 
U/L segment ratio: 0.91
Fundoscopic exam was benign. He had a 2/6 soft systolic ejection murmur at the left lower sternal border. There was no axillary hair. The remainder of the exam was unremarkable.

Impression: Robert has displayed a good initial response to his testosterone therapy, both in terms of growth velocity as well as genital development. I will increase his dose to 75 mg im every 4 weeks, and will see him back again in 4 months time.

Thank you for letting us share in the care of Robert.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology


CONTENT:
Shahin Pulanski, M.D.          RE: Robert Polo
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/22/75

Dear Dr. Pulanski:

We had the pleasure of seeing Robert in the Endocrine Program on 4/6/93 for .

History: I most recently saw Robert in Endocrinology on April 6, 1993.  As you know, he is a 17 and 9/12 year old boy whom I am following for Kallman's syndrome.  He has been on testosterone therapy since last April, and is currently receiving 100 mg IM each month.  Since his last visit, his intercurrent history and review of systems are unremarkable.

Physical exam:  
Height: 168.0 cm        Weight: 73 kg           BP: 111/43              
Pubic hair: Tanner V    R. testis: 2.0 x 1.4 cm L. testis: 2.2 x 1.2 cm 
Phallus length: 9.0 cm  Mid-shaft diam.: 2.4 cm 
Fundoscopic exam was benign.  There was no thyromegaly.  He had trace facial hair above his upper lip.  He had axillary hair.  There was mild gynecomastia present.  The remainder of the exam was unremarkable.

Impression: Robert continues to do well on his testosterone therapy, progressing with normal pubertal development.  I have obtained a bone age, and have increased Robert's testosterone dose to 100 mg every three weeks.  I plan to see Robert again in four months time in order to follow his course.

Thank you for allowing me to participate in Pauls' care.  Please don't hesitate to call if you have any specific questions.

Thank you for letting us share in the care of Robert.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology   


Addendum: Bone age=15 years.


CONTENT:
Pericles Meisner, M.D.          RE: Edmund Devereux
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/21/76

Dear Dr. Meisner:

     We had the pleasure of seeing Edmund in the Renal Program on 10/12/95 for hypertension.
     Edmund comes in today for an interval visit.  It has been over two years  since his last Renal Program evaluation.  He has kept in intermittent  phone follow-up with the program.  He continues to take his Enalapril  without problems.  He has not been checking his blood pressure as an  outpatient. 
Edmund's blood pressure appears to be adequately maintained on his  current dose of Enalapril.  During his appointment today, his initial  blood pressure readings showed a somewhat elevated systolic reading.  On  repeated measurements, Edmund's blood pressure improved.  I therefore  suspect that there was an element of anxiety in the initial higher  readings. 

I spent some time discussing hypertension with Edmund.  I reemphasized  that we believe he has essential hypertension since his extensive  work-up has not revealed any organic abnormalities.  I emphasized that  hypertension is a risk factor for cardiac disease and also warned Edmund  that obesity and a sedentary lifestyle can complicate his health in the  future.  I advised him to maintain some sort of aerobic exercise program  and also counseled him regarding weight loss.  Over the last 2 years,  Edmund has gained some 20 kg. 

I also explained to Edmund that we would like to follow at a somewhat  regular interval for the next two years to assess his hypertension a bit  more closely.  I told him that it would be important to have his blood  pressure checked if he were to go to a health care provider for an  interval visit.  I do not believe, however, that we need to establish  some sort of regular blood pressure checks as an outpatient besides the  interval checks at clinic here.

His current medications include:
     Enalapril 10 mgm q am

Edmund's physical exam was notable for:
     BP 137/63   P 86   R    T    HT cm   WT 110kg
     In general he's an  alert, active young man in no distress.  
     HEENT:  exam is remarkable for tympanostomy tubes bilaterally.  
     Neck:   supple without thyromegaly or adenopathy. 
     Chest:   The configuration and  excursion is normal.  
     Lungs:  are clear to auscultation.   
     Heart:  Cardiac exam is without murmur.  S1 and S2 are normal.  Pulses are full.   
     The abdomen: is obese.  There is no organomegaly or mass.  
     Extremities:  are well-formed.  
     Skin exam is remarkable for scattered striae on the  abdomen.
A urinalysis revealed:
     SPEC GR UA  1.028           BLOOD UA    N          
     PH UA       5               PROTEIN UA  trace      
     WBC ENZ UA  N          

Laboratory studies at this time include:


     Thank you for the opportunity to participate in Edmund's care.  We would like to review him in six months.  In the interim, we would be happy to discuss or review Edmund should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Attending, Pediatric Nephrology


CONTENT:
Ludlow Meisner, M.D.          RE: Edmund Devereux
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/21/76

Dear Dr. Meisner:

We had the pleasure of seeing Edmund in the Renal Program on 12/19/96 for hypertension.
Edmund comes in today for an interval visit.  It has been just over one year since his last Renal Program evaluation.  During that  time, he has been doing well.  He is attending college full time and is studying nursing.  He has had no interval health problems.  He continues to take his enalapril on a daily basis without difficulty. 

Edmund does tell me that he tries to follow a no added salt diet.  He  reports that he has little if any exercise and is generally rather  sedentary.  He was referred by his primary provider to a nutritionist, but has missed that appointment.

His current medications include:
     ENALAPRIL MALEATE 10 mg q.a.m.
     ENALAPRIL MALEATE 5 mg q.p.m.


Edmund's physical exam was notable for:
     BP 142/51   P 76   R    T    HT cm   WT 115.5kg
     On physical examination, Edmund was an alert and active young man in no distress.  The HEENT exam was benign.  
     Neck was supple, without thyromegaly or adenopathy.  
     The configuration and the excursion of the chest was normal.  
     Lungs are clear to auscultation.   
     Cardiac exam was without murmur.  S1 and S2 are normal.  Pulses are full.  
     The abdomen was obese.  There was no hepatosplenomegaly or mass.   
     Extremities were well formed, without edema.  
     Skin exam is remarkable for some striae present on the abdomen.
A urinalysis revealed:
     SPEC GR UA  1.032           PROTEIN UA  -          
     WBC ENZ UA  -               BLOOD UA    -          
     PH UA       5               GLUCOSE UA  -          

Edmund continues to have adequate blood pressure  control on his current therapy.  He has had a tendency to have slightly elevated systolic pressures, but these have improved markedly on his enalapril, and his diastolic pressures are well within an acceptable range.   

I spent some time discussing with Edmund the importance of his trying to  increase his exercise and lose some weight.  I also discussed this issue with his family.  Edmund is going to attempt to get more aerobic exercise as well as to discuss his diet with the nutritionist at his primary care center.   

I also spent some time with Edmund about transitioning his medical care  to an internist who has an interest in hypertension.  Edmund has had a thorough workup for hypertension including angiography, all of which has been negative.  As he completes his college studies, I believe it is reasonable for us to consider a transfer to an adult provider who will be able to focus on issues more germane to a patient of Edmund's age.

Thank you for the opportunity to participate in Edmund's care. We would be happy to discuss or review Edmund should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Attending, Pediatric Nephrology


CONTENT:
Montse Simpson, M.D.          RE: Timothy Quincy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/20/85

Dear Dr. Simpson:

We had the pleasure of seeing Timothy in the Endocrine Program on 2/27/92 for  evaluation of premature adrenarche.

History: Timothy is a 6 and 7/12 year old male referred to Endocrine Clinic for evaluation of early onset of pubic hair.  He has had short fine pubic hair, by his mother's report, since the first year of life, but this hair has become longer and coarser for the past year.  He has fine dark hair over his legs and back which has increased only slightly over the past year.  He has no axillary hair or apocrine, no facial hair, and has experienced no change in his voice, although mother notes that Timothy's voice has always been slightly lower than his friends voices.  He has not experienced a growth spurt and is average height in comparison to his friends.  He has no history of acne.  

Timothy has a 5 year old brother who has no signs of early pubic hair.  Mother thinks that Dad was a "early developer" with his pubertal onset between 11 and 12 years of age.

Past medical history:  Review of systems:  General: Timothy experiences no fatigue, no insomnia, no weight loss.  Skin:  He has no history of rashes, bleeding or bruising disorders or acne.  HEENT:  He does not wear corrective lenses and senses of hearing and smell are normal.  Cardiovascular:  He has no history of murmur.  Pulmonary:  No history of asthma or wheezing or pneumonia.  G.I.:  Timothy has had a recent history of staining his underwear intermittently.  G.U.:  He experiences no dysuria, hematuria or nocturia.  Extremities:  He denies joint pain or weakness.

Family history:  Mother's height 5'5", father's height 6'.

Physical exam:  
Height: 120.5 cm        Weight: 19.4 kg         BP: 90/68               
Pulse: 90               R. testis: 2.0 x 1.0 cm L. testis: 2.1 x 1.0 cm 
Phallus length: 8.0 cm  Mid-shaft diam.: 1.8 cm 
General: Timothy is a healthy appearing, active male in no apparent distress.
Skin: No acne, no cafe-au-lait spots.
HEENT: Normocephalic, pupils equal, round and reactive to light.  Extraocular movements intact.  Fundi normal.  Tympanic membranes clear.  Hearing intact.  Pharynx clear.
Neck: No adenopathy.  Thyroid not palpable.
Axillae:  No axillary hair, no apocrine odor.
Chest: Clear to auscultation.
Heart: S1, S2 regular rate and rhythm without murmur.
Abdomen: Nondistended.  Soft and nontender.
Extremities: No clubbing cyanosis or edema.
Genitourinary:  2-3 cm dark hairs distributed over scrotum.

Impression: Impression:  Timothy is a 6 and 7/12 year old with early appearance of pubic hair.  Typically the first sign of puberty in boys is enlargement of the testis; that Timothy's testis measure < 2.5 cm suggests that he has not entered puberty.  Vincet has no testicular masses suggestive of a tumor, and no cafe-au-lait spots suggestive of McCune-Albright syndrome.  The early growth of pubic hair does suggest hyperandrogenism, though probably mild given his lack of axillary or facial hair, acne or growth spurt.  The most likely cause of increased adrenal androgens in Timothy would be congenital adrenal hyperplasia.  The slow course of pubic hair development as described in the history, argued against adrenal tumor, which would be uncommon in this age group to begin with.

Disposition: 1.  We will order an x-ray of the left hand and wrist for a bone age determination.  2.  We will schedule Timothy for an ACTH stimulation test to evaluate the possibility of congenital adrenal hyperplasia.  We have explained the test to his mother and have given her an information pamphlet, and we will call her with an appointment date.

Thank you for letting us share in the care of Timothy.

Sincerely,


Elmo Domingo, MD                 
Attending in Endocrinology


CONTENT:
Avani Neptune, M.D.          RE: Asa Martens
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/04/83

Dear Dr. Neptune:

We had the pleasure of seeing Asa in the Endocrine Program on 2/6/92 for  evaluation of premature adrenarche.

History: Asa is an 8 year and 4 month old girl who was referred for a second opinion of her premature adrenarche.  She was always noted to be taller than her peers and her siblings age.  At about 5 years of age she was noted to have body odor for which she has been using a deodorant.  In November of 1991 she was noted on her physical exam to have pubic hair.  Retrospectively this pubic hair was probably present since the spring of 1991.  She has occasional acne from time.  She denies having armpit hair or, breast development.  She denies any headaches, visual or hearing problems.  She has a good appetite and denies any abdominal pain or bowel changes.  She also denies polyuria and polydipsia.  She has a negative  past medical history.  She is on no medications.

Family history:  Mother is 31 years old and has hypothyroidism.  Maternal grandfather has Type II diabetes.  Mother's height is 5'8", father's height is 5'6".  She has two brothers and one sister who are in the 50th percentile for height and weight.

Physical exam:  
Height: 140.3 cm        Weight: 42.7 kg         BP: 110/60              
Pulse: 88               Breast Tanner: 1        Pubic hair Tanner: 2    
General: Healthy looking, in no distress.  Has normal facies.  Skin:  Normal texture.  No abnormal pigmentation.
HEENT: Normocephalic, atraumatic.  Extraocular movements intact.  Fundi: normal.  Oropharynx: normal.
Neck: Thyroid not enlarged.
Chest: Clear to auscultation and percussion.
Heart: Heart sounds 1 and 2 were normal.  No murmurs heard.
Abdomen: Soft, no masses or tenderness.  No hepatosplenomegaly.
Genitourinary: Tanner II pubic hair with sparse, slightly pigmented, slightly curly pubic hair.  There was no hair growth on the labia.  
Extremities: Normal.
Neurological: Cranial and peripheral nerves exam was intact.

Laboratory studies:  Testosterone was 11 ng/ml (normal 15-110), FSH 0.3, LH 1, Prolactin 6, T4 7.7, TSH 1.9, DHEA 182 (normal 74-180) ng/dl.  X-ray of the left hand showed a bone age between 10 and 11 years old.  Skull x-ray was normal.

In summary: Impression:  Asa is an 8 year and 4 month old girl who most likely has Idiopathic Premature Adrenarche.  It is very unlikely that she has a tumor given her good health the lack of change in her linear growth since infancy and the minimally elevated DHEA level.  The other cause of premature adrenarche is a non-classical form of congenital adrenal hyperplasia which one cannot exclude by baseline studies.  ACTH stimulation test necessary to make such a diagnosis was deferred at this time.  She needs however to be monitored for her linear growth and further development over the next six to twelve months.


Thank you for letting us share in the care of Asa.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Per Adler, M.D.          RE: Floyd Justin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/25/56

Dear Dr. Adler:

     We had the pleasure of seeing Floyd in the Renal Program on 6/22/95 for Hypertension.
     Floyd Justin presents today to the Renal Clinic for follow-up evaluation  of his hypertension and renal insufficiency.  Floyd is a 39 year old man  with cystic fibrosis who is status post bilateral lung transplant in May  of 1994.  He had a single episode of Grade III rejection and has been on  high doses of cyclosporine in the past.  He has been well since we saw  him last September and his cyclosporine has been tapered from 350 mg bid  to 75 mg bid.  In addition, he is on Imuran and prednisone, current dose  7.5 alternating with 5 mg every day.  He has been noted to have erratic  cyclosporine levels, both with his last level 1 week ago at 172 meg/l.  His  creatinine last week was 2.5 mg/dl. and he has noted that his creatinine tends  to vary directly with his cyclosporine level.  In addition, Floyd eats a  high protein diet with both protein supplement milkshakes which he has  discontinued as well as a relatively high intake of meat, both red meat  and chicken.  Otherwise, Floyd has been well.  He reports some mild  trouble sleeping and has a stiff neck when he wakes up, which resolves  quickly with movement.  He reports no headaches, vision changes,  swelling, hematuria or dysuria.

His current medications include:
     CgA 75 mg. b.i.d. 
     Imuran 150 mg. q.d.
     Prednisone 7.5/5 mg. q.d.
     Vasotec 5 mg. b.i.d.
     Tobnomgel 160 mg. b.i.d. inhaler
     Bactrim 1 tsb. 2 x week 
     Zantac 150 mg. q.d.
     CA - 1000 mg. q.d.
     Sporomax 400 mg. b.i.d.
     Vit K 5 mg. 3 x week
     Folic Acid 1 mg. q.d.
     MVI q.d.

Floyd's physical exam was notable for:
     BP 135/90   P 70   R    T    HT cm   WT 76kg
     General: :  Floyd is well appearing.  His fundi do not reveal any  vascular or disc changes.  His oropharynx is clear.   
     Neck:  Does not reveal any  vascular or disc changes.  His oropharynx is clear.  His neck is supple  without lymphadenopathy.  His lung exam is clear to auscultation  bilaterally with good aeration.  He has a well healed transverse  thoracotomy scar. 
     Heart: Regular rate and rhythm without murmur.  
     Abdomen: Soft and nontender without palpable  masses or audible bruits 
     Genitourinary: Remarkable
     Extremities:  Without clubbing, cyanosis or edema. 
     Neurological: Nonfocal.
A urinalysis revealed:
     SPEC GR UA  1.018           RBC/HPF     0-2        
     PH UA       6               WBC/HPF     None       
     BLOOD UA    None            SQUAM EPI   None       
     PROTEIN UA  None  mg/dl     BACTERIA    None  
     GLUCOSE UA  None       
     CASTS       Rare hyaline casts

Laboratory studies at this time include:
          CR          1.8   MG/DL (H)   LYMPH A     .75   K/MM3    
          CYA         100   MCG/L       EOS ABS     .13   K/MM3    
          BUN          59   MG/DL (C)   NEUT-A     76.7       % (H)
          WBC        5.91   K/MM3       LYMPH-A    12.7       % (L)
          RBC        3.94   M/MM3 (L)   MONO-A      5.8       %    
          HGB        11.4    G/DL (L)   EOS-A       2.1       %    
          HCT.       35.2       % (L)   BASO-A       .9       %    
          MCV        89.4      FL       ATYP LY     1.8       %    
          MCH        28.9      PG       HYPO-A        +    
          MCHC       32.4    G/DL       PT         10.9     SEC (L)
          RDW        14.7       %       EST. FI     336   MG/DL    
          HDW        2.60    G/DL       PTT        21.4     SEC    
          PLT UNC     125   K/MM3 (L)   BT          8.5     MIN (H)
          NEUT AB    4.53   K/MM3       

     It seems apparent that Mr. Justin's renal insufficiency is  secondary to his history of multiple nephrotoxins including his  cyclosporine and long history of aminoglycosides in the past.  We are  encouraged that his renal function appears to be improving and this is  likely due to his lower dose of cyclosporine.  Mr. Justin's high protein  diet may be contributing to his renal dysfunction and we have asked the  renal nutritionist to discuss his diet with him, with the hope of  minimizing the possible impact of his diet on his renal function.  It  may be appropriate in the future to perform a radiomiclide functional study of his  kidneys but their apparent improvement does not warrant this  intervention at this time.  His blood pressure appears adequately  controlled on his regimen.  We will plan to see Mr. Justin back in  mid-August prior to his transfer of care to another facility.  In  addition, we will obtain laboratory studies today and repeat in  2 weeks to be sure return to baseline is continuing.

     Thank you for the opportunity to participate in Floyd's care.  We would like to review him in Mid August.  In the interim, we would be happy to discuss or review Floyd should you or the family wish it.

               Sincerely,


               Floyd Khan, MD
               Director, Clinical Services


CONTENT:
Antony McPherson, M.D.          RE: Art Hopper
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/07/60

Dear Dr. McPherson:

We had the pleasure of seeing Art in the Endocrine Program on 12/27/94 for hashimoto's thyroiditis.

History: This is a follow up visit for Melissa who is an 8 and 11/12 year old female who was seen in our Endocrine Clinic three months ago and diagnosed at that time as Hashimoto's thyroiditis and hypothyroidism.  She was started on Synthroid 75 mcg per day.     She is here today for follow up.    

For the past three months, mother noted marked increaes in Melissa's appetite with marked increased in weight where she has put on approximately 3 kg.   She is more active and she denies intolerance to cold, heat or palpitations.

Review of systems:  Skin is less dry.   HEENT:  No headache.  Cardiorespiratory:  No palpitation.  GI:  Normal bowel movement.  GU:  No polyuria or frequency.

Physical exam:  
Height: 124.6 cm        Weight: 36.8 kg         BP: 78/60               
Pulse: 87               Breast: Tanner I        Pubic hair: Tanner I    
General:  Alert.  She does not seem to be in any distress.   
Skin:  Moist.   
HEENT:  Within normal limits.
Neck:  Thyroid is palpable.   
Chest:  Clear.
Heart:  Within normal limits.
Abdomen:   No hepatosplenomegaly.
GU:  Within normal limits.
Extremities:   Within normal limits.

Impression: Melissa seems to be in euthyroid state at the present time.  Her weight gain is due to excess of eating secondary to increased appetite after starting thyroid medication.   It will be advisible to cut down on her foot intake to overcome this problem.   We would like to see Melissa back in six months.  A thyroid function test will be ordered today.

Thank you for letting us share in the care of Art.

Sincerely,


Nanae Welby, MD              Kendell Simpson, MD             
                             Clinical Director            


Problem list to date:
                             

Transcribed by Nortonsh


CONTENT:
Antony Adler, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Adler:

     We had the pleasure of seeing Abraham in the Renal Program on 6/23/94 for hypertension, and William's Syndrome.

     Abraham comes in today with his mother.  Since his last renal evaluation six months ago, he has been doing quite well.  He has been maintained on his 250mg b.i.d. Labetalol and has blood pressure readings in the 130-140/70 at home.  He has had no significant interim illnesses.

Current medications include:
     LABETALOL 250 mg b.i.d.

Physical exam:
     BP 147/76   P 74   WT 78.8kg
     General: Alert, pleasant man with the obvious stigmata of William's Syndrome.
     Skin: Hyperpigmented nipples bilaterally as well as small angiomatous lesions scattered on the abdomen.
     HEENT: Unremarkable
     Neck: Supple and broad.  There was no adenopathy.
     Chest: Configuration and excursion was remarkable for pectus excavatum.  Lungs were clear.
     Heart: Grade 2/6 systolic ejection murmur left upper sternal border.  Pulses were full and symmetrical.
     Abdomen: Soft without organomegaly or mass.
     Extremities: Without edema or rash.

Urinalysis:
     SPEC GR UA  1.010           PH UA       6          
     PROTEIN UA  N               BLOOD UA    N     
     GLUCOSE UA  N               WBC/HPF     N     
     RBC/HPF     N               SQUAM EPI   N     
     CASTS       N

     Abraham continues to be quite clinically stable.  His blood pressure remains under excellent control on his Labetalol therapy.  I did review with Mrs. Henle the fact that Abraham has not had a renal ultrasound in sometime.  I believe that it may be useful to obtain a renal ultrasound with Doppler flow at some point in the future to assess his renal anatomy as well as to get some impression regarding his vascular integrity.  In the interim, I do not believe that we need to pursue any further diagnostic work-up and I would maintain Abraham on his current anti-hypertensive regimen.

     We have asked Abraham to return in six months.  Thank you for allowing us to share in Abraham's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Antonio Khan, MD
               Instructor in Pediatrics


CONTENT:
Antony Adler, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Adler:

     We had the pleasure of seeing Abraham in the Renal Program on 12/15/94 for hypertension, William's Syndrome.
     Abraham comes in today with his mother.  Since his last evaluation six months ago, he has been doing well.  He has been maintained on 250mg twice a day of Labetalol.  The family has not been checking blood pressures at home.  Abraham continues to try to lose weight.  His family states that they believe he has lost approximately 8 lbs since his last visit.  He has had no significant interim illnesses.

Current medications include:
     LABETALOL 250 mg b.i.d.


Physical exam:
     BP 118/74   P 84   R    T    HT cm   WT 75kg
     General: Alert, pleasant man with the obvious stigmata of William's Syndrome.
     Skin: Hyperpigmented nipples bilaterally as well as small angiomatous lesions scattered on the abdomen.
     HEENT: Unremarkable except for a moderate cerumen bilaterally.
     Neck: Supple.
     Lymph Nodes:  Without adenopathy.
     Chest: Clear throughout.  There is a pectus excavatum.
     Heart: Grade 2/6 systolic ejection murmur left upper sternal border.  Pulses were full bilaterally.
     Abdomen: Soft without organomegaly or mass.
     Extremities: Well formed without edema.
     
     Renal Ultrasound:  Reveals the right kidney to be 10cm and the left kidney 11cm.  
     Renal Indicees:  Normal.  There was no hydronephrosis or mass.
Urinalysis:
     SPEC GR UA  1.005           PH UA       7          
     PROTEIN UA  N               BLOOD UA    N     
     GLUCOSE UA  N          WBC/HPF     N     
     RBC/HPF     N          SQUAM EPI   N     


Laboratory studies at this time include:

          CASTS       N

     Abraham continues to maintain adequate blood pressure control on his current twice a day regimen of Labetalol.  In addition, he has manifested a 4-5kg weight loss over the last six months which, most likely will also help with his hypertension.  Abraham's renal ultrasound was unremarkable and revealed no evidence of large renal vessel disease which is sometimes seen with William's Syndrome.  In addition, the kidneys were of normal size and contour.  

At his point, I see no need to alter Abraham's therapeutic regimen.  I advised the family to periodically check Abraham's blood pressure at home.  If they were to obtain diastolic readings which are consistently greater than 90,  then they should contact my office.

     We have asked Abraham to return in .

     Thank you for allowing us to share in Abraham's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Antonio Khan, MD
               Instructor in Pediatrics


CONTENT:
Antony Adler, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Adler:

     We had the pleasure of seeing Abraham in the Renal Program on 6/22/95 for Williams Syndrome.
     Abraham comes in today with his mother.  It has been six  months since his last Renal Program Evaluation.  During that time, he has  been doing well.  His blood pressures at home have been in the 120 to 130/80 range.  He has been taking his labetalol regularly.  There have  been no episodes of low blood pressure recorded by the family.  Abraham  has not had any significant medical illnesses. 



His current medications include:
     Labetalol 250 mg. b.i.d.
     Vitamins

Abraham's physical exam was notable for:
     BP 131/73   P 91   R    T    HT cm   WT 78.8kg
     General:  An active, alert, pleasant man with the obvious  stigmata of William's syndrome.
     Skin: Hyperpigmented nipples bilaterally as well as  small angiomatous lesions scattered on the abdomen.
     HEENT:  Moderate cerumen bilaterally.  
     Neck:  Supple without thyromegaly or adenopathy.  The configuration and excursion of the chest  reveals a moderate pectus excavatum.
     Chest: Clear
     Heart: Remarkable for a grade II/VI systolic ejection murmur at the left  upper sternal border. 
     Abdomen: Soft without organomegaly or  masses. 
     Extremities: Well formed without cyanosis or edema.
A urinalysis revealed:
     PH UA       6               RBC/HPF     0          
     BLOOD UA    Neg.  mg/dl     WBC/HPF     0          
     PROTEIN UA  Trace           SQUAM EPI   Neg.  
     GLUCOSE UA  100   mg/dl     BACTERIA    Neg.  

Laboratory studies at this time include:
          CR          0.9   MG/DL       GLY HGB     6.7       %    
          GLU         129   MG/DL (H)   

     Abraham's blood pressure continues to be well controlled on  his current dose of labetalol.  He did have a modest amount of  glucosuria today.  In the past, Abraham has also had episodes where he  has been spilling glucose.  He had an extensive evaluation approximately  six years ago including an endocrine consult.  At that point, it was  felt that Abraham may have a predisposition to diabetes mellitus, type  II.  Abraham's blood sugar today is modestly increased above normal.  I  did obtain a glycolyted hemoglobin to assess his glucose metabolism over the last several months which returned within normal limits.  In the past, when Abraham has spilled sugar, he  has always responded to closer attention to his diet and his weight.  At  this point, I have reinforced with the family the importance of trying  to maintain his weight at approximately 160 pounds.  I have also advised  them to follow the dietary parameters which have been outlined to them  in the past.

     Thank you for the opportunity to participate in Abraham's care.  We would like to review him in six months.  In the interim, we would be happy to discuss or review Abraham should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Instructor in Pediatrics


CONTENT:
Antony Adler, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Adler:

     We had the pleasure of seeing Abraham in the Renal Program on 1/11/96 for hypertension.
     Abraham comes in today with his mother.  It has been six  months since his last renal program visit.  During that time, Abraham has  generally been doing well.  He has been watching his weight closely and  has had no significant weight gain.  His blood pressures have remained  in the 140/70-80 range.  He is taking his labetalol without difficulty  on a regular basis.  Abraham did have a lipoma removed from his left arm  in November and also an oral lesion removed later that same month.  Otherwise, he has had no significant interim illnesses.

His current medications include:
     LABETALD 250  b.i.d.

Abraham's physical exam was notable for:
     BP 140/84   P 87   R    T    HT cm   WT 78.5kg
     General: alert, active, pleasant man with obvious stigmata of William's syndrome.  HEENT: remarkable for a very high arched palate.  There is moderate cerumen bilaterally.  
     Neck is supple without thyromegaly or adenopathy.  The configuration and excursion of the chest is remarkable for a pectus excavatum.  
     The lungs are clear throughout.   
     Cardiac exam is with grade II/VI systolic ejection murmur at the left upper sternal border.  The pulses are normal bilaterally. 
     The abdomen is soft without organomegaly or mass.  
     Extremities are well formed without edema.  
     Skin: remarkable for hyperpigmented lesions on the chest as well as angiomatous lesions scattered on the abdomen and the back.
A urinalysis revealed:
     SPEC GR UA  1.010           PROTEIN UA  trace      
     PH UA       5               GLUCOSE UA  -          
     WBC ENZ UA  -               BACTERIA    -     
     BLOOD UA    -          

Laboratory studies at this time include:


     Clinically, Abraham continues to do quite well.  His blood pressure is being adequately maintained on his current medication regimen.  At this  point, I believe we should maintain our current regimen.  I did reiterate the importance of him following a prudent diet and maintaining his weight at approximately 160 lb.  I also discussed with his mother the importance of Abraham having a primary care provider.

     Thank you for the opportunity to participate in Abraham's care.  We would like to review him in  six months.  In the interim, we would be happy to discuss or review Abraham should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Attending, Pediatric Nephrology


CONTENT:
Loocie Gorby, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Gorby:

We had the pleasure of seeing Abraham in the Renal Program on 10/31/96 for hypertension.
Abraham comes in today with his parents.  It has been approximately nine months since his last Renal Program visit.   During that time, Abraham has been doing well.  He has been taking his Labetalol without difficulty.  Occasionally, his blood pressure has been checked at home and has been in the 130/70 range. 

Abraham continues to work and was involved at the William's Syndrome Camp this summer.  He has begun seeing a new primary care physician and the family is happy with that arrangement.  Abraham has also made an effort to lose weight since his last visit.  He has been trying to eat  healthier foods and also to watch his activity level.

His current medications include:
LABETALOL 250 mg b.i.d.

Abraham's physical exam was notable for:
     BP 125/67   P 80   R    T    HT cm   WT 69.8kg
     Abraham was an alert and active young man with  obvious stigmata of Williams syndrome.  
     HEENT was remarkable for a very  high arched palate.  The neck was supple, without thyromegaly or adenopathy.  
     His chest examination was remarkable for a pectus excavatum.  His lungs were clear throughout.  
     Cardiac exam was with grade I-II/VI systolic ejection murmur at the left upper sternal border without radiation.  Pulses were normal.  
     The abdomen was soft, without organomegaly or masses.  
     Extremities were well formed, without edema.   
     Skin exam was remarkable for several hyperpigmented lesions on the chest as well as many angiomatous lesions scattered on the back, as well as the abdomen.
A urinalysis revealed:
     SPEC GR UA  1.020           WBC/HPF     -          
     WBC ENZ UA  -               RBC/HPF     -          
     PH UA       6.5             WBC CAST    -          
     PROTEIN UA  -               RBC CAST    -          
     BLOOD UA    -               GRAN CAST   -          
     GLUCOSE UA  -               WAXY CAST   -     
     BACTERIA    -               SQUAM EPI   -     

Abraham's blood pressure is remaining quite stable.   It does appear to be somewhat sensitive to his weight, and as he brings  his weight down to a more ideal range, his blood pressure continues to  improve. 

I spent some time discussing his blood pressure with the family.  I told  them that Abraham is on a dose of Labetalol which I would feel comfortable lowering or discontinuing if his blood pressures remained in the normal range.  During this trial off the medication, his blood  pressure would need to be monitored carefully at home, and if he began showing persistent elevations, then we could reinstitute the Labetalol.   

At this point, the family would like to continue monitoring his blood pressure to see if it remains in the 120/60-70 range before they make a  decision regarding a trial off the therapy.   

Otherwise, I see no reason to embark on any further diagnostic or therapeutic intervention.  I asked Abraham to continue increasing his  activity level as well as to continue monitoring his food intake.  I told him that we would be happy with his weight somewhere between 150  and 160 lbs.

Thank you for the opportunity to participate in Abraham's care.  We would like to review him in nine months. In the interim, we would be happy to discuss or review Abraham should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Attending, Pediatric Nephrology


CONTENT:
Leary, M.D.          RE: Abraham Henle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/15/62

Dear Dr. Guay-Leary:

We had the pleasure of seeing Abraham in the Endocrine Program on 8/5/93 for follow-up of glycosuria.

History: Abraham is a 31 year old male with Williams syndrome, a history of cardiac disease, hypertension and glucose intolerance in the past.  He is followed in the Renal Clinic for hypertension and was noted to have some glucosuria, hence, the referral to the Endocrine Clinic.

Physical exam:  
Weight: 78.9 kg         
Physical examination was deferred.

Laboratory studies:  Random blood sugar 125 mg/dL, HbA1 6.9%.

Impression: Abraham's blood work is reassuring and suggests that his degree of glucose intolerance -- if any is present, is minimal.

Disposition: Follow-up with Dr. Guay-Leary in five months and further follow-up with Dr. Mansfield at the Joslin Diabetes Center as needed.
Thank you for letting us share in the care of Abraham.

Sincerely,


Rahul Ibsen, MD                
Attending in Endocrinology


CONTENT:
Lemar Hagen, M.D.          RE: Jeff Werner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/01/75

Dear Dr. Hagen:

We had the pleasure of seeing Jeff in the Endocrine Program on 3/21/94 for neonatal adrenal leukodystrophy, to rule out adrenal insufficiency.

History: Jeff is an 18 year old who has neonatal adrenal leukodystrophy who was referred to assess his adrenal gland function.   He is very handicapped, blind, deaf and wheelchair bound, apparently eats with  spoon.  He resides at the Perkins school.  There has not been any recent illnesses.   He apparently has decreased motivation for activities but the school thinks it is of a behavioral nature.  He has gained 20 pounds gradually over the last two years and has weight checks every month.  He has not had any abnormal skin pigmentation.   Salt craving cannot be assessed as his development level does not allow it.

Current medications: Tegretol qid, Intal two puffs bid, Ventolin by nebulizer, Tums, one tablet bid, Prevident gel on brush for his teeth.

Past medical history:  Neonatal adrenal leukodystrophy as well as asthma, osteoporosis, blindness and deafness. The review of systems was noncontributory.

Physical exam:  
BP: 90/                 Pulse: 80               
Revealed a wheelchair bound child who grimaces and moves constantly.  He does not respond to commands.  He was extremely uncooperative and belligerent and therefore the physical examination was very limited. Skin and mucous membranes: No hyperpigmentation. Chest: Clear. Cardiac: Normal.

Laboratory studies:  Showed fasting ACTH of 79 in March 1992.  This is elevated (normal 9 to 52).  An ACTH stimulation test in July of 1992 showed a fasting ACTH of 65, a plasma renin activity of 2.4 (normal) and an appropriate Cortisol response which went from 8.7 fasting to 18.2 after stimulation.  On February 14, 1994 he had a repeat fasting ACTH which is at 81, which is the highest it has ever been and a cortisol is 10 which is still within normal range.

Impression: Our impression is that he does have compensated adrenal insufficiency.  We have therefore, arranged for an ACTH stimulation test on April 7, 1994 with DHEAS, prolactin, sodium and potassium.  If the ACTH stimulation test shows poor adrenal reserve, we may have to start Prednisone replacement.

Disposition: We will see him in follow up on May 16, 1994. Thank you for letting us share in the care of Jeff.

Sincerely,


Zinoviy Neptune, MD               Kendell Simpson, MD             
Adolescent Fellow                  Clinical Director            

cc: Talie Sagie, M.D. / Department of Genetics/Metabolism / Children's Hospital


CONTENT:
Lemar Hagen, M.D.          RE: Jeff Werner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/01/75

Dear Dr. Hagen:

We had the pleasure of seeing Jeff in the Endocrine Program on 5/16/94 for follow-up of neonatal adrenal leukodystrophy.

History: Jeff is an 18 year old with neonatal adrenal leukodystrophy who was last seen April 6, 1994.  Since his last visit he has maintained the same activity level, there has been no weight loss and there has not been any hyperpigmentation noted.  Mother today reports for the last two years he has had occasional episodes of weakness  (legs buckling) and episodes of tremors in his arms lasting for 5 seconds and with absent look on his face.  The neurologist however does not think that this represents seizures. Jeff remains at the Perkins School of the Blind.

Current medications: Tegretol qid, Intal two puffs bid, Ventolin by nebulizer, Tums bid.

Physical exam:  
R. testis: 2.5 x 1.2 cm L. testis: 2.5 x 1.2 cm 
Tanner Pubic hair:  III-IV  
 Penis is approximately Tanner IV  
General:  Jeff's interact with his environment are limited.  He is in a wheelchair but is able to stand and walk with support.
Skin: No hyperpigmentation of skin.
Neck: No thyromegaly
GU:  Small testes.

Laboratory studies:  From April 7, 1994:  sodium 141 mEq/L, potassium 3.5 (normal range 3-4.5), plasma renin activity 4.5, normal.  His cortisol went from 14.2 to 20.6 on ACTH stimulation test which is an adequate response.

Impression: Jeff is a patient with neonatal adrenal leukodystophy who currently seems to have adequate adrenal reserve.  On exam he has some hypogonadism. However, the size of his penis and pubic hair suggest reasonable testosterone production at this point.


Laboratory studies pending at this time include: In six months we would like a morning cortisol,sodium, potassium, LH and FSH to be drawn at the Perkins School two weeks prior to his visit here. We will obtain an afternoon ACTH during his visit here.
Disposition: We decided not to start adrenal steroid replacement, however, mother should contact us for any febrile or significant illness and at that point we will consider giving him stress doses of steroids.  We will continue to watch his gonadal function carefully because since he is immobile he is at risk for  osteoporosis.  Should his gonadal function become inadequate we will consider testosterone replacement.  We would like to see him again in six months.
Thank you for letting us share in the care of Jeff.

Sincerely,


Zinoviy Neptune, MD         Lancelot Blue, MD             
                             Attending in Endocrinology


CONTENT:
Lemar Hagen, M.D.          RE: Jeff Werner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/01/75

Dear Dr. Hagen:

We had the pleasure of seeing Jeff in the Endocrine Program on 11/21/94 for adrenal leukodystrophy.

History: Jeff is a 19 year old with neonatal leukodystrophy which does not appear to have adrenal glucocorticoid insufficiency but does have primary gonadal failure.   He has symptoms of tremor and hyperpnea which are not likely to be Endocrine in nature.

Current medications: Ventolin, Tegretol, Tums.

Physical exam:  



Laboratory studies:  9/15/94: 7:00 am LH 13.7 IU/L, FSH 48.7 IU/L, Cortisol 13.7 IU/L.

Impression: Jeff is essentially unchanged from his last visit.   He does not have adrenal deficiency but does have primary gonadal failure.   Of note, since he is on Tegretol he is at risk for hypothyroidism.  Although it may cause some behavioral changes, Jeff's osteoporosis suggests that we may have to seriously consider testosterone supplementation with densitometry measurements to track effect.

Disposition: Please obtain T4 and TSH as well as calcium and magnesium level.
Thank you for letting us share in the care of Jeff.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology


CONTENT:
Milorad Swartzenegger, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. Swartzenegger:

Enclosed are the initial Children's Hospital Discharge Summary of 4/23 to 5/4/76 when the diagnosis of congenital adrenocortical hyperplasia (CAH) was made and Milt begun on glucocorticoid therapy.  Along with this, I have included his adrenal record and growth charts which summarize his subsequent course over the years I have followed him.

I last saw Milt on 1/6/94 at which time he was taking prednisone 5 mg around 8 AM and 2.5 mg around 8 PM with 0.1 mg of Florinef with each prednisone dose.  On this therapy, he showed significant adrenal suppression as indicated by the laboratory studies obtained (see Adrenal Record sheets).

If I can be of further help, please let me know.

Sincerely yours,




Mitsuko F. Simpson, Jr. M.D.
Chief emeritus


CONTENT:
Hugo, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. Hugo:

We had the pleasure of seeing Milt in the Endocrine Program on 7/27/94 for .

History: Thank you for your kind note.  I, also, have enjoyed seeing you over all of these years as you have grown into a fine young man with your family's strong support and guidance.

I sent Dr. Garber copies of the information gathered over the years and know that he will give you good care in the future.  If you remember to take the prednisone and Florinef as recommended, you should do well and have no problems from your adrenal disorder.

Let me know if I can be of any help in the future.  Warm regards to you and your parents and best wishes for a very happy and successful life.

Physical exam:  




Thank you for letting us share in the care of Milt.

Sincerely,


Mitsuko Simpson, MD             
Chief Emeritus


CONTENT:
Mitsuko Simpson, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. :

We had the pleasure of seeing Milt in the Endocrine Program on 11/26/91 for evaluation of .

History: The above named patient is followed in the Endocrine Program at the Children's Hospital for treatment of congenital adrenocortical hyperplasia.  He was last seen on June 17, 1991 at which time he was receiving cortisol 7.5 Mg in the AM, 5 mg at 1 and 8 PM and Florinef 0.1 mg X2/day.  On this therapy, his general health has been excellent and he has no restrictions on physical activity of any kind.

Physical exam:  



In summary: 

Disposition: 

Thank you for letting us share in the care of Milt.

Sincerely,


Mitsuko Simpson, MD             Mitsuko Simpson, MD             
Chief, Emeritus              Chief, Emeritus


CONTENT:
Red, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Ms. Red:

Mrs. Steele has asked me to write in support of their request for health insurance for Milt under the family policy.

I have followed Milt since April 1976 for treatment of congenial adrenocortical hyperplasia.  Over this period of time, he has had a completely uneventful course.  Currently, he takes cortisol 10 mg at 7 am, and 5 mg at 1 pm and 8 pm and Florinef 0.1 mg at 7 am and 8 pm.  he is not in the 10 grade and doing exceedingly well.  I monitor his adrenal substitution therapy three times a year.  He had had no increased incidence of other illnesses and on appropriate therapy should have a normal life span without extra requirements for medical care.

If I can be of further assistance, please let me know.

Sincerely Yours,






Mitsuko F. Simpson, Jr., M.D.
Chief, Emeritus

cc:  Mr. and Mrs. Jeffrey Steele
     5 Timothy Drive
     Andover, MA  01810


CONTENT:
Etta Shields, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. Shields:

I saw Milt and his mother in the Endocrine Program on 04/28/92.  Since my previous note, he has done well on Cortisol 10 mg at 7-8 am, 5 mg at 1-2 pm and 8 pm and Florinef 0.1 mg at 7 am and 8 pm.  he is completing the 10th grade.

On examination he appears to be nearly fully sexually matured with a height of 170.9 cm and weight of 69.3 kg. His pulse was 80 and PB 124/60.  The remains examination including fundi was negative.  Testes are large (4.5 x 2.9 cm each) but not nodular.

In December, plasma and urinary steroid determinations revealed values consistent with appropriate therapy and compliance although the adrenal was not as suppressed as might be desirable.  I have checked these values again today.  If they are similar, it may be advisable to change Milt to prednisone which he could take twice instead of three times daily.  I'll let you know if I recommend this change.  I expect to check him again in October 1992.

Thanks for letting me follow him with you.

Sincerely yours,




Mitsuko F. Simpson, Jr., M.D.
Chief, Emeritus


CONTENT:
Etta Shields, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. Shields:

I saw Milt and his mother in the Endocrine Program on January 4, 1993 for further revaluation of his treatment for congenital adrenocorticol hyperplasia.  He currently is taking cortisol 10 mg. at 7:15 AM, 5 mg. at 1:30 PM and 8:30 PM and Florinef 0.1 mg. at 7:15 AM and 8:30 PM.  On this therapy he seems to be doing quite well.  He is now in the 11th grade at Andover High School.  His review of systems was not remarkable.

Physical exam:  
Height: 171.4 cm        Weight: 68.4 kg         BP: 114.66/80           
Pulse: 80               U/L ratio:  0.92        Arm span: 177.6         
Pubic hair: Tanner I    R. testis: 5.0 x 2.8 cm L. testis: 5.0 x 2.5 cm 
Phallus length: 13.0 cm Mid-shaft diam.: 3 cm   
On examination at 16 and 9/12 years of age, he appeared well and is nearing completion of his linear growth.  His height was 171.4 cm. and weight 68.4 kg.   His skin was clear with a significant beard.  He is fully masculinized with a penis that measures 13x3.2 cm. (stretch length x mid-shaft diameter) and testis of 5.0x2.8 cm.  There were no evidences of any nodules in this testis although they are somewhat large.  Aside from this his general examination including fundoscopic and fields by confrontation were unremarkable.

Laboratory studies:  The following laboratory studies were obtained:  Cortisol 9.8 mcg/dL, 17 hydroxy progesterone > 4500 ng/dL DHEA 162 ng/dL, delta 4 401 ng/dL dehydroepiandrosterone sulfate 177 mcg/dL, ACTH 151 pg/mL, and renin 9.6 ng/mL/hr. A 24 hour urine showed 17-ketosteroids of > 20 mg/total volume with creatinine of 2202 mg/TV suggesting that the sample represented more than a 24 hour period.

Impression: In light of the above clinical and laboratory findings, I felt a change in his therapy was indicated at this time since these findings demonstrate inadequate adrenal suppression. While this may be only to short-term non-compliance, it also may be due to inadequate dose on his current medication and schedule (3x day schedule is a difficult one). For this reason I have recommended to Milt that he take prednisone 5 mg at 7 to 8 am and 2.5 mg at 7 to 8 pm with the same Florinef dose and schedule.  After six weeks on this medication, he will send in another 24 hour urine for 17 Ketosteroids so if necessary, the dose can be further adjusted. I expect to continue to check him at six month intervals.

Thank you for letting us share in the care of Milt.

Sincerely,


Mitsuko Simpson, MD             
Chief, Emeritus


CONTENT:
Etta Shields, M.D.          RE: Milt Steele
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/05/76

Dear Dr. Shields:

We had the pleasure of seeing Milt in the Endocrine Program on 04/13/93 for follow up of  his congenital adrenal hyperplasia.

History: Since he was changed to Prednisone bid, Milt has not noticed any changes except that it is much easier and simpler being on a bid, rather than tid, schedule; he admits his compliance had greatly improved.  Otherwise he has been well, he had no significant illness and the review of systems was negative.  He shaves daily.

Current medications: Prednisone 5 mg in am and 2.5 mg in pm.  Florinef 0.1 mg bid.

Physical exam:  
Height: 171.6 cm        Weight: 67.4 kg         BP: 110/70              
Pulse: 72               Pubic hair: Tanner V    R. testis: 5.0 x 3.0 cm 
L. testis: 5.0 x 3.0 cm Phallus length: 13.0 cm 
Milt was healthy looking, well developed.  Skin: Normal texture.  Slightly oily.  No acne.  HEENT:  Negative.  Neck:  Thyroid not palpable.  Lymph Nodes:  No lymphadenopathy.  Chest:  Clear lungs.  Cor:  Normal S1 and S2, no murmurs.  Abdomen:  Soft, no tenderness, no hepatosplenomegaly.   Extremities:  Normal.  Neurological:  Non-focal.

Laboratory studies:  (04/01/92) 17 ks:  14.2 ng/24 hours, Cr 158 mg/dL, volume 980 mL.

Laboratory studies pending at this time include: ACTH, 17 OH Progesterone, D4, DHEAS, PRA.

Impression: Milt seems to be doing well on his present regimen.  The 24 hour excretion of 17 ks is probably not significantly different from the previous collection when expressed per amount of creatinine excreted in the urine.  Will await the results of blood levels.

Disposition: He is to continue on the same medications pending results of laboratory tests and return to clinic in six months.

Thank you for letting us share in the care of Milt.

Sincerely,




Kendell Simpson, MD             
Clinical Director


CONTENT:
Mandy DeNiro, M.D.          RE: Alicia Craigie
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/77

Dear Dr. DeNiro:

We had the pleasure of seeing Alicia in the Endocrine Program on 8/16/93 for evaluation of hirsutism.

History: Alicia was seen by Adolescent Clinic last June and found to be hirsute.  She, herself, was not worried about the problem, but thinks that it dates back three years.  Hair is noted especially in the side burn area, chest, and on the lower abdomen.  Irregular menses date from menarche, and occur every 1 to 3 months.  She experiences pain in the lower abdomen approximately twice a week, thought unrelated to menstrual periods.  

Alicia has always been of average height.  She describes tiredness, decreased appetite, weight loss, and occasional mild headaches.  She is currently sexually active, and denies taking exogenous anabolic steroids.  A pelvic ultrasound last year at HCHP is said have shown "large ovaries".

Current medications: None.

Past medical history:  Unremarkable.

Family history:  She lives with her mother and two sisters in Dorchester.  The family is on pubic support, and the father is out of the picture.  No endocrine diseases are reported in the family.

Physical exam:  
Height: 163.1 cm        Weight: 57.3 kg         BP: 100/60              
Breast: Tanner IV       Pubic hair: Tanner V    
General: Well developed, healthy, flat affect. Skin: Hirsutism in the
sideburn area, chest, and abdomen.  HEENT: PERRL, EOMI, optic discs sharp. Neck: Thyroid easily palpable, symmetrical, approximately 20 gm. Chest: Clear to auscultation. Heart: Benign. Abdomen: No masses. Genitourinary: Normal clitoris approximately 2 cm in length,  vaginal mucosa well estrogenized. Extremities: Without clubbing, cyanosis or edema. Neurological: Brisk reflexes.

Laboratory studies:  June 1993: Testosterone 39 ng/dL, free testosterone 4.7 pg/mL, LH 6.4 IU/L, FSH 4.9 IU/L, prolactin 10.6 ng/mL, DHEAS 467 ng/dL, T4 7.1 mcg/dL, TSH 0.8 mcU/mL, August 18, 1993: B-HCG negative.

Impression: Alicia is a 16 and 7/12 year old girl with early puberty, irregular menses, hirsutism and weight loss.  Laboratory results are notable for an elevated DHEAS.  While the etiology of the weight loss is unexplained (? depression), the other symptoms may be caused by nonclassical congenital adrenal hyperplasia, 21 hydroxylase or 3-betahydroxysteroid dehydrogenase deficiency, or polycystic ovary syndrome.

As per our telephone conversation, and at your request, we are redirecting Alicia's care back to Adolescent Clinic.  We recommend that you consider an ACTH stimulation test and possibly a repeat pelvic ultrasound.

Disposition: Appointment with Mandy DeNiro August 19, 1993 at 9:45AM.
Thank you for letting us share in the care of Alicia.

Sincerely,


Gleide Crane, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 7/7/94 for lupus nephritis, focal proliferative glomerulonephritis.

     Dawn was seen in follow-up for her lupus focal proliferative glomerulonephritis.  In the interval since her last visit both Dawn and her mother have been following a weight watchers diet and have lost considerable amounts of weight.  In addition, Dawn continues to be very active doing aerobic exercises as well as walking and running.  The family is looking forward to Dawn's sisters wedding next week.  In anticipation of the wedding, Dawn has been getting a considerable amount of sun and has had a red facial rash for at least a few weeks.   Her most recent labs on June 27, include a BUN of 35, creatinine 1.0, 24 hour urine protein of 1800 mg, WBCs 3.4, hematocrit 34.5, ANA 1:640, ESR 85, C3 38,  C4 11 and a non fasting cholesterol level of 549.  Dawn has had no complaints of arthralgia, arthritis, fever or malaise.

Current medications include:
     LASIX 40 mg q.d.
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.


Physical exam:
     BP 114/68   P 68   HT 153.1cm   WT 48.7kg
     General: Well appearing
     Skin: Prominent malar erythematous rash
     HEENT: No evidence of infection, fundi benign
     Chest: Lungs clear with good aeration
     Heart: Regular rhythm without murmur
     Abdomen: Positive bowel sounds, soft, non-tender without palpable organomegaly
     Extremities: Normal form, no edema
     Neurological:Non focal exam

Urinalysis:
     SPEC GR UA  1.006           PH UA       5          
     PROTEIN UA  3+              BLOOD UA    mod        
     GLUCOSE UA  N               WBC/HPF     1-3        
     RBC/HPF     1-3             SQUAM EPI   N     
     CASTS       N

     Dawn continues to have very active lupus serologies.  It is not clear to me whether her malar rash represents worsening disease or the effects of sun exposure.  Although there was no evidence of renal disease or arthritis it may be reasonable to have Dawn evaluated by a Rheumatologist at this point.  I have also noted that Dawn has persistently very high lipid levels.  Although she is restricting her fat intake somewhat with the weight watchers diet, this would not be sufficient reduction for treatment of hypercholesterolemia.  As it is likely that this hypercholesterolemia will persist, treatment with lipid lowering agents should be considered.

     We have asked Dawn to return in three months.  Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 9/15/94 for lupus, focal proliferative glomerulonephritis.

     Dawn was seen in conjunction with Dr. Robert Sundell from Rheumatology following her recent hospitalization.  Dawn had been hospitalized from August 19th through August 24th with systemic symptoms consistent with a flare of her lupus.  (Her discharge summary is enclosed).  Dawn was treated with aJ3 day solumedrol pulse and then started on prednisolone, 25 mg PO BID.  She tolerated the hospitalization well and reported feeling better at the time of her discharge.  In the interval since hospitalization, Dawn says she has been feeling well.  She is quite tiredJgoing to school and takes a nap each day.  She has had no complaints of arthralgias or rash.  Her pedal edema persists and at the end of the day her legs do feel tired from lifting the weight.


Current medications include:
     LASIX 40 mg q.d.
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.
     PREDNISONE 25 mg b.i.d.

Physical exam:
     BP 122/82   P 72   R 18   HT 152.3cm   WT 50.3kg
     General: A pale, tired adolescent in no distress
     Skin: Erythematous malar rash with mild acne
     HEENT: Periorbital edema, no evidence of infection.  Fundi benign
     Neck: Supple
     Chest: Lungs clear with decreased excursion at the bases
     Heart: Regular rhythm without murmur, gallop or rub
     Abdomen: Full, positive bowel sounds, soft and non-tenderJwith a positive fluid wave and no palpable masses or organomegaly
     Extremities: Pitting edema to the thigh
     Neurologic: Non-focal exam


     Dawn has had a minimal response to steroids.  Her albumin on September 6th was 1.7.  Her edema is likely due to a combination of hypoalbuminemia and salt and water retention with the prednisone.  I did not increase her diuretics due to concerns of causing intervascular depletion and an increased risk for thrombosis.  We asked Dawn to have labs drawn at HCHP on the day of this visit.

The main issue now will be what to do if Dawn does not respond well to the prednisone.  Following her renal biopsy in 1991 which showed focal proliferative glomerulonephritis, she was treated with steroids and a course of monthly cytoxan.  Neither treatment had any effect on her C3, C4 levels or her degree of nephrosis.  A repeat renal biopsy in 1993 showed mesangial proliferative glomerulonephritis with minimal immune complex deposition.  This biopsy showed less proliferation than the previous study.  Since immunosuppressive therapy had little response, she had been started on the Enalapril.  We will continue to closely follow Dawn.  If she does have a change in her urinary findings suggestive of a more proliferative lesion, she may merit a further biopsy before considering additional treatment.  In the meantime, we will have to maximize the supportive treatments that she is receiving.  Dawn will return for follow-up on October 12.

     We have asked Dawn to return on October 12, 1994.  Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 10/12/94 for lupus, focal proliferative glomerulonephritis.

     Dawn has been feeling well since her last visit.  She decided to taper herself off the prednisone due to concerns of being dependent on it and desires to try an alternative approach of healthy diet, exercise and positive thinking.  The taper was apparently over four weeks with the last dose being approximately at the time of the October 3 labs.  The October HCHP labs were remarkable for a WBC of 4.4, with 46 polys and 44 lymphs, hematocrit 28.3%, BUN 31, creatinine 1.1, C3 60, C4 17 and ANA of 1:640.  In comparison to the middle of September she has less fatigue, she is running and walking and takes after school naps less frequently.  She continues to take her Enalapril and takes the diuretics prn edema.  She is no longer taking the benzoyl peroxide, which she felt did slightly improve her facial rash.  She had no arthralgias or edema.

Dawn is continuing with plans for attending college next year.  At this time she is applying to the University of Florida in Gainesville as well as Tufts.


Current medications include:
     LASIX 40 mg q.d.
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.

Physical exam:
     BP 107/57   P 78   R 16   HT 152.6cm   WT 45.8kg
     General: Pale but well appearing adolescent in no distress
     Skin: Faint erythematous malar rash with mild acne
     HEENT: No evidence of infection, fundi benign
     Neck: Supple 
     Chest: Lungs clear with good aeration
     Heart: Regular rhythm, without murmur
     Abdomen: Positive bowel sounds, soft and non-tender, without appreciable ascites or organomegaly
     Extremities: No edema

Urinalysis:
     SPEC GR UA  1.023           PH UA       5          
     PROTEIN UA  3+              BLOOD UA    3+         
     GLUCOSE UA  N               WBC/HPF     2-6        
     RBC/HPF     40-60           SQUAM EPI   N     
     CASTS       N


     Overall, Dawn's lupus appears to be less active than in August, however, there is evidence for a more active renal disease than in the spring.  Her most recent creatinine was improved and her lack of edema on intermittent diuretics suggests that her albumin is increased from previous measures.  On the other hand she has evidence of a proliferative lesion on her urinalysis.  The extent of her hematuria is greater than it had been.  The fact that there are no casts is somewhat encouraging.  As we discussed, I am very concerned about the fact that Dawn discontinued the prednisone.  Her significant flair was only six weeks ago and she continues to have evidence of proliferative renal disease.  I have asked her to have her labs repeated including BUN, Creatinine, CBC, complement and a spot urine for protein to creatinine next week at HCHP.  We will have to determine if her disease has remained quiescent off prednisone.  I also suggested that she follow-up with these labs in two months.  I discussed Dawn's current status with Dr. Rob Sundel in Rheumatology.  He shared my concerns about the prednisone and suggested that Plaquenil be considered.  Plaquenil has been shown to prolong the period of remission in some patients.  I am not sure that this would be a viable solution for Dawn but we can certainly suggest it.  

We also discussed Dawn's plans for college.  I would be able to arrange appropriate long term follow-up in Gainesville if she decides to go there.  I told Dawn and her mother that I did not think that this would be a problem and that we could refer her to an internist.

     We have asked Dawn to return in two months.  Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 11/3/94 for lupus, focal proliferative glomerulonephritis.

     Dawn is seen today in follow-up for her lupus nephritis.  Dawn had evidence of a lupus flare on October 24.  At that time her labs were remarkable for a BUN 73, creatinine 2.1, albumin 1.6, hematocrit of 23%, spot urine protein to creatinine ratio of 8.8 and ESR of 140.  She was treated with a 5 day solumedrol pulse and given 1 dose of IV cytoxan.  At the time of admission her weight was 50 kg which increased to 53 kg at discharge.  in addition to her admission medications Nifedipine XL was added to her regimen to better control her blood pressure.  In the time since her discharge, Dawn has been having increasing anorexia and fatigue.  She has also complained of dull, diffuse abdominal pain that is not relieved by Mylanta.  Dawn was seen by Dr. Bonaparte on November 1.  At that time her physical exam was remarkable for a blood pressure  of 120/84, weight 53.6 kg and prominent ascites without pedal edema.  She had no abdominal tenderness.  She was given a trial of 2 days of increased Lasix 80 mg b.i.d. and scheduled for a visit with me today.  In the interval since that visit Dawn feels more fatigued.  She has difficulty sleeping due to her abdominal discomfort.  She is able to sleep lying down or sitting up, although she does have some dyspnea on mild exertion.  She has had no cough or fever.  Her abdominal pain continues to be dull and diffuse.  She has had no other localized symptoms.

Current medications include:
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.
     LASIX 80 mg b.i.d.
     PREDNISONE 30 mg b.i.d.
     MYLANTA 30 cc t.i.d.
     NIFEDIPINE XL 30 mg b.i.d.

Physical exam:
     BP 127/77   P 81   R 20   T 36   WT 53kg
     General: Pale and tired appearing
     Skin: Erythematous malar rash with some fine papules
     HEENT: Fundi benign, no evidence of infection
     Neck: Supple
     Chest: Lungs clear with decreased aeration at both bases bilaterally
     Heart: Regular rhythm, without murmur or rub appreciated
     Abdomen: Full, positive bowel sounds, soft with mild diffuse tenderness.  No appreciable organomegaly and no rebound tenderness
     Extremities: Minimal pedal edema

Urinalysis:
     SPEC GR UA  1.015           PH UA       5          
     PROTEIN UA  3+              BLOOD UA    large      
     GLUCOSE UA  N               WBC/HPF     3-12       
     RBC/HPF     loaded          GRAN CAST   occ   
     CELLULAR CASTS      occ

     Dawn has had no apparent response to the medrol pulse and cytoxan.  It is likely too early appreciate the effects of the cytoxan.  She continues to have active urinary sediment with constitutional symptoms consistent with lupus.  Her current symptoms of anorexia may be due to bowel wall edema or to some degree of uremia.  I would expect her BUN to be elevated due to the prednisone as well as her low dietary calorie intake.  The fact that DawnUs ascites is out of proportion to her pedal edema may reflect her amount of bed rest.  We will also have to rule out an abdominal venous thrombosis.  

Laboratory values from HCHP today are remarkable for BUN of 174, creatinine 3.4, sodium 141, chloride 107, potassium 4.7, TCO2 23, and hematocrit 24.7.  It is clear that many of DawnUs symptoms are related to uremia.  We will therefore plan to admit Dawn for dialysis.  At that time we can also infuse albumin to ultrafilter her.  Additional labs pending at HCHP include an albumin and a urine culture.  I have discussed this plan with Dr. Bonaparte at HCHP

     We have asked Dawn to return in two weeks.  Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 11/23/94 for lupus, focal proliferative glomerulonephritis.

     Dawn had been hospitalized from 11/3-9/94.  At that time she required dialysis for a BUN of 174.  Since the time of her discharge she is feeling better, although she continues to have some fatigue and anorexia.  Her most recent laboratory values at HCHP were on 11/18/94.  At that time her evaluation was remarkable for a BUN 48, creatinine 3.0, WBC 3.7, HCT 21.1, ERS 80, and albumin 2.4.  

Dawn continues to take numerous naps and is receiving home tutoring.  She plans to visit an uncle in Florida next week and hopefully will be ready for a return to school after that period.

Current medications include:
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.
     PREDNISONE 30 mg b.i.d.
     LASIX 40 mg q.d.
     NIFEDIPINE XL 30 mg b.i.d.

Physical exam:
     BP 140/93   P 76    WT 46.3kg
     General: tired appearing.  She had a very minimal malar erythema. 
     HEENT: no evidence of infection.  Fundi benign.  
     Chest: clear with good aeration 
     Heart: regular rhythm with a 1/6 systolic ejection murmur.
     Extremities: normal form, no edema, no arthritis. 
     Neurological: non-focal.

Urinalysis:
     SPEC GR UA  1.014           WBC ENZ UA  N          
     PH UA       5               PROTEIN UA  4+         
     BLOOD UA    mod.            GLUCOSE UA  N     mg/dl
     WBC/HPF     1-3             RBC/HPF     16-20      
     WBC CAST    0               RBC CAST    0          
     GRAN CAST   0               WAXY CAST   0          
     SQUAM EPI   0               BACTERIA    0     
     HYALINE CAST  occ.

     Although Dawn continues to have significant renal insufficiency with a creatinine of 3.0, she has had some improvement in her renal status.  Her albumin has increased and her edema has largely resolved.  Her urinary sediment is less active than the last visit.  She tolerated the Cytoxan pulse with a white count of 2.87 and an absolute nutrofil count of 1900.  At this time our plan will be to continue  with six monthly courses of Cytoxan.  At the end of that period we will reassess her status.  At that time a renal biopsy may be useful to determine the best therapeutic approach to follow.

Dawn's blood pressure control has been adequate.  Following her blood pressure of 140/90 in clinic it lowered to 135/76.  I will plan to continue to follow her and may need to increase her antihypertensive medications if her blood pressures do not improve.  

I also discussed with Dawn and her mother approaches to try to preserve gonadal function.  Apparently during her last course of Cytoxan she received Lupron.  It had been recommended by Dr. Mark Laufer and administered at HCHP.  She is scheduled to have a follow-up visit with Dr. Laufer to discuss repetition of this therapy.  At this point Dawn's last menstrual period was in April 1994.  The visit with Dr. Laufer will enable her to discuss her current prognosis as well as therapeutic actions.

     Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 1/6/95 for lupus, focal proliferative glomerulonephritis.

     Dawn is evaluated prior to IV Cytoxan on January 6, 1995.  She had hospitalized on December 6th for her second cytoxan infusion.  Labs at that time were remarkable for a creatinine of 1.4,  an albumin of 1.7, a C3 of 85, an ANA of 1:80 in speckled pattern, a urine protein/creatinine ratio of 15.8 and a hematocrit of 27.0%.  She tolerated the IV cytoxan without complication.  Follow up laboratory values on December 12th at HCHP included a BUN of 53, creatinine of 1.6, albumin of 2.7, white count of 3.3 and hematocrit 25.1.  Dawn was seen by Dr. Laufer on December 29th for evalation of possible Lupron treatment.  Evaluation included normal FSH and LH levels.  She also had bone density, which showed that her density was decreased to 72% of age matched controls.  This decrease in bone density was thought to be an absolute contraindication to Lupron use.  

Dawn has been feeling better.  She attended full days of school this week without problems.  She continues to take ocassional naps.  Her blood pressures at home have had systolics in the 90's and 100's.  Her appetite is improved and she eats three meals a day with ocassional snacks.

Current medications include:
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     ENALAPRIL MALEATE 10 mg b.i.d.
     PREDNISONE 30 mg b.i.d.
     LASIX 40 mg q.d.
     NIFEDIPINE XL 30 mg b.i.d.

Physical exam:
     BP 100/68   P 88   R 20   WT 42.6kg
     General: She was a pale girl in no acute distress 
     Skin: no rash
     HEENT: no evidence of infection.   
     Neck: supple
     Chest: clear with good aeration 
     Heart: regular rhythm with a 1/6 systolic ejection murmur.
     Abdomen: postitive bowel sounds, soft, non-tender without palpable organomegly
     Extremities: normal form, no edema, no joint swelling. 
     Neurological: non-focal.

Laboratory studies at this time include:
          ALB         2.3    G/DL (L)   ALKP         51     U/L    
          C3           64   MG/DL (L)   CA          8.3   MG/DL    
          C4           26   MG/DL       CAU          <3   MG/DL (*)
          CRU        33.7   MG/DL       CR          1.4   MG/DL (H)
          IGG         186   MG/DL (L)   PHOS        5.8   MG/DL (H)
          BUN          58   MG/DL (C)   NA          138  MMOL/L    
          K           4.0  MMOL/L       CL          108  MMOL/L    
          PLT         252   K/MM3       WBC        4.43   K/MM3 (L)
          RBC        3.24   M/MM3 (L)   HGB         9.1    G/DL (L)
          HCT.       26.4       % (L)   MCV        81.3      FL    
          NEUT AB    2.95   K/MM3    
          LYMPH A    1.03   K/MM3       NEUT-A     66.7       %    
          LYMPH-A    23.2       % (L)   MONO-A      6.2       %    
          EOS-A       1.4       %       BASO-A       .3       %    
          ATYP LY     2.2       %       MICRO-A       +    
          ESR          75   MM/HR (H)     
          SPEC GR   1.007               PH UA       5.0
          PROTEIN      2+ (*)           GLUCOSE     NEG    
          BLOOD U      2+ (*)           RBC/HPF     2-4 (*)
          U PROT       95   MG/DL (H)   ANA         NEGATIVE

     Dawn's C3, ESR, immunoglobulins are essentially unchanged from previous visits, suggesting no change in disease activity.  Her ANA is negative.  Her protein excretion is markedly decreased with a urine protein/creatinine ratio of 2.8 and a albumin of 2.3.  I believe this shows a response to the cytoxan treatment.  Given her good response I think it is reasonable to decrease her prednisone.  We will taper her dose to 50 mg daily.  She will receive her third dose of IV cytoxan today.  Her dose is one gram, which is 750 mg/m2.  She has maintained an adequate white count on this dose.

I am concerned about the fact that Dawn has lost 2.5 kg in the last month.  She was seen by Nancy Spinozzi, the renal dietitian.  Review of her dietary history suggests inadquate intake.  I have recommended that she try to increase her caloric intake and have her weight checked in Dr. Bonaparte's office in two weeks.  If she continues to lose weight we may need to consider caloric supplementation.  The decrease in Dawn's bone density reflects her long term corticosteroid treatment.  Steroids decrease bone calcification through a combination of mechanisms.  These mechanisms include a decreased intestinal calcium absorption, increased bone resorption, decreased bone formation, and increased urinary calcium excretion.  The chlorthiazide that she is taking decreases the calcium excretion.  I think it is reasonable to initiate vitamin D treatment to try to improve Dawn's bone density.  She will start on DHT (dihydrotachysterol) .125 mg biw.  Her urinary calcium excretion is currently negligible.  We will follow her serum calcium level.  As long as she remains in the normal calcium range we will be able to slowly increase the vitamin D.

I have scheduled Dawn for her fourth dose of cytoxan on February 15th.  She will follow up at HCHP in two weeks.

     We have asked Dawn to return on February 15 for Cytoxan treatment.  Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Jerald Bonaparte, M.D.          RE: Dawn Mosher
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/13/77

Dear Dr. Bonaparte:

     We had the pleasure of seeing Dawn in the Renal Program on 2/15/95 for lupus, focal proliferative glomerulonephritis.
     Dawn is in today for evaluation and her fourth monthly dose of cytoxan.  Since her last visit on January 6th, she has generally been feeling well.  She is lifting weights with her father approximately three times a week and has an after school job at Dunkin Donuts.  Her energy level continues to improve.  Her main concern is some hair thinning that she has noted.  She feels that large amounts of hair come out in the shower.

Current medications include:
     HYDROCHLOROTHIAZIDE 250 mg b.i.d.
     LASIX 40 mg q.d.
     
     PREDNISONE 50 mg   q.d.
     ENALOPRIL 5 mg b.i.d.
     DHT .125 mg q.d.

Physical exam:
     BP 100/41   P 85   R 20   T    HT cm   WT 44.6kg
     General: Dawn was well appearing.
     Skin: No evidence of infection or cataracts.   
     Neck: Supple.
     Chest:  Lungs were clear with good aeration.
     Heart: Regular rate without murmur.
     Abdomen:  Positive bowel sounds, soft and non-tender without palpable masses or organomegaly.
     Extremities: No edema or joint swelling.
     Neurological: Non-focal.
Urinalysis:
     PH UA       6               PROTEIN UA  1+         
     BLOOD UA    1+              GLUCOSE UA  N          


Laboratory studies at this time include:
          ALB         2.8    G/DL (L)   ALKP         81     U/L    
          CO 2         25   MEQ/L       C3           84   MG/DL    
          CA          8.6   MG/DL       C4           32   MG/DL    
          CAU          <3   MG/DL (*)   CRU        27.7   MG/DL    
          CR          1.7   MG/DL (H)   IGG         356   MG/DL (L)
          PHOS        5.2   MG/DL (H)   BUN          74   MG/DL (C)
          NA          141  MMOL/L       K           4.4  MMOL/L    
          CL          108  MMOL/L       PLT         408   K/MM3 (H)
          WBC        4.47   K/MM3 (L)   RBC        3.50   M/MM3 (L)
          HGB         9.3    G/DL (L)   HCT.       28.4       % (L)
          MCV        81.0      FL       MCH        26.6      PG        
          HDW        3.43    G/DL (H)   PLT UNC     408   K/MM3 (H)
          NEUT AB    3.32   K/MM3       LYMPH A     .54   K/MM3    
          LYMPH-A    12.0       % (L)   MONO-A      9.4       % (H)
          EOS-A       1.8       %       BASO-A       .4       %    
          ATYP LY     2.1       %       MICRO-A       +    
          HYPO-A        +               ESR          82   MM/HR (H)
          U PROT       36   MG/DL (H)

     Dawn's status continues to improve.  Her weight has increased approximately 2kg in the last month.  As she had previously lost over 2kg, this is encouraging.  Her general status also continues to improve as she has increased activity tolerance.  In general, the laboratory evidence of her disease has improved.  Her hematocrit, white count and platelet counts are higher.  In addition, the degree of proteinuria has dramatically decreased.  Her urine protein to creatinine ratio of 1.3 is below the nephrotic cut off of 3.0.  Her albumin has also increased to 2.8.  I am concerned that her creatinine continues to be considerably above baseline.  As her urinalysis and serum albumin are consistent with a lesser degree disease activity, the increase in creatinine may reflect increased sclerosis.  Since she has evidence for less degree disease activity I would like to continue with her prednisone taper.   I therefore decreased her prednisone to 45mg each day.  If her serologies continue to look improved, it would be reasonable to decrease her dose to 40mg daily in 2 weeks.  After that time, I would like to begin to slowly taper her dose to an alternate day schedule.  This would be accomplished by sequentially decreasing the dose on the alternate day.  At this time my plan is to continue with the 6 monthly doses of cytoxan.  If her renal function continues unchanged, I think that we should consider a repeat biopsy at that time.  This would help to determine if it is reasonable to continue with every three months cytoxan treatment after that.  We also will provide helpful prognostic information for the test.  

Dawn seems to be tolerating the DHT for osteopenia.  There has been no change in her calcium level or the degree of calcium excretion.  It is difficult to find any short term marker of bone disease.  The Moshers were concerned about this, however, I think that the best we can do is follow-up her bone studies in approximately one year.  The degree of Dawn's anemia is improving.  I did note that her MCV is depressed at 81.  This might be consistent with iron deficiency.  Dawn's final concern is her hair thinning.  This is likely due to her disease as well as the cytoxan and high dose steroids.  I suggested that this should resolve with time.  I don't think that there is any specific treatment, however, it is reasonable to try to avoid increased trauma to the hair.  

Dawn is scheduled for her 5th dose of cytoxan on March 15.  Today's dose was received in the outpatient unit without problem.  Dawn will follow-up with Dr. Bonaparte in two weeks.

     We have asked Dawn to return in .

     Thank you for allowing us to share in Dawn's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Tucker Hugo, M.D.          RE: Lacey Sanford
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/76

Dear Dr. Hugo:

We had the pleasure of seeing Lacey, a 15 and 9/12 year old female, in the Endocrine Program on 11/11/91 for evaluation of short stature.

History:  Patient had an uncomplicated prenatal course and no abnormal childhood diseases.  Patient was seen in Orthopedic Clinic at age 22 months with the complaint of chronic familial short stature.  She has been followed since that time by her regular pediatrician.  She denies headache, blurred vision, nausea or vomiting.  She states she has a good appetite.  She has no heat or cold intolerance, no polydypsia, or polyuria.  She had menarche at age 12-13 and now with regular periods every 28-30 days.  Patient has been followed in the past with the work up of short stature showing a normal bone age at 4 and 2/12 years old and normal TFT's.

Family history:  Mother's height is 5'1", father is 5'7".  Brother now at age 22 is 5'8".  Maternal grandmother is 5'1", maternal grandfather is 6'.  Maternal aunts range from 5'1" to 5'6".  Paternal grandparents are reported as normal height.

Physical exam:  
Height: 146.8 cm        Weight: 52.6 kg         BP: 80/50               
Pulse: 68               Breast Tanner: 5        Pubic hair Tanner: 5    
General: Pleasant, well-appearing white female with no apparent distress.  Skin: Axillary hair is shaven with terminal hair follicles visable.  Neck: Thyroid is nonpalpable.  Chest: Clear.  Heart: Regular rate and rhythm.
Abdomen: Soft and nontender.  No masses.  Extremities: Negative.  Neurological: Normal.

In summary: Patient is a post menarchal 15 and 9/12 year old white female with familial short stature.  Familial short stature was discussed with the patient and it was impressed on Lacey and her mother that her final stature will be approximately what it is at this time.  It was stressed to the patient that this a normal short stature and that no further work up is indicated at this time.  Questions from Lacey and her mother were answered. 

Thank you for letting us share in the care of Lacey.

Sincerely,



Mark Laufer, M.D.            Kendell Simpson, MD             
BWH Fellow                   Clinical Director


CONTENT:
Cala Bonaparte, M.D.          RE: Alexandria Baskin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/02/78

Dear Dr. Bonaparte:

We had the pleasure of seeing Alexandria in the Endocrine Program on 7/18/94 for .

History: I saw Alexandria, age 16 years 2 1/2 months, in follow-up for autoimmune thyroiditis.

She feels well and currently has no complaints.  She will be entering her junior year in high school.  She sleeps well without disturbance.  She has no insomnia.  She denies any constipation or change in bowel movements.  She has regular menstruation lasting 5 days on a 33 day cycle.  She reports no heat or cold intolerance. 

She is currently on no medications.

Physical exam:  
Height: 159. cm         Weight: 53.8 kg         BP: 102/66              
Pulse: 78               
General: Well-developed, well-nourished, good color and hydration and a healthy appearing young woman.  
Skin: Smooth and warm.
HEENT: Unremarkable.
Neck: Thyroid gland was ~ 1.5 normal size and firm in consistency; has a prominent isthmus.  Lymph nodes were palpable upper deep cervical lymph nodes bilaterally.
Chest: Clear to percussion and auscultation.
Heart: Regular rate and rhythm, no murmur
Abdomen: No organ enlargement, no masses. 
Genitourinary: 
Extremities: Normal.
Neurological: DTR's 2+ and symmetric in the lower extremities; vibration sense intact.

Laboratory studies:  Serum thyroxine 7.4 mcg/dL, TBGI 1.03, TSH 2.7 mcU/mL, all normal.  Thyroid peroxidase and thyroglobulin antibodies were both positive.  The former was 6.9 and the latter 1.2 u/mL (normal 0-3).

Impression: Alexandria is clinically and biochemically euthyroid.  She does have a modest goiter.  There are no other signs of endocrinopathies.

Disposition: Alexandria should continue to have thyroid function monitored at approximately 12 month intervals, or more frequently if symptoms warrant.

Thank you for the opportunity to share in the care of this lovely young lady.
Thank you for letting us share in the care of Alexandria.

Sincerely,


Bernardo Bird, MD         
Clinical Director
Associate Professor  of Pediatrics, HMS


CONTENT:
Blue, M.D.          RE: Alexandria Baskin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/02/78

Dear Dr. Morin,

     we had the pleasure of seeing Alexandria for evaluation of subacute thyroiditis in the Endocrine Program on 1/31/91.

Blue: 12 8/12 y.o, product of a former 2 lb 10 oz., 3 month premature gestation, pretreated in utero with Dexamethasone, delivered withour complications. She never required ventilatory assistance. 6/78, she had an episode of rectal bleeding though to be due to a Meckel's diverticulum. 12/80 she had an epidermal inclusion cyst removed from her anterior neck. She is being followed by Dr. Michali of orthopedics for bilaterally "high patellae" for which she wears "orthotic" shoes. Her growth has reportedly been normal. She denies heat or cold intolerance, sleeps from 11 p.m. to 6 a.m. and has a calorically adequate and broadly balanced diet. She drinks 2 glasses of milk per day. She denies menarche, acne, or body odor but does report the recent onset of breast development. Prior to this visit, after "flu"-like symptoms, a set of TFT's and thyroid antibodies were obtained primarily because of her sister's history of Hashimoto's disease. The values obtained were: T3 Uptake: 27%, T4 8.5 mcg/dl, TSH 5.0 mcIU/ml, anti-thyroglobulin antibodies 1:10, anti-microsomal antibodies 1:400.


Family history: History of endocrinological disease: sister and Mother have Hashimoto's. A maternal great aunt had a thyroidectomy and a paternal grandmother and paternal aunt had an unspecified form of hypothyroidism.
Mother's hgt: 5'
Father's hgt: 5'8"
Mother's age at menarche: 12 years old

Physical exam:
Weight: 41 kg, height: 146.4 cm, breast Tanner stage: 4, pubic hair Tanner stage: 1
General: Active female in no apparent distress
Skin: two pigmented nevi on abdomen; no axillary hair
HEENT: fundi benign, otherwise unremarkable
Neck:  no thyromegaly, delphian node present
Chest: CTA
Heart:  RRR without murmur
Abdomen:  no hepatosplenomegaly
Genitorurinary:  no pubic hair
Extremeties: no deformities
Neurological: non-focal exam

In summary: Given the positive antibodies, and the delphian node on exam, it appears that Alexandria does, like her sister and other family members, have Hashimoto's disease. However here TFTs are normal and she is asymptomatic. Therefore, treatment at this time is not indicated.

Recommendations: We would like to see Alexandria again in 6 months at which point we will re-assess her thyroid status.

     Thank you for letting us share in the care of Alexandria.

            Sincerely,


                        Blue, Lancelot MD                        Simpson, Kendell MD
                        Fellow in Endocrinology              Clinical Director


CONTENT:
Gleide Swartzenegger, M.D.          RE: Alexandria Baskin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/02/78

Dear Dr. Swartzenegger:

We had the pleasure of seeing Alexandria in the Endocrine Program on 03/05/92 for  follow up of hashimoto's thyroiditis.

History: Since last seen on 08/20/91 Alexandria has been well.  She had no symptoms suggestive of hypo of hyperthyroidism.  Her neck and thyroid gland did not increase in size.  She did have her menarche.  The review of systems was negative.

Thyroid function tests obtained on 11/03/91 revealed a T4 of 6.0 mcg/dl, TBGI 1.05, TT3 140 ng/dl and TSH 2.5 mcU/ml.  It was of interest that the TSH dropped to normal spontaneously.

Physical exam:  
Height: 153.3 cm        Weight: 45.0 kg         BP: 86/60               
Pulse: 92               
She had a normal skin texture.  She had no exophthalmos.  
The left lobe of the thyroid was enlarged and measured 2 x 1 cm, it was thin but firm in consistency.  The isthmus was minimally enlarged and there was have very small Delphian node.  The remainder of the examination was non-revealing.

Laboratory studies:  (drawn 03/05/91)  T4 was 7.6 mcg/dl, TBGI 0.99, TT3 113 ng/dl, TSH 2.80 mcU/ml.

Impression: Alexandria remains euthyroid and the TSH within normal limits.  No therapy is needed at present.


Thank you for letting us share in the care of Alexandria.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Gleide Swartzenegger, M.D.          RE: Alexandria Baskin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/02/78

Dear Dr. Swartzenegger:

We had the pleasure of seeing Alexandria in the Endocrine Program on 9/22/92 for for Hashimoto's thyroiditis.

History: Alexandria claims to be tired but Mrs. Baskin attributes this to her life-style - she stays up very late and wakes up early.  No history of constipation, cold intolerance, or dry hair and skin.  Menses remain regular.  The review of systems was negative.

Current medications: none.

Physical exam:  
Height: 156.2 cm        Weight: 46.7 kg         BP: 104/68              
Breast: Tanner V        Pubic hair: Tanner V    
Skin:  Normal texture.  HEENT: No exophthalmos, no lid lag.   Neck:   Mildly enlarged left lobe.   Chest:  Clear lungs.  Cor:  Normal S1 and S2, no murmurs.  Abdomen:  Soft, no masses.  No hepatosplenomegaly.  Extremities:  Normal.  Neurologic:  Non-focal.

Impression: Alexandria is clinicaly euthyroid.

Disposition: T4 and TSH were obtained.  She is to be re-assessed in six months.

Thank you for letting us share in the care of Alexandria.

Sincerely,





Kendell Simpson, MD             
Clinical Director


CONTENT:
Yvette Seinfeld, M.D.          RE: Alexandria Baskin
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/02/78

Dear Dr. Seinfeld:

We had the pleasure of seeing Alexandria in the Endocrine Program on 5/17/93 for evaluation of hashimoto's thyroiditis.

History: Alexandria is 15 years old and has a family history of Hashimoto's thyroiditis.  She has been euthyroid and has not required any treatment to date.  Her energy level is normal although she is often fidgety and has a rapid rate of speech. She sleeps from 10 or 11 p.m. until 5 a.m. and often takes an afternoon nap from 4 p.m. to 7 p.m. Her weight has been stable. She has no heat or cold intolerance and no constipation. Menses began at age 13 and have been regular every month. She has not yet been to a gynecologist.

Current medications: None.

Physical exam:  
Height: 157.6 cm        Weight: 47.8 kg         BP: 90/56               
Pulse: 78               
General: She was thin and slightly fidgety.  Skin: Mild facial acne and some oiliness to her facial skin.  HEENT: PERRL, EOMI, no lid lag, no edema.  Neck: Supple. There is a well circumcised, slightly enlarged left lobe of the thyroid and a palpable isthmus although the right lobe is barely palpable. The overall texture feels normal. There is a small Delphian node present but no other adenopathy.  Chest: Lungs clear.  Heart: Regular rate and rhythm, normal S1, S2, no murmurs.  Abdomen: Soft, nontender. Normal active bowel sounds. Liver 6 cm from the midclavicular line.   Genitourinary: She refused a breast exam today.  Extremities: No clubbing, cyanosis or edema.  Neurological: 2+ normal relaxation phase of her reflexes.

Laboratory studies:  July 1992:  thyroid function tests normal.  May 1993: T4 6.1 mcg/dL, TBGI 0.93, TSH 2.90 mcU/mL, thyroglboulin antibody positive; thyroid peroxidase antibody postiive.  Hemoglboin 12.3 g/dL, hematocrit 36.5%, MCV 82.5.

Impression: Alexandria is a 15 year old female with Hashimoto's thyroiditis who remains clinically euthyroid. We will repeat her thyroid function tests and antibodies today. The reason for repeating the antibodies is that she has not had them in a while and the assay has recently changed. We recommend that she have an annual follow-up ; but should return sooner if she has any symptoms of hypo- or hyperthyroidism. We will also check a CBC today as she is due for one on her annual exam and she is at a slightly increased risk of pernicious anemia although the onset would be unusual at this early age. She is also at increased risk of other autoimmune diseases as discussed with the family.

Disposition: Return to clinic in twelve months.
Thank you for letting us share in the care of Alexandria.

Sincerely,


Bernardo Bird, MD         
Associate in Endocrinology   


Addendum: Cecilia Larson, MD
Fellow in Endocrinology


CONTENT:
Ana Neptune, M.D.          RE: Floyd Angstrom
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/13/69

Dear Dr. Neptune:

We had the pleasure of seeing Floyd in the Endocrine Program on 08/08/91 for evaluation of Camurati Engelmann syndrome.

History: As you know, Floyd is a 21 year old man with Camurati Engelmann Syndrome, hypothyroidism, obsessive compulsive disorder, and an empty sella turcica, here for follow-up.  Since his last visit in the Endocrine program, Floyd has had no medical problems.  He remains on Prozac and complains of less trouble than previously with his headaches.  He denies constipation or diarrhea, change in the amount of sweating, but does seem somewhat more tired than previously.  Over the summer he has not had much sun exposure, but is drinking one glass of milk daily.  At the time of his last visit, his evaluation was notable for a somewhat low 25 hydroxy-vitamin D and an elevated LH response after GnRH stimulation.

Current medications: Prozac 25mg q AM, Synthroid 125mcg q AM, and tetracycline 500mg q 12 hours.

Physical exam:  
Weight: 69.5 kg         BP: 124/86              Pulse: 88
Pubic hair Tanner: 5    R. testis: 3.8 x 1.8 cm 
L. testis: 3.8 x 1.8 cm 
Floyd remained a mildly dysmorphic man in no distress.  Skin turgor was good and there were no cutaneous lesions.  Thyroid gland was not palpable.  HEENT exam revealed unchanged proptosis and flat optic discs.  Chest was without gynecomastia.  Lungs were clear to auscultation and there were no cardiac murmurs.  Abdomen was soft and without hepatosplenomegaly.  Testes measured 3.8 x 1.8cm bilaterally with phallus measuring 4cm, in mid-shaft diameter, and 12cm in stretch length.  Extremities were notable for unchanged, thick bones.  Neurologic exam revealed 2+ and equal deep tendon reflexes with normal relaxation time.

In summary: Since his last visit, Floyd has exhibited a stable course.  We hope to see Floyd again when he returns for vacation during the winter.

Since he plans on going away to school at the end of August, we scheduled Floyd for repeat GnRH stimulation testing today to determine if indeed he has primary gonadal failure.  Also, with that, we obtained repeat 25 and 1,25 di-hydroxy vitamin D levels, as well as PTH, calcium phosphorous, alkaline phosphatase, and thyroid function tests.  We will make any doses in Floyd's medication based on these results.

Thank you for letting us share in the care of Floyd.

Sincerely,


Maureen Shields, MD                      Samir Najjar, MD
Fellow in Endocrinology               Clinical Director

LM/JW/smp

Addendum:  Floyd's repeat GnRH stimulation test was notable for baseline LH 8.3mIU/ml, FSH 7.8mIU/ml with last LH 60.1mIU/nl, falling to 34.6 by + 120 min.  Thyroid function tests were normal.  Repeat 25 HO Vitamin D level is pending, though repeat CaH was 8.3mg/dl.  Of interest, Floyd had a somewhat low free Testosterone suggesting his high LH may reflect a mild primary hypogonadal state.


CONTENT:
Ana Neptune, M.D.          RE: Floyd Angstrom
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/13/69

Dear Dr. Neptune:

We had the pleasure of seeing Floyd in the Endocrine Program on 2/21/92 for  in follow-up of camurati-engelmann syndrome and hypothyroidism.

History: Floyd is a now 22 and 2/12 year old whose recent medical course has been complicated by the onset of a seizure disorder in January of 1992.  He was noted to have a cry of pain, broke into a cold sweat and had the onset of tonic movement of the extremities with fine tremor which lasted approximately 20 minutes.  He was taken to Beth Israel Hospital in New York where a work-up was unremarkable including a CT scan.  He was started on Dilantin with no subsequent seizure-like episodes.  However, three weeks after its initiation, he was significantly dizzy and was subsequently found to have a rash believed to be an allergic reaction.  He was switched to Valproic Acid with continued good control of seizures.  Floyd's main complaint is increased leg, arm and intermittently head pain which has been unresponsive to usual analgesics.  He has occasional blurry vision which was worse while on Dilantin.

Floyd was seen at a Joint Clinic in New York, where they are considering beginning him on a therapeutic course of steroids to decrease bone growth in the near future.

Current medications: Valproic Acid 500 mg q AM, 250 q afternoon, and 500 mg qhs; Synthroid 0.125 mg q day; and Prozac 20 mg q day.

Physical exam:  
Weight: 140 kg          BP: 138/86              Pulse: 92               
General: 
Skin: Mild acne but no new cutaneous lesions.
HEENT: Unchanged proptosis, flat optic discs and a moist oropharynx.
Neck: Without thyromegaly.
Chest: Clear to auscultation and no gynecomastia.
Heart: Unremarkable.
Abdomen: Mildly obese but without hepatosplenomegaly or masses.
Genitourinary: Mid-shaft diameter 3.6 cm, testis 2.2 x 4.0 bilaterally, Tanner V pubic hair.
Extremities: Unchanged thickening of the bones. 
Neurological: Nonfocal.

In summary: Our impression is that currently Floyd is clinically euthyroid and based on the size of his testes and virilization has an intact pituitary-gonadal axis despite abnormal provocative testing previously.  Since Floyd had a relatively low vitamin D and calcium on previous checks, we repeated calcium  phosphorous, alkaline phosphatase, vitamin D, and PTH levels as well as thyroid function tests, LH, FSH, and testosterone to continue his ongoing monitoring.  Should any of these require further intervention we will contact the mother.


Thank you for letting us share in the care of Floyd.

Sincerely,


Maureen Shields, MD             Bernardo Bird, MD         
Fellow in Endocrinology      Associate in Endocrinology


CONTENT:
Jonnie Daley, M.D.          RE: Floyd Angstrom
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/13/69

Dear Dr. Daley:

We had the pleasure of seeing Floyd in the Endocrine Program on 8/18/93 for follow-up of camurati-engelmann syndrome and hypothyroidism, and elevated gonadotropins on provocative testing.

History: Since his last visit, approximately 18 months ago, Floyd has been doing relatively well overall. He received a short course of steroids but reported little improvement in his joint pains during this trial. Since this time, his energy level has occasionally been low and he reports having to sleep at an increased frequency compared to previously. He is able to have erections normally and denies heat or cold intolerance, headaches, change in vision, constipation or diarrhea.  Floyd's medications are Synthroid .125 mg q day, Prozac 40 mg q day, Valproate 250 mg q day, and multivitamin.

Physical exam:  
Height: 169 cm          Weight: 79.3 kg         BP: 140/84              
Pulse: 88               Pubic hair: Tanner V    R. testis: 4 x 2 cm     
L. testis: 4 x 2 cm     
General: Notable for significant frontal bossing, mild acne.  Skin: Texture normal, turgor good.  HEENT: Flat optic discs, moist oropharynx. Intact extraocular movements with mild proptosis.  Neck: Without thyromegaly.  Chest: Clear to auscultation.  Heart: Regular rate and rhythm without murmur.  Abdomen: Soft without hepatosplenomegaly or masses.  Genitourinary: Normal texture of the testes.  Extremities: Notable for unchanged, tubular, thickened long bones,  nontender to palpation. Neurological: Nonfocal.

Impression: Our impression is overall, Floyd continues to do relatively well. His only notable symptomatology is his report of increased fatigue which could be due to inadequate thyroid replacement though he has been on a stable dose for several years or possibly worsening testicular functioning. To evaluate this we obtained gonadotropins, testosterone, and thyroid function tests and also to reassess Floyd's  calcium/phosphorous status, we obtained PTH, calcium phosphorous, albumin and vitamin D level. We will communicate these results to Floyd when they are available and recommend any additional therapy should they be needed.  Because of Floyd's living in New York, primarily at this stage, it probably would be most appropriate for him to find an endocrinologist locally. When this occurs, we would be glad to forward any information that would be helpful.

Thank you for letting us share in the care of Floyd.

Sincerely,


Maureen Shields, MD             
Attending in Endocrinology   


Addendum: Floyd's thyroid function tests were normal.  LH and FSH were 8.3 U/L and 6.8 U/L respectively, near the upper end of the normal range.  Testosterone is pending.  PTH level was normal, though calcium level was slightly low at 8.5 mg/dL.  Vitamin D metabolites are pending.  We have made no changes in Floyd's managment, but will await testosterone and vitamin D levels.


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

We had the pleasure of seeing Ingrid in the Renal Transplant Program on May 3, 1994.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on June 28, 1984.

Ingrid has had no complaints since her last visit to our program.  Her blood pressure has been normal at home.

Current medications include: IMURAN 150 mg q.d., PREDNISONE 15 mg q.o.d., LASIX 40 mg q.d., ROCALTROL 0.25 mcg b.i.d., PROCARDIA XL 30 mg b.i.d., BACTRIM SS 1 tablet q.h.s., ESTINYL 10 mg day 1-25 and PROVERA 10 mg day 14-25.

Physical exam:
     BP 137/95     P 86     R 18      WT 73.9kg
     HEENT:  oropharynx is clear moist
     Neck: supple without any masses
     Chest: clear to auscultation
     Heart: regular rate and rhythm
     Abdomen: soft, there is no tenderness or bruit over the graft site
     Extremities: no peripheral edema

Urinalysis:
          SPEC GR UA  1.015                PH UA       7          
          PROTEIN UA  3+    mg/dl          GLUCOSE UA  N     mg/dl
          BLOOD UA    trace                WBC ENZ UA  0          
          RBC/HPF     0     /HPF           WBC/HPF     0     /HPF 
          WBC CAST    0     /LPF           RBC CAST    0     /LPF 
          GRAN CAST   0     /LPF           WAXY CAST   0     /LPF 
          Leucocyte Esterase N

Laboratory studies at this time include:
          BUN 29      Cr 1.9     K 4.3       Ca 8.0      PO4 3.5     PRO/Cr= 5
          Cl 105      Na 140     GLUC 91     ALB 2.6     UCR 98.4    UPRO 502
          WBC 7.85    HGB 10.6   HCT 31.5    MCV 86.2    PLT 267
          N 69.4      L 19.3     B 1.2       ABS N 5.45
        

Ingrid is currently doing well and has no complaints.  Her blood pressure is slightly elevated here but has been normal at home.  We have asked Ingrid to monitor her blood pressure at home and if it is elevated we will increase her Procardia.  The dietician will discuss diet, dairy product intake since the calcium and phosphorous are low.  She may need supplementation with Tums if her diet is insufficient.

She will return in two months.

Thank you for allowing us to assist in the care of Ingrid.

Sincerely,


Kinze Brando, MD               
Assistant in Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, July 26, 1994.  She has chronic renal failure secondary to .  She had a renal transplant on .
     Ingrid has had no problems since her last visit.  She states that her  blood pressures have been normal at home.  Of note, Ingrid has had a 24 blood pressure monitoring in the past which was normal.  Her blood pressures have always been elevated while in the clinic.

Current medications include:
     IMURAN 150 mg   q.d.
     PREDNISONE 15 mg   q.o.d.
     LASIX 40 mg   q.d.
     ROCALTROL .50 mcg   q.d.
     PROCARDIA XL 60 mg am, 30 mg pm
     BACTRIM SS qhs
     ESTENOL .02 mg days 1-25
     PROVERA 10 mg days 14-25

Physical exam:
     BP 135/95   P 75   R 20   T    HT cm   WT 72.3kg   HC cm
     General: healthy
     HEENT: moist mucous membranes without exudates.  No signs of cataracts
     Chest: clear 
     Heart: regular rate and rhythm without murmurs, gallops, or rubs
     Abdomen: soft non-tender,  non-distended without bruits over the abdominal site.
     Extremities: warm and well profused.  An ultrasound that was done today, it  revealed normal flow and normal sized transplanted kidney.
     Neurological: non-focal
Urinalysis:
     SPEC GR UA  1.007           WBC ENZ UA  N          
     PH UA       6               PROTEIN UA  2+         
     BLOOD UA    1+              GLUCOSE UA  N          
     WBC/HPF     0               RBC/HPF     0          
     WBC CAST    0               RBC CAST    0          
     GRAN CAST   0               WAXY CAST   0          
     SQUAM EPI   few             BACTERIA    N     


Laboratory studies at this time include:
          BUN 32    Cr 2.0    K 4.1    Ca 8.5    PO4 4.0
          Na 140    Cl 106    GLUC 89    CO2 26
          WBC 7.25    HGB 9.2    HCT 27.7    MCV 85.1    PLT 248
          N 53    L 40    ABS N 3.84

     Ingrid is currently doing well status post transplant for glomerulosclerosis.  Her renal function is stable.

     We have asked Ingrid to return in .

     Thank you for allowing us to share in Ingrid's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Conrado Seinfeld, MD         Clotilde Goldwater, MD


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, September 20, 1994.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on June 28, 1984.
     Ingrid has been well since her last visit.  She has no new problems.

Current medications include:
     IMURAN 150 mg q.d.
     PREDNISONE 15 mg q.o.d.
     ROCALTROL .25 mcg b.i.d.
     LASIX 40 mg q.d.
     PROCARDIA XL 60 mg am, 30 mg pm
     BACTRIM SS q.h.s.
     ESTINAL .02 mg day 1-25
     PROVERA 10 mg day 14-25

Physical exam:
     BP 150/95   P 80   R 18     WT 70.6kg   
     General: appears well 
     HEENT: normal 
     Neck: supple with no masses, lymph nodes not palpable
     Chest: clear
     Heart: two heart sounds with no murmurs
     Abdomen: soft, multiple striae and surgical scars from transplant and a nephrectomy
     Genitourinary: Tanner 5
     Extremities: no edema

Urinalysis:
     SPEC GR UA  0               WBC ENZ UA  0          
     PH UA       6               PROTEIN UA  2+         
     BLOOD UA    N               GLUCOSE UA  N     mg/dl
     WBC/HPF     0               RBC/HPF     1-2        
     WBC CAST    1               RBC CAST    1          
     GRAN CAST   1               WAXY CAST   1          
     SQUAM EPI   1               BACTERIA    trace 


Laboratory studies at this time include:
          CO 2         23   MEQ/L       CA          8.2   MG/DL (L)
          CR          1.7   MG/DL (H)   GLU          89   MG/DL    
          PHOS        3.4   MG/DL       BUN          26   MG/DL (H)
          NA          136  MMOL/L       K           4.4  MMOL/L    
          CL          100  MMOL/L       WBC        9.15   K/MM3    
          RBC        3.05   M/MM3 (L)   HGB         9.5    G/DL (L)
          HCT.       27.5       % (L)   MCV        90.4      FL    
          MCH        31.3      PG       MCHC       34.6    G/DL    
          RDW        15.6       % (H)   HDW        2.43    G/DL    
          PLT UNC     224   K/MM3       NEUT AB    6.08   K/MM3    
          LYMPH A    2.23   K/MM3       EOS ABS     .17   K/MM3    
          NEUT-A     66.4       %       LYMPH-A    24.3       % (L)
          MONO-A      4.5       %       EOS-A       1.9       %    
          BASO-A      1.2       %       ATYP LY     1.6       %    


     Ingrid is well since last visit.  She has no new problems.  Her blood pressure continues to be elevated in clinic, but previous monitoring has demonstrated more normal levels at home.  Her calcium is slightly low but I suspect that she has not taken her Rocaltrol consistantly.

     We have asked Ingrid to return in three months.

     Thank you for allowing us to share in Ingrid's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Clotilde Goldwater, MD
               Director, Pediatric Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, January 17, 1995.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on June 28, 1984.
     Ingrid is doing well.  She has no new complaints.

Current medications include:
     IMURAN 150 mg q.d.
     PREDNISONE 15 mg q.o.d.
     ROCALTROL .50 mcg q.d.
     LASIX 40 mg q.d.
     PROCARDIA XL 60 mg, 30 mg
     BACTRIM (TRIMETHOPRIN 8 mg, SULFAMETHOXAZOLE 40 mg) q.h.s.
     ESTINOL 2 (1-25 days)
     PROVERA 10 (14-25 days)

Physical exam:
     BP 140/95   P 75    R 18     WT 71.6kg   
     General: appears well.
     SKIN: striae from past steroids.
     HEENT: normal. 
     Neck: supple with no masses, lymph nodes not palpable.
     Chest: clear.
     Heart: two heart sounds with no murmurs.
     Abdomen: soft, non-tender.
     Genitourinary: Tanner 5.
     Extremities: no edema.
     Neurological: normal

Urinalysis:
     SPEC GR UA  1.010           WBC ENZ UA  0          
     PH UA       5               PROTEIN UA  3+         
     BLOOD UA    1+              GLUCOSE UA  0     mg/dl
     WBC/HPF     5               RBC/HPF     5          
     WBC CAST    0               RBC CAST    0          
     GRAN CAST   0               WAXY CAST   0          
     SQUAM EPI   0               BACTERIA    0     


Laboratory studies at this time include:
          CO 2         26   MEQ/L       CA          8.3   MG/DL (L)
          CR          1.7   MG/DL (H)   GLU          89   MG/DL    
          FE           62  MCG/DL (L)   PHOS        3.3   MG/DL    
          BUN          33   MG/DL (H)   NA          140  MMOL/L    
          K           4.1  MMOL/L       CL          105  MMOL/L    
          TIBC        308  MCG/DL       WBC        8.02   K/MM3    
          RBC        2.77   M/MM3 (L)   HGB         8.3    G/DL (L)
          HCT.       25.4       % (L)   MCV        91.8      FL    
          MCH        29.9      PG       MCHC       32.6    G/DL    
          RDW        16.6       % (H)   HDW        2.69    G/DL    
          PLT UNC     250   K/MM3       NEUT AB    5.85   K/MM3    
          LYMPH A    1.55   K/MM3       NEUT-A     72.9       % (H)
          LYMPH-A    19.3       % (L)   MONO-A      3.6       %    
          EOS-A       1.5       %       BASO-A       .8       %    
          ATYP LY     1.8       %      
        
     Ingrid is doing well.  Her renal function is stable.  We will start an iron supplement now to try to increase her hematocrit.

     We have asked Ingrid to return in three months.

     Thank you for allowing us to share in Ingrid's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, April 11, 1995.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on June 28, 1984.
     Ingrid is now a 24 year old lady who had a renal transplant 11 years ago from her mother for glomerulosclerosis.  Ingrid was seen today in the clinic and in general she is doing well.

Current medications include:
     IMURAN 150 mg q.d.
     PREDNISONE 15 mg q.o.d.
     ROCALTROL .50 mcg q.d.
     LASIX 40 mg q.d.
     PROCARDIA XL 60 mg b.i.d.
     FERGON 325 mg b.i.d.
     BACTRIM (TRIMETHOPRIN 8 mg, SULFAMETHOXAZOLE 40 mg) q.h.s.
     ESTINOL 2 mg (1-25 days)
     PROVERA 10 mg (14-25 days)

Physical exam:
     BP 160/110   P 70   R 18     WT 70.8kg   
     General: good looking lady who is not in any distress although she is relating that most of the time she is very stressed and even the visit here makes her worry.  She also relays that her blood pressure is high only because she is very stressed but at home she usually runs 130-80.
     SKIN: clean, no rash
     HEENT: normal. 
     Neck: supple, lymph nodes not palpable.
     Chest: clear.
     Heart: no murmurs.
     Abdomen: soft, right lower quadrant transplanted kidney.
     Extremities: no edema.
     Neurological: intact
Urinalysis:
     SPEC GR UA  1.010           WBC ENZ UA  0          
     PH UA       5               PROTEIN UA  3+         
     BLOOD UA    0               GLUCOSE UA  0     mg/dl
     WBC/HPF     0               RBC/HPF     0          
     WBC CAST    0               RBC CAST    0          
     GRAN CAST   0               WAXY CAST   0          
     SQUAM EPI   1-2             BACTERIA    0     


Laboratory studies at this time include:
          CO 2         24   MEQ/L       CA          8.4   MG/DL    
          CRU        54.3   MG/DL       CR          2.2   MG/DL (H)
          GLU         106   MG/DL       PHOS        3.5   MG/DL    
          BUN          40   MG/DL (H)   NA          140  MMOL/L    
          K           4.5  MMOL/L       CL          107  MMOL/L    
          WBC       12.24   K/MM3 (H)   RBC        3.18   M/MM3 (L)
          HGB         9.3    G/DL (L)   HCT.       28.3       % (L)
          MCV        89.1      FL       MCH        29.2      PG    
          MCHC       32.8    G/DL       RDW        14.9       %    
          HDW        2.78    G/DL       PLT UNC     265   K/MM3    
          NEUT         95       % (H)   LYMPH         1       % (L)
          BASO          2       %       BAND          2       %    
          CELLS C     100               ABS NEU   11.87   K/MM3    
          ABS LYM     .12   K/MM3       ABS EOS     .00   K/MM3    
          MORPH    NORMAL               U PROT      256   MG/DL (H)


     Sand is hypertensive and was evaluated two years ago with a holter monitor which showed normal blood pressures at home.  We will perform a 24 hour blood pressure monitoring and plan treatment.  The protein/creatinine ratio is significantly elevated consistent with chronic transplant glomerulopathy.  At the present time we will not adjust her hypertensive medications and follow closely.

     We have asked Ingrid to return in two months.

     Thank you for allowing us to share in Ingrid's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, June 27, 1995.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.
     Ingrid has done well since her last visit with us.  She had a 24 hour blood pressure monitoring which demonstrated the majority of her diastolic blood pressures to be below 90.  She has some mildly elevated diastolics during her time of work, these are never over 94.

Her current medications include:
     IMURAN 150 mg q.d.
     PREDNISONE 15 mg q.o.d.
     ROCALTROL .50 mcg q.d.
     LASIX 40 mg q.d.
     BACTRIM (TRIMETHOPRIN 8 mg, SULFAMETHOXAZOLE 40 mg) q.h.s.
     ESTINOL 2 mg (1-25 days)
     PROVERA 10 mg (14-25 days)
     PROCARDIA XL 90 mg   b.i.d.

Ingrid's physical exam was notable for:
     BP 150/95   P 78   R 20      WT 69.3kg   
     General:  No acute distress.
     HEENT: Moist mucous membranes without masses or exudates.  
     Chest: Clear.
     Heart: Regular rate and rhythm without murmurs, gallops or rubs.
     Abdomen: Soft non-tender and non-distended without tenderness or bruits over the graft site. 
     Extremities: Warm and well perfused without edema.  
     Neurological:  Non-focal.
A urinalysis revealed:
     SPEC GR UA  1.008           RBC/HPF     N          
     WBC ENZ UA  0               WBC CAST    N          
     PH UA       6               RBC CAST    N          
     PROTEIN UA  3+              GRAN CAST   N          
     BLOOD UA    +               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          CO 2         22   MEQ/L       CA          8.2   MG/DL (L)
          CR          2.2   MG/DL (H)   GLU         101   MG/DL    
          PHOS        4.0   MG/DL       BUN          25   MG/DL (H)
          NA          137  MMOL/L       K           4.7  MMOL/L (H)
          CL          106  MMOL/L       WBC        8.51   K/MM3    
          RBC        2.73   M/MM3 (L)   HGB         8.3    G/DL (L)
          HCT.       25.2       % (L)   MCV        92.4      FL    
          MCH        30.4      PG       MCHC       32.9    G/DL    
          RDW        17.1       % (H)   HDW        2.98    G/DL    
          PLT UNC     263   K/MM3       NEUT AB    7.38   K/MM3    
          LYMPH A     .71   K/MM3       EOS ABS     .05   K/MM3    
          NEUT-A     86.7       % (H)   LYMPH-A     8.3       % (L)
          MONO-A      2.8       %       EOS-A        .6       %    
          BASO-A       .6       %       ATYP LY     1.0       %    
          ANISO-A       +               MACRO-A       +    


     Ingrid has white coat hypertension.  This was documented two years ago and has been reconfirmed with this latest blood pressure monitoring.  As such we will not alter her antihypertensive regimen.

     Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in three months.  In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Gleide Blue, MD
               Attending, Pediatric Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, October 17, 1995.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

     Ingrid appears comfortable in no acute distress.

Her current medications include:
     IMURAN 150 mg q.d.
     PREDNISONE 15 mg q.o.d.
     ROCALTROL .50 mcg q.d.
     LASIX 40 mg q.d.
     BACTRIM (TRIMETHOPRIN 8 mg, SULFAMETHOXAZOLE 400 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 5 mg (14-25 days)
     PROCARDIA XL 90 mg   b.i.d.

Ingrid's physical exam was notable for:
     BP 140/90   P 80   R 20     WT 69.5kg   
     General:  No acute distress.
     Skin:  no rash
     HEENT: clear sclera.  Oropharynx clear and moist.  
     Neck: supple with no palpable lymph nodes.
     Chest: Clear to auscultation.  There is no wheezing or crackles heard.
     Heart: S1, S2, regular rhythm without murmur.
     Abdomen: Soft non-tender and no organomegaly.
     Extremities: no edema.  
     Neurological:  grossly intact.

A urinalysis revealed:
     SPEC GR UA  1.010           RBC/HPF     N          
     WBC ENZ UA  0               WBC CAST    N          
     PH UA       7               RBC CAST    N          
     PROTEIN UA  2+              GRAN CAST   N          
     BLOOD UA    +               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          CRU        47.2   MG/DL       HGB         7.8    G/DL (C)
          K           4.6  MMOL/L (H)   HCT.       23.7       % (L)
          CA          7.6   MG/DL (L)   MCV        91.7      FL    
          CO 2         21   MEQ/L (L)   MCH        30.2      PG    
          CR          2.2   MG/DL (H)   MCHC       33.0    G/DL    
          PHOS        3.6   MG/DL       RDW        17.0       % (H)
          NA          139  MMOL/L       HDW        3.22    G/DL (H)
          CL          107  MMOL/L       PLT UNC     251   K/MM3    
          WBC        6.56   K/MM3       U PROT      164   MG/DL (H)
          RBC        2.58   M/MM3 (L)   

     Ingrid is doing well clinically with no complaints.  She has high protein output and potential for decrease in serum albumin.

     Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in three months with serum total/serum albumin in addition to routine medications.  In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, May 21, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.
     Ingrid has been stable.  Her last visit with us was in October 1995.  She has documented white coat hypertension.  It appears that she has not been taking the prescribed dose of Nifedipine.  We will adjust her meds according to her response.  Ingrid has inadvertently stopped taking Rocaltrol.  Her calcium in October was 7.6 mg/dl.  She has also been having leg cramps which may be attributed to hypocalcemia.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     IMURAN 100 mg q.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 5 mg (14-25 days)
     PROCARDIA XL 90 mg qam, 30 mg qpm
     MVI 1 q.d.

Ingrid's physical exam was notable for:
     BP 160/105    R 12   WT 69kg 
     General: comfortable in no distress.
     Skin: without lesions.
     HEENT: within normal limits. 
     Neck: supple without lymphadenopathy.
     Chest: clear.
     Heart: without murmur. 
     Abdomen: soft without tenderness over graft site.
     Extremities: no edema.

A urinalysis revealed:
     SPEC GR UA  1.008           RBC/HPF     N          
     WBC ENZ UA  0               WBC CAST    N          
     PH UA       5               RBC CAST    N          
     PROTEIN UA  2+              GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   many       
     WBC/HPF     0-1             BACTERIA    N     

Laboratory studies at this time include:
          CRU        38.9   MG/DL       MCHC       32.7    G/DL    
          K           5.8  MMOL/L (H)   RDW        16.9       % (H)
          CA          7.4   MG/DL (C)   HDW        3.19    G/DL    
          CR          3.0   MG/DL (H)   MPV         8.3      FL    
          PHOS        4.6   MG/DL (H)   PLT UNC     195   K/MM3    
          BUN          41   MG/DL (H)   NEUT         90       % (H)
          NA          136  MMOL/L       LYMPH         6       % (L)
          CL          106  MMOL/L       BAND          4       %    
          CO 2         18   MEQ/L (L)   CELLS C     100    
          WBC        5.74   K/MM3       ABS NEU    5.40   K/MM3    
          RBC        2.35   M/MM3 (L)   ABS LYM     .34   K/MM3    
          HGB         6.3    G/DL (C)   ABS EOS     .00   K/MM3    
          HCT.       19.2       % (C)   MICRO        1+    
          MCV        81.9      FL       WBC COR    5.74   K/MM3    
          MCH        26.8      PG       U PROT       61   MG/DL (H)

     Ingrid's creatinine is elevated to 3.0 mg/dl and has protein excretion by semiquantitative analysis to approximately 2 g.  We will repeat her creatinine, quantify her protein excretion and investigate the etiology of her anemia, these may be secondary to chronic transplant neuropathy.  Her blood pressure remains elevated and we will increase her procardia to 60 mg b.i.d.  She will be calling us next week with blood pressure readings once she has adjusted the dose.  Because she is symptomatic and her calciums have been low, we are going to start Rocaltrol .25 mcg q.d.  We will be repeating her calcium in two weeks.

     Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in  three months.  In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

                Sincerely,


                Mitsuko Khan, MBBS, FRACP
                Director, Clinical Services


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, June 11, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

     Ingrid has been doing reasonably well until recently.  She has had multiple problems within the last week.  She has had no complaints of shortness of breath and dyspnea on exertion.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     IMURAN 100 mg q.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 5 mg (14-25 days)
     PROCARDIA XL 90 mg b.i.d.
     MVI 1 q.d.

Ingrid's physical exam was notable for:
     BP 160/90   P 89    WT 70.9kg   
     General: yellow tint, pale. 
     HEENT: within normal limits. 
     Neck: supple no adenopathy.
     Chest: clear to auscultation
     Heart: regular rate and rhythm
     Abdomen: soft, non-tender.
     Extremities: no edema.

A urinalysis revealed:
     SPEC GR UA  N               RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       N               RBC CAST    N          
     PROTEIN UA  1+              GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          AST          40     U/L       WBC        4.96   K/MM3    
          K           5.1  MMOL/L (H)   RBC        2.22   M/MM3 (L)
          CA          7.0   MG/DL (C)   HGB         5.8    G/DL (C)
          CR          2.8   MG/DL (H)   HCT.       18.5       % (C)
          GLU         115   MG/DL       MCV        83.2      FL    
          FE           60  MCG/DL (L)   MCH        26.1      PG    
          PHOS        3.4   MG/DL       MCHC       31.4    G/DL    
          ALT          68     U/L (H)   RDW        18.1       % (H)
          BUN          40   MG/DL (H)   HDW        3.19    G/DL    
          NA          133  MMOL/L (L)   MPV         7.6      FL    
          CL          105  MMOL/L       PLT UNC     293   K/MM3    
          CO 2         18   MEQ/L (L)   RET         1.4       %    
          TIBC        277  MCG/DL       

     Ingrid's hematocrit is very low.  We will arrange for a blood transfusion for her.  We will also start EPO 3000 units sq biw.  We will check her serum albumin prior to transfusion.  To help reduce her blood pressure we will add Hydralazine 10 mg q.d.

     Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in three months.  In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

     We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, June 18, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

     Ingrid reports improved blood pressures with diastolics ranging from 70's-80's and systolics in the 130's since starting on Hydralazine.   Todays blood pressure may be secondary to white coat hypertension as her home blood pressures and readings done in CAT/CR appear to be stable.  

She had a blood transfusion for a hematocrit of 18.  She was also initiated on erythropoietin.  Her 24 hour protein excretion came back as 1.1 grams per day.  Her creatinine has stabilized at 2.8 mg/dl consistent with chronic transplant nephropathy.  She has also recently started back on Rocaltrol for hypocalcemia with her most recent serum calcium being 7.  That was with a serum albumin of 2.7 and she therefore needs to be on calcium supplements.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     EPOGEN 4000 units b.i.w.
     HYDRALAZINE HCL 10 mg q.i.d.
     IMURAN 100 mg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 10 mg (14-25 days)
     PROCARDIA 90 mg b.i.d.
     MVI 1 q.d.

Ingrid's physical exam was notable for:
     BP 151/103   P 86   R 14    WT 71.2kg   
     General: comfortable, well hydrated in no distress. 
     Skin: without lesions.
     HEENT: within normal limits. 
     Neck: supple no adenopathy.
     Chest: clear. 
     Heart: without murmur.
     Abdomen: soft and flat without masses nor tenderness.
     Extremities: no edema.
     Neurological: no lateralizing signs.

A urinalysis revealed:
     SPEC GR UA  N               RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  1-2+            GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   few        
     WBC/HPF     0-1             BACTERIA    N     

Laboratory studies at this time include:
          K           4.6  MMOL/L (H)   PLT UNC     235   K/MM3    
          CA          7.2   MG/DL (C)   NEUT         70       %    
          CR          3.1   MG/DL (H)   LYMPH        25       %    
          PHOS        4.3   MG/DL       MONOS         2       %    
          BUN          48   MG/DL (H)   EOS           1       %    
          NA          138  MMOL/L       BASO          1       %    
          CL          106  MMOL/L       NF-2001       1       %    
          CO 2         21   MEQ/L (L)   CELLS C     100    
          WBC        5.93   K/MM3       ABS NEU    4.15   K/MM3    
          RBC        2.98   M/MM3 (L)   ABS LYM    1.48   K/MM3    
          HGB         8.1    G/DL (L)   ABS EOS     .06   K/MM3    
          HCT.       25.3       % (L)   ANISO        1+    
          MCV        84.9      FL       MICRO        1+    
          MCH        27.2      PG       POIK         1+    
          MCHC       32.0    G/DL       OVALO        1+    
          RDW        17.0       % (H)   TEAR       RARE (*)
          HDW        2.89    G/DL       SCHISTO    RARE (*)
          MPV         7.6      FL       WBC COR    5.93   K/MM3    

     In summary Ingrid's blood pressures appear to be better controlled since Hydralazine was initiated last week.  We have asked her to document her home blood pressure readings so that we can better assess her blood pressure status.  We have also initiated calcium carbonate for calcium replacement.  We anticipate beginning Ferrous Sulfate supplements over the next couple of weeks.  We will change her Imuran to Cellcept 1 gram bid po next week.

     Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in one week.  In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, June 25, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.
Ingrid comes in for follow up.  Her problems include:

1.  Gradual decline in renal function with creatinine around 3.0 mg/dl.  Her previous biopsy showed chronic transplant nephropathy.  2.  Hypertension, she is both on Hydralazine and Procardia.  Yesterday her blood pressure was 160/105 accompanied by bilateral pedal edema.  She increased her Lasix dose to 80 mg X1.  On home monitoring, Ingrid reports diastolic pressures of 100's.  3.  Anemia, she has been on erythropoietin for three doses.  She was recently transfused for a hematocrit of 18.5.  Her hematocrit last week was 25.3.  4.  Low calcium, she was started on Tums last week, she also continues to take Rocaltrol daily.  5.  Proteinuria, 24 hours protein excretion 1.1 g/day.  

Otherwise Ingrid has actually felt better ever since her blood transfusion.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     EPOGEN 4000 units b.i.w.
     HYDRALAZINE HCL 10 mg q.i.d.
     IMURAN 100 mg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 10 mg (14-25 days)
     PROCARDIA 90 mg b.i.d.
     TUMS one t.i.d.

Ingrid's physical exam was notable for:
     BP 157/90   P 91   R 12   WT 69.3kg   
     General: well hydrated in no distress. 
     Skin: without lesions.
     HEENT: within normal limits. 
     Neck: supple without lymphadenopathy.
     Chest: clear. 
     Heart: without murmur.
     Abdomen: soft without tenderness over the graft site.
     Extremities: no edema.
     Neurological: no lateralizing signs.

A urinalysis revealed:
     SPEC GR UA  1.006           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  2+              GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   many       
     WBC/HPF     3-5             BACTERIA    occ   

Laboratory studies at this time include:
          K           5.1  MMOL/L (H)   RDW        18.0       % (H)
          ALB         3.3    G/DL       HDW        3.11    G/DL    
          CA          7.9   MG/DL (L)   MPV         7.7      FL    
          CR          3.0   MG/DL (H)   PLT UNC     273   K/MM3    
          PHOS        3.8   MG/DL       NEUT AB    4.73   K/MM3    
          BUN          42   MG/DL (H)   LYMPH A     .40   K/MM3    
          NA          136  MMOL/L       EOS ABS     .02   K/MM3    
          CL          108  MMOL/L       NEUT-A     87.8       % (H)
          CO 2         19   MEQ/L (L)   LYMPH-A     7.5       % (L)
          WBC        5.39   K/MM3       MONO-A      3.1       %    
          RBC        3.06   M/MM3 (L)   EOS-A        .3       %    
          HGB         8.5    G/DL (L)   BASO-A       .5       %    
          HCT.       25.9       % (L)   ATYP LY      .7       %    
          MCV        84.7      FL       ANISO-A      ++ (*)
          MCH        27.8      PG       MICRO-A       +    
          MCHC       32.8    G/DL       LEFT SH  ABSENT    

Ingrid continues to have a gradual decline in renal function from chronic transplant nephropathy.  She has had continuing problems with hypertension.  When questioned, she admits to missing her middle of the day Hydralazine dose.  We have advised her to do a strict diary of her blood pressures to guide us with her blood pressure management.  We have also decided to initiate her on Atenolol 50 mg q.d..  If her diastolic pressures are less than 90 in the middle of the day, she may hold her Hydralazine.  We will reassess her blood pressure regimen in clinic next week.

Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in one week. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Gleide Blue, MD
               Attending, Pediatric Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

    We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, July 2, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

    Ingrid comes to the renal transplant program today for follow up.  She does have chronic transplant nephropathy.  During her last visit with us, we initiated her Atenolol with note of decrease in pulse rate.  She still has occasional rises in blood pressure.  She has dropped her late afternoon Hydralazine dose.  At this point we would like her to continue monitoring her blood pressure.  

    Ingrid also continues on Erythropoietin.  Her hematocrit is stable at 25%.  It appears that she develops bruising with the volume of Erythropoietin that she gets.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     EPOGEN 4000 units b.i.w.
     HYDRALAZINE HCL 10 mg q.i.d.
     IMURAN 100 mg q.d.
     ROCALTROL 0.25 mcg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.d.
     ESTINOL 2 mg (1-25 days)
     PROVERA 10 mg (14-25 days)
     PROCARDIA XL 90 mg b.i.d.
     TUMS one t.i.d.

Ingrid's physical exam was notable for:
     BP 150/95   P 65   R 12    WT 71kg 
     General: comfortable, in no distress. 
     Skin: positive bruising at injection sites.
     HEENT: within normal limits. 
     Neck: supple without lymphadenopathy.
     Chest: clear to auscultation. 
     Heart: without murmur.
     Abdomen: soft without tenderness nor bruits over the graft site.
     Extremities: positive bilateral ankle edema.
     Neurological: no lateralizing signs.

A urinalysis revealed:
     SPEC GR UA  1.002           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  1+              GRAN CAST   N          
     BLOOD UA    trace           WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           5.0  MMOL/L (H)   HDW        3.11    G/DL    
          ALB         3.2    G/DL       MPV         7.7      FL    
          CA          7.9   MG/DL (L)   PLT UNC     297   K/MM3    
          CO 2         19   MEQ/L (L)   NEUT AB    3.95   K/MM3    
          CL          106  MMOL/L       LYMPH A    1.66   K/MM3    
          CR          3.3   MG/DL (H)   EOS ABS     .08   K/MM3    
          GLU          96   MG/DL       NEUT-A     63.0       %    
          PHOS        4.8   MG/DL (H)   LYMPH-A    26.5       %    
          NA          135  MMOL/L       MONO-A      6.7       %    
          BUN          53   MG/DL (H)   EOS-A       1.2       %    
          WBC        6.27   K/MM3       BASO-A       .8       %    
          RBC        3.02   M/MM3 (L)   ATYP LY     1.9       %    
          HGB         8.8    G/DL (L)   ANISO-A      ++ (*)
          HCT.       25.8       % (L)   MICRO-A       +    
          MCV        85.4      FL       MACRO-A       +    
          MCH        29.1      PG       LEFT SH  ABSENT    
          MCHC       34.1    G/DL       RET         2.4       % (H)
          RDW        19.5       % (H)   

    Ingrid appears to have better control of her blood pressure.  At this point we would like to continue monitoring blood pressures before adjusting any of her medications.  Because she is having bruising at sites of Erythropoietin, we have suggested a higher concentration of Erythropoietin with a multiuse vial.  She needs to also monitor her urine outputs as well as fluid intakes as she has recently developed pedal edema.

    Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in  one week. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, July 9, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.
Ingrid was seen one week ago, at that time she had some increasing pedal edema.  She was asked to record her intake and output for three days at home.  Ingrid recorded and average daily intake of roughly 2500-3000 cc of fluid, output was roughly 3000 cc/day.  Systolic blood pressures at home are running 130-140 with diastolic blood pressures  running 80-90.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     HYDRALAZINE HCL 10 mg q.i.d.
     IMURAN 100 mg q.d.
     ATENOLOL 50 mg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.d.
     PROCARDIA XL 90 mg b.i.d.
     TUMS one t.i.d.

Ingrid's physical exam was notable for:
     BP 155/88   P 60   R    T    HT cm   WT 70.3kg   HC cm
     General: alert and feeling well.
     HEENT: within normal limits. 
     Neck: normal.
     Chest: clear to auscultation. 
     Heart: regular rate and rhythm without murmur.
     Abdomen: soft, graft was palpable on the right side and was not tender.  
     Extremities: trace edema.

A urinalysis revealed:
     SPEC GR UA  1.005           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  1+              GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           5.6  MMOL/L (H)   MPV         7.9      FL    
          CA          8.0   MG/DL (L)   PLT UNC     259   K/MM3    
          CR          3.0   MG/DL (H)   NEUT AB    5.37   K/MM3    
          PHOS        3.4   MG/DL       LYMPH A     .53   K/MM3    
          BUN          39   MG/DL (H)   EOS ABS     .04   K/MM3    
          NA          136  MMOL/L       NEUT-A     86.4       % (H)
          CL          108  MMOL/L       LYMPH-A     8.6       % (L)
          CO 2         19   MEQ/L (L)   MONO-A      2.9       %    
          WBC        6.22   K/MM3       EOS-A        .7       %    
          RBC        3.14   M/MM3 (L)   BASO-A       .9       %    
          HGB         9.1    G/DL (L)   ATYP LY      .6       %    
          HCT.       27.4       % (L)   ANISO-A      ++ (*)
          MCV        87.3      FL       MICRO-A       +    
          MCH        29.0      PG       MACRO-A       +    
          MCHC       33.2    G/DL       LEFT SH PRESENT    
          RDW        19.5       % (H)   RET         1.6       %    
          HDW        3.13    G/DL       

Ingrid shows adequate blood pressure control at home on her current regimen.  Her pedal edema could be due to the large fluid intake at home.  We recommend decreasing her po intake to roughly 1800 cc of fluid per day.

Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in two weeks. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Nazek Carerras, MD            Clotilde Goldwater, MD
                                            Director of Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

    We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, July 9, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

    Ingrid was seen one week ago, at that time she had some increasing pedal edema.  She was asked to record her intake and output for three days at home.  Ingrid recorded and average daily intake of roughly 2500-3000 cc of fluid, output was roughly 3000 cc/day.  Systolic blood pressures at home are running 130-140 with diastolic blood pressures  running 80-90.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     HYDRALAZINE HCL 10 mg q.i.d.
     IMURAN 100 mg q.d.
     ATENOLOL 50 mg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.d.
     PROCARDIA XL 90 mg b.i.d.
     TUMS one t.i.d.

Ingrid's physical exam was notable for:
     BP 155/88   P 60     WT 70.3kg  
     General: alert and feeling well.
     HEENT: within normal limits. 
     Neck: normal.
     Chest: clear to auscultation. 
     Heart: regular rate and rhythm without murmur.
     Abdomen: soft, graft was palpable on the right side and was not tender.  
     Extremities: trace edema.

A urinalysis revealed:
     SPEC GR UA  1.005           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  1+              GRAN CAST   N          
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   N          
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           5.6  MMOL/L (H)   MPV         7.9      FL    
          CA          8.0   MG/DL (L)   PLT UNC     259   K/MM3    
          CR          3.0   MG/DL (H)   NEUT AB    5.37   K/MM3    
          PHOS        3.4   MG/DL       LYMPH A     .53   K/MM3    
          BUN          39   MG/DL (H)   EOS ABS     .04   K/MM3    
          NA          136  MMOL/L       NEUT-A     86.4       % (H)
          CL          108  MMOL/L       LYMPH-A     8.6       % (L)
          CO 2         19   MEQ/L (L)   MONO-A      2.9       %    
          WBC        6.22   K/MM3       EOS-A        .7       %    
          RBC        3.14   M/MM3 (L)   BASO-A       .9       %    
          HGB         9.1    G/DL (L)   ATYP LY      .6       %    
          HCT.       27.4       % (L)   ANISO-A      ++ (*)
          MCV        87.3      FL       MICRO-A       +    
          MCH        29.0      PG       MACRO-A       +    
          MCHC       33.2    G/DL       LEFT SH PRESENT    
          RDW        19.5       % (H)   RET         1.6       %    
          HDW        3.13    G/DL       

    Ingrid shows adequate blood pressure control at home on her current regimen.  Her pedal edema could be due to the large fluid intake at home.  We recommend decreasing her po intake to roughly 1800 cc of fluid per day.

    Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in two weeks. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, July 23, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

Ingrid says that she has been doing well.  She has decreased the amount of water that she takes during the day to roughly 3 L/day.  She notices that she still has some edema at home.  Otherwise, she states that she has been doing well.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     IMURAN 100 mg q.d.
     ATENOLOL 50 mg q.d.
     HYDRALAZINE HCL 10 mg t.i.d.
     SODIUM BICARBONATE 650 mg t.i.d.
     ERYTHROMYCIN 3000 units b.i.w.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.h.s.
     PROCARDIA XL 90 mg b.i.d.
     TUMS one t.i.d.

Ingrid's physical exam was notable for:
     BP 162/95   P 53    WT 72.2kg  
     General: well developed, well nourished young woman.
     Skin: normal.
     HEENT: normal.
     Neck: normal.
     Chest: clear.
     Heart: regular rate and rhythm.
     Abdomen: soft, graft was palpable on the right side and was not tender.  
     Genitourinary: normal.
     Extremities: 1+ edema at the ankles.
     Neurological: normal.

A urinalysis revealed:
     SPEC GR UA  1.010           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  2+              GRAN CAST   N          
     BLOOD UA    2+              WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   many       
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           5.2  MMOL/L (H)   HGB         9.5    G/DL (L)
          CA          7.3   MG/DL (C)   HCT.       28.7       % (L)
          CR          2.9   MG/DL (H)   MCV        89.9      FL    
          PHOS        4.0   MG/DL       MCH        29.8      PG    
          BUN          51   MG/DL (H)   MCHC       33.1    G/DL    
          NA          140  MMOL/L       RDW        19.6       % (H)
          CL          111  MMOL/L (H)   HDW        3.07    G/DL    
          CO 2         20   MEQ/L (L)   MPV         8.3      FL    
          WBC        7.57   K/MM3       PLT UNC     169   K/MM3    
          RBC        3.19   M/MM3 (L)   

Ingrid is doing well.  She has stable renal function.  Her metabolic status is satisfactory.

Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in one month. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Loocie Welby, M.D.          RE: Ingrid Sand
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/31/71

Dear Dr. Welby:

We had the pleasure of seeing Ingrid in the Renal Transplant Program on Tuesday, August 27, 1996.  She has chronic renal failure secondary to glomerulosclerosis.  She had a renal transplant on 6/28/84.

Ingrid offers no complaints today.  At home her blood pressure reading have been normal tensive with diastolic in the 80's.  She also stopped taking her sodium bicarbonate.

Her current medications include:
     PREDNISONE 15 mg q.o.d.
     LASIX 40 mg q.d.
     IMURAN 100 mg q.d.
     ATENOLOL 50 mg q.d.
     HYDRALAZINE HCL 10 mg t.i.d.
     ROCALTROL .25 mcg q.d.
     BACTRIM SS (Trimethoprim 80 mg, Sulfamethoxazole 400 mg) q.h.s.
     PROCARDIA XL 90 mg b.i.d.
     TUMS EX 750 mg t.i.d.
     ESTINOL 0.20 1-25
     PROVERA 5 mg 16-25

Ingrid's physical exam was notable for:
     BP 147/100   P 53   R 14   HT 158.5cm   WT 69.4kg   
     General: well developed, and well nourished in no distress.
     Skin: positive bruises that are in various stages of resolution.
     HEENT: TM's not injected.
     Neck: supple without lymphadenopathy.
     Chest: clear to auscultation.
     Heart: regular rate and rhythm.
     Abdomen: soft without masses or tenderness.
     Extremities: warm and well perfused.
     Neurological: no lateralizing signs.

A urinalysis revealed:
     SPEC GR UA  1.006           RBC/HPF     N          
     WBC ENZ UA  N               WBC CAST    N          
     PH UA       5.0             RBC CAST    N          
     PROTEIN UA  2+              GRAN CAST   0-1        
     BLOOD UA    N               WAXY CAST   N          
     GLUCOSE UA  N     mg/dl     SQUAM EPI   few        
     WBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           5.1  MMOL/L (H)   RDW        17.9       % (H)
          ALB         3.2    G/DL       HDW        3.28    G/DL (H)
          CA          7.9   MG/DL (L)   MPV         8.0      FL    
          CR          3.1   MG/DL (H)   PLT UNC     208   K/MM3    
          FE           50  MCG/DL (L)   NEUT AB    7.39   K/MM3    
          PHOS        4.6   MG/DL (H)   LYMPH A     .53   K/MM3    
          TRF         324   MG/DL       EOS ABS     .12   K/MM3    
          BUN          50   MG/DL (H)   NEUT-A     89.3       % (H)
          NA          138  MMOL/L       LYMPH-A     6.4       % (L)
          CL          108  MMOL/L       MONO-A      2.1       %    
          CO 2         19   MEQ/L (L)   EOS-A       1.4       %    
          TIBC        321  MCG/DL       BASO-A       .6       %    
          WBC        8.28   K/MM3       ATYP LY      .3       %    
          RBC        3.64   M/MM3 (L)   ANISO-A       +    
          HGB        10.7    G/DL (L)   MICRO-A       +    
          HCT.       31.9       % (L)   LEFT SH  ABSENT    
          MCV        87.6      FL       RET         0.7       %    
          MCH        29.4      PG       FER        15.1   NG/ML    
          MCHC       33.6    G/DL       

Ingrid offers no complaints today with moderate stable renal function.  We checked her iron studies as her reticulocyte count is on the low side for her hematocrit.  We will initiate iron therapy if her iron studies are low.  We have advised her to continue monitoring her blood pressure and if they continue to be normal we will discontinue the hydralazine.  We will continue communicating with the Brigham Renal Service in order to transition her care.

Thank you for the opportunity to participate in Ingrid's care.  We would like to review her in one month. In the interim, we would be happy to discuss or review Ingrid should you or the family wish it.

               Sincerely,


               Antonio Khan, MD
               Attending, Pediatric Nephrology


cc:
Jean B Emans, M.D.
Adolescent Clinic
Hunnewell Ground
Children's Hospital
300 Longwood Avenue
Boston, MA 02115


CONTENT:
Marietta Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert in the Endocrine Program on 08/29/94 for hypopituitarism.

History: Robert is a 17 2/12 years old male with acquired growth hormone deficiency, last seen 03/15/93 and who has since missed multiple follow up appointments.  He has been receiving Lupron and growth hormone supplimentation but it was not clear how compliant he was with the therapy.  His last dose of growth hormone was about two months ago.  He cannot recall his last dose of Lupron.  Robert has nocturia highly.  He has occasional heat intolerance.  He will be starting his sophomore year in school.

Current medications: None.

Physical exam:  
Height: 154.0 cm        Weight: 49.4 kg         Pulse: 80               
Mid-shaft diam.: 2.6 cm 
Robert was a comfortable black male.  Skin: warm.  Neck:  No thyromegaly.  Abdomen:  No hepatosplenomegaly.  GU late pubertal genitalia.

Laboratory studies:  A bone age obtained 08/29/94 was read as follows:  A PA view of the left hand and wrist shows no bony abnormalities.  The epiphyses are still open.  The chronological age is 17 years 2 months, bone age is 15 years.   Standard
deviation is 13 months.   Osmolality  293  mosmkg,  osmou 1183  mosmkg, Na           143  mmol/L, nau 192 mmol/L, T4  5.0  mcg/dL, TBGI 1.04

Impression: In all likelihood, Robert's epiphyses are fused or close to fusion.  there is little additional height to be gained therefore with further treatment with growth hormone.  consequently, if this is bore out by a bone age today, I would no recommend further treatment.  I will also obtain a standard set of neuroendocrine screening studies.

Disposition: Robert is to return to clinic in six months.
Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology   


Addendum: Robert should be started on thyroid supplementation.  He likely has central hypothyroidism.


CONTENT:
Marietta Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

I saw Robert in the Endocrine Program on 3/15/96 for follow-up of hypopituitarism.

History: Robert is an 18 and 10/12-year-old status post chemotherapy and radiation for rhabdomyosarcoma.  He reports that his interval history has been remarkable for intermittent leg pain and occasional back pain.  These appear to be unrelated to exercise.  He also reports "shortness of breath" and dyspnea on exertion, however the dyspnea and shortness of breath are intermittent, sometimes occurring at night, sometimes occurring at the end of strenuous activity.  Robert reports nocturia three to four times a night.  He is currently doing well, and has a lot of  friends.

Physical exam:
HT: 167 cm    WT: 50.3 kg       BP: 90/70       Pulse: 60               
Slight male with no apparent distress.   Skin:  Warm.  HEENT:  No thyromegaly.  Chest exam:  No murmur, breath sounds clear throughout.

Laboratory studies:  CR = 0.8 mg/dL,  BUN =13 mg/dL, WBC = 5.97k/mm3, RBC = 4.56 k/mm3, hemoglobin = 14.3 g/dL, hct = 40.9%, MCV = 89.8 Fl, MCH = 31.4 pg, MCHC = 349 g/dL, RDW = 12.2%, HDW = 2.61 g/dL, PLT = 230 k/mm3, ESR = 5 mm/hr. T4 = 4.8  ug/dL, TBGI  = 1.01, TSH = 1.40  uU/mL.  Urinalysis: SPEC GR = 1.021, pH = >9.0, negative WBC enzymes, nitrite, glucose, ketones, trace protein

Impression: Robert has grown reasonably well considering his intermittent  compliance with growth hormone.  He has now been off growth hormone for  approximately 1 year but has achieved a quite acceptable height of 5'2".   Although tertiary hypothyroidism was noted to be likely on his last visit he has not received therapy for that since and that may contribute to some fatigability.  His nocturia is suggestive of diabetes insipidus  However, on his prior visit he was found to have a urine osmolality well above 1100 mOsm/kg which is inconsistent with diabetes insipidus.  Incidentally, Robert's mother reports that she has similar behaviors, that is, she wakes up 3 to 4 times a night for urination.  Robert's shortness of breath is of some concern.  Although I could not find any abnormality on exam I nonetheless worry that he may have some late complication of his treatment and therefore have elected to obtain a chest X-ray.

Disposition: 1.  Repeat neuroendocrine screening for thyroid status 2.  Initiation of thyroid supplementation  3.  Chest X-ray 4.  Sedimentation rate to screen him for rheumatologic disease



Sincerely,




Lancelot Blue, MD             
Attending in Endocrinology   


Addendum: T4 is low.  Robert should start taking Synthroid 50 mcg qd.  Also, the chest x-ray was normal.


CONTENT:
Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert for evaluation of short  stature in the Endocrine Program on 5/16/91.

History: Robert is a 13 and 11/12 year old with a right orbital rhabdomyosarcoma removed on 7/11/83.  5400 Rads were delivered to the right orbit, and 400 Rads to the right anterior neck. On 10/84 he underwent enucleation of the right eye.  His has subsequently been noted to have decreased growth velocity.  He also reports waking up a few times a night to urinate and drinks at least 6 to 8 oz glasses of fluid per day.  However, prior urine testing has revealed specific gravities of 1.025 and greater.  He has 2 to 4 bowel movements a day, and has occasional headaches and reports a adequate energy level.  He is currently in 6th grade and has good grades.  He reports body odor of 8 months duration and pubic hair of 6 months duration. He also has noted a small amount of axillary hair.  He complains of being the shortest in his class.

Current medications: None.


Family history: Father 5'4",  mother 5'1". A 9 year old half brother has nephrotic syndrome.

Physical exam: HR: 70, BP: 105/60, weight: 34.6 kg, height: 139.9 cm, upper:lower segment ratio:  0.77, pubic hair Tanner stage:  4, right testicle: 3.2 x 1.5 cm, left testicle: 3.2 x 1.5 cm, penile stretch length: 12.1 cm, mid-shaft diameter: 2.5 cm
General: comfortable, black male.  Skin no apparent  lesions, and a small amount of axillary hair.  HEENT exam was unremarkable except for right zygomatic hypoplasia and a right eye prosthesis.  Neck: there was no thyromegaly.  Chest: clear to auscultation.   Heart exam was unremarkable.  Abdominal exam: no hepatosplenomegaly and soft.  Extremities: no deformities, cyanosis, or edema. Neurological exam was unremarkable except for significantly decreased reflexes in all extremities.

Laboratory studies:
On 3/20/91 Robert underwent an insulin GnRH, TRH glucagon stimulation tests to investigate anterior pituitary, these studies revealed a sub-normal growth hormone response both to insulin induced hypoglycemia and to glucagon.  Cortisol response was adequate.  TSH response was slightly blunted but within normal range and LH and FSH response was in the pubertal range.  T4 was 6.8 mcg/dl, TBGI was 1.02, TT3 was 132 ng/dl, and baseline TSH was 1.1 mcU/ml.

In summary: Robert is a 13 and 11/12 year who has biochemical evidence of growth hormone deficiency and a physical exam and biochemical evidence of puberty.  Although his height remains substantially below normal, his most recent measurement reveals an acceleration since his last visit approximately one year ago.  If his bone age does not show significant acceleration it may still be worthwhile to follow his growth without further intervention as it has been shown that at least a sub-population of these patients can grow adequately without growth hormone supplementation despite biochemical evidence of growth hormone deficiency.

Recommendations: Due to Robert's mother being under some time pressure, we were unable to obtain  a bone age today.  However, we have discussed with the mother  the necessity of obtaining such a bone age and she has also expressed interest in meeting again next month to discuss again the pros and cons of both growth hormone therapy and the use of a GnRH analog to block pubertal advance.

Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD                      Etta Kramer, MD
Fellow in Endocrinology               Assistant in Endocrinology


CONTENT:
Ferle Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert in the Endocrine Program on 08/15/91 for evaluation of growth hormone deficiency.

History: Robert is a 14 and 2/12 year old status post right orbital rhabdomyosarcoma treated on 7/83 with 5400 rads to the right orbit and 4000 to the anterior neck.  On 10/84 he underwent enucleation of the right eye.  He has had body odor for one year and pubic hair for three quarters of a year.  He has not had acne.  He continues to be bothered by his being the smallest in the class.  He has bowel movements one to two times per day and occasional nocturia.  Prior functional testing has revealed him to have a subnormal growth hormone response and a pubertal gonadotropin response.

Current medications: None.

Physical exam:  
Height: 142.2 cm        Weight: 34.8 kg         BP: 90/58
Pulse: 64               Pubic hair Tanner: 4    
R. testis: 3.8 x 2.0 cm              L. testis: 3.8 x 1.9 cm 
Penile stretch length: 12 cm         Mid-shaft diam.: 2.2 cm 
Comfortable male in a no apparent distress.  Skin: no lesions.  HEENT: prosthetic eye.  Pupillary reflex and extraocular movements intact in native eye.  Neck: no thyromegaly.  Chest: clear to ausciltation.  Heart: regular rate and rhythm.  Abdomen: no hepatosplenomegaly.  GU: pubertal male.  Extremities: no edema or cyanosis.  Neuro: nonfocal.

In summary: Robert is a 14 and 2/12 year old who is showing a mild accelleration of his growth, which is probably due to sex steroids elaborated by his testes.  Since this is probably accellerating his bone age disproportionately to his bone length because of the documented growth hormone deficiency, he would be best served by both blocking his puberty at this point, as well as giving him growth hormone supplementation.  We discussed at length with both Robert and his mother the risks and benefits of growth hormone therapy.  Among the risks, we included the possibility of tumor recurrence and secondary malignancy such as leukemia.  We reported that we had no firm data to indicate to us what the extent of such a risk was.  Both Robert and his mother indicated that they understood these risks, but nonetheless would be very interested in continuing with our plan.

Disposition: We will arrange that Robert and his mother meet with our clinic nurse to learn how to administer daily growth hormone and we will also arrange for monthly injections with GNRH analog to block pubertal advancement.  At that time, we will also obtain a bone age.

Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD                      Kendell Simpson, MD
Fellow in Endocrinology               Clinical Director

IK/SN/smp


CONTENT:
Ferle Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert in the Endocrine Program on 12/12/91 for evaluation of growth hormone deficiency status post irradiation.

History: 14 and 6/12 year old status post cranial irradiation for orbital rhabdomyosarcoma.  He is currently receiving daily doses of growth hormone  at 0.025 mg/kg/day.  He is also receiving monthly injections of Lupron Depot, but he has only received one dose so far.  Since his last visit, Robert has noted marked increase in his stature and his muscular bulk and he also feels more energetic.  He is currently joining several organized athletic activities.  He does report having nocturia 3-4 times a night but on the day of his visit he had not drunk any fluid since morning and did not have increased thirst.  He has bowel movements once a day.  He reports his appetite is significantly increased and he notes that he has increased body odor as well as increased phallic size and pubic hair.

Current medications: Lupron Depot 7.5 mg q 28 days.  Growth hormone 0.025 mg/kg/day.

Physical exam:  
Height: 145.7 cm        Weight: 37.5 kg         BP: 94/60               
Pulse: 68               Pubic hair Tanner: 4    R. testis: 3.2 x 1.2 cm 
L. testis: 3.2 x 2 cm   Phallus length: 14 cm   Mid-shaft diam.: 2.5 cm 
GENERAL: Comfortable, smiling male in no acute distress.  SKIN: Unremarkable.  HEENT: Mucus membranes were moist.  NECK: No thyromegaly.  CHEST: Clear to auscultation.  HEART: Regular rate and rhythm.  ABDOMEN: No hepatosplenomegaly.  GENITOURINARY: Pubertal genitalia.  EXTREMITIES: No apparent deformities.

In summary: Robert appears to be having an excellent response to growth hormone, but continues to show signs of advancing puberty.

Disposition: At the time of this visit, we gave Robert his second dose of Lupron Depot.  We have also obtained a bone age (unchanged over last year) to assess his remaining growth potential and predicted adult height.

Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD             Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Ferle Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert in the Endocrine Program on 9/14/92 for follow-up of growth hormone deficiency.

History: Robert is a 15 and 3/12 year old with growth hormone deficiency status-post cranial irradiation. Over interval history he reports occasional aches. He notes very slight increase in testicular size and none in penile size. He denies headaches, and has had nocturia twice a night over the past three weeks. He has approximately five bowel movements/day. He reports an overall increase in his energy level and strength. He also reports polydipsia (1 glass/hour, principally orange juice). Urinary frequency is approximately 4 times a day.

Current medications: Lupron 7.5 mg q 28 days.  Growth hormone 0.9 mg/day.

Physical exam:  
Height: 147.8 cm        Weight: 46.6 kg         BP: 120/70              
Pulse: 84               Pubic hair: Tanner III  R. testis: 3.2 x 1.6 cm 
L. testis: 3.3 x 1.8 cm Phallus length: 11.5 cm Mid-shaft diam.: 2.6 cm 
Skin:  Axillary hair present. 
HEENT: Tongue slightly coated.
Neck: No thyromegaly. 
Chest: Clear to auscultation.
Heart: Regular rate and rhythm. 
Abdomen:  No hepatosplenomegaly. 
Genitourinary: Mid-pubertal. 
Extremities: No cyanosis or edema. 
Neurological: Non-focal.

Impression: Robert is a 15 and 3/12 year old who has not visited this clinic regularly enough to provide adequate timely changes of his dosing. His current growth velocity is inadequate at approximately 3 cm/year. His history of nocturia and polydipsia may be due to growth hormone related glucose intolerance or diabetes insipidus.

Disposition: 1) Increase growth hormone to 1.15 mg/day. 2) Continue Lupron. 3) Follow-up on screening for diabetes insipidus.

Laboratory studies pending at this time include: Hemoglobin-BA1, BS, lytes, osmolantis, urinalysis, LH, FSH, glucose intolerance.
Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology   


Problem list to date:
GH-DEFICIENCY                Start: 06/11/91  End:          Invalid: 
CNS-TUMOR                    Start: 12/12/91  End:          Invalid: 

Transcribed by Cassells


CONTENT:
Ferle Stone, M.D.          RE: Robert Lafitte
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/19/77

Dear Dr. Stone:

We had the pleasure of seeing Robert in the Endocrine Program on 12/7/92 for follow-up of growth hormone deficiency.

History: Robert is now 15 and 6/12 years old. He has been taking growth hormone daily as well as a Con RH ogermist to block further pubertal development. His growth velocity over the last 3 months is 6.8 cm/year but only 4 cm/year when compared to a year ago. He reports increased compliance with his dosage. He continues to have nocturia  about 2-3 times a night. He reports "pus" and significant soreness after his intramuscular Lupron doses. He reports occasional headaches. He denies acne, increase in the size of his genitals or an increase in pubic hair.

Current medications: Protropin 0.024 mg/kg/day. Growth Hormone 1.15 mg/day. Lupron 7.5 mg q 28 days.

Physical exam:  
Height: 149.5 cm        Weight: 49.6 kg         BP: 100/60              
Pulse: 82               Pubic hair: Tanner III  R. testis: 3 x 1.5 cm   
L. testis: 3 x 1.6 cm   Phallus length: 12.2 cm Mid-shaft diam.: 2.6 cm 
Skin: Indented, scarred area where most Lupron doses are injected.  Neck: Thyroid palpable but not enlarged.   Chest: Clear to auscultation.   Heart: Regular rate and rhythm.   Abdomen: No hepatosplenomegaly.   Genitourinary: 1 cm slightly tender inguinal node.   Extremities: No cyanosis or edema.   Neurological: Unchanged.

Laboratory studies:  September 14, 1992: Specific gravity 1.019, glycosylated hemoglobin 4.7%, sodium 139 mEq/L, osmolality 282 mm/kg, T4 7.7 mcg/dL.

Impression: With better compliance, Robert is showing improved growth velocity. However, reviewing of how he draws up his daily dose suggest that he may still be not fully compliant. Injection technique needs to be reviewed with UNA and Robert needs to be made aware of proper care of IM sites.

Disposition: Return to clinic in three months. Review presence of node at that time.  Increase GH dose to 1.25 mg/day.
Thank you for letting us share in the care of Robert.

Sincerely,


Lancelot Blue, MD             
Attending in Endocrinology


CONTENT:
Crane, M.D.          RE: Hallie MacBridge
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/20/74

Dear Dr. Crane:

We had the pleasure of seeing Hallie in the Endocrine Program on 11/21/91 for evaluation of .

History: Your labs from November 21st, 1991 were normal, including normal thyroid functions (T4 of 9.1mcg/dl, and TSH 1.3 mcU/ml) and normal, nondiabetic average blood sugars (hemoglobin A1 5.8%).  Your cholesterol was 169.  A bone age was read to be 15 years, indicating a year or so of potential further growth.  

As we discussed, I have changed your medications to Premarin, receiving 0.9 mg on days 1-25 of each month, and Provera, receiving 10 mg on days 13-25 each month.  I would anticipate that  you would have a lot less bleeding this month, and you should have a more substantial menstrual period within a few days after the 25th of the month.  Feel free to call if you have any specific questions.

Physical exam:  





Thank you for letting us share in the care of Hallie.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology


CONTENT:
Crane, M.D.          RE: Hallie MacBridge
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/20/74

Dear Dr. Crane:

We had the pleasure of seeing Hallie in the Endocrine Program on 6/8/93 for follow-up.

History: All of your laboratory studies obtained on June 8, 1993 were normal. Specifically, your thyroid hormones were normal with a T4 of 10.6 mcg/dL and a TSH 2.1 mcu/mL. Other hormones revealed FSH 11.5 miu/mL, LH 6 miu/mL, DHEA sulfate 276 mcg/dL, and estradiol 99 ng/dL. Your hemoglobin-A1 was 5.7% (normal, nondiabetic), and your cholesterol was 176.

I have enjoyed seeing you as a patient over the years. If you have any questions, please do not hesitate to contact me.

Physical exam:  




Thank you for letting us share in the care of Hallie.

Sincerely,


Etta Kramer, MD           
Attending in Endocrinology


CONTENT:
Antony Plutarch, M.D.          RE: Andie Austen
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/24/78

Dear Dr. Plutarch:

We had the pleasure of seeing Andie in the Endocrine Program on 4/29/93 for evaluation of hypothyroidism.

History: As you know,  Andie is a 14 and 8/12 year old female who was previously well until about one week ago when her great-aunt noticed that she had a thick neck. Her mother brought her in to see you at which time you drew thyroid function tests and referred her to endocrine clinic for possible hypothyroidism. Andie states that she noticed that her neck was thicker a bit more than a month ago but thought that this was her Adam's Apple and didn't tell anyone. She has noted increased tiredness necessitating rests during the day, however, she has been more active and is involved in softball practice daily. She sleeps about 8-9 hours a day with no noted change in sleep patterns. She has had no change in appetite, no recent illnesses, no fevers, nausea, vomiting, diarrhea, constipation, change in her bowel movement, hyperactivity, neck pain, change in menses, change in hair. She has always had dry skin and has had no change in that as well. She is now in high school and was previously an A student but is now having more difficulties and attributes this to harder work in high school.  Her menses began about one year ago in July of 1992 and has been regular. Her flow is for three days. She does not experience significant cramps.

Past medical history:  Significant for reactive airways disease diagnosed at 5 years of age for which she is presently on Tornelate Inhaler, Seldane, and Provental Inhaler. She has multiple allergies, mostly seasonal to pollen etc. In July of 1990 she underwent a tonsillectomy for multiple strep infections, prior to this and has had no problems since.

Family history:  Remarkable for thyroid problems. Her father reportedly has "thyroid problems". Her paternal grandmother has hypothyroidism and is on medication. She has "cold nodule". Her paternal grandmother's sister also has hypothyroidism and is on medication. There is another aunt of theirs who has thyroid problems.  Her maternal great aunt also had thyroid problems of unknown etiology.

Physical exam:  
Height: 157.8 cm        Weight: 62.7 kg         BP: 100/60              
Pulse: 78               Breast: Tanner IV       Pubic hair: Tanner IV   
General: She was a well appearing female in no apparent distress.  Skin: No rashes or dry patches noted.  HEENT: Essentially negative.  There were no nodules palpated.  Neck: Diffusely enlarged thyroid gland. Chest: Clear.  Heart: Regular rate and rhythm without murmurs, gallops, or rubs.  Abdomen: Soft, nontender. No hepatosplenomegaly.  No masses.  Neurological: Unremarkable. DTRs were 2+ bilaterally.

Laboratory studies:  from April 23, 1993: T4 4.8 (normal 4.5-11.8), T3 uptake 27 (normal 25-37), T7 1.3 (normal 1-4.4), TSH 18.9 (normal .5-6.1), thyroglobulin antibodies 15.1.

Impression: Andie is a 14 year old female with no overt clinical symptoms except for  a large goiter and laboratory testing which is consistent with hypothyroidism.  Also, there is a strong family history for hypothyroidism. The most likely etiology is Hashimoto thyroiditis which is believed to be autoimmune mediated.  Since Andie is not symptomatic at this time, we would recommend starting Synthroid at a lower dose of 50 mcg daily. We would like to have her return to clinic in six weeks to monitor this dose which may need adjustment.

Thank you for letting us share in the care of Andie.

Sincerely,


Fozzie Swartzenegger, MD        Kendell Simpson, MD             
                             Clinical Director


CONTENT:
Yvette Seinfeld, M.D.          RE: Agatha Cobbett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Seinfeld:

We had the pleasure of seeing Agatha in the Endocrine Program on 10/21/94 for hashimoto's thyroiditis.

History: Agatha is a 16 year old girl with Hashimoto's thyroiditis and a slight increase in her thyroid peroxadase and anti- thyroglobulin antibodies at the time of her November 1993 exam.  Her recent past medical history reveals that she has had the flu with persistent headaches that were not relieved by Ibuprofen.  The symptoms are currently abaded.  Agatha describes her energy level as "good" now that she is skating and playing field hockey several times a week.  She denies dry skin.  She has a bowel movement every other day.   Her diet is balanced although she does not like eating meat.   She notices no change in the size or tenderness of her thyroid gland.   She denies recent gum or tooth infections.  Her last visit with Dr. Ferraro was in the summer of 1993 and her next visit will be in six months.  Her previous menstrual period was 30 days ago and she is menstruating at present.  She states that her last several menstrual periods have been 30 days apart.  She also complains of a painful serous fluid producing small lesion at the base of her spinal column.

Current medications: None.

Physical exam:  
Height: 169.2 cm        Weight: 57.6 kg         BP: 106/59              
Pulse: 86               Breast: Tanner V        Pubic hair: Tanner V    
General: She is a thin, well-nourished and well-developed female in no apparent distress.
Skin: clear except for the skin of her medial upper right buttock where she has a one quarter to one half cm diameter reddened, non- raised area that at present expresses no fluid.  It is slightly tender at the site of inflammation.  There is no area of induration beneath the erythematous region.  HEENT: negative.
Neck: No thyromegaly.  Lymph Nodes:  No adenopathy.
Chest: Lungs clear.  Heart: Regular rate and rhythm without murmur.
Abdomen: No hepatosplenomegaly.
Extremities and neurological:  unremarkable.

Laboratory studies:  10/21/94 T4 7.0 mcg/dl, TBGI 1.00, T3 125 ng/dl, TSH 2.5 U/L, TPOAb 193.8 U/ml (N <0.3), TGAb 50.8 U/ml (N <0.3).

Impression: Hashimoto's thyroiditis, clinically and biochemically euthyroid with significantly elevated thyroid auto-antibodies.  Pilonidal cyst.

Disposition: Referred to Dr. Craig Lillehei after speaking with Dr. Barbara Segal for removal of pilonidal cyst (done 11/22/94). Return to clinic in 12 months.  We shall discuss the findings of thyroid function tests with Agatha and her mother.
Thank you for letting us share in the care of Agatha.

Sincerely,


Rahul Hagen, MD           Mitsuko Simpson, MD             
Fellow in Endocrinology      Chief Emeritus               


Addendum: TPO antibodies 193.8 and TG antibodies 50.8 are both elevated.


CONTENT:
Yvette Seinfeld, M.D.          RE: Agatha Cobbett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Seinfeld:

I saw Agatha in the Endocrine Program on 11/7/95 for Hashimoto's thyroiditis.

History: Sara is a 17 year old high school junior who is seen for follow up of Hashimoto's thyroiditis.  She had an intercurrent illness two weeks ago, sore throat and fever that has now resolved. She is active in sports  and doing well at Wellesley High School.  Her last menstrual period was in October 1995 and she has her menstrual periods every four weeks and the length of the periods is only two days.  She has bowel movements every day to every other day; she has had no recent gum infections or thyroid pain and she denies cold intolerance.   Two weeks ago when she had the sore throat, the school nurse noted that she had an enlarged thyroid gland.

Current medications: None.

Physical exam:
Ht: 170.8 cm       Wt: 59.4 kg
BP: 98/60          Pulse: 78               
General appearance:  She is a well appearing, well nourished, pink cheeked young lady in no apparent distress.  Her skin is not dry.  She has no acne, no tonsillar erythema, exudate or enlargement.  Thyroid gland has palpable edges, no lymph nodes.  Lungs are clear.  Heart:  regular rate and rhythm without murmur. Abdomen: soft, no hepatosplenomegaly.  Deep tendon reflexes are +2/4 bilaterally in her  upper and lower extremities.

Previous laboratory studies:  She had normal thyroid function tests one year ago, but elevated TPO's and anti-thyroglobulin antibodies.  Today, her TSH was 2.6 mcU/mL (normal), T4 5.2 mcg/dL (slightly below normal); TT3 119 ng/dL (normal) and TBGI 0.99 (normal).  Her thyroid antibodies were elevated again.

Impression: Sara has Hashimoto's thyroiditis that appears to be mild but possibly had been activated during an intercurrent illness two weeks ago.  Her current thyroid function is normal.  We will ask Agatha to repeat the TFT's in 3 months because the antibodies were elevated.  We would like Sara to return to clinic in 12 months.



Sincerely,




Rahul Hagen, MD           
Attending in Endocrinology


CONTENT:
Yvette Seinfeld, M.D.          RE: Agatha Cobbett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Seinfeld:

We had the pleasure of seeing Agatha in the Endocrine Program on 7/20/92 for follow-up of hashimoto's thyroiditis.

History: Agatha is a 3 and 9/12 year old female who presented in November 1991 to Mass. General with chief complaints of fatigue, headaches, and neck pain.  She denied weight gain or loss, change in appetite and intolerance to heat or cold.  Mother states that she was told that her thyroid gland was enlarged.  Agatha missed more than half a year of school from November 1991 to the present.  The findings noted on her work-up at Mass. General include increase in thyroid antibodies and a low cortisol level.  An MRI on March 28, 1992 revealed no pituitary lesions but did show sphenoid cysts on one of her right sinuses.  At Mass. General, in April 1992 she was told she had Hashimoto's thyroiditis and was told she had an 80% chance of her thyroid becoming inactive within the next few years.  Recently, she was "possibly less active than usual is at camp" in the past two weeks.  She has had no palpitations; her appetite has been good. She has grown taller and gained weight in the past year.  She has no constipation. Menarche was at 13 and 4/12  years old and menses are normal.

Current medications: Amoxicillin 250 mg tid for otitis media.

Past medical history:  Mother was a 37 year old G2P2 when Agatha was born. Agatha was delivered by an uncomplicated Caesarean section. Birth weight: 10 lbs. 9.5 oz. Infectious Disease: Work-up at Mass General in spring 1992 negative. Hospitalizations: none. Injuries: Temporal mandibular joint injury at age 6. Surgery: None.

Review of systems:  Skin: complains of heat rash. HEENT: has Seborrheic dermatitis, and blepharitis. The remainder of the review of systems is negative.

Family history:  Positive for a paternal family history of thyroid disease and goiter. Positive for osteogenesis imperfecta in her mother. Positive for whooping cough in Agatha after birth. Maternal grandmother had uterine cancer in her 30's after taking DES. Mother, brother, and maternal aunts and uncles have asthma. Mother also has "immature platelets". Agatha's mother has hypertension controlled with methyl dopa and chronic bronchitis. Father's height is 5'10.5". Mother's height is 168 cm.

Physical exam:  
Height: 165.1 cm        Weight: 52.8 kg         BP: 96/66               
Pulse: 72               U/L ratio:  0.97        Arm span: 169           
Breast: Tanner III      Pubic hair: Tanner IV   
General: Agatha is well- nourished and well-developed and in no acute distress.  Skin: Dry, no lesions.   HEENT: No adenopathy, no exophthalmus.  Neck: No thyromegaly.   Chest: Clear.   Heart: Regular rate and rhythm. No murmur.  Abdomen: Soft, nontender.  Extremities: No clubbing, cyanosis or edema.  Neurological: Normal.

Laboratory studies:  January 14, 1992 UA negative, phosphorous 5.3, total bilirubin 2.1, throat culture negative, blood culture negative.  January 15, 1992 Negative Lime antibodies. January 16, 1992: ANA negative, IGG 1030, IGA 32 (low), IGM 54 (low).  January 22, 1992: thyroid microsomal antibodies elevated 1-1600, thyroid thyroglobulin antibodies that were elevated at 1-640. CMV titer negative, toxoplasmosis antibodies negative, total bilirubin 1.8/0.3, alkaline phosphatase 233, SGOT 23.  July 20, 1992:  T4 7.4 mcg/dL, TSH 2.0 mcU/mL, microsomal Ab 1:400, thyroglobulin Ab 1:320.

Impression: Agatha has Hashimoto's thyroiditis.  We explained to Agatha and her mother that one third of cases of Hashimoto's thyroiditis improve, another third do not change and another third progress to hypothyroiditis.  At present, she is clinically and biochemically euthyroid and needs no therapeutic intervention.

Disposition: Follow-up in six months.
Thank you for letting us share in the care of Agatha.

Sincerely,


Rahul Hagen, MD           Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Yvette Seinfeld, M.D.          RE: Agatha Cobbett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Seinfeld:

We had the pleasure of seeing Agatha in the Endocrine Program on 1/11/93 for evaluation of Hashimoto's thyroiditis.

History: Agatha is now a 14 and 4/12 year old here for follow-up of Hashimoto's thyroiditis. She has no complaints of tiredness. She does have a problem falling asleep at night; she goes to bed at 11 p.m. but does not fall asleep until 1 a.m. She has no neck pain or tenderness although she did have neck pain on exam in July 1992. She does have heat rash and  scaling dry skin most pronounced behind her ears. Her last menstrual period was two weeks ago. Her periods occur regularly at 28 day intervals. Menarche was at age 13 and 4/12 years of age. She is in eighth grade and gets excellent grades. She will attend Wellsley High School next fall. She is having an increase in participation in field hockey from September through October 1992. She has also been skating twice a week either before or after school from September 1992 to present

Review of systems:  She denies headaches, has cold intolerance, denies constipation. The thyroid gland looks smaller to her mother. Her skin is and always has been dry. She does not have any palpitations. Mother is going to Al-Anon for counseling concerning Agatha's fathers alcohol abuse problem.

Physical exam:  
Height: 166.2 cm        Weight: 53.5 kg         BP: 98/56               
Pulse: 60               Breast: Tanner III      Pubic hair: Tanner IV   
General: Her weight is up 0.5 kilos since July. Her height is up 1.1 cm since July.  Skin: Dry skin on her face, arms, legs and trunk. She does have axillary hair.  HEENT: Pupils equal, round, and reactive to light. Mucous membranes moist. No tonsillar enlargement.  Neck: She has a diffusely enlarged almost twice enlarged thyroid gland. It is not nodule.  Chest: Lungs were clear.  Heart: Regular rate and rhythm without a murmur.   Abdomen: Soft, nontender.  Neurological: Deep tendon reflexes +1-2/4 bilaterally in her upper and lower extremities.

Impression: Agatha has an increase in the size of her thyroid gland consistent with Hashimoto's thyroiditis.  Thyroid function tests were obtained and we will call her mother with the results and send her prescription for Synthroid if needed. Agatha also has a history of a low/normal cortisol level drawn in the afternoon at Mass. General. We would obtain an a.m. cortisol level if other draw a.m. labs were to be drawn.

Thank you for letting us share in the care of Agatha.

Sincerely,



Rahul Hagen, MD           Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Yvette Seinfeld, M.D.          RE: Agatha Cobbett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/05/78

Dear Dr. Seinfeld:

We had the pleasure of seeing Agatha in the Endocrine Program on 11/18/93 for follow up of hashimoto thyroiditis.

History: Agatha is a 15 and 1/12 year old with Hashimoto thyroiditis who gives a history of persistent dry skin and slight cold and heat intolerance.  She has been active sports.  She sleeps 7-8 hours per night.  She is able to keep up with her classmates.  She denies tenderness of her thyroid gland.  She is also s/p osteomyelitis of her left jaw in March 1993.  her last menstrual period was November 3, 1993; her periods last from 1 to 5 days and she follows a consistent 28 day cycle.

Current medications: Doxycycline 250  mg BID since June 1993 and Ibuprofen as needed for jaw pain.

Physical exam:  
Height: 167.4 cm        Weight: 55.5 kg         BP: 90/62               
Pulse: 60               Pubic hair: Tanner V    
General: Quiet, cooperative, adolescent girl in no apparent distress. Skin: Two cheloid scars in her mid-sternal area.  Neck: Slightly, diffusely enlarged thyroid gland.  Lymph nodes were not palpable.  Chest: Clear.  Heart: Regular rate and rhythm without murmur.  Abdomen: Soft, nontender.  The remainder of her exam was normal.

Laboratory studies:  January 1993: thyroid function tests were normal and she had a normal ACTH stimulation test during her March hospitalization. 

On this visit: T4 6.6, T3 135, TBGI 1.01, TSH 1.0.  TPO Ab and TG Ab were elevated at 50.6 and 27.8 respectively.

Impression: Agatha is clinically and biochemically euthyroid.

Disposition: Return to Clinic in six months.
Thank you for letting us share in the care of Agatha.

Sincerely,


Rahul Hagen, MD           Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Brett Carter, M.D.          RE: Abraham Spinoza
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/10/74

Dear Dr. Carter:

     We had the pleasure of seeing Abraham in the Renal Transplant Program on Tuesday, July 26, 1994.  He has chronic renal failure secondary to .  He had a Renal Transplant on .
     Abraham has no complaints since his last visit.  He is status post closure of vesicocutaneous fistula in May 1994 and he has no problems post-operatively.

Current medications include:
     ROCALTROL .25 mcg   q.d.
     BACTRIM REGULAR STRENGTH 2 q.d.
     TUMS 1 with meals

Physical exam:
     BP 122/72   P 76   R    T    HT 166.7cm   WT 54.8kg   HC cm
     General: healthy.
     HEENT: moist mucous membranes without exudate.
     Chest: clear.
     Heart: regular rate and rhythm without murmurs, gallops, or rubs.
     Abdomen: soft, nontender and nondistended without any masses palpable.
     Extremities: warm and well profused without clubbing, cyanosis or edema.
     Neurological: nonfocal.
Urinalysis:
     SPEC GR UA  1.005           WBC ENZ UA  2+         
     PH UA       6               PROTEIN UA  2+         
     BLOOD UA    2+              GLUCOSE UA  2+    mg/dl
     WBC/HPF     50              RBC/HPF     2-5        
     WBC CAST    N               RBC CAST    N          
     GRAN CAST   N               WAXY CAST   N          
     SQUAM EPI   N               BACTERIA    N     


Laboratory studies at this time include:
          BUN 45    Cr 7.0    K 3.9    Ca 8.3    PO4 4.5
          Na 141    Cl 108    GLUC 94    CO2 23
          WBC 3.15    HGB 11.8    HCT 34.6    MCV 88.5    PLT 124

     Abraham has no new complaints since his last visit.  We will send a urine culture because of the high number of WBC on urinalysis.  However, it should be noted that he is colonized with pseudomonas and has not been treated for this in quite some time as it has not made any difference in his course.  We will begin histocompatability testing with possible donors soon.  His renal function is stable.

     We have asked Abraham to return in .

     Thank you for allowing us to share in Abraham's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Conrado Seinfeld, MD         Clotilde Goldwater, MD


CONTENT:
Brett Carter, M.D.          RE: Abraham Spinoza
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/10/74

Dear Dr. Carter:

     We had the pleasure of seeing Abraham in the Renal Transplant Program on Tuesday, December 20, 1994.  He has chronic renal failure secondary to obstructive uropathy.  
     Abraham has been well since his last visit six months ago.

Current medications include:
     ROCALTROL .25 mcg q.d.
     BACTRIM DS q.d.

Physical exam:
     BP 140/80   P 80    WT 58.2kg   
     General: appeared well.
     Skin: no rashes
     HEENT: normal. 
     Neck: supple with no masses, lymph nodes not palpable.
     Chest: clear
     Heart: two heart sounds.
     Abdomen: central scar Metrofanoff fistula epispadias.
     Genitourinary: Tanner 1
     Extremities: no edema

Urinalysis:
     SPEC GR UA  1.015           WBC ENZ UA  1+         
     PH UA       5               PROTEIN UA  3+         
     BLOOD UA    1+              GLUCOSE UA  N     mg/dl
     WBC/HPF     0               RBC/HPF     0          
     WBC CAST    0               RBC CAST    0          
     GRAN CAST   0               WAXY CAST   0          
     SQUAM EPI   0               BACTERIA    0     


Laboratory studies at this time include:
          CO 2         20   MEQ/L (L)   CA          8.0   MG/DL (L)
          CR          7.8   MG/DL (C)   GLU          92   MG/DL    
          PHOS        4.6   MG/DL (H)   BUN          77   MG/DL (C)
          NA          140  MMOL/L       K           3.6  MMOL/L    
          CL          106  MMOL/L       WBC        3.83   K/MM3 (L)
          RBC        3.55   M/MM3 (L)   HGB        10.7    G/DL (L)
          HCT.       31.9       % (L)   MCV        89.9      FL    
          MCH        30.3      PG       MCHC       33.7    G/DL    
          RDW        14.1       %       HDW        2.99    G/DL    
          PLT UNC     157   K/MM3       NEUT AB    2.25   K/MM3    
          LYMPH A    1.20   K/MM3       NEUT-A     58.6       %    
          LYMPH-A    31.4       %       MONO-A      5.9       %    
          EOS-A       1.1       %       BASO-A       .7       %    
          ATYP LY     2.2       %       


     Abraham has deteriorating renal function.  We will increase his Rocaltrol to 0.5 mcg qd to improve his metabolic control and check a PTH level at his next visit.

     We have asked Abraham to return in two months.

     Thank you for allowing us to share in Abraham's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Animal, M.D.          RE: Denise Meitner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/09/58

Dear Dr. Animal:

We had the pleasure of seeing Denise in the Endocrine Program on 12/3/92 for evaluation of estrogen replacement.

History: Denise is a 34 year old female with past medical history significant for Down Syndrome and bilateral ovarian dysgerminomas. She was referred  for evaluation of hormone replacement therapy. You have seen Denise approximately 3 times and state that she has lost bone mass stating that her left arm is larger than her right arm. Denise was concerned because she was never put on hormones after the ovaries were removed 15 years ago. Mom also claims that she has lost a lot of hair in the year and occasionally complains of pain in her right arm when she awakens in the morning but seems to improve later in the day.

Current medications: Levoxine.

Past medical history:  Denise was diagnosed with Down Syndrome at birth. She had a tonsillectomy  in 1960. She was diagnosed with ovarian disgerminoma in 1977 by her pediatrician Dr. Lee. It was found that she had an ovarian tumor. She was operated on at South Shore Hospital and received approximately six months of radiation. She was fine until the final year when it was noted that the other ovary had a mass. The operation was done at Children's Hospital and she had one year of chemotherapy. She was born at 40 weeks gestation at Quincy Hospital. She was 6 lbs. 6 oz. She stayed in the hospital one week because of jaundice which was treated with phototherapy. There was trouble with feeding due to a large tongue. She takes in a normal diet with a good intake of milk products. Her periods began at age 13 and had normal 28 day cycles that lasted 5-6 days. Her last period was when she was 19. She has never had a full pelvic exam.

Review of systems:  Skin is dry with psoriasis. She wears glasses. Her last eye exam was four to five years ago. No constipation or diarrhea. She is very anxious and she stutters.

Family history:  Mother is 58 and father is 57. She has three brothers that are 30, 29 and 22. There is no family history of Down syndrome or ovarian cancer. There  is a positive family history on the mother and father's side of vascular disease and the maternal  grandmother had lung cancer.

Physical exam:  
Height: 143.1 cm        Weight: 55.1 kg         BP: 110/68              
Breast: Tanner V        Pubic hair: Tanner V    
General:  Exam was difficult due to lack of cooperation.  Skin: Very dry. Evidence of psoriasis and hirsutism.   HEENT:  Normocephalic atraumic. Extraocular muscles intact. PERRL. No nasal discharge. No pharyngitis., no large tongue.   Neck: No thyromegaly or lymphadenopathy.   Chest: Clear to auscultation.   Heart: Regular rate and rhythm. 
Abdomen: Deferred.   Genitourinary: Unable to evaluate genitalia due to lack of cooperation.  Extremities: No clubbing, cyanosis or edema. Good distal pulses.   Neurological: Alert and oriented. Cranial nerves grossly intact.

Laboratory studies:  LH, FSH, estradiol, testosterone, DHEAS, T4, TBGI, Total T3 and TSH.

Impression: Denise is a 34 year old female status-post bilateral ovarian removal secondary to ovarian disgerminoma 15 years ago who presents at endocrine clinic for estrogen replacement. Studies ordered were a pelvic ultrasound.

Disposition: Follow-up on December 17 at Reproductive Endocrinology with Dr. Hammer.
Thank you for letting us share in the care of Denise.

Sincerely,


M Joan Hammer, MD         
Associate in Endocrinology   


Addendum: Donna Lombardi, MD
Medical Student-IV


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of evaluating Michel in the Renal Program on 11/3/94 for Alport's Syndrome.

     A good 6 months without significant symptomatology and now with clearing of his edema and more stable weight with only n intermittent need for furosemide.  His weight is stable at 105-110 pounds and surprisingly he has experienced an improvement in hearing and at present is not using his hearing aid although there is still a defect present.  Increased appetite and intake have resulted in stable and mildly elevated true weight. 

Of some concern is the fact that Michel has dropped out of school and is in a tutorial program for his G.E.D. and hopefully will continue to pursue this route.

Current medications include:
     LOPID 300 mg b.i.d.
     VASOTEC 7.5 mg q.d.
     FUROSEMIDE  20 mg p.r.n. swelling

Past medical history:
     Well known for his Alport's Syndrome.

Review of systems:
     No specific symptomatic change.

Physical exam:
     BP 120/50   P 75   HT 158.8cm   WT 51kg
     General: Short statured, well covered, well hydrated boy
     Skin: Intact, no evidence for striae
     HEENT: Normal fundi and lenses without evidence for cataracts or keratoconus
     Neck: Supple, no lymph nodes present
     Chest, heart and abdomen were within normal limits
     Genitourinary: Tanner 3-4

Urinalysis:
     SPEC GR UA  1.012           PH UA       7.5        
     PROTEIN UA  3+              BLOOD UA    3+         
     GLUCOSE UA  .25             WBC/HPF     2-4        
     RBC/HPF     8-10            GRAN CAST   fine  
     SQUAM EPI   occ             HYALINE CAST broad    
     EPITHELIAL CAST   1
   
Laboratory studies at this time include:
          ALB         2.8    G/DL (L)   ALKP        189     U/L    
          MG          2.1   MG/DL       CO 2         16   MEQ/L (L)
          CA          8.5   MG/DL       CRU        46.5   MG/DL    
          CR          3.3   MG/DL (H)   GLU          91   MG/DL    
          OSMO        294  MOSMKG       PHOS        5.2   MG/DL (H)
          TP          6.3   GM/DL       AST          19     U/L    
          BUN          31   MG/DL (H)   URIC        4.8   MG/DL    
          NA          139  MMOL/L       K           4.4  MMOL/L    
          CL          109  MMOL/L       PLT         270   K/MM3    
          WBC        6.22   K/MM3       RBC        3.91   M/MM3 (L)
          HGB        11.1    G/DL (L)   HCT.       32.2       % (L)
          MCV        82.4      FL       MCH        28.4      PG    
          MCHC       34.5    G/DL       RDW        13.5       %    
          HDW        2.92    G/DL       PLT UNC     270   K/MM3    
          NEUT         35       % (L)   LYMPH        57       % (H)
          MONOS         5       %       EOS           1       %    
          BASO          1       %       BAND          1       %    
          CELLS C     100               ABS NEU    2.24   K/MM3    
          ABS LYM    3.55   K/MM3       ABS EOS     .06   K/MM3    
          MORPH    NORMAL               U PROT      427   MG/DL (H)


     1)  Alport's syndrome  2)  Mild hearing deficit  3)  Acidosis  4)  Stable to mildly decreased renal insufficiency  5)  Significant proteinuria

Diagnostic studies were repeated with an elevated serum creatinine now at 3.3 mg% showing further steady decrease in glomerular clearance function.  Proteinuria has remained relatively stable with a urinary protein to creatinine ratio of 9.2.  

Michel should have his hearing rechecked and push to obtain new hearing aids so he can function fully.  Add sodium bicarbonate at 650 mg q.d. in an attempt to correct his acidosis and stabilize renal function and bony growth.  He should have his chemistries rechecked through Dr. Pulanski in 4 weeks and titrate sodium bicarbonate if necessary.

     We have asked Michel to return in six months.  Thank you for allowing us to share in Michel's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Mitsuko Khan, MBBS, FRACP
               Director, Clinical Services


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Program on 6/22/95 for Alports Syndrome.
     
Michel returns today to clinic for ongoing evaluation of his chronic  renal insufficiency.  Michel has a history of Alport's syndrome and  describes being well since his last visit but noting increasing  tiredness over the past few months.  Michel has been on escalating  doses of Rocaltrol and Tums due to hypocalcemia. 



His current medications include:
     Rocaltrol .50 mcq. q.d.
     Sodium Bicarbonate 10 grains q.i.d.
     Tums Ex 3 tablets t.i.d.
     Lopid 300 mgm. b.i.d.
     Vasotec 1.25 mgm. t.i.d.

Michel's physical exam was notable for:
     BP 125/56   P 69   R    T    HT 161.1cm   WT 56kg
     General: Notable for bilateral hearing  aids.  His oropharynx was clear. 
     Neck: Supple, he had no lymphadenopathy.
     Chest: Clear 
     Heart:   Regular rate and rhythm without murmur. 
     Abdomen: Soft and  nontender, without masses or audible bruits.
     Genitourinary: Tanner V.   
     Extremities:  Warm and well perfused without clubbing, cyanosis  or edema and his skin revealed no rashes. 
     Neurological: Nonfocal with the exception of hearing, which was not evaluated on this  visit.
A urinalysis revealed:
     SPEC GR UA  1.012           GLUCOSE UA  Neg.       
     PH UA       6.0             RBC/HPF     20-50      
     BLOOD UA    3+              WBC/HPF     Few        
     PROTEIN UA  4+    mg/dl     BACTERIA    Neg.  
     CASTS       Broad hyaline
Squam        Few plus oval fat bodies

Laboratory studies at this time include:


     Michel presents with worsening renal failure, as evidenced  by his increasing requirement for supplementation, his dropping  hematocrit and active sediment.  We will check lytes, BUN, creatinine,  magnesium and phosphorus today and adjust his medications according to  these results.  We discussed starting erythropoietin in the near future  for assistance with his anemia and in addition, we discussed the need  for transplantation in the future.  We will plan to see Michel in 1  month, and he will have labs drawn in 2 weeks.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him in One month.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Program on 7/19/95 for alpouts syndrome.
     Michel returns today to clinic for ongoing evaluation of his chronic  renal insufficiency.  Michel has a history of Alport's syndrome and  describes being well since his last visit but noting increasing  tiredness over the past few months.  Michel has been on escalating  doses of Rocaltrol and Tums due to hypocalcemia.

His current medications include:
     Rocaltrol 0.5 mcg. q.d.
     Sodium bicarbonate 10 gram (650 mg.)
     Tums Ex 3 tabs t.i.d.
     Lopid 300 mg. b.i.d.
     Vasotec 1.25 mg. t.i.d.

Michel's physical exam was notable for:
     BP 129/67   P 64   R 18   T    HT 161cm   WT 55.2kg
     General:  His physical exam today is notable for bilateral hearing aids.  His oropharynx was clear. 
     Skin: No rashes 
     Neck:  Supple,  no lymphadenopathy
     Chest: Clear
     Heart:  Regular rate and rhythm without murmur.  
     Abdomen: Soft and nontender, without masses or audible bruits.   
     Genitourinary: Tanner V. 
     Extremities:  Warm and well perfused without clubbing, cyanosis or edema 
     Neurological: Nonfocal with the exception of hearing, which was not evaluated on this  visit
A urinalysis revealed:
     SPEC GR UA  1.013           GLUCOSE UA  ++         
     PH UA       7.0             RBC/HPF     50-80      
     BLOOD UA    +               WBC/HPF     0          
     PROTEIN UA  +++             BACTERIA    Neg.  
     CASTS       0-1 epith cast/HPF
Squam       + fat globules oval fat bodies

Laboratory studies at this time include:
          CRU        62.7   MG/DL       U PROT      670   MG/DL (H)

     Michel presents with worsening renal failure, as evidenced  by his increasing requirement for supplementation, his dropping  hematocrit and active sediment.  We will check lytes, BUN, creatinine,  magnesium and phosphorus today and adjust his medications according to  these results.  We discussed starting erythropoietin in the near future  for assistance with his anemia and in addition, we discussed the need  for transplantation in the future.  We will plan to see Michel in 1  month, and he will have labs drawn in 2 weeks.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him in Four weeks.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of evaluating Michel in the Renal Program on 8/9/95 for .
     Michel returns after the creation of a  left arteriovenous fistula for dialysis access at the left wrist (ten days ago).  The fistula has healed well and has an exceptionally good  bruit and thrill.  There is pulsation above the elbow joint on moderate  occlusion of the vessels on that side showing excellent potential for  development.

His current medications include:
     LOPID  300 mg b.i.d. (1/2 x 600mg)
     VASOTEC 3.7 mg (2.5mg x 1/2 t.i.d.)
     ROCALTROL 0.5 mcg q.d
     SODIUM BICARBONATE  650 mg q.i.d.
     CALCIUM CARBONATE 750 mg (TUMS 4 t.i.d.)

Past medical history:
     Well known for Alport's syndrome with chronic renal  insufficiency progressing toward end-stage renal failure.  Review of  systems, asymptomatic on supplemental medications which are well tolerated.   Presently commencing on erythropoietin injections for anemia of chronic  renal failure.

Michel's physical exam was notable for:
     BP 125/85   P 76   R 16   T    HT 160cm   WT 54.5kg
     General Appearance: A well tanned short statured  boy.  
     HEENT:  Within normal limits.  No keratoconus or cataracts.  
     Neck:  Supple   
     Heart: Clinically clear.  S1, S2 readily audible without change in rate or rhythm but with a soft systolic bruit.   
     Abdomen: Non-tender.  
     Genitourinary: Tanner IV.  
     Extremities:  No edema  or rash.  
     Skin:  Intact.  
     Neurological: No focalizing signs.
A urinalysis revealed:
     SPEC GR UA  1.008           GLUCOSE UA  N          
     PH UA       7               RBC/HPF     10-12      
     BLOOD UA    1+              WBC/HPF     0-3   
     PROTEIN UA  2+         
     CASTS   rare broad fine granular

Laboratory studies at this time include:
          ALKP        149     U/L       HCT.       22.4       % (L)
          CO 2         22   MEQ/L       MCV        83.0      FL    
          CA          9.1   MG/DL       MCH        30.0      PG    
          CHOL        278   MG/DL       MCHC       36.0    G/DL    
          CR          8.2   MG/DL (C)   RDW        13.8       %    
          GLU         103   MG/DL       HDW        2.54    G/DL    
          FE           69  MCG/DL       PLT UNC     262   K/MM3    
          OSMO        313  MOSMKG (H)   NEUT         48       %    
          PHOS        5.0   MG/DL (H)   LYMPH        45       %    
          AST          18     U/L       MONOS         2       %    
          ALT           9     U/L       EOS           2       %    
          TRIG        211   MG/DL       ATYP LY       1       %    
          BUN          83   MG/DL (C)   BAND          2       %    
          NA          138  MMOL/L       CELLS C     100    
          K           5.6  MMOL/L (H)   ABS NEU    3.11   K/MM3    
          CL          103  MMOL/L       ABS LYM    2.80   K/MM3    
          HDLC       32.1   MG/DL       ABS EOS     .12   K/MM3    
          TIBC        163  MCG/DL (C)   ANISO        1+    
          WBC        6.22   K/MM3       MICRO        1+    
          RBC        2.70   M/MM3 (L)   POIK         1+    
          HGB         8.1    G/DL (L)   OVALO        1+    

     Michel shows stabilization of his falling renal function  with better control of acidosis and calcium on heavier supplementation.   His AV fistula is working well although still immature but showing  adequate flow and excellent venous dilatation.  He was counselled not to  have blood pressure or blood drawing from the left arm to allow the  fistula to adequately develop and to be fully protected.  

Starting EPO therapy, he will require supplemental iron since his iron  binding capacity is low and his serum iron at the low end of normal.   Iron stores would be expected to be on the low side.  Ferrous sulfate  350 mg orally twice a day will be added.   

The family will be coming for a family meeting on August 22 and at that  stage will transfer to the end-stage renal service for transplant  planning.  A large number of relatives at present are in consideration  for potential living related donation.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him in  August 22nd in in transplant clinic.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Kinze Brando, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, August 22, 1995.  He has chronic renal failure secondary to Alport's Syndrome.  
     Michel has been complaining of mild pain over the fistula on and off for the last few days, which has resolved spontaneously.  He had a fistula placed 8/1/95.

His current medications include:
     ROCALTROL 0.50 mcg q.d.
     EPOGEN 3000 units sc t.i.w.
     VASOTEC 1.25 mg t.i.d.
     SODIUM BICARBONATE 1 tablet t.i.d.
     TUMS EX 750 mg tablets, 4 tablets pc
     GEMFIBROZIL (Lopid) 300 mg b.i.d.

Michel's physical exam was notable for:
     BP 120/82   P 100   R 18    WT 55.1kg   
     General: in no distress.
     Skin: no rashes.
     HEENT: normal cranial atraumatic.
     Neck: supple, lymph nodes not palpable.
     Chest: clear to auscultation.
     Heart: no murmurs.
     Abdomen: soft, bowel sounds present. 
     Extremities: free range of motion.  The fistula examination revealed no tenderness or erythema or swelling and has good bruit.  
     Neurological: no meningeal signs.
A urinalysis revealed:
     SPEC GR UA  1.015           WBC/HPF     0          
     PH UA       7               SQUAM EPI   0          
     WBC ENZ UA  0               RBC CAST    0          
     BLOOD UA    2+              WBC CAST    0          
     PROTEIN UA  3+              GRAN CAST   0          
     GLUCOSE UA  0     mg/dl     WAXY CAST   0          
     RBC/HPF     5-10            BACTERIA    0     

Laboratory studies at this time include:
          ALB         2.2    G/DL (L)   MG          1.8   MG/DL    
          CO 2         23   MEQ/L       CA          8.2   MG/DL    
          CR          9.2   MG/DL (C)   GLU          95   MG/DL    
          PHOS        6.6   MG/DL (H)   TP          5.0   GM/DL (L)
          BUN          69   MG/DL (C)   NA          141  MMOL/L    
          K           5.6  MMOL/L (H)   CL          108  MMOL/L    
          WBC        5.72   K/MM3       RBC        2.88   M/MM3 (L)
          HGB         8.6    G/DL (L)   HCT.       25.1       % (L)
          MCV        87.0      FL       MCH        29.9      PG    
          MCHC       34.3    G/DL       RDW        18.6       % (H)
          HDW        3.35    G/DL (H)   PLT UNC     266   K/MM3    
          NEUT         47       %       LYMPH        43       %    
          MONOS         6       %       EOS           3       %    
          ATYP LY       1       %       CELLS C     100    
          ABS NEU    2.69   K/MM3       ABS LYM    2.46   K/MM3    
          ABS EOS     .17   K/MM3       ANISO        1+    
          MICRO        1+       


     Michel is a patient with chronic renal failure who has had a fistula placed just recently.  He has been stable.  His fistula seems to be working fine.  His low albumin is due to nephrosis.  We will do iron studies at his next visit.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him as scheduled.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, February 27, 1996.  He has chronic renal failure secondary to Alport's Syndrome.  He had a renal transplant on 2/8/96.

     Michel has been doing well since his discharge from the hospital on 2/23/96.  His only complaints are of some mild facial acne.  His blood pressures at home have been 130-140/80-90.  His intakes have been 2,500 - 3,500 cc's.  He noticed that there is scant drainage at JP site.

His current medications include:
     PREDNISONE 18 mg b.i.d.
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 350 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F
     MYLANTA 30 cc t.i.d.

Michel's physical exam was notable for:
     BP 130/88   P 76   R 18   HT 162.5cm   WT 56.5kg   
     General: Ken has had mild cushingoid features with mild facial acne. 
     HEENT: no evidence of infection or cataracts. 
     Chest: suture at site of CVL line in place.  Lungs clear with good aeration. 
     Heart: regular rhythm without murmur.
     Abdomen: biopsy site clear and healing well.  JP site with mild erythema and scant drainage and dressed.  Positive bowel sounds.  Soft non-tender without organomegaly and there was no graft tenderness.
     Extremities: no edema.

A urinalysis revealed:
     SPEC GR UA  1.018           WBC/HPF     0-2        
     PH UA       5               SQUAM EPI   0          
     WBC ENZ UA  0               RBC CAST    0          
     BLOOD UA    2+              WBC CAST    0          
     PROTEIN UA  1+              GRAN CAST   0          
     GLUCOSE UA  100   mg/dl     WAXY CAST   0          
     RBC/HPF     0               BACTERIA    0     

Laboratory studies at this time include:
          AST          10     U/L       MCHC       32.0    G/DL    
          K           4.5  MMOL/L       RDW        14.9       %    
          CA          8.9   MG/DL       HDW        3.24    G/DL (H)
          CR          1.2   MG/DL (H)   PLT UNC     373   K/MM3    
          CYA         439   MCG/L       NEUT AB   10.46   K/MM3    
          GLU         122   MG/DL (H)   LYMPH A    2.06   K/MM3    
          PHOS        1.2   MG/DL (C)   EOS ABS     .08   K/MM3    
          ALT          12     U/L       NEUT-A     78.1       % (H)
          BUN          36   MG/DL (H)   LYMPH-A    15.4       % (L)
          URIC        4.5   MG/DL       MONO-A      4.5       %    
          NA          142  MMOL/L       EOS-A        .6       %    
          CL          106  MMOL/L       BASO-A       .2       %    
          CO 2         26   MEQ/L       ATYP LY     1.2       %    
          BILI TO     0.9   MG/DL       LEFT SH  ABSENT    
          BILI DI     0.3   MG/DL       MORPH       YES    
          WBC       13.39   K/MM3 (H)   ANISO        1+    
          RBC        3.82   M/MM3 (L)   MACRO        1+    
          HGB        11.4    G/DL (L)   HYPO         1+    
          HCT.       35.6       % (L)   POIK         1+    
          MCV        93.3      FL       OVALO        1+    
          MCH        29.8      PG       TEAR       RARE (*)

     Michel is doing well post transplant.  His creatinine is slightly elevated but the CYA level is also too high.  We will lower the cyclosporine and recheck both labs later this week.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Friday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director of Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, March 5, 1996.  He has chronic renal failure secondary to Alport's Syndrome.  He had a renal transplant on 2/8/96.

     Kenny is doing well at the present time.  His abdominal pain has completely resolved.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 300 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F

Michel's physical exam was notable for:
     BP 124/82   P 80   R 12    HT 161.5cm   WT 57.2kg   
     General: comfortable, alert
     Skin: no lesions
     HEENT: eyes, nose and throat clear
     Neck: supple, no significant lymphadenopathy 
     Chest: clear to auscultation 
     Heart: S1 S2 normal, no murmur
     Abdomen: soft, no tenderness over the graft site  
     Extremities: no edema
     Neurological: no focal deficits or meningeal signs

A urinalysis revealed:
     SPEC GR UA  1.015           WBC/HPF     4-5        
     PH UA       5.0             SQUAM EPI   N          
     WBC ENZ UA  N               RBC CAST    N          
     BLOOD UA    trace           WBC CAST    N          
     PROTEIN UA  trace           GRAN CAST   N          
     GLUCOSE UA  trace mg/dl     WAXY CAST   N          
     RBC/HPF     0-2             BACTERIA    N     
     HYALINE CAST

Laboratory studies at this time include:
          AST           8     U/L       HCT.       36.2       %    
          K           5.1  MMOL/L (H)   MCV        90.8      FL    
          CA          9.7   MG/DL       MCH        29.6      PG    
          CR          1.4   MG/DL (H)   MCHC       32.6    G/DL    
          CYA         362   MCG/L       RDW        15.0       %    
          GLU          93   MG/DL       HDW        3.12    G/DL    
          PHOS        2.7   MG/DL       PLT UNC     200   K/MM3    
          ALT           7     U/L       NEUT AB    8.16   K/MM3    
          BUN          37   MG/DL (H)   LYMPH A    1.67   K/MM3    
          URIC        6.6   MG/DL (H)   EOS ABS     .18   K/MM3    
          NA          141  MMOL/L       NEUT-A     75.7       % (H)
          CL          106  MMOL/L       LYMPH-A    15.5       % (L)
          CO 2         25   MEQ/L       MONO-A      5.2       %    
          BILI TO     0.8   MG/DL       EOS-A       1.7       %    
          BILI DI     0.3   MG/DL       BASO-A       .6       %    
          WBC       10.78   K/MM3       ATYP LY     1.2       %    
          RBC        3.99   M/MM3 (L)   HYPO-A        +    
          HGB        11.8    G/DL (L)   LEFT SH  ABSENT    

     We have advised Kenny to stop the Almacone and continue Zantac.  We will start oral potassium phosphate in view of his low phosphorous on his last visit.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Tuesday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, March 12, 1996.  He has chronic renal failure secondary to Alport's Syndrome.  He had a renal transplant on 2/8/96.

     Kenny has no specific complaints except for increased facial acne.  His appetite has been good.  His output remains at 2-3 liters per day.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 275 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F
     KPHOS 2 tablets t.i.d.
     OXY 10 topical b.i.d.

Michel's physical exam was notable for:
     BP 124/78   P 100   R 16    HT 161.7cm   WT 59.7kg   
     General: alert and oriented in no acute distress.
     Skin: diffuse facial acne, otherwise there are no significant other lesions.
     HEENT: benign, no thrush or exudate.  
     Neck: no JVD or lymph nodes palpable. 
     Chest: clear.
     Heart: S1 S2 normal, no murmurs, gallops, or rubs.
     Abdomen: positive bowel sounds, non-tender.  
     Genitourinary:
     Extremities: no cyanosis, clubbing or edema.

A urinalysis revealed:
     SPEC GR UA  1.016           WBC/HPF     0-1        
     PH UA       5.0             SQUAM EPI   0-2        
     WBC ENZ UA  N               RBC CAST    N          
     BLOOD UA    trace           WBC CAST    N          
     PROTEIN UA  trace           GRAN CAST   N          
     GLUCOSE UA  N     mg/dl     WAXY CAST   N          
     RBC/HPF     0-1             BACTERIA    N     
     0-1 cast

Laboratory studies at this time include:
          AST          11     U/L       HCT.       33.2       % (L)
          K           4.0  MMOL/L       MCV        89.7      FL    
          CA          8.5   MG/DL       MCH        29.2      PG    
          CR          1.2   MG/DL (H)   MCHC       32.5    G/DL    
          CYA         266   MCG/L       RDW        14.8       %    
          GLU          87   MG/DL       HDW        3.25    G/DL (H)
          PHOS        2.6   MG/DL (L)   PLT UNC     203   K/MM3    
          ALT           7     U/L       NEUT AB    8.19   K/MM3    
          BUN          40   MG/DL (H)   LYMPH A    2.01   K/MM3    
          URIC        5.5   MG/DL       EOS ABS     .16   K/MM3    
          NA          142  MMOL/L       NEUT-A     73.3       % (H)
          CL          108  MMOL/L       LYMPH-A    18.0       % (L)
          CO 2         25   MEQ/L       MONO-A      5.4       %    
          BILI TO     0.7   MG/DL       EOS-A       1.4       %    
          BILI DI     0.2   MG/DL       BASO-A       .6       %    
          WBC       11.17   K/MM3       ATYP LY     1.4       %    
          RBC        3.70   M/MM3 (L)   HYPO-A        +    
          HGB        10.8    G/DL (L)   LEFT SH  ABSENT    

     Kenny has been doing extremely well.  His renal function has been stable and his phosphorus level is satisfactory with K-Phos supplement.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Friday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Friday, March 8, 1996.  He has chronic renal failure secondary to Alport's Syndrome.  He had a renal transplant on 2/8/96.

     Kenny comes to the renal transplant program today for follow up.  His mother states that he had a lower output of 2 liters yesterday as opposed to his usual urine output of 3 liters.  He is on a 3 liter minimum fluid goal at present.  He has some mild abdominal pain and mild headache yesterday which spontaneously resolved.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 300 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F

Michel's physical exam was notable for:
     BP 131/80   P 82   R 12    WT 53.2kg  
     General: comfortable, alert
     Skin: mild facial acne
     HEENT: eyes, nose and throat clear
     Neck: supple, no significant lymphadenopathy 
     Chest: clear to auscultation 
     Heart: S1 S2 normal, no murmur
     Abdomen: soft, no tenderness over the graft site, bowel sounds well heard  
     Genitourinary:
     Extremities: no edema
     Neurological: no focal deficits or meningeal signs

A urinalysis revealed:
     SPEC GR UA  1.020           WBC/HPF     N          
     PH UA       5.0             SQUAM EPI   N          
     WBC ENZ UA  N               RBC CAST    N          
     BLOOD UA    N               WBC CAST    N          
     PROTEIN UA  N               GRAN CAST   N          
     GLUCOSE UA  N     mg/dl     WAXY CAST   N          
     RBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          K           4.2  MMOL/L       MCHC       32.3    G/DL    
          CA          9.5   MG/DL       RDW        14.8       %    
          CR          1.1   MG/DL (H)   HDW        3.22    G/DL (H)
          CYA         308   MCG/L       PLT UNC     177   K/MM3    
          GLU          79   MG/DL       NEUT AB    8.00   K/MM3    
          PHOS        2.8   MG/DL       LYMPH A    2.15   K/MM3    
          BUN          31   MG/DL (H)   EOS ABS     .17   K/MM3    
          NA          140  MMOL/L       NEUT-A     71.0       % (H)
          CL          105  MMOL/L       LYMPH-A    19.1       % (L)
          CO 2         25   MEQ/L       MONO-A      6.3       %    
          WBC       11.27   K/MM3       EOS-A       1.5       %    
          RBC        3.86   M/MM3 (L)   BASO-A       .6       %    
          HGB        11.4    G/DL (L)   ATYP LY     1.5       %    
          HCT.       35.3       % (L)   HYPO-A        +    
          MCV        91.4      FL       LEFT SH  ABSENT    
          MCH        29.5      PG       

     Kenny is still doing very well one month post transplant.  We had advised him to cut down his fluid minimum to 2.5 liter per day.  We will reduce his CYA study drug dose to 275 mg bid.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Tuesday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, March 19, 1996.  He has chronic renal failure secondary to alport's syndrome.  He had a renal transplant on 2/8/96.

     Kenny is doing well.  Since he was last seen four days ago, he had some transient right knee pain which spontaneously subsided.  His facial acne is improving with topical treatment.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 275 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F
     KPHOS 2 tablets t.i.d.
     OXY 10 cream to face b.i.d.

Michel's physical exam was notable for:
     BP 134/88   P 84   R 12   HT 161.3cm   WT 60.9kg   
     General: comfortable, alert.
     Skin: facial acne.
     HEENT: eyes, nose and throat clear.  
     Neck: supple, no significant lymphadenopathy. 
     Chest: clear to auscultation.
     Heart: S1 S2 normal, no murmurs.
     Abdomen: soft, no tenderness over the graft site.  
     Genitourinary:
     Extremities: both knees show full range of motion.  There is no swelling or tenderness evident.
     Neurological: no focal deficits or meningeal signs.

A urinalysis revealed:
     SPEC GR UA  1.015           WBC/HPF     0-1        
     PH UA       5.0             SQUAM EPI   N          
     WBC ENZ UA  N               RBC CAST    occ.       
     BLOOD UA    N               WBC CAST    occ.       
     PROTEIN UA  N               GRAN CAST   occ.       
     GLUCOSE UA  rare  mg/dl     WAXY CAST   occ.       
     RBC/HPF     0-1             BACTERIA    N     

Laboratory studies at this time include:
          AST          12     U/L       HCT.       32.8       % (L)
          K           4.3  MMOL/L       MCV        87.1      FL    
          CA          8.9   MG/DL       MCH        29.7      PG    
          CR          1.3   MG/DL (H)   MCHC       34.1    G/DL    
          CYA         264   MCG/L       RDW        14.9       %    
          GLU         113   MG/DL       HDW        3.16    G/DL    
          PHOS        3.4   MG/DL       PLT UNC     261   K/MM3    
          ALT           9     U/L       NEUT AB    7.80   K/MM3    
          BUN          26   MG/DL (H)   LYMPH A    1.71   K/MM3    
          URIC        6.2   MG/DL (H)   EOS ABS     .07   K/MM3    
          NA          140  MMOL/L       NEUT-A     74.8       % (H)
          CL          104  MMOL/L       LYMPH-A    16.4       % (L)
          CO 2         28   MEQ/L       MONO-A      5.6       %    
          BILI TO     0.8   MG/DL       EOS-A        .7       %    
          BILI DI     0.2   MG/DL       BASO-A       .6       %    
          WBC       10.42   K/MM3       ATYP LY     1.9       %    
          RBC        3.77   M/MM3 (L)   LEFT SH  ABSENT    
          HGB        11.2    G/DL (L)   

     Kenny continues to do well six weeks post kidney transplant.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Tuesday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Friday, March 22, 1996.  He has chronic renal failure secondary to alport's syndrome.  He had a renal transplant on 2/8/96.
     Kenny comes to the renal transplant program today for the second time this week status post renal transplant seven weeks.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 275 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F
     KPHOS 2 tablets t.i.d.
     OXY 10 cream to face b.i.d.

Michel's physical exam was notable for:
     BP 138/73   P 89   R    T    HT cm   WT 60.8kg   HC cm
     General: interactive, olive skin boy, cushingoid.
     Skin: facial acne.
     HEENT: normal
     Neck: supple, no significant lymphadenopathy. 
     Chest: clear 
     Heart: S1 S2 normal
     Abdomen: non-tender graft site, no bruits.  He has a fistula on his left arm which had a good thrill  
     Genitourinary:
     Extremities: both knees show full range of motion.  There is no swelling or tenderness evident.
     Neurological: no focal deficits or meningeal signs.
A urinalysis revealed:
     SPEC GR UA  1.015           WBC/HPF     0-2        
     PH UA       6               SQUAM EPI   5-10       
     WBC ENZ UA  N               RBC CAST    0          
     BLOOD UA    N               WBC CAST    0          
     PROTEIN UA  trace           GRAN CAST   0          
     GLUCOSE UA  rare  mg/dl     WAXY CAST   0          
     RBC/HPF     2-4             BACTERIA    N     
     numerous hyaline 10-15 small hyaline casts
5 small fine granular
5-10 bladder cells

Laboratory studies at this time include:
          K           4.2  MMOL/L       MCH        29.7      PG    
          CA          8.8   MG/DL       MCHC       33.8    G/DL    
          CR          1.2   MG/DL (H)   RDW        14.9       %    
          CYA         423   MCG/L       HDW        3.27    G/DL (H)
          GLU          89   MG/DL       PLT UNC     288   K/MM3    
          PHOS        3.0   MG/DL       NEUT AB    8.47   K/MM3    
          BUN          28   MG/DL (H)   LYMPH A    2.18   K/MM3    
          NA          140  MMOL/L       EOS ABS     .07   K/MM3    
          CL          104  MMOL/L       NEUT-A     72.8       % (H)
          CO 2         27   MEQ/L       LYMPH-A    18.8       % (L)
          WBC       11.63   K/MM3       MONO-A      5.8       %    
          RBC        3.80   M/MM3 (L)   EOS-A        .6       %    
          HGB        11.3    G/DL (L)   BASO-A       .4       %    
          HCT.       33.4       % (L)   ATYP LY     1.6       %    
          MCV        87.9      FL       LEFT SH  ABSENT    

     Kenny is doing well.  His urinalysis today appears to be somewhat changed from previous.  This may be explained by the change in his kidney function as demonstrated by his urine output which has been diminishing over the course of the seven week period.  It is still within the normal range taking into account sensible losses for this 60 kilo boy.  We will review the sediment again on Tuesday.  Laboratory values from today showed the white count 11, hematocrit 33 and a platelet count of 288.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him in Tuesday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


CONTENT:
Mitsuko Pulanski, M.D.          RE: Michel Evans
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/78

Dear Dr. Pulanski:

     We had the pleasure of seeing Michel in the Renal Transplant Program on Tuesday, March 26, 1996.  He has chronic renal failure secondary to alport's syndrome.  He had a renal transplant on 2/8/96.

     Kenny returns to the renal transplant program for routine follow up.  Since his last visit Kenny has been complaining of slowly increasing blood pressures into the 130's/90's with increase in weight as well.  Mom has also noted that he has had facial swelling and lip swelling especially in the morning which generally resolves by the afternoon.  Kenny has also had some difficulty with epigastric pain and nausea lasting transiently after brief spurts of running.  Kenny has none of this pain with any other form of exercise and has had no vomiting, diarrhea, constipation or any other abdominal symptoms during the day.  He has markedly increase his intake of cheese.

His current medications include:
     IMURAN 100 mg q.d.
     ZANTAC 150 mg b.i.d.
     PREDNISONE 12 mg b.i.d.
     PROCARDIA XL 30 mg b.i.d.
     CYCLOSPORINE STUDY #073-C 275 mg b.i.d.
     BACTRIM DS (Trimethoprim 160 mg, Sulfamethoxazole 800 mg) M-W-F
     KPHOS NEUT 2 tablets t.i.d.
     OXY 10 cream to face b.i.d.

Michel's physical exam was notable for:
     BP 130/100   P 80   HT 162.2cm   WT 62.8kg 
     General: well developed, well nourished male in no apparent distress.
     Skin: without rash
     HEENT: within normal limits with no oral lesions.
     Neck: supple without masses
     Chest: clear to auscultation bilaterally with no wheezes, rales or rhonchi
     Heart: regular rate and rhythm with no murmur 
     Abdomen: soft, non-distended, non-tender with well healed surgical scars from transplant and nephrectomy, bowel sounds were present 
     Extremities: warm and well perfused with no edema except in face
     Neurological: grossly intact.

A urinalysis revealed:
     SPEC GR UA  1.015           WBC/HPF     0          
     PH UA       5               SQUAM EPI   0          
     WBC ENZ UA  N               RBC CAST    0          
     BLOOD UA    N               WBC CAST    0          
     PROTEIN UA  trace           GRAN CAST   0          
     GLUCOSE UA  N     mg/dl     WAXY CAST   0          
     RBC/HPF     occ             BACTERIA    N     

Laboratory studies at this time include:
          K           4.3  MMOL/L       MCH        30.5      PG    
          CA          9.5   MG/DL       MCHC       34.9    G/DL    
          CR          1.4   MG/DL (H)   RDW        14.8       %    
          CYA         342   MCG/L       HDW        3.16    G/DL    
          GLU         126   MG/DL (H)   PLT UNC     262   K/MM3    
          PHOS        3.0   MG/DL       NEUT AB    8.39   K/MM3    
          BUN          29   MG/DL (H)   LYMPH A    1.88   K/MM3    
          NA          141  MMOL/L       EOS ABS     .11   K/MM3    
          CL          105  MMOL/L       NEUT-A     75.7       % (H)
          CO 2         25   MEQ/L       LYMPH-A    17.0       % (L)
          WBC       11.08   K/MM3       MONO-A      4.9       %    
          RBC        3.70   M/MM3 (L)   EOS-A       1.0       %    
          HGB        11.3    G/DL (L)   BASO-A       .4       %    
          HCT.       32.4       % (L)   ATYP LY     1.1       %    
          MCV        87.5      FL       LEFT SH  ABSENT    

     Kenny appears to be doing well except for probable fluid retention and increased blood pressure over the last week.  We will start Lasix 40 mg po qd and his fluid and blood pressure status will be re-assessed on Friday.  We will lower his cyclosporine to 250 mg bid.

     Thank you for the opportunity to participate in Michel's care.  We would like to review him on Friday.  In the interim, we would be happy to discuss or review Michel should you or the family wish it.

               Sincerely,


               Kinze Brando, MD
               Assistant in Nephrology


