# BIGPATS_DOC_STORE

CONTENT:
Fozzie Steinbeck, M.D.          RE: Leontyne Brisbane
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/11/92

Dear Dr. Steinbeck:

     We had the pleasure of seeing Leontyne in the Renal Program on 5/9/96 for ureteral reflux.
     Leontyne has done well since her last visit here.  She has had  no documented urinary tract infections.  She continues on Furadantin at  a dose that her mother describes as "one-quarter teaspoon" at bedtime  each night.  She seems to be growing and developing well.  She had a  VCUG performed at the New England Medical Center last month.  That  showed grade II reflux on the right side with no reflux on the left  side.  The summation of her bladder studies, therefore, is as follows:   

03/93: MGH, VCUG: Grade III, bilateral. 
03/94: CH, RNC: Grade I, left.  
04/94: CH, VCUG: Grade II, bilateral. 
04/95: CH, RNC: Grade I, left. 
04/96: NEMC, VCUG: Grade II, right.

Her current medications include:
     FURADANTIN 25 mg q.h.s.


Leontyne's physical exam was notable for:
     BP 103/66   P 109   R 12   T    HT 90.9cm   WT 13.6kg
     Appears well and in no distress.  
     HEENT:  negative; fundi are benign, no significant adenopathy.  
     Chest: clear to  auscultation, no rales or wheezes.  
     COR: S1 normal, S2 split, no S3, S4  or murmur.  
     Abdomen: soft and flat, no masses, nontender or  organomegaly. 
     Extremities: no edema.
A urinalysis revealed:
     SPEC GR UA  1.010           WBC/HPF     0-1        
     PH UA       8               RBC CAST    none       
     WBC ENZ UA  neg             WBC CAST    none       
     BLOOD UA    neg             GRAN CAST   none       
     PROTEIN UA  neg             WAXY CAST   none  
     GLUCOSE UA  neg             BACTERIA    neg   
     RBC/HPF     0          
     URINE CULTURE:  less than 1,000

Laboratory studies at this time include:


     Overall, Leontyne seems to be doing quite well.  She continues to have  grade II reflux on the right, but the left reflux seems to have cleared.   Thus, she is following the usual course with gradual lessening of the  reflux.  I reiterated to her mother that she must continue on the  antibiotics until the reflux is entirely gone.  I could not predict, of  course, when that would happen.  She seems to be tolerating the  Furadantin, but the dose does seem to be somewhat low.  However, even at  that level, if it is working, I don't see any reason to change it.  I  would evaluate her again in one year.  Routinely, we should get a  radionuclide cystogram.  Her cystograms have seemed to underestimate the  amount of reflux.  Nonetheless, as she gets older, they may be  technically more easy to do, and I would continue to try to get a  radionuclide study rather than a regular VCUG because of the decreased  amount of radiation exposure.  I think we may also want to get a DMSA  scan at the next visit also, to see if there is any sign of renal  scarring.  Certainly her blood pressure and urinalysis would not suggest  that, but remember that there was a previous ultrasound that suggested a  radiolucent area in the right upper pole.  Alternatively, of course, we  could just repeat the ultrasound, but I think if there is any  abnormality go with the DMSA and probably it is better just to do that  study.  In any case, I would be happy to discuss this prior to the next  visit.

     Thank you for the opportunity to participate in Leontyne's care.  We would like to review her in 1 year with RNC.  In the interim, we would be happy to discuss or review Leontyne should you or the family wish it.

               Sincerely,


               Clotilde Goldwater, MD
               Director, Pediatric Nephrology


CONTENT:
Saurabh Montague, M.D.          RE: Rich Metz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/03/79

Dear Dr. Montague:

We had the pleasure of seeing Rich in the Endocrine Program on 3/22/94 for follow up of his pubertal development.

History: Since last seen on March 15, 1993 Rich has been well.  neither he nor his father noted marked changes in his height or his pubertal development.  Rich thinks he did grow more pubic hair and grew some in height.  He claims to be socially well adjusted and not concerned about his size or his pubertal status.  Review of systems was negative.  HE did have a few pimples over the face.  He does not use a deodorant.  He has been more physically active.

Current medications: None.

Physical exam:  
Height: 151.5 cm        Weight: 39.2 kg         Pubic hair: Tanner II   
R. testis: 4.0 x 2.0 cm L. testis: 4.0 x 2.0 cm Phallus length: 6.0 cm  
Mid-shaft diam.: 2.0 cm 
Rich was healthy looking.
Skin: Normal texture, no acne.  No axillary hair.
HEENT: Negative.
Neck: Thyroid not enlarged.  No lymphadenopathy.
Chest: Clear lungs.
Heart: Normal S1 and S2, no murmurs.
Abdomen: Soft, not tender, no hepatosplenomegaly.
Extremities: Normal.
Neurological: Non-focal.

Impression: Constitutional delay in growth and pubertal development.  Rich has had further evidence of pubertal development over the past year albeit not a very dramatic one.

Disposition: Rich was reassured that his growth and development is normal.
Thank you for letting us share in the care of Rich.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Saurabh Montague, M.D.          RE: Rich Metz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/03/79

Dear Dr. Montague:

We had the pleasure of seeing Rich in the Endocrine Program on 3/15/93 for evaluation of short stature.

History: Rich is a 13 and 7/12 year old white male referred for evaluation of short stature. Rich was the full term product of a c-section for prolonged labor. He was well throughout childhood with no major diseases and no hospitalizations. He comes from a family of "short adults". Mother is 5'2" by report. Father is 5'5.5" by report. He has no siblings. He has one older cousin who was short during early teen years (almost 20 years ago), who was treated with ? testosterone. Father reports going through puberty at approximately 13-13 1/2. 

Rich was previously growing on the 10th percentile till approximately age 12 when he fell to the 5th percentile. He reports having mild body odor during the past year, occasionally using deodorant. He has no axillary hair and occasional pimples on his face. He has noticed a small of amount of light facial hair on his upper lip only. He has 1-2 light pubic hairs. He reports his genital size to be behind that of his peers in PE. He is in the 7th grade and is doing fine. He has no headaches, and no symptoms of thyroid dysfunction (heat or cold intolerance, change in appetite, concentration etc.). TFTs and Somatomedin-C were normal from 10/91.

Current medications: None. No allergies.

Past medical history:  Benign.

Review of systems:  He has occasional facial pimples. No blurry or double vision. No palpitations. No constipation or diarrhea. No cold or heat intolerance.

Family history:  Rich is an only child with no known endocrinologic disorders running in his family. Mother's height measured 62 1/10 inches. Father's measured height 64 7/10 inches.

Physical exam:  
Height: 146.8 cm        Weight: 40.5 kg         BP: 105/60              
Pulse: 72               Arm span: 56            Pubic hair: Tanner I    
R. testis: 3.5 x 2 cm   L. testis: 3.5 x 2 cm   
Phallus length: 6 cm    Mid-shaft diam.: 1.5 cm 
General:  A well appearing boy who looks younger than his stated age.  Skin: 1-2 small resolved acne form lesions on face and back.  HEENT: PERRL, DC, EOMs intact. Fields full, no nystagmus. Fundi normal with disc margins sharp. Oropharynx clear.  Neck: No nodes. Thyroid not palpable.  Chest: Clear.  Heart: Normal S1, S2. No murmurs, rubs or gallops.  Abdomen: Soft. No hepatosplenomegaly.  Genitourinary: No pubic hair visible.  Extremities: Normal.  Neurological: DTRs 2+ throughout.

Laboratory studies:  10/91 Somatomedin-C 198 (158-222), TSH 1.78 (.3-4.80), T3U 44.6 (35-45), T4 8.2 (4.5-12), T4 index 3.7 (1.6-5.4).  Bone age on 03/15/93 was12 years 6 months.

Impression: There is no evidence of his short stature being due to an endocrine problem such as growth hormone deficiency or hypothyroidism. Bone age today and physical exam along with parental heights suggest that he has familial short stature with slight constitutional delay. With his bone age as above, his predicted adult height is 5'6"-5'8". This is taller than his father and a height with which Rich seems to be very satisfied. Given that his bone age is within a year of his chronological age,  we would not recommend accelerating his puberty with testosterone. Also, his testicular size at this time suggests that he has begun to enter puberty and should be going into his growth spurt in the next six to twelve months.

Disposition: Follow-up in one year. Thank you for letting us share in the care of Rich.

Sincerely,



Sharon Housman, MD                     Kendell Simpson, MD             
Medical Student, HMS IV                Clinical Director


CONTENT:
Fai Pluto, M.D.          RE: Edith Meiggs
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/12/82

Dear Dr. Pluto:

We had the pleasure of seeing Edith in the Endocrine Program on 4/29/39 for short stature.

History: Edith is a 10 and 10/12 year girl with a long standing history of being the shortest in her class but it was recently noted that she had fallen off her growth curve.  Prior to her referral, she had a bone age done at Children's Hospital which was 10 at the time of chronologic age of 10 and 9/12.  She is an otherwise healthy active fifth grader.  She is an A- to B+ student and is currently active in three sports. 

Current medications: None, no known drug allergies.

Past medical history:  Edith was the product of a full term spontaneous vaginal delivery of an uncomplicated pregnancy.  Birth weight was 9 lbs 4 oz.  Growth and development have been normal; she walked at 11 months.  She has never been a big eater, but does seem to eat a balanced diet.  No infectious diseases other than chicken pox.  Immunizations are up to date.  She has never been hospitalized or had any serious injuries or operations.

Review of systems:  Negative in detail.

Family history:  No cancer, diabetes or thyroid disease.  Hypercholestolemia in her 64 year old maternal grandmother and hypertension in her paternal grandfather.  Mother's height is 5'3", onset of menses at age 14.  Father's height is 5'10".  Mother has sisters who were  5'5" and  5'9".  Father has brothers who are 5'10 and greater in height, some being >6'.  Edith has one brother, age 6 and is at about the 50th percentile for height.

Physical exam:  
Height: 130.3 cm        Weight: 26 kg           BP: 80/50               
Pulse: 72               U/L ratio:  0.86        Arm span: 128           
Breast: Tanner I        Pubic hair: Tanner I    
General: Pleasant, polite, petite, cooperative girl in no distress.  Skin: Scattered small moles but they do not meet the criteria for displasitc nevi.  HEENT: PERRL, EOMI.  Red reflex bilaterally.  Neck: Supple, normal width and no thyromegaly.    Chest: Clear to auscultation and percussion.  Nipples are slightly widely spaced.  There is no breast development.  Heart: Regular rate and rhythm, normal S1, S2,  I/VI systolic murmur.  Abdomen: Soft, flat, nontender.  No hepatosplenomegaly, no organomegaly.  Genitourinary: There is no estrogen effect noted.  There is no clitoromegaly.  Extremities: No clubbing, cyanosis or edema, no tremor.  Neurological: Normal, motor 5/5 and symmetric.  Reflexes 2+ and symmetric, no tremor.  Light tough is intact throughout.

Laboratory studies:  Bone age 10 at 10 and 9/12 chronological age.

Impression: Edith is a 10 10/12 year old girl who appears to have recently fallen off an already petite growth chart.  Of most concern however, is her recent decline in growth velocity over the past 1 to 2 years.  Associated with this, her weight has continued to increase though she remains slightly underweight  but less than 15% underweight.  Because of her recent change in growth velocity we feel that it is important to rule out medical causes and will check her thyroid functions tests today as well as an antiendomysial antibody, bicarb, creatinine, CBC and Somatomedin-C C.  It is also possible that she may be a Turner mosaic and a karyotype was also requested.

Disposition: Return to the Endocrine Clinic in three months.  If all her test are normal and her growth resumes at its prior velocity, Edith may opt to continue follow-up with you.  Parents are aware that they will be changing insurance soon and not sure if they will continue to follow-up at Children's.  We encouraged them to obtain a copy of Edith's medical chart should they seek care elsewhere, so that these tests to not have to be repeated.

Laboratory studies pending at this time include: Chem 7, CBC, antiendomysial antibodies, Somatomedin C, TSH, T4 and karyotype.
Thank you for letting us share in the care of Edith.

Sincerely,


Ariel Adler, MD           
Attending in Endocrinology   


Addendum: Cecilia Larson, MD
Fellow in Endocrinology


CONTENT:
Mitsuko Piggy, M.D.          RE: Art Dandridge
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/10/88

Dear Dr. Piggy:

We had the pleasure of seeing Art in the Endocrine Program on 12/22/95 for .

History: I had the pleasure of meeting the Dandridges and Art on 12/22/94 for evaluation of possible hypoglycemia.   

As you know, this now 6 and 6/12 year old boy was evaluated here initially a year and a half ago for the same problem.   He has continued to have some difficulties and much is related to his difficulties with eating.  He is now in kindergarten and three days a week he has school from 11:45 to 3:15 during which time he usually has very little difficulty.  Sometimes having eaten an excellent lunch at 11:15 he will feel a little bit agitated, may have somewhat glassy eyes and respond well to being given something to eat around 1:30 pm.   Two days a week he has more difficulty because he goes from an extended day program from 9:00 am to 11:15 am directly to his kindergarten program and is supposed to eat his lunch at extended day at 11:15 am.   Mom packs him a lunch quite similar to that which she normally gives him at home:  some pasta, usually sprinkled with cheese.  Because he is often eating a snack in "extended day" in the morning very close to the lunch time and because he often prefers cookies and chips that he obtains at the snack time, he is usually not hungry for lunch and often doesn't eat it at all.  By 2-2:30 in the afternoon at kindergarten he is quite symptomatic and responds quickly to being fed.   He has never had any seizures.   He has never been faint. He does at times get sweaty.   

Although he is somewhat difficult to awaken in the morning, he is not described as glassy eyed, ketone-smelling or by any definition hypoglycemic in terms of symptoms on fasting.   He has not had blood sugars measured during any of these episodes nor has he had urine ketone testing.  He has been remarkably well so there has been very little opportunity to assess how he would do the day after a significant decrease in intake secondary to illness.  Most young children with "Ketotic Hypoglycemia" tend to have their most problematic symptoms under those conditions.   

There is some family history of reported hypoglycemia in one grandfather and a somewhat distant history of late onset diabetes in father's side.

Physical exam:  

On physical today he is clearly growing and gaining beautifully to a present 119.1 cm, 24.5 kg, blood pressure 99/58.  He looks generally quite well and has no thyromegaly.  Dentition is normal and he has no hepatosplenomegaly or abdominal masses.

Impression: I think it is necessary to find out a bit more what is going on and why he is having what appears to be post-prandial hypoglycemia.   I have instructed the parents in the use of the blood glucose testing machine (a One Touch "Basic") and to obtain urine for ketones using "chem strip K" testing strips on any occasion when he appears to be low.  Mother is going to maintain a diary to fax results to me and communicate with me about this so that we can decide whether further testing needs to be done.   He may require an overnight fasting test at the Ambulatory Test Center at Children's or we may do a mixed meal challenge or even a 6 hour oral glucose tolerance test.   

In the meantime, a concerted effort is going to be made to try to make sure that he has a lunch that contains both complex carbohydrates and protein.   

As further details become available I will get back to you.

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

Sincerely,


Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Mitsuko Piggy, M.D.          RE: Art Dandridge
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/10/88

Dear Dr. Piggy:

We had the pleasure of seeing Art in the Endocrine Program on 5/6/93 for rule out hypoglycemia.

History: Art is a 4 and 11/12 year old male referred for evaluation of possible hypoglycemia. In September of last year he was noted to become hyperactive, agitated and subsequently diaphoretic approximately 3-4 hours after meals. These symptoms were also noted in the morning upon awakening and following exercise. Mother notes that improvement was noted within 20-30 minutes of ingestion of food. Since then, his diet has been adjusted to include snacks between each meal and a snack at bedtime. On this regime he has had noticeable improvement in his symptoms. The only documented glucose is one of 64 that was done on April 2,1 993 which was approximately three hours after Art had breakfast.

Current medications: Vitamins.

Past medical history:  Pregnancy term. Delivery  was Caesarean section for indication of failure to progress and prolonged rupture of membranes. Art was hospitalized in the neonatal intensive unit for seven days with presumed infection. He was breast fed for six months exclusively then subsequently with formulas. He had eczema until age 2 1/2 but this has resolved spontaneously.

Review of systems:  Negative.

Family history:  Unremarkable. Mother's height is 5'1". Father's height is 5'11".

Physical exam:  
Height: 107 cm          Weight: 19.9 kg         BP: 112/80              
Pulse: 100              R. testis: 1.5 x 1 cm   L. testis: 2 x 1 cm     
Phallus length: 4.5 cm  
General: Art was a well appearing young male who was comfortable.  Skin: Normal.  HEENT: Normal.  Neck: No thyromegaly or masses.  Chest: Lungs clear.  Heart: Normal.  Abdomen: Soft, nontender. There was no organomegaly.  Genitourinary: Normal.  Extremities:  No edema.  Neurological:  Grossly normal.

Laboratory studies:  April 2, 1993: Glucose 64

Impression: It is difficult to be sure if Art is hypoglycemic. We would ideally need documentation of his glucoses  at the time of symptoms.  If in fact he is hypoglycemic then knowing his insulin levels and the status of his urinary and blood ketones would also provide valuable information. If hypoglycemia is occurring the most likely differential diagnosis is that of idiopathic ketotic hypoglycemia which is a benign condition which resolves spontaneously. His symptoms are unlikely secondary to hyperinsulinism given his normal growth and unlikely to be secondary to glycogen storage diseases given the fact that he is only now presenting at age 4.

Disposition: Mother was given lab slips for measurement of blood glucose an urine dip sticks for ketones. In the event he has symptoms she has been instructed to take him to the nearest medical facility to have these labs drawn. We discussed the benign course of this condition with the parents and Art will continue to stay on the dietary program that he is currently on. Mother will contact us in three months for a follow-up visit.
Thank you for letting us share in the care of Art.

Sincerely,


Rahul Ibsen, MD                
Attending in Endocrinology   


Addendum: Evan Dale Able, MD
Fellow in Endocrinology


CONTENT:
Yvette Seinfeld, M.D.          RE: Douglas Barnini
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/29/92

Dear Dr. Seinfeld:

We had the pleasure of seeing Douglas in the Endocrine Program on 6/26/95 for short stature.

History: This is a 3 and 3/12 year old boy with a history of short stature as well as low weight since the last 3 years.  His height and weight have always been around the 5th percentile.  His birth weight was 6 lb 15 oz.   According to his mother, he has been a fussy eater and is not taking adequate food intake. He was seen by "Nutrition" earlier, and his calorie intake was calculated to 92 cal/kg/dy.  The ideal intake is 102 cal/kg/dy.  There has been no sudden decrease in weight in the recent past.  There is no history of polyuria, polydipsia, blurry vision, or repeated infections.  He is an active child and has had normal milestones.  He is currently in playschool and can read the alphabet.

Past medical history:  It is not significant for any medical problems.

Review of systems:  Within normal limits.

Family history:  He has one sister, 7 years old.  Her height and weight have always been around the 50th percentile.  His mother's height is 5 ft 5 in, and father's is 5 ft 7 in.  His mid-parental height is 5 ft 8 in.  His father's family has a tendency to short stature.  His father sister's is only 5 ft.  There is no history of delayed growth in the father, however, his mother's menarche was at 15 years of age.

Physical exam:  
Height: 90.8 cm                    Weight: 12.7 kg         
BP: 110/70                         Pulse: 88               
U/L ratio:  1.24                   Arm span: 88.5 cm.          
Right testis: 2.0 x 1.2 cm         Left testis: 2.2 x 1.2 cm 
Phallus length: 4.8 cm             Mid-shaft diameter: 1.8 cm 
General: Normal
Skin: Within normal limits.
HEENT: Extraocular movements, normal.
Neck: No thyromegaly.
Chest: Clear to auscultation
Heart: S1S2 were normal.
Abdomen: Soft, bowel sounds present, nontender, and no organomegaly.
Genitourinary: 
Extremities: Normal.
Neurological: Reflexes 2+ with normal relaxation phase. No focal deficits.

Laboratory studies:  IGF-I 101 ng/mL, T4 9.6 ug/dL, TBGI  0.93, TSH  1.00 uU/mL

Impression: This is a 3 and 3/12 year old child with short stature.  He has a height which is around the 5th percentile, and a weight which is at about the 5th percentile.  His decreased height and weight are likely due to a combination of genetic factors given the mid-parental height is about 5ft 8 in, as well as a constitutional growth delay.  His mother does have a history of late onset of menarche.  We will, however, get his IGF-1 and IGFBP-3 levels.

Disposition: He has been advised to follow-up in the endocrine clinic in 6 months.  We will continue to monitor his height and weight.

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


Sincerely,





Mandeep Bajaj, M.D.                 Bernardo Bird, M.D.         
Fellow in Endocrinology             Clinical Director of Endocrinology
                                    Associate Professor of Pediatrics, HMS


CONTENT:
Yvette Seinfeld, M.D.          RE: Douglas Barnini
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/29/92

Dear Dr. Seinfeld:

I saw Douglas, age 3 years 8 months, for follow-up of short stature. 

He has been a healthy and happy boy who attends nursery school.  He is energetic and active.  He sleeps well without disturbance.  Mother stated that he has an improved appetite.  He normally has formed bowel movements, but has had diarrhea in the week prior to this visit.

Current medications: None.

Physical exam:
HT: 94 cm               WT: 13.5 kg
BP: 76/44               Pulse: 90               
He looked well and had smooth warm skin.  He had crusted nares; tympanic  membranes were healthy.  The remainder of the examination was normal.

Impression: Weight-for-height was in the 25th-50th percentile.  Height is just above 5th percentile.  He is growing at a normal rate. 

I reviewed his growth pattern with both his parents and explained that he appears to be following a pattern of constitutional growth delay on a background of average midparental height.  I would be pleased to monitor his growth and development at 6-12 month intervals.


Sincerely,






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


CONTENT:
Yvette Seinfeld, M.D.          RE: Douglas Barnini
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  03/29/92

Dear Dr. Seinfeld:

I saw Elliot, age 4 years 3 months, for follow-up of short stature. 

Medications:  None. 

He has enjoyed good health except for an episode of uncomplicated varicella.  His appetite has improved and he has two good meals and a small lunch daily.  He consumes approximately 12 ounces of milk, yogurt and cheese, and other sources of first class protein.  He sleeps well without disturbance and has a normal energy level.

Physical exam:
HT: 97.8 cm             WT: 14.4 kg    
Arm span: 95.5 cm       U/L ratio:  1.17        HC: 51.6 cm
BP: 99/56               Pulse: 98                      
Breast: Tanner I        Pubic hair: Tanner I    
He appeared healthy, well-nourished, and had good color and hydration.  Skin was smooth and warm.  HEENT was unremarkable.  His physical examination was entirely normal.

Impression: He has grown 3.8 cm and gained 0.9 kg in seven months, an annualized growth velocity of 6.5 cm per year, just above 25th percentile.  Weight-for-height is between 25th and 50th percentile.  His pattern of growth is consistent with constitutional growth delay.  I explained the findings to his parents, and reassured them that there is no evidence of an endocrinopathy.  I would appreciate the opportunity to continue to see him at annual visits.


Sincerely,






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


CONTENT:
Sabie Piggy, M.D.          RE: Lillian Mahler
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/20/93

Dear Dr. Piggy:

We saw Lillian in the Endocrine Program on 4/25/96 for ambiguous genitalia.

History: Lillian is a 3-year old white female with ambiguous genitalia.  She was last seen in Endocrine Clinic on November 29, 1993, by Dr. Najjar.  She had a full evaluation of her ambiguous genitalia as a newborn, which revealed a karyotype of 46 XY, as well as an ultrasound evaluation which revealed a uterus and intra-abdominal gonads.  Exploratory surgery at ten days of life revealed atretic fallopian tubes bilaterally, as well as small intra-abdominal gonads, which upon biopsy revealed testicular material.  An MIF level obtained at that time was 5.5 ng/ml.  Normal values for male newborns is between 30 and 50 ng/ml, with normal values for females usually being unmeasurable.  The decision was made to raise Lillian as a girl, and she underwent surgery at 5-1/2 months for removal of her intra-abdominal gonads and fallopian tubes, as well as a clitoroplasty for correction of her mild clitoromegaly.   

Since that time she has done exceptionally well with no further hospitalizations and only the usual numbers of viral intercurrent illnesses.  She has had no history of UTIs or vaginal symptoms.  She is growing well.   

She is scheduled to see Dr. Retick in Urology Clinic again today for follow up.

Current medications: None.

Family history:  Mother is currently pregnant, and amniocentesis has revealed that the fetus's karyotype is XY.  A sonogram, which was also performed revealed a normal appearing phallus.

Physical exam:
Length: 92.5 cm         Weight: 13.8 kg
Breast: Tanner I        Pubic hair: Tanner I    
General appearance: alert, active, healthy appearing white female.  
Skin:  No lesions, no hyperpigmented areas.  
HEENT:  Normocephalic atraumatic.  pupils equal and reactive to light.  
Neck: supple, no thyromegaly.  Lymph nodes within normal limits.  
Lungs: clear to auscultation.  
Breasts:  Tanner I.  
Heart:  Regular rate and rhythm, normal S1 and S2, no murmur.  
Abdomen:  Soft, nontender, nondistended, no masses, no hepatosplenomegaly, well healed transverse scar just below umbilicus.  
GU:  External female genitalia, somewhat generous labia majora.  No clitoromegaly.  No vaginal discharge.  Prepubertal appearing vaginal mucosa.  Normal introitus.  Extremities:   No clubbing, cyanosis, or edema.  
Neurological:  Grossly within normal limits.

Impression: Lillian is a 3-year old white female with ambiguous genitalia, who has a 46 XY karyotype and who was diagnosed with  dysgenetic gonads in infancy.  She is status post clitoroplasty and removal of her gonads.   

Lillian will need sex hormone replacement beginning at the time of normal puberty.  She does not require any hormonal intervention prior to that time.   

In addition, we discussed fertility issues with Lillian's parents today, focusing on the fact that Lillian should theoretically be able to carry a pregnancy using a donor oocyte.

Disposition: We would like to see Lillian back in Endocrine Clinic in two year's time.



Sincerely,




Yale Neptune, MD            Rahul Ibsen, MD                
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Sabie Hammer, M.D.          RE: Lillian Mahler
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  04/20/93

Dear Dr. Hammer:

We had the pleasure of seeing Lillian in the Endocrine Program on 11/29/93 for folllow-up of ambiguous genitalia.

History: Lillian is a 7 month old female who was born with ambiguous genitalia secondary to a dysgenetic gonad.  She is had reconstructive surgery to correct her ambiguous genitalia on the 25th of August and has had no problems since then.  She is doing well, gaining weight and growing.  She is presently on Amoxicillin for otitis media.

Physical exam:  
Height: 70.3 cm         Weight: 9.44 kg         
Length:  70.3 cm      Weight: 9.44 kg
General: Well developed, well nourished girl.  Skin: No lesions.  HEENT: Atraumatic, normocephalic.  Neck: Supple, thyroid was not palpable. Chest: Clear to auscultation.  Heart: Regular rate and rhythm, no murmurs, gallops or rubs.  S1, S2 was normal.  Abdomen: Soft, nontender, positive bowel sounds.  Surgical scar on her lower abdomen was noted.  Genitourinary: Normal appearing external female genitalia.    Extremities: Free range of motion, good tone.  Neurological:  Alert, active, nonfocal.

Impression: Our impression is that Lillian appears to be doing well after reconstructive surgery.  We will see Lillian again in about two years time for follow up.

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

Sincerely,


Mitsuko Leary, MD           Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Saurabh Domingo, M.D.          RE: Lorraine Harper
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/18/85

Dear Dr. Domingo:

We had the pleasure of seeing Lorraine in the Endocrine Program on 4/26/93 for .

History: Lorraine is a 7 and 101/2 year old girl who has been followed for a midline neck mass which has been infected and treated with antibiotics over the past month.  Two weeks go, Lorraine was seen by an ENT specialist who diagnosed a thyroglossal duct cyst.  Lorraine has been on antibiotics during this periods and is presently on Amoxicillin 250 mg a day.  She has no other significant past medical history.  She reports mild weight gain and an increase in growth over the past six months.  She denies any heat or cold intolerance.  She reports mild constipation which she has had for a long period of time.  History is also negative for polyuria or polydipsia.  She is presently in the second grade and doing well.  No other signs or symptoms of hypo- or hyperthyroidism.

Past medical history:  Lorraine is the product of a full term repeat Cesarean section.  Her birth weigh was 7 lbs. 6 oz.  No complications were reported.  Her growth and development have been normal.

Family history:  Significant for Type II diabetes and obesity.  There is also a family history of hypertension and constitutional delay.  There is no family history of any thyroid disease.  Mother's height was 5'4", father's height 6'4".

Physical exam:  
Height: 129.2 cm        Weight: 35.4 kg         Pulse: 76               
Arm span: 127.5         Breast: Tanner I        Pubic hair: Tanner I    
U/L ratio: 0.93
General: Lorraine was a well developed, well nourished girl, pleasant and active.  Skin: No lesions.  HEENT: Atraumatic/normocephalic.  Pupils were equally reactive.  Neck: Supple, no thyromegaly, masses or cysts.  Chest: Clear to auscultation.  Heart: Regular rate and rhythm,  No murmurs, gallops or rubs.  Abdomen: Soft, nontender.  Positive bowel sounds.
Extremities: Free range of motion.  Good tone.  Neurological: Alert and active.  Nonfocal.

Laboratory studies:  TSH 0.7, total T4 10.3, T3 resin uptake 29.6%.

Impression: Lorraine's history is consistent with a thyroglossal duct cyst.  There is no need for further work-up at this time.  However, if indeed Lorraine's cyst becomes re-infected we would like to re-examine Lorraine and obtain a technetium thyroid scan and proceed to the removal of the cyst.  Until then, we will continue to follow her closely.

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

Sincerely,


 , MD                        Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Antony McPherson, M.D.          RE: Raymond Updike
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/29/79

Dear Dr. McPherson:

We had the pleasure of seeing Raymond in the Endocrine Program on 5/13/93 for evaluation of short stature.

History: Raymond is a 13 and 6/12 year old boy brought in by his parents for concern about delayed growth. He was 5 lb. 12 oz. breached delivery with normal growth until about 13 months of age when he had diarrhea which required hospitalization. He was found to have klebsiella positive stool but it is unclear whether this was in any way related to the diarrhea. Coincidentally he was also found to have an ecoli urinary tract infection for which he received antibiotics and has had no recurrence of urinary infections since then. Since then he has had intermittent diarrhea and postparandial bloating and indigestion which has increased over the past two to three years. He currently rarely takes Tums for indigestion. His bowel movements are two times a day formed, brown and do not float, and are not noted to be fowl smelling.  He occasionally has loose bowel movements every six weeks which last for one day and resolve on its own. His diet consists of cereal with milk, yogurt,  sandwich, chocolate milk, three snacks, meat and vegetables each day. He has no emesis now but postparandial emesis did occur at age 4-5 years. His energy level is normal and he sleeps ten hours a night without any nighttime awakens. He has not had any change in his hair, skin or fingernails. His weight has always been appropriate for his height. There has been no deepening of his voice yet and his first pubic hairs have just begun to appear. There has been no axillary hair although he does have some axillary sweat.  He has had no acne or facial hair and scant facial hair is starting to appear. He is still growing but he only needs new clothes only once a year.

Current medications: Multivitamins and zinc occasionally.

Past medical history:  Pregnancy to mom who was 26 years old at the time. He was 5 lbs. 12 oz., 19 inches at birth. He was delivered full term, spontaneous vaginal delivery, in a breech position. About age 2 growth seemed to be slower than his brother. At 13 months he began to walk. He had noted fine motor skill delay at the age of preschool and attended a preschool for one extra year before being enrolled  in regular school and is currently in the sixth grade. He was hospitalized at 13 months of age for diarrhea and a urinary tract infection as noted above.

Family history:  Maternal grandfather has cardiac disease. Paternal grandfather has hypertension. Maternal grandmother has colisystechtomy. Maternal height 5'4". Paternal height 6'3". Paternal uncle 6'1". Paternal aunt 5'10". Paternal grandmother 5'8". Paternal grandfather 5'11".. Maternal aunts 5'6", 5'2", 5'3". Mother 5'4". Maternal grandmother 5'5". Maternal grandfather 5'10". He has two siblings, one age 9 years who is 56.5 inches and one age 15 years who is 5'6".

Physical exam:  
Height: 141.5 cm        Weight: 38.2 kg         BP: 110/70              
Pulse: 72               U/L ratio:  0.86        Arm span: 144           
Breast: Tanner I        Pubic hair: Tanner I    R. testis: 3 x 2 cm     
L. testis: 2.8 x 1.8 cm Phallus length: 4.2 cm  Mid-shaft diam.: 2.1 cm 
General: Healthy appearing and cooperative young boy in no acute distress.  Skin: No rashes or lesions.  HEENT: Normocephalic, atraumatic. PERRL. EOMI. Fundi flat discs, no palpable edema.  Neck: Supple, no thyromegaly. A few shoddy nodes.  Chest: Lungs clear. No gynecomastia.  Heart: Regular rate and rhythm, normal S1, S2. 2/6 systolic ejection murmur at the apex and across the picordium.  Abdomen: Soft, flat, nontender. Normal active bowel sounds. No hepatosplenomegaly.  Genitourinary:  Normal.  Extremities: No clubbing, cyanosis or edema. No axillary hair or sweat.  Neurological: Reflexes 2+ and symmetric. Motor 5/5 and symmetric.

Impression: Raymond is a 13 and 6/12 year old boy currently only two growth points which appear to demonstrate delayed case. We checked a bone age today and found that it was in fact 11 years with a chronological age of 13 and 6/12 and his predicted height is 5'8". He is delayed in pubertal development but appears to be in early puberty with an increase in his testicular size and mid-shaft penile diameter. Given his history of diarrhea, we will also check and anti-endomyseal antibody as well as routine CBC, chem 7, Somatomedin-C and thyroid function tests.  We will call the family with the results of these tests when they are available. They were also instructed to monitor his growth over the next 2-3 years when he should enter a growth spurt as he enters puberty.

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

Sincerely,


Elmo Domingo, MD                 
Attending in Endocrinology   


Addendum: Cecilia Larson, MD
Fellow in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We had the pleasure of seeing Roscoe in the Endocrine Program on 7/5/94 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is a 14 and 10/12 year-old boy who was diagnosed with diabetes two months ago, when he presented with polyuria, polydipsia, hyperglycemia, and mild ketonuria.  He was hospitalized at that time at the Children's Hospital for initiation of therapy.  He has been well since discharge, except for an automobile accident on July 4, in which he sustained a forehead laceration requiring stitches.  Blood glucose levels are measured three to four times per day and have remained generally between 70 and 180 mg/dl.

Current medications: Novolin human insulin 14-15 U NPH qam, 1-3 U regular predinner, 8-9 U NPH qhs (0.4 U/kg/day).

Family history:  The paternal grandfather takes insulin for adult-onset diabetes mellitus.

Physical exam:  
Height: 172.4 cm        Weight: 60.5 kg         BP: 118/70              
Pulse: 64               Pubic hair: Tanner VI   
Slim, healthy-looking.  Skin with normal texture; no hypertrophy at injection sites.  Thyroid not palpable.  Heart without murmur.  Deep tendon reflexes 2+ without delay.

Laboratory studies:  5/3/94 - hemoglobin A1 14.7%
7/5/94 - hemoglobin A1 8.8%, plasma glucose 124 mg/dl (5:30pm).

Impression: Roscoe currently has very good control glycemic control with a glycosylated hemoglobin reflecting an average blood glucose of 150 mg/dl over the last two to three months.

Disposition: We recommended standing NPH doses of 14 U qam and 8 U qhs.  The sliding scale of regular insulin at dinner was formalized.  We would like to see Roscoe again in three months.
Thank you for letting us share in the care of Roscoe.

Sincerely,


Rene Daley, MD         Bernardo Bird, MD         
Fellow in Endocrinology      Clinical Director
Associate Professor  of Pediatrics, HMS


Problem list to date:
INSULIN-DEPENDENT-DM                         07/13/94  
Transcribed by Sullivan_ma


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We had the pleasure of seeing Roscoe in the Endocrine Program on 7/5/94 for follow-up of insulin-dependent diabete mellitus.

History: Roscoe is a 14 and 10/12 year-old boy who was diagnosed with diabetes two months ago, when he presented with polyuria, polydipsia, hyperglycemia, and mild ketonuria.  He was hospitalized at that time at the Children's Hospital for initiation of therapy.  He has been well since discharge, except for an automobile accident on July 4, in which he sustained a forehead laceration requiring stitches.  Blood glucose levels are measured three to four times per day and have remained generally between 70 and 180 mg/dl.

Current medications: Novolin human insulin 14-15 U NPH qam, 1-3 U regular predinner, 8-9 U NPH qhs (0.4 U/kg/day).

Family history:  The paternal grandfather takes insulin for adult-onset diabetes mellitus.

Physical exam:  
Height: 172.4 cm        Weight: 60.5 kg         BP: 118/70              
Pulse: 64               Pubic hair: Tanner VI   
Slim, healthy-looking.  Skin with normal texture; no hypertrophy at injection sites.  Thyroid not palpable.   Heart without murmur.  Deep tendon reflexes 2+ without delay.

Laboratory studies:  5/3/94 - hemoglobin A1 14.7%
7/5/94 - hemoglobin A1 8.8%, plasma glucose 124 mg/dl (5:30pm).

Impression: Roscoe currently has very good control glycemic control with a glycosylated hemoglobin reflecting an average blood glucose of 150 mg/dl over the last two to three months.

Disposition: We recommended standing NPH doses of	14 U qam and	8 U qhs.	The sliding scale of regular insulin at dinner was formalized.	We would like to see Roscoe again in three months.
Thank you for letting us share in the care of Roscoe.

Sincerely,


Rene Daley, MD         Bernardo Bird, MD         
Fellow in Endocrinology      Clinical Director
Associate Professor  of Pediatrics, HMS


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We had the pleasure of seeing Roscoe in the Endocrine Program on 10/18/94 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is a 15 and 2/12 year-old boy who was diagnosed with diabetes five months ago.  Since we last saw him in July, he has been well.  Dose adjustments made since then involve the addition of more regular insulin.  His blood glucose levels are 80-150 mg/dl before breakfast, and 100-200 mg/dl throughout the rest of the day.   He has had only mild hypoglycemic episodes, and these have been infrequent.  He complains of feeling hungry, especially in the afternoon.

Current medications: Humulin insulin 0-2 U regular and 15 U NPH qam, 4-7 U regular predinner, 9-10.5 U NPH qhs (0.5 U/kg/day).

Physical exam:  
Height: 172.8 cm        Weight: 62.5 kg         BP: 118/78              
Healthy-looking.  Skin without hypertrophy at injection sites. Deep tendon reflexes 2+ without delay.

Laboratory studies:  hemoglobin A1 8.8%, plasma glucose 219 mg/dl (5:30pm)

Impression: Roscoe continues in partial remission from diabetes, but is requiring more regular insulin.  He is doing an excellent job at monitoring and responding to changes in his blood glucose.   However, the use of varying NPH doses based on the glucose level is to be discouraged.

Disposition: We recommended NPH doses of 15 U qam and 9 U qhs, and the sliding scale of regular was expanded to include occasional bedtime regular for high glucose levels.  We would like to see Roscoe again in three months.
Thank you for letting us share in the care of Roscoe.

Sincerely,


Rene Daley, MD         Bernardo Bird, MD         
Fellow in Endocrinology      Clinical Director
Associate Professor  of Pediatrics, HMS


Problem list to date:
INSULIN-DEPENDENT-DM                         07/13/94  
Transcribed by Sullivan_ma


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We had the pleasure of seeing Roscoe in the Endocrine Program on 8/22/95 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is 16 years old and was diagnosed with diabetes fifteen months ago.  Since his last visit in March, he has been well.  The blood glucose levels, measured four times per day, are mostly within the normal range, with only occasional hypoglycemia.  Roscoe recognizes hypoglycemia readily, with no apparent blunting of symptoms.  He is active in sports and follows the prescribed diet closely.

Current medications: Humulin 6-14 U regular and 26 U NPH qam, 5-14 U regular predinner, 11 U NPH qhs (average 0.78 U/kg/day).

Physical exam:  
Height: 173.4 cm        Weight: 67.9 kg         BP: 122/82              
General:  Healthy-looking.  Skin without hypertrophy at injection sites.  Thyroid not palpable.  Heart without murmur.

Laboratory studies:  hemoglobin A1 7.0% (normal 5.2-7.8)

Impression: Roscoe continues to maintain excellent glycemic control, with remarkably few problems with hypoglycemia.  Nonetheless the increased risk of severe hypoglycemic episodes with such good control was emphasized.  A more portable glucometer would be useful for testing around sports activities.

Disposition: No changes in the insulin doses are needed at this time.  Occasional blood glucose measurements in the middle of the nights were encouraged.  We would like to see Roscoe again in four months.
Thank you for letting us share in the care of Roscoe.

Sincerely,


Rene Daley, MD         Maureen Shields, MD             
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We had the pleasure of seeing Roscoe in the Endocrine Program on 8/22/95 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is 16 years old and was diagnosed with diabetes fifteen months ago.  Since his last visit in March, he has been well.  The blood glucose levels, measured four times per day, are mostly within the normal range, with only occasional hypoglycemia.  Roscoe recognizes hypoglycemia readily, with no apparent blunting of symptoms.  He is active in sports and follows the prescribed diet closely.

Current medications: Humulin 6-14 U regular and 26 U NPH qam, 5-14 U regular predinner, 11 U NPH qhs (average 0.78 U/kg/day).

Physical exam:  
Height: 173.4 cm        Weight: 67.9 kg         BP: 122/82              
General:  Healthy-looking.  Skin without hypertrophy at injection sites.  Thyroid not palpable.  Heart without murmur.

Laboratory studies:  hemoglobin A1 7.0% (normal 5.2-7.8)

Impression: Roscoe continues to maintain excellent glycemic control, with remarkably few problems with hypoglycemia.  Nonetheless the increased risk of severe hypoglycemic episodes with such good control was emphasized.  A more portable glucometer would be useful for testing around sports activities.

Disposition: No changes in the insulin doses are needed at this time.  Occasional blood glucose measurements in the middle of the nights were encouraged.  We would like to see Roscoe again in four months.

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

Sincerely,



Rene Daley, MD         Maureen Shields, MD             
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We saw Roscoe in the Diabetes Program on 12/26/95 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is 16 4/12 years old and was diagnosed with diabetes one and one-half years ago.  Since his visit in August, he has been well.  Alex forgot his blood glucose log book today but reports levels generally within the normal range.  He has occasional mild hypoglycemia but has had no severe reactions.  Alex continues to adjust his own insulin doses, according to activity and trends in the glucose measurements.  The review of systems revealed no new symptoms.

Current medications: Humulin 6-12 U regular and 27 U NPH qam, 6-12 U regular predinner, 14 U NPH qhs (average 0.77 U/kg/day).

Physical exam:     HT: 174.0 cm    WT: 71.7 kg  BP: 124/74              
Healthy looking.  Skin without hypertrophy at injection sites.  Thyroid not palpable.  Heart without murmur.  Deep tendon reflexes 2+ without delay in relaxation.

Laboratory studies:  hemoglobin A1c 5.8% (normal 4.0-6.0), urine microalbuminuria 3 mg/g creatinine (normal 0-15)

Impression: Alex' glycemic control continues to be excellent.  A baseline microalbuminuria test was normal.  This screening test for diabetic nephropathy should be performed yearly from now on.

Disposition: Once again the increased risk of severe hypoglycemia with such good glycemic control was emphasized.  A middle-of-the-night glucose measurement every two weeks was advised.  Decreased insulin after evening sports was also discussed.  We would like to see Roscoe again in four months.



Sincerely,




Rene Daley, MD             Lancelot Blue, MD             
Fellow in Endocrinology          Attending in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

We saw Roscoe in the Endocrine Program on 3/28/96 for insulin dependent diabetes mellitus.

History: Roscoe is 16 7/12 years old and was diagnosed with diabetes almost two years ago.  Since his last visit in December, he has been well.  In January and February, he adjusted his insulin doses down to accommodate the increased activity of the wrestling season.   His doses have since been increased again, but next month he will begin playing lacrosse.  The blood glucose levels are measured three to four times daily and are almost always in the recommended target ranges of 80-180 mg/dl (80-150 before breakfast), except for some lower numbers in the 60-80 range during the day.  Occasional middle of the night measurements have shown levels of about 100 mg/dl at 2-3am.  There have been no severe hypoglycemic episodes.

Current medications: Humulin 4 or more U Regular and 26 U NPH 6:30am, 8 or more U Regular 6-7pm, 13 U NPH 9pm (average 0.73 U/kg/day).

Physical exam:
HT: 173.8 cm         WT: 69.3 kg
BP: 130/70              

Laboratory studies:  hemoglobin A1c 6.3% (normal 4.0-6.0)

Impression: Alex continues to do a beautiful job of monitoring and adjusting his insulin, demonstrating a thorough understanding of the principles involved.

Disposition: Methods of varying the insulin dosing to accommodate changes in his schedule were discussed.  The regular use of the Medic Alert tag was recommended.  We would like to see Roscoe again in four months.

Sincerely,






Rene Daley, MD         Rahul Ibsen, MD                
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

I saw Roscoe in the Endocrine Program on 8/20/96 for follow-up of insulin-dependent diabetes mellitus.

History: Roscoe is 17 years old and has had diabetes for two years.  Since his last visit in March, he has been well.  He is working an evening shift at a restaurant this summer and has adjusted his insulin appropriately for the change in dose timing.  His blood glucose is measured four or more times daily and has ranged in the last six days from 66 to 126 mg/dL before breakfast, 68 to 170 before lunch, 82 to 185 before dinner, 91 to 135 and in the evening.  There have been no severe hypoglycemic episodes.  In fact hypoglycemia is infrequent in general.  However, he has had a couple episodes recently when he was hypoglycemic (glucose <60) and had few warning symptoms.  Alex has checked his glucose levels late at night following various doses of bedtime Regular insulin and has not been hypoglycemic.

Current medications: Novolin 2-8 U Regular and 27 U NPH qam, 4-10 U Regular before dinner, 0-4 U Regular and 14 U NPH qhs (average 0.70 U/kg/day).

Physical exam:
HT: 174.5 cm    WT: 69.6 kg    Pulse: 72               
Healthy looking.  Skin without hypertrophy at injection sites. Thyroid not palpable.

Laboratory studies:  hemoglobin A1c 6.3% (normal 4.0-6.0)
                     urine negative for ketones and glucose

Impression: Alex's glycemic control is excellent.  He is very mature and intelligent and appears to incorporate diabetes care into his life with minimal disruption of his lifestyle.  With near normal glucose levels, his major risk is hypoglycemia.  Not atypically, hypoglycemia awareness may be diminished with such good glucose control, and Alex has experienced this already.

Disposition: Alex was provided with some information about how to recognize hypoglycemia when the adrenergic warning symptoms are lacking and how to prevent severe hypoglycemia.  I would like to see him again in four months.

Sincerely,



Rene Daley, MD         
Attending in Endocrinology


CONTENT:
Oanh Welby, M.D.          RE: Roscoe Farley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/17/79

Dear Dr. Welby:

I saw Roscoe in the Diabetes Program on 12/19/96 for insulin-dependent diabetes mellitus.

History: Alex is 17 4/12 years old and has had diabetes for two and one-half years.  Since his last visit in August, he has been well.  He is a junior in high school and is doing well academically.  He keeps active with weight-lifting.  The blood glucose is measured three to four times daily and ranges between 60 and 150 mg/dL throughout the day, with only rare values less than 50 or greater than 200.  He has had no severe hypoglycemic episodes.

Current medications: Novolin 4-10 U Regular and 30 U NPH qam, 6-20 U Regular before dinner, 17 U NPH qhs (average 0.88 U/kg/day)

Physical exam:
HT: 174.9 cm    WT: 72.4 kg
BP: 122/70              Pulse: 72               
Healthy looking.  Skin with normal texture, no hypertrophy. Thyroid not palpable.  Heart regular rate and rhythm.

Laboratory studies:  hemoglobin A1c 6.5% (target 6.0-8.0), microalbumin level in random urine specimen 3 mg/g creatinine (normal <20)

Impression: Alex continues to maintain excellent glycemic control.  As before, his main risk is hypoglycemia.  His insulin requirement has increased over the last few months, perhaps because of decreased exercise.

Disposition: An occasional glucose level should be measured in the middle of the night to see if there is hypoglycemia (about once a month).  If so, the nighttime insulin dose should be decreased.  I would like to see Alex again in four months.


Sincerely,



Rene Daley, MD         
Attending in Endocrinology   


cc: Roscoe Farley, 45 Saco Street, Newton, MA  02164


CONTENT:
Ricot Bear, M.D.          RE: Miguel Jansen
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/09/92

Dear Dr. Bear:

We had the pleasure of seeing Miguel in the Endocrine Program on 11/22/93 for short stature.

History: Miguel is an 18-month-old boy who was had poor weight and statural gains.  He was born measuring 2.5 kg and 44 cm at 37 weeks gestation.  There was mild jaundice that was gone by 8-9 days.  He gained well during the first two months while on breast milk, but thereafter, upon discontinuing breastfeeding and entering daycare, he did not take bottled formula well.  His weight gain between two and four months of age was markedly decreased, and since four  months, his weight has been well below the fifth percentile.  His length fell to less than the fifth percentile by six months.  He was admitted to the Rhode Island Hospital for failure to thrive and begun on nasogastric tube feedings at nine months.  At 14 months, a gastrostomy was placed.  He is currently fed Pediasure 28 oz/day via the gastrostomy.  He tastes food with pleasure but does not swallow it.  He has been vomiting since the nasogastric tube was introduced and still vomits once every day.  Stools are  frequent and watery.

Propulcid 10 mg qd, Zantac 2 ml (30 mg) qd

Past medical history:  Miguel had cryptorchidism and bilateral inguinal hernias at birth.  At three weeks, a herniorrhaphy was performed.  The testes descended on their own.  A myringotomy with tube placement was performed for frequent otitis media.  He walked by twelve months
          and now says a few words.

Family history:  The mother is 28 years old and the father 29 years old.  Both are healthy.  There are two cousins on the father's side with a probable genetic disorder associated with carnitine or cytochrome oxidase deficiency (The parents were unsure of this information).  Mother's height 159.7 cm, father's height 165.1 cm.

Physical exam:  
Height: 75.5 cm         Weight: 8.75 kg         Pulse: 120              
Breast: Tanner I        Pubic hair: Tanner I    R. testis: 1.8 x 1.0 cm 
L. testis: 1.8 x 1.0 cm Phallus length: 4.1 cm  Mid-shaft diam.: 1.3 cm 
General:  Alert, active boy. HEENT: Head circumference: 46.5 cm. Abdomen:  Presence of G-tube.  Genitourinary: Mild rash in the diaper area.  The remainder of the exam was normal.

Impression: The growth records and history provide strong evidence for a nutritional etiology for Miguel's short stature.  His statural growth rate has been greater than average since 12 months of age, and his length is moving back up towards the fifth percentile.  However, since mild hypothyroidism can subtly affect growth, it is wise to check for this.

Disposition: We recommend obtaining a baseline bone age and thyroid function tests (T4, TSH, and T3-resin uptake).  The parents asked if these could be done in your office.  If these are normal, we would observe his growth prior to any further endocrine evaluation.  We would like to see Miguel again in six months.
Thank you for letting us share in the care of Miguel.

Sincerely,


Rene Daley, MD         Mitsuko Simpson, MD             
                             Chief Emeritus


CONTENT:
Ricot Bear, M.D.          RE: Miguel Jansen
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/09/92

Dear Dr. Bear:

We had the pleasure of seeing Miguel in the Endocrine Program on 8/15/94 for .

History: Miguel is a 27-month-old boy with slow statural growth.  He was last seen by us in November, 1993, at which time we felt that inadequate nutrition was the most likely etiology.  Since then there has been a decrease in the amount of vomiting, allowing for a significant increase in intake.  He is now able to take by gastrostomy 40 oz/day of Pediasure supplemented to 32 cal/oz, totaling 1230 cal/day.  Ranitidine was stopped several months ago and cisapride six weeks ago.  Miguel still refuses to eat by mouth.

Current medications: Septra for an ear infection

Past medical history:  The persistent elevation of ALT and AST serum levels led to a liver biopsy in May, 1994, which was inconclusive but showed some changes suggestive of a mild chronic hepatitis.

An otolaryngology evaluation was performed because of frequent loud snoring.  Markedly enlarged tonsils and adenoids were found, and Miguel is scheduled for a tonsillectomy and adenoidectomy in September.

Physical exam:  
Height: 83.8 cm         Weight: 11.1 kg         Pulse: 90               
Breast: Tanner I        Pubic hair: Tanner I    
head circumference: 47.5 cm
Active and playful boy.  Skin with normal texture.  Thyroid not enlarged. Heart without murmur.  Abdomen with gastrostomy.

Laboratory studies:  February 24, 1994 - T4 18.2 mcg/dl, TSH 2.5 mcIU/ml, thyroxine binding globulin (TBG) 59.2 mcg/ml (normal 16-34 for adults, norms for infants not available from this laboratory), bone age 12-15 months at chronological age of 21 months.

Impression: Miguel's weight has reached the fifth percentile for age, the highest it has been since he was four months old.  The statural growth rate has also improved.  We expect that he will show significant catch-up growth over the next few months if he can continue to take in adequate calories.

The thyroid function tests demonstrate TBG excess, which is generally a benign, euthyroid condition.  Although the T4 is high, the free T4, which is the active fraction of hormone, should be normal.  This condition can be inherited or acquired.

Disposition: At his next blood test at Children's Hospital, repeat thyroid tests will be drawn (T4, TSH, TBGI, and TBG) to see if the excess TBG has persisted.   Follow-up will be determined based on those findings.
Thank you for letting us share in the care of Miguel.

Sincerely,


Rene Daley, MD         Ariel Adler, MD           
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Fai Stalin, M.D.          RE: Mildred Sage
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/30/76

Dear Dr. Stalin:

We had the pleasure of seeing Mildred in the Endocrine Program on 5/24/93 for evaluation of .

History: Mildred is a 16 and 6/12 year old girl whom we saw in Endocrine clinic today for goiter. Mildred has had several episodes of sore throat since August of 1992 which were thought to be of bacterial etiology or viral though mono spots were negative. On May 10 she saw you for a swollen lymph node in front of her left ear. At that time you noticed an enlarged thyroid gland and obtained TFTs which showed an unmeasurable TSH and normal T3, T4 and which showed positive microsomal antibodies and positive thyroglobulin antibodies. She began antibiotic treatment and has been on Tetracycline for three days.

Past medical history:  The pregnancy was normal. Delivery at term was normal. According to father, she has always had retarded bone growth. She has had chicken pox. She has had no severe illness, no injuries, hospitalizations, or surgery. She has been tired for several months. She has noticed that her skin was dry lately without a rash. She has intermittent frontal headaches about once a week for several months. These occur in the daytime and go away without special measures. Vision and hearing are good. She has had a cough and a runny nose off and on since 8/92. Father feels that her appetite has been decreased for a year. She has gained 3 lbs. in two months. Stools are once a day and of normal consistency. There is no dysuria. Her menarche was at 11 years of age and she has had regular periods once a month sometimes with cramps. The last period ended a day ago. One month ago she had left flank pain which is now resolved. She has been especially tired for several months. In addition to sleeping 8-10 hours a night, has sometimes taken 2-3 hour naps during the daytime.

Family history:  She has a healthy 14 year old brother. Father is healthy. He has three brothers and one sister. They are all healthy. Father's uncle has diabetes. Mother and mother's sister have thyroid disease probably hypothyroidism according to the father. Mother's height 5'7". Father's height 5'8".

Physical exam:  
Height: 156.1 cm        Weight: 45.9 kg         BP: 100/75              
Pulse: 72               
General: Pleasant, alert, 16 year old girl in no distress. 
Skin: Cool with no rash.
HEENT: PERRL, EOMI, discs sharp bilaterally. Visual fields full to confrontation. Timpanic membranes and throat were clear. There were shoddy anterior cervical lymph nodes bilaterally. 
Neck: Thyroid was enlarged with an ill defined border and not nodular. 
Chest: Lungs clear. 
Heart:  Normal S1 and physiologically split S2. A 2/6 systolic ejection murmur on the left lower sternal border lying down. 
Abdomen: Soft and nontender. No hepatosplenomegaly or mass. 
Genitourinary: Normal. 
Extremities: Without edema. 
Neurological: DTRs 1-2+ symmetrically. Toes downgoing.

Impression: Mildred is a 16 and 6/12 year old girl with goiter. No clinical signs of hypo or hyperthyroidism. Positive microsomal and thyroglobulin antibodies, normal T3, T4 and an increased TSH. We felt the most likely etiology to be Hashimoto's thyroiditis. We obtained TSI and TBII antibodies and rechecked TSH, T3, and T4. Since she is euthyroid at this point, we see no role for hormone therapy but feel her TFTs should be checked again in two months either with you or in our clinic. Father was encouraged to call Dr. Hagen with any questions or change in Mildred's status.

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

Sincerely,


Valentine Domingo, MD            Penelope Hagen, MD              
                             Attending in Endocrinology   


Problem list to date:
                             

Transcribed by Cassells


CONTENT:
Fai Stalin, M.D.          RE: Mildred Sage
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/30/76

Dear Dr. Stalin:

We had the pleasure of seeing Mildred in the Endocrine Program on 5/24/93 for evaluation of a goiter.

History: Mildred is a 16 and 6/12 year old girl who has had several episodes of sore throat since August of 1992, thought to be of bacterial or viral origin.  On May 10 she was seen by you for a swollen lymph node in front of her left ear and was noted to have an enlarged thyroid gland.  Thyroid function tests showed an unmeasurable TSH, normal T3,  and T4, and positive microsomal thyroglobulin antibodies. She was begun on antibiotic treatment, and has been on Tetracycline for three days.

Past medical history:  Normal pregnancy, labor and delivery.  She has had no severe illness, no injuries, hospitalizations, or surgery.

Review of systems:  Mildred has been tired for several months.  In addition to sleeping 8-10 hours a night, has sometimes taken 2-3 hour naps during the daytime.  Dry skin without a rash. She has intermittent frontal headaches about once a week for several months. These occur in the daytime and go away without special measures. Vision and hearing are good. She has had a cough and a runny nose off and on since 8/92. Father feels that her appetite has been decreased for a year. She has gained 3 lbs. in two months. Stools are once a day and of normal consistency. There is no dysuria. Her menarche was at 11 years of age and she has had regular periods once a month sometimes with cramps. The last period ended a day ago. One month ago she had left flank pain which is now resolved.

Family history:  She has a healthy 14 year old brother. Father and his three brothers and one sister are all healthy. Father's uncle has diabetes. Mother and mother's sister have thyroid disease, probably hypothyroidism according to the father. Mother's height 5'7". Father's height 5'8".

Physical exam:  
Height: 156.1 cm        Weight: 45.9 kg         BP: 100/75              
Pulse: 72               
General: Pleasant, alert, 16 year old girl in no distress.  Skin: Cool with no rash. HEENT: PERRL, EOMI, discs sharp bilaterally. Visual fields full to confrontation. Timpanic membranes and throat were clear. There were shoddy anterior cervical lymph nodes bilaterally.  Neck: Thyroid was enlarged with an ill defined border and not nodular.  Chest: Lungs clear.  Heart:  Normal S1 and physiologically split S2. A 2/6 systolic ejection murmur on the left lower sternal border lying down.  Abdomen: Soft and nontender. No hepatosplenomegaly or mass.  Genitourinary: Normal.  Extremities: Without edema.  Neurological: DTRs 1-2+ symmetrically. Toes downgoing.

Impression: Mildred has a mildly enlarged thyroid gland but no clinical signs of hypo-  or hyperthyroidism.  We felt the most likely etiology to be Hashimoto's thyroiditis. We obtained TSI and TBII antibodies and rechecked TSH, T3, and T4. Since she is euthyroid at this point, we see no role for hormone therapy but feel her thyroid function tests should be checked again in two months either with you or in our clinic, and she should be carefully followed for changed in her thyroid status.

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

Sincerely,


Valentine Domingo, MD            Penelope Hagen, MD              
                             Attending in Endocrinology


CONTENT:
Vanderlei Piggy, M.D.          RE: Vivian Hoban
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/30/93

Dear Dr. Piggy:

We saw Vivian in the Endocrine Program on 11/20/95 for osteopenia.

History: Vivian is now 2-1/2 years old.  She was previously seen by the  endocrinology service as an inpatient in 8-95 for evaluation of failure to thrive and a history of two femur fractures.   

She has a very complex past medical history; she was a twin born of a 33 week  gestation, C section for twin to twin transfusion where Vivian was the donor.  Her twin sister is absolutely healthy but Vivian was born with the VACTERL association and the following problems:  esophageal atresia, duodenal atresia, and imperforate anus, rectovaginal fistula, coarctation of the aorta, hemivertebra and a biphid rib of T12.  She has needed multiple surgeries and has been intermittently on a ventilator.   

Her past surgeries include gastrostomy, duodenal duodenostomy, repair of  coarctation, revision of duodenostomy and partial resection of the distal duodenum, colon interposition for reconstruction of her esophagus, aortopexy, repair of cloacal malformation, and most recently in 11-95 reversal of her colostomy. 

For the first 9-10 months of life she was mostly on total parenteral nutrition overlapping with Tolerex G tube feeds from age six months.  She started walking at age one and had been walking for about four months when she had her first femur fracture on the left side after a minimal fall.  Reportedly those films did not show any osteopenia.  In 4-95 she broke her right femur also with minimal trauma.   

In 8-95 when we were first consulted, she had been on a blended tablefood diet for about six weeks which in retrospect was probably calcium deficient.  On our evaluation in the hospital her vitamin D levels were normal, calcium and phosphates were also normal, but she had an elevated alkaline phosphatase most likely attributable to her recent surgery with cloacal reconstruction.  Her bone age at that time was two years at a chronologic age of two years two months and her hand x-rays showed osteopenia with cortical thinning but at the same time, x-rays of her knee did not show any demineralization.  In 2-94 she had been diagnosed with villous atrophy on a small bowel biopsy and thought to have a malabsorptive syndrome.  Based on this and pending PTH and vitamin D levels, she was started on vitamin D supplements and calcium supplements.  In August her urine calcium creatinine ratio was normal at 0.1 and her tubular reabsorption of phosphate also normal at 0.82.   

Since discharge she has been ambulating normally and has not sustained any new fractures.  She is doing well developmentally although she is still behind her sister in speech.  Her weight gain and growth have also improved and she is currently in the 10th percentile for both length and weight, where she was previously well below the 5th percentile in both.  This is attributed to increased caloric intake both via G tube, a more balanced diet and increased PO intake after a long period of oral aversion.   

Her current diet consists of blenderized table food with Pediasure and MCT oil supplements.  She is currently followed by a nutritionist.

Current medications: Propulsid 10 mg per day, multi-vitamins 1 cc per day, Fer-in-Sol 0.6 cc per day, vitamin D 0.2 ml or 1600 IU per day, Neo-Calglucon 1 tbsp per day equalling 345 mg or 31.4 mg. elemental calcium per kilo per day and Spironolactone with hydrochlorothiazide 25 mg p.r.n. when she looks puffy or has respiratory infections.  She has no known drug allergies.

Review of systems:  Skin:  occasional dryness. HEENT: has had not had any ear infections.  Cardiorespiratory:  frequent aspiration pneumonias, occasionally needing 02 supplements at night and p.r.n. diuretics.   Cardiac defect has been corrected.  GI:  anatomical reconstruction is completed.  She still has GE reflux with poor GI motility and she was diagnosed with dumping syndrome in 2-95 with Villous atrophy and question of fat malabsorption.  These symptoms with hypoglycemia have resolved clinically after she started on regular table foods and her parents are not following blood glucoses at home.  On her last admission, vitamin E, A, and B12 and folic acid levels were all within normal limits.  She  currently has a G tube and is improving on her oral intake.  Genitourinary:  She has not had any urinary tract infections or urinary tract defects and she is almost potty trained.  Neurologic:  she had two seizures in 2-95 thought to be due to her dumping syndrome.  Imaging studies and EEG have been normal.

Family history:  Healthy twin sister, both parents are healthy.  A paternal aunt has a thyroid problem, there is no history of diabetes melitis, no history of bone disease or osteopenia.

Physical exam:
Length: 85 cm           WT: 11.03 kg
Pulse: 105              Breast: Tanner I        Pubic hair: Tanner I    
Very pleasant and cooperative young child, slightly dysmorphic with low nasal bridge and frontal bossing.  Skin is normal except for multiple scars on her abdomen and chest.  TMs are normal and she has normal dentition and normal enamel.   Neck is supple without thyromegaly.  She has no palpable nodes.  Lungs are clear to ausculation and she has good air entry.  Her heart has no murmurs and she has regular rate and rhythm with good pulses.  No palpable breast tissue.   Abdomen is soft with no hepatosplenomegaly and no masses.  G tube button  is in place.  Genitalia are normal prepubertal female status post reconstruction.  Extremities:  show no signs of rickets and she is not bow-legged.  She has normal deep tendon reflexes and no clonus.

Laboratory studies:  On 11-2-95 she had a phosphorous of 2.5, urine phosphorous of 8.4 with a TPR of 0.93, creatinine in the urine was 12.8, and serum 0.3, magnesium 1.8.  On 11-7 she had 21, 25-hydroxy vitamin D level (pending), a 25- hydroxy vitamin D level was 27, PTH was 11, and a serum calcium was 9.6.

Impression: 2-1/2 year old girl with a complex past medical history.  Failure to thrive seems to be resolving with increasing intake.  Her osteopenia is more perplexing as her knee films are normal and reportedly were normal in terms of mineralization at the time of her fractures.  All her metabolic labs as above have also been within normal limits.  It is surprising that her vitamin D level would not go up more significantly after she started on replacement.  Long-term  hyperalimentation and diuretics are known to cause hypercalciuria in infants and that is a possibility in her case even though her calcium/creatinine ratio in August was back to normal.  Most likely her problems are multi-factorial with additional efects of long-term immobilization and possibly some component of malabsorption and/or malnutrition.

Disposition: Currently continue the same regimen of supplementation and repeat a calcium and phosphorous locally.  Vivian will return in February and her parents will bring copies of her previous bone films from Vermont.  We also asked the father to inquire if anti-endomesial antibodies had been sent previously.


Sincerely,






Fuli Meisner, MD     Maritza Brezny, MD             
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Vanderlei Piggy, M.D.          RE: Vivian Hoban
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/30/93

Dear Dr. Piggy:

I saw Vivian, age 2 years 9 months, for follow up of osteopenia and slow growth. 

Her past medical history is well known to you and will not be restated here.   

Medications:  Neo-Calglucon 1 tablespoon daily; vitamin D (calciferol) 0.2 ml daily; Pol-vi-sol 1 ml daily; ferrous sulfate 0.6 ml daily; Propulsid 7.5 mg daily in 3 divided doses; 1 to 3 tablespoons of MCT oil per day.   

Since January 1996, she has taken all her food by mouth for the first time in her life.  She currently has three meals and three snacks each day.  All food is blenderized.  Review of her diet indicates that she has a varied and nutritious diet with adequate representation from all the major food groups.  In addition, her diet is fortified with additional fat in the form of butter, vegetable oil, sour cream, and mayonnaise.   

Although bowel movements are softer than normal, she does have occasional firm stools.  She is beginning to gain control of bowel function and has begun to use a potty.  Stool frequency is every 2 to 3 hours in the day, but often not during the night. 

There have been no new fractures or bone problems.  There have been no major illnesses since her last visit to the Children's Hospital, with the exception of otitis media once and a few minor upper respiratory infections.

Current medications: None.

Physical exam:   Length: 87 cm    WT: 11.68 kg    HC: 46.5 cm
She was anxious, but calmed down during the examination.  She has dolicocephaly and a prominent brow.  She had 20 erupted teeth.  Skin was smooth and warm.  HEENT was unremarkable.  She had shotty cervical lymphadenopathy; no thyromegaly.  Chest was clear and heart sounds were normal; no murmurs.  Abdomen revealed no organ enlargement or masses.  She had numerous well healed scars.  A button gastrostomy was in place.  She has normal female external genitalia.  She had good muscle development and tone.  She stands and walks normally.  

Her length and weight are in the10th centile.  She is making normal developmental progress.

Laboratory studies: I ordered x-rays of her hands and knees to evaluate the status of her previous osteopenia.  "Studies demonstrate normal mineralization. The epiphyses are open and otherwise unremarkable.  Some growth recovery lines are seen in the proximal tibiae bilaterally.  The cortex is slightly thin, but within normal limits.  

UPPER GI SERIES:  "The upper GI was performed in the standard fashion. 
The patient has undergone a colonic interposition.  The prior
exam is from 1995.  There is no change in the appearance of the
colonic interposition.  The pharyngeocolonic anastomosis is open
and unremarkable.  The colonic gastric anastomosis is open and
unremarkable as well.  Note is made, however, of free reflux
from the stomach into the colon.  There is no evidence of
gastric emptying abnormality.  
Impression:  Free reflux is noted from the stomach into the
colonic interposition.  This is unchanged from prior exams."

Hemoglobin 12.4 g/dl, hematocrit 36.7%, MCV 70.9, MCH 24, MCHC 33.8, white cell count 11.62 k, platelets 344 K, retics 1%; serum electrolytes were normal.  BUN 14, creatinine 0.5, calcium 10.0, phosphorus 4.1, magnesium 2.2 mg/dl.  AST, ALT, LDH: 30, 10, 182 U/l, respectively; alkaline phosphatase 157 U/L within the normal range.  Spot urine calcium/creatinine ratio 0.07 mg, within the normal range.  Serum vitamin D levels are pending.

My impression is that Vivian is making marvelous progress physically and developmentally.  Bones are not osteopenic and appear well mineralized.  Her serum and urinary calcium are nrormal and alkaline phosphatase is back in the normal range.  I have no new recommendations concerning her treatment.  

Please feel free to have Lilly see us again if the need arises in the future.



Sincerely,




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

cc:  Samuel Nurko, M.D. Children's Hospital 300 Longwood Avenue
     GI Clinic Boston, MA 02115

     Mr. John MacArthur  RR2 Box 6  Dickinson Center, NY 02930


CONTENT:
Elizabeth Swartzenegger, M.D.          RE: Bruce Scott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/21/81

Dear Dr. Swartzenegger:

We had the pleasure of seeing Bruce in the Endocrine Program on 1/20/94 for evaluation of endocrine function status post radiation therapy  after incidental radiation to the pituitary                                                                                                                                                                                                   after incidental radiation to the pituitary.

History: He was healthy until this past year when he had blurred vision and an optic nerve meningioma was found.  He is s/p surgery and radiation from October to December 1993.  He is scheduled for another MRI in three months.   He has no vision in his left eye.   He had incidental radiation to the pituitary and was referred to us for evaluation of any hypopituitary findings.   The mother and the patient deny the need for deodorant.    He has some erections.   He sleeps through the night with no accidents and no need to urinate or to drink.   He has no signs of hypothyroidism.

He is on Intal and Ventolin inhaler for his asthma.    He was given steroids after his surgery.    At this time he is on non-steroidal inhalers.

Past medical history:  He is the product of an uncomplicated, normal vaginal delivery.   Growth and development were normal.    No hospitalizations or operations except as noted above.

Review of systems:  Normal except for asthma and the neurological findings of the past year.

Family history:  He has three older siblings (ages 22, 19 and 16 years of age).   Father's height is 5'9.5".  Mother's height is 164.3 cm.

Physical exam:  
Height: 154.3 cm        Weight: 56.1 kg         BP: 109/64              
Pulse: 97               U/L ratio:  0.9         Arm span: 156.5         
R. testis: 3.6 x 2.1 cm L. testis: 3.5 x 2.3 cm Phallus length: 8 cm    
Mid-shaft diam.: 2 cm   
Skin: No facial hairs.
HEENT: Normal.
Neck:  No thyromegaly.   No lymph node enlargement.   
Chest: Clear.
Heart: S1, S2.
Abdomen: Soft, no organomegaly.
Genitourinary: 
Extremities: Normal.
Neurological: Normal except he has no vision in the left eye.   Achilles normal.

Laboratory studies:  Bone age 12 years.  Testosereon 69 ng/dL (early pubertal), LH 0.75 IU/L (early pubertal), FSH 3.3 IU/L (early pubertal), AM Cortisol 5.1 mcg/dL (low), IGF-1 384 ng/ml ( normal).  T4 8.6 mcg/dl, TBGI 0.9, TSH 1.30 mcU/mL, all nroaml>  Electrolytes were normal.   In the future we will consider doing ACTH stimulation test.

Impression: Bruce is s/p surgery and incidental radiation to the pituitary.   We will follow Bruce for hypopituitarism.   There are signs of puberty developing as seen by the increased size of his testes.

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

Sincerely,


Nilto Carter, MD             
Attending in Endocrinology   


Addendum: Judith Carroll, M.D.
Fellow in Endocrinology


CONTENT:
Shahin Adler, M.D.          RE: Bruce Scott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/21/81

Dear Dr. Adler:

We had the pleasure of seeing Bruce in the Endocrine Program on 6/2/94 for .

History: I wanted to report my visit with Bruce June 2, 1994.   As you know, this now 12 and 7/12 year old boy is being followed for the potential effects of cranial irradiation on his neuro-endocrine axis.   I understand from his mother and from his that record, his last MRI in April showed no change from his previous studies and the next one will be done in six months.   There are no symptoms suggestive of diabetes insipidus.   He has developed further into puberty and he has grown 3 cm in the past four and a half months.

Physical exam:  
Height: 157.3 cm        Weight: 56.3 kg         Pubic hair: Tanner II   
GU:  Left testis is 15 cc in volume, right testis is 17 cc.   Penile stretch length is 8.5 cm.  Mid shaft diameter is 2 cm.  He looks approximately a year older than his chronological age and the remainder of his exam is unchanged.

Impression: With the normal growth rate in the stages of early puberty, I certainly see no evidence of deletion of neuro-endocrine function.   A more appropriate question is whether or not his treatment may have initiated gonadotropin output.   

It is not necessary, to pursue neuro-endocrine or even growth hormone testing at this point although the latter will be watched for with respect to his growth velocity.   

Because an am Cortisol done several months ago was only 5 mcg/dL, I have asked Mrs. Scott to bring him into the Lexington Center early in the morning for another repeat of this and if it is again low, we may do an ACTH stimulation test to look at his adrenal capacity to respond.   This might have some impact on how he might need to be treated at the time of any trauma or surgery. 

Thanks for allowing me to share in his care.  I will see him again in 9 months.

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

Sincerely,


Nilto Carter, MD             
Attending in Endocrinology


CONTENT:
Shahin Adler, M.D.          RE: Bruce Scott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/21/81

Dear Dr. Adler:

We had the pleasure of seeing Bruce in the Endocrine Program on 2/23/95 for .

History: I had the pleasure of seeing Bruce again on 2/23/95 at 13 4/12 for evaluation  of pubertal and growth response status post radiation of his central nervous system.  As you know, this was done for an optic nerve meningioma, following surgery.  Some pituitary radiation did occur.

Bruce is definitely in mind puberty at this point and has had nothing to suggest any compromising growth.  In fact, he grew 8.7 cm in the last seven months.  

He has been quite well; has not had any alteration in visual status in his right eye, but did have a somewhat severe headache this morning, lasting approximately 15 minutes and relieved by Tylenol,  occurring after waking from sleep, while he was still in bed.  This is the first time he has had such a headache.

Current medications: None.

Physical exam:  
Height: 166 cm          Weight: 65.4 kg         BP: 117/53              
Pulse: 80               U/L ratio:  0.94        Arm span: 166           
Breast: Tanner III      Pubic hair: Tanner IV   R. testis: 4.5 x 2.2 cm 
L. testis: 4.5 x 2.2 cm Phallus length: 10 cm   Mid-shaft diam.: 2.5 cm 
Upper/Lower Segment Ratio:  0.94
General:   
Skin: 
HEENT:  Obvious left optic atrophy on the left side, but the right eye looks entirely normal, with normal disk margins and pulsations.  
Neck: 
Chest:  Clear. 
Heart:   No murmurs.
Abdomen:  
Genitourinary:  Normal and quite pubertal. 
Extremities: 
Neurological:
Bone age: 14 years


Impression: With this bone age, it appears he has a predicted height of approximately 71", which is an adult height which is consistent with "mid parental" based on the heights of his parents.

Several weeks ago he had a DHEAS level of 85 mcg/dL, which is normal and the morning cortisol was slightly low at  7 mcg/dL.  An ACTH level was 14 pg/ml.

Disposition: I will be content to see him again in a year.
Thank you for letting us share in the care of Bruce.

Sincerely,


Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Nilto Carter, M.D.          RE: Bruce Scott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/21/81

Dear


CONTENT:
Macarthur Adler, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Adler:

We had the pleasure of seeing Bart in the Endocrine Program on 3/10/94 for craniopharyngioma.

History: Bart underwent surgery for craniopharyngioma in July of 1993, during which the pituitary stock was transected.   Ensuing complications have included obesity, panhypopituitarism and diabetes insipidus.   Since his last visit, Bart had one febrile illness but did not require stress dose steroid replacement.  Obesity continues to be a significant problem.  He was evaluated by a nutritionist who recommended an 800 k/cal diet, down from his current 1,100 k/cal per day.   The Verwoerd's recognize that this will be a difficult goal, and would like the nutritionist to provide more specific advice about how to plan his diet.  

Thyroid replacement is taken on a daily basis.  He appears to be more energetic and experiences less cold intolerance on the recently increased dosage of Synthroid.  Diabetes insipidus is treated with DDAVP before bed time, and extra doses as needed in the morning.   Evening dose typically lasts 12 to 20 hours.   Thirst mechanism is intact, as demonstrated by water craving when polyuria develops.

Current medications: Synthroid 0.0625 mg po qd, DDAVP 0.075-0.1 cc qhs intranasal, Hydrocortisone 2.5 mg po bid.

Physical exam:  
Height: 94.7 cm         Weight: 23.3 kg         BP: 90/60               
L. testis: 2 x 1 cm     Phallus length: 4.3 cm  Mid-shaft diam.: 1.5 cm 
General: Well appearing, obese.  
Neck: Without thyromegaly. 
Chest: Clear.
Heart: Regular rate and rhythm, no murmurs.
Genitourinary: Right testicle is undescended.  Left testicle in scrotum.

Laboratory studies:  (2/11/94):  T4 9.0, T3 resin uptake 1.17 (0.72-1.24), TSH <0.3 mcU/mL.

Impression: Bart is a 3 and 2/12 year old boy status post craniopharyngioma surgery, generally doing well.   The Verwoerds are attempting to limit Bart to an 800 k/cal diet, though specific advice and guidance from the nutritionist would probably be appreciated.  Linear growth continues to appear good, despite almost certain growth hormone deficiency.   Provocative neuro-endocrine testing and growth hormone replacement will be deferred until growth rate becomes inadequate.  

Diabetes insipidus is adequately treated with DDAVP, but an additional dose of 0.025-0.05 cc in the morning may allow Bart to get through the day with less difficulty.  The importance of a breakthrough period of diuresis lasting at least two hours, in every 24 hour period, has been stressed to the parents.   Bart is currently clinically euthyroid, though repeat thyroid function testing would be recommended in one month since Synthroid dose was recently increased.   Should Bart experience significant stress (car accident, surgery, serious infection, etc.), stress dose steroid would be required at a dosage of 5 mg po q6 hours.    Finally, we have referred Bart to Urology for work up of an apparent undescended right testicle.

Disposition: We would like to see Bart back in clinic in four months.
Thank you for letting us share in the care of Bart.

Sincerely,


Gleide Crane, MD             Penelope Hagen, MD              
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Antonio Lear, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Lear:

We had the pleasure of seeing Bart in the Endocrine Program on 7/21/94 for follow-up of craniopharyngioma.

History: Bart is a 3 and a half year old status post craniopharyngioma operated upon July 1993.  His DI needs better DDAVP regiment to allow sleeping through the night without enuresis, but breakthrough during the next day.  His pediatrician could not feel his right testicle.  His major problem remains his obesity and hyperphagia.  He was recently seen by an ophthalmologist (May 1994) with stable but decreased acuity.  MRI May 1994 showed no change in area of the tumor.

Current medications: Synthroid 0.0625 mg daily, hydrocortisone 2.5 mg bid, DDAVP 0.05 mg qhs.

Physical exam:  
Height: 98.3 cm         Weight: 26.0 kg         BP: 90/60               
Pulse: 100              U/L ratio:  1.14        Arm span: 96            
Pubic hair: Tanner I    

Genitourinary: Left testis easily palpable in squatting position.  Right testis small (~5 mm) in sac, slightly granular in texture.

Laboratory studies:  T4 11.93 (4.5-12), TBGI 1.07 (4/94), TSH <0.3.

Impression: 1.  Major current problem is obesity.  He continues to grow well, possibly without growth hormone secretion.  I emphasized the need to limit oral intake to the parents.  
2.  I would give DDAVP 0.05 ml at bedtime, and 0.025 ml during the day as needed, with breakthrough diuresis after each dose.  
3.  Re-evaluate right testis on his next exam.  4.  Consider full neuroendocrine work-up in ~1 year.

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

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Antonio Lear, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Lear:

We had the pleasure of seeing Bart in the Endocrine Program on 2/22/95 for craniopharyngeoma.

History: On 2/22/95, Bart had testing for growth hormone and cortisol deficiency.  Following adequate hypoglycemia (nadir 35 mg/dL), there was no rise in either growth hormone or cortisol, both which remained below the limit of assay detection.

Physical exam:  



Impression: Based on this, Bart may require growth hormone therapy.  Even though his linear growth is adequate at this point, his obesity may in part be due to growth hormone deficiency.  Regarding cortisol deficiency, he is currently doing well without cortisol replacement, although his parents have been instructed in giving it during times of stress.  His diabetes insipidus is better controlled on the spray form of DDAVP.  I will discuss these issues when I see Bart later this month.

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

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Macarthur Adler, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Adler:

We had the pleasure of seeing Bart in the Endocrine Program on 3/23/95 for craniopharyngeoma.

History: Bart is a 4 and 2/12 year old boy with craniopharyngioma, seen 2/22/95 for growth hormone stimulation.  Following adequate hypoglycemia, he had no detectable rise in growth hormone or cortisol.  For the past 3 months he has been off of cortisol.  His energy level may be slightly less off of cortisol, and his appetite is also decreased.

Current medications: DDAVP 10ug/spray,  two puffs in afternoon, Synthroid 0.0625 mcg daily.  Off cortisol for three months.

Physical exam:  
Height: 103.7 cm        Weight: 29.2 kg         BP: 90/60               
Pulse: 80               Phallus length: 5.5 cm  Mid-shaft diam.: 1.4 cm 
General:  Obese, serious boy, no distress.  

HEENT:  Right eye exotopia.

Genitourinary:  Testes bilaterally palpable, less than 1 cm. length, difficult to feel.

Impression: 1.  He is gaining less weight off of cortisol, but may be more tired.  I will ask the parents to monitor his activity (amount of sleep, level of physical activity) before making another change in cortisol dosage.

2.  Growth hormone deficiency -- adequate growth rate but may be due to obesity and high insulin.  His obesity might improve on growth hormone therapy.

I will discuss treating with growth hormone or GRF, as well as doing CRH/GRF/ACTH stimulation test with Dr. Adler and parents.

Disposition: Parents will call me next week to discuss.  Return to clinic in three months.  Whether he might benefit from GRF therapy is of importance, since there may be a lower risk of second malignancy or possibly tumor recurrence with GRF, given that GH levels are likely more physiologic with this form of therapy.  It has just been approved by the FDA, and I will look into its availability.
Thank you for letting us share in the care of Bart.

Sincerely,


 , MD


CONTENT:
Macarthur Adler, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Adler:

We had the pleasure of seeing Bart in the Endocrine Program on 6/15/95 for follow-up of craniopharyngioma.

History: Bart has done well.  He has had two episodes of rectal bleeding.  He was scheduled for an evaluation on 6/16/95 in the GI division at Children's.   His parents and I have discussed rhGH therapy for control of weight and metabolism.  He has started taking one nap day daily while off of cortisol treatment. 

He also needs protocol to be written for school, which he will be starting in the fall (medications, etc).

Current medications: 
Synthroid 0.0625 mg daily,  DDAVP 10 mcg at night, 2.5 mcg in a.m.  Still off cortisol.

Physical exam:  
Height: 106.6 cm        Weight: 38.0 kg         BP: 80/60               
Arm span: 107           Breast: Tanner I        Pubic hair: Tanner I    
General:  obese.
Genitourinary: testes not palpable.

Impression: 1.  GH deficiency.  Growing at a normal rate,  but massively obese.  Growth hormone therapy may improve this.
2.  Cortisol deficiency -- because of fatigue, we should re-start at 2.5 mg daily.

Disposition: 1.  Restart hydrocortisone at 2.5 mg q a.m.
2.  Petition HCHP re-starting GH therapy.
3.  Return to clinic in three months.
Thank you for letting us share in the care of Bart.

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Bernardo Red, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear


CONTENT:
Brehane Matlock, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Matlock:

I saw Bart in the Endocrine Program on 1/25/96 for follow-up of craniopharyngioma and growth hormone deficiency.

History: The patient was last seen on 09/26/95, at  which time he was started on human growth hormone Humatrope 0.025  mg/kg/day which amounts to 0.75 mg a day.  He has done well since that  time, with a significant increase in height above the growth track that  he had been on and with an increase but not commensurate of weight, so  that he has become slightly more lean with respect to his height.  His  appetite is unchanged from before, although he may have been more active  up till recently, when his parents report that he is perhaps less  active.  In addition, the patient has been doing well in his school,  with a substantial amount of support from ancillary helpers both in  physical activities as well as in cognitive function.

Current medications: Humatrope as above, Synthroid 0.0625 mg a day,  hydrocortisone 2.5 mg in the morning and DDAVP 2.5-10 mcg b.i.d.,  depending on urine output and thirst.

Physical exam:
HT: 113.8 cm    WT: 32.5 kg    Arm span: 116 cm    U/L ratio:  1.11
BP: 90/60               Pulse: 88               Breast: Tanner I        
Pubic hair: Tanner I    Phallus length: 6 cm    Mid-shaft diam.: 1.5 cm 
General appearance: slightly less obese than before, but still moderately so, and otherwise no change from prior exam.  Testes are both palpable and prepubertal, but were not measurable due to their retractile nature.

Laboratory studies:   Prior laboratory studies from 12/18/95 include a T4  of 7.4, T3 resin uptake of 27.4 and a TSH of 0.01.

Impression: 1.  Growth hormone treatment: The patient appears to be somewhat more  lean after four months of growth hormone therapy.  His height appears  to have increased above the growth track that he has been on prior to  institution of therapy.  Synthroid dose has been increased to 75 mcg a  day because of the somewhat low TFTs associated with his feeling of  tiredness.  In addition, I have increased cortisol to 2.5 mg twice a  day, again because of his known cortisol deficiency and fatigue.  He is  to come back in six months, and we appreciate your consultation with us.



Sincerely,




Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Brehane Matlock, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Matlock:

I saw Bart in the Endocrine Program on 6/19/96 for obesity.

History: Bart underwent surgery for craniopharyngioma in July 1993, complicated by reported resection of the pituitary stock.  He subsequently develped panhypopituitarism, requiring growth hormone treatment, beginning September, 1995.  Last April, the dose of glucocorticoid replacement was increased because of a seizure attributed to hypoglycemia.  Otherwise, he is generally doing well, with good strength, normal sleep pattern, no cold intolerance, and no constipation or diarrhea.   

Excessive weight gain was first noted shortly after craniopharyngioma surgery.  He is now described as always hungry, waking at 6:00 a.m. wanting breakfast.  a typical day's diet is described as:  cold cereal, skim milk for breakfast; banana for morning snack; bread, peanut butter with cheese, pretzels and fruit for lunch; crackers and skim milk for afternoon snack; meat, pasta, carrots, salad, and frozen yogurt for dinner.  In addition, he drinks about one cup of fruit juice per day.  He watches two hours of television per day and has a very sedentary life style.

Current medications: Synthroid 0.075 mg per day, hydrocortisone 5 mg PO b.i.d., growth hormone 0.75 mg per day, and DDAVP intranasally.

Past medical history:  As described above, partially blind in the right eye following craniopharyngioma surgery.  Normal pregnancy, labor and delivery, birth weight 9 pounds 2 ounces, length 22 inches.

Physical exam:
HT: 118.9 cm            WT: 37.1 kg
BP: 99/52               Pubic hair: Tanner I    
Appearance:  significant, generalized obesity, no buffalo hump, no moon facies.  Skin without striae, warm and dry.  HEENT examination showed right eye with partial blindness, neck without thyromegaly, lungs clear.  Cardiac regular rate and rhythm, no murmur.  Abdomen without hepatosplenomegaly.  GU normal pre-pubertal male.   Extremities normal fourth metacarpal.  Neurologic, normal strength and tone.

Laboratory studies:  12/18/95 include a T4 at 7.4 mcg/dl, and a TSH of 0.01 mcu/ml.

Impression: Bart is a 5-5/12 year old boy status post craniopharyngioma surgery, now with panhypopituitarism and progressive obesity.  His diet is very heavy in sweet and starchy foods, complicated by an underlying picture of hypothalamic obesity.

Disposition: The risks of uncontrolled obesity, including cardiovascular disease, diabetes mellitus, musculoskeletal problems, and psychosocial issues were explained.  The nature of a well-balanced diet was discussed in detail.  In particular, Bart should decrease consumption of sweet and starchy foods, increase fruits and vegetables, and eat moderate amounts of protein and (monounsaturated) oils.  Furthermore, increased exercise and decreased television viewing will contribute to the overall program of weight management.  Follow-up is recommended for three months. 

Thank you for referring the Verwoerd's to our obesity clinic.


Sincerely,






Gleide Crane, MD, PhD             
Attending in Endocrinology   

cc: Joseph Majzoub, MD, Endocrine Division, Children's Hospital


CONTENT:
Brehane Matlock, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Matlock:

I saw Bart in the Endocrine Program on 7/11/96 for craniopharyngeoma.

History: Bart was last seen in Endocrine Clinic on  01/25/96.  Since that time, he has been admitted in  April of this year to Children's Hospital for a hypoglycemic seizure.  Since that time,  hydrocortisone has been increased to 5 mg b.i.d. and his parents have  been told to triple his dose of hydrocortisone should he become febrile or unable to eat.  He also has been given a Medi-Alert bracelet detailing his medications.  He was seen by Dr. David Ludwig in our Obesity Clinic for evaluation of obesity.  Dr. Ludwig recommended a low carbohydrate diet higher in protein and fat.  The patient is having trouble adhering to this, principally because his parents find a lack of flexibility and choice in the diet.  I gave the parents some additional suggestions for foods that would conform to this diet.  His activity is  still not as great as we would like, but he is trying to use his  bicycle.  Growth hormone treatment has been reapproved for the following  six months and he is otherwise doing well.

Current medications: Synthroid 0.075 mg a day, hydrocortisone 5 mg a.m./5 mg  p.m.  Recombinant human growth hormone 0.75 mg per day.  DDAVP 0.2 mg PO  b.i.d.

Physical exam:  
HT: 118.4 cm    WT: 37.6 kg      BP: 105/63        Pulse: 87               
Physical examination unchanged from before.   Pre-pubertal.

Laboratory studies:  No studies ordered.  Will repeat thyroid  function studies in six months.

Impression: Doing well.  The patient needs to attempt to adhere better  to his diet and continue present medications.

Disposition: Return to clinic in six months.



Sincerely,




Bernardo Red, MD           
Chief, Division of Endocrinology

cc:  David Ludwig, M.D.    Endocrinology  Children's Hospital  

     R. Michael Scott, M.D.  Department of Neurosurgery  Bader 3  
     Children's Hospital  300 Longwood Avenue  Boston, MA 02115

     James Verwoerd, 65 Lawton Rd   Shirley , MA  01464


CONTENT:
Brehane Matlock, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Matlock:

I saw Bart in the Endocrine Program on 12/18/96 for obesity.

History: Bart's past medical history is notable for craniopharyngioma, S/P surgical resection.  He currently has panhypopituitarism, and is receiving hormone replacement therapy.   He was initially evaluated in the obesity clinic on 6/19/96.  Interval health has been good.  His weight appeared to remain stable through the fall, though he has put on a few pounds in the last couple of months.  His appetite remains essentially unchanged, still being very  hungry in the afternoon.  Encouragingly however, his mother notes an increase in pants length, but not waist size.  Also of note, both  parents have lost weight on the prescribed diet.  A typical day's diet  includes toast and egg, milk or yogurt (sweetened with NutraSweet) for  breakfast, tuna, vegetable, and fruit for lunch, a snack of fruit,  yogurt, with cheese and crackers at 1:30 p.m., another snack of apple  and peanut butter, etc., at 3:30 p.m., and meat and vegetables for  dinner.  A bedtime snack of frozen yogurt is sometimes given.

Current medications: Synthroid, growth hormone, DDAVP, and hydrocortisone.

Physical exam:  Deferred.  HT: 123.3 cm    WT: 40.6 kg

Impression: Bart is a 5 year 11 month old boy, S/P  craniopharyngioma resection with obesity, showing a moderate response to  a low glycemic index diet.  His weight has increased 3.5 kg in the past  six months, but this interval has been characterized by rapid linear growth (annualized to 8.8 cm) on growth hormone therapy.  Encouragingly, his mother reports no  increase in waist size, suggesting conversion of body fat to muscular tissue.

Disposition: Continue generally as previously recommended.  Bart should try to eat a larger lunch and avoid snacking in the early afternoon.   Snacks at 2:30 and 5:00 are acceptable, if limited to low glycemic index foods.  Finally, it was emphasized that obesity is likely to be a  life-long problem for Bart, necessitating a long-term approach to dietary therapy.  Follow-up is recommended for six months.


Sincerely,



Gleide Crane, MD, PhD             
Attending in Endocrinology   


cc: Joseph Majzoub, M.D.,   Chief,  Division of Endocrinology  Children's Hospital


CONTENT:
Macarthur Adler, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Adler:

We had the pleasure of seeing Bart in the Endocrine Program on 8/26/93 for craniopharyngioma.

History: Bart was diagnosed with a craniopharyngioma in July, 1993 after presenting with polydipsia and failure to thrive. He underwent surgical resection, during which a  pituitary stalk was sectioned.  He completed a perioperative course of Decadron in early August.  Diabetes insipidus is controlled with PRN DDAVP, each dose lasts 6-18 hours, and parents allow a 2-4 hour breakthrough period before each dose. Obesity remains a concern, but appetite has diminished somewhat after discontinuing Decadron. Thyroid medication is taken regularly and energy level is good.

Current medications: DDAVP 0.05 mL nasal PRN. Hydrocortisone 5 mg morning, and 2.5 mg evening, Synthroid 37.5 mcg q day.

Family history:  Mother's height 5'5". Father's height 5'9".

Physical exam:  
Height: 90.1 cm         Weight: 17.4 kg         BP: 90/53               
Pulse: 110              Pubic hair: Tanner I    
General: Obese, mildly developmentally delayed, pleasant.  Skin: Without lesions, warm and dry.  HEENT: PERRL, EOMI.  Neck: Thyroid normal. 
Chest: Clear.  Heart: Regular rate and rhythm with no murmurs.  Abdomen: Without hepatosplenomegaly.  Genitourinary: Normal prepubertal male. Extremities: Without clubbing, cyanosis or edema. Neurological: Nonfocal.

Laboratory studies:  7/27/93 TBGI 0.92, T4 5.3 mcg/dL, Total T3 64 ng/dL, TSH < 0.05 mcu/mL.  Current (from your lab):  TSH 0.2 mcU/mL, T4 8.3 mcg/mL.

Impression: Bart is a 2 and 7/12 year old boy status-post craniopharyngioma resection now with panhypopituitarism on hormone replacement. Diabetes insipidus appears reasonably well controlled. We have encouraged the Verwoerd's to strive for regular qhs DDAVP dosing. Obesity is improving off Decadron, but we will considering lowering replacement hydrocortisone dose if still a problem at the next visit.  Growth over the past two months has been negligible, also probably attributable to Decadron treatment. Although John is most probably growth hormone deficient, many patients with craniopharyngioma obtain adequate growth without replacement therapy. Should interval growth prove to be inadequate over the next 6-12 months the status of his growht hormone secretion will be evaluated.

Disposition: We would like to see Bart back in clinic in three months.  Thyroid function tests, IGF-1 and prolactin should be rechecked at the end of September.
Thank you for letting us share in the care of Bart.

Sincerely,


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


CONTENT:
Macarthur Adler, M.D.          RE: Bart Verwoerd
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/18/91

Dear Dr. Adler:

We had the pleasure of seeing Bart in the Endocrine Program on 12/6/93 for craniopharyngioma panhypopituitarism..

History: Bart underwent craniopharyngioma resection in July 1993, with sectioning of the pituitary stalk.    He completed a peri-operative course of Decadron in August.  Interval history is notable for progressive obesity.   Recent MRI of the head is said to show no residual tumor.    

The obesity is associated with a large appetite, but hunger is more easily controlled now that Decadron has been stopped.   He takes frequent snacks, but mother tries to avoid excessive fats, etc.  He has not received a nutrition evaluation since undergoing surgery.   Diabetes insipidus seems adequately controlled with DDAVP 0.1 cc qhs.   Each dose lasts 14 to 18 hours, and thirst mechanism is intact as demonstrated by avid thirst when diuresing.   Thyroid medication is taken regularly, energy level remains variable and he tires easily.   There is no constipation or cold intolerance.

Current medications: Synthroid 0.05 mg qd, Hydrocortisone 2.5 mg bid, DDAVP 0.1 cc qhs.

Physical exam:  
Height: 92.3 cm         Weight: 22.9 kg         BP: 100/80              
Pubic hair: Tanner I    Phallus length: 4.2 cm  Mid-shaft diam.: 1.6 cm 
General: Morbidly obese, well appearing and pleasant.     Skin:  Without striae, warm and dry.     HEENT:  PERRL, EOMI  Neck:  Without thyromegaly. Chest:  Clear.    Cardiac:  Regular rate and rhythm with no murmurs.  Abdomen:  Without hepatosplenomegaly.  GU:  Normal male, testes descended bilaterally.    Extremities:  No clubbing, cyanosis or edema.   Neurologic:  Nonfocal.

Laboratory studies:  Prior laboratories (10/27/93 at Acton Medical Associates) T4 7.8 mg/dL, TSH 0.0, T3 uptake 1.07.

Impression: Bart is a 2 and 10/12 year old boy with panhypopituitarism status post craniopharyngioma surgery.    Linear growth remains normal without growth hormone replacement, despite presumed growth hormone deficiency.    Obesity now constitutes his major medical problem.   We recommend a careful calorie count, strict calorie restriction, and a formal evaluation by a nutritionist.    Diabetes insipidus is well controlled with qhs DDAVP.   We have explained to the mother that a second dose of DDAVP can be used during the day as needed, provided that two to three hours of breakthrough diuresis is allowed.   Bart remains euthyroid, but thyroid function tests should be checked on a regular basis, approximately every four months.    The Hydrocortisone dose of 2.5 mg bid appears appropriate at the current time.    

Lastly, a formal neuroendocrine evaluation by insulin tolerance test should be considered if linear growth becomes inadequate.

Disposition: Return to clinic in three to four months.
Thank you for letting us share in the care of Bart.

Sincerely,


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


CONTENT:
McPherson, M.D.          RE: Eric Raeburn
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/27/73

Dear Dr. McPherson:

We had the pleasure of seeing Eric in the Endocrine Program on 8/12/93 for evaluation of hormonal imbalance.

History: Eric is a 19 and 3/4 year old young man with a history of depression since age 16.  H was the 7 lbs. 6 oz, 21" product of an uncomplicated pregnancy and delivery to a 28 years old mother.  No problems during his first year of life.  He had normal developmental milestones.  HE started school at 5 years and did well.  During his sophomore years, Eric began losing weight intentionally after experiences adverse comments by friends.  He lost 25 lbs. to the point where he was significantly underweight for height and growth ceased.  His school performance also fell off.  He resumed eating and began weight training approximately one year later with good muscular development but no further growth in height.  At about this time he also began experiencing moodiness, emotional lability, loos of spontaneity without drugs, alcohol at that time.  After graduating from high school, he went into counseling and this year began seeing an analyst and started treatment with Zoloft.  Eric was disappointed by not being accepted into the military school as his father had been.  Present, he feel fatigued frequently and occasionally experiences some chest discomfort akin to anxiety.

Current medications: Zoloft 50 mcg bid.  His compliance is good.

Review of systems:  GU:  notable for decreased libido.  He has normal erections and nocturnal emissions.  Neurologic: dysphoric.

Family history:  Mother's height is 5' 1/4" and had normal menarche.  Father's height is 5'10" and also had normal puberty.  He has a brother with ectopic thyroid.  No other thyroid/hormone disorders.

Physical exam:  
Height: 165.2 cm        Weight: 63.7 kg         BP: 120/74              
Pulse: 55               
General: Muscular, well developed young man.
Skin: Normal temperature and texture.
HEENT: PERRLA, EOM's, fundi normal.
Neck: No goiter.
Chest: No gynecomastia.
Heart: No murmurs.
Abdomen: Benign.
Genitourinary: Normal adult male genitalia.
Neurological: CNN II-XII groslly normal.  Slight decerase in recovery at ankles.

Impression: Endogenous depression.  R/o hypothyroidism

Disposition: Return for follow-up as needed.

Laboratory studies pending at this time include: Thyroid function tests, urinalysis, CBC with differential, LH, FSH, testosterone.
Thank you for letting us share in the care of Eric.

Sincerely,


Rahul Ibsen, MD                
Attending in Endocrinology


CONTENT:
Rosimeire Green, M.D.          RE: Margaret Russell
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/23/89

Dear Dr. Green:

     We had the pleasure of seeing Margaret in the Renal Program on 8/2/95 for hemolytic uremic syndrome.
     Margaret is now six years old. She was diagnosed with hemolytic  uremic syndrome and was hospitalized in July, 1993.  At that time she received  hemodialysis for about four days.  Since that time she has had no  residual problems and has had normal renal function.  Her mother reports  that she has been healthy and has had no recent illnesses.  She is  starting first grade in the fall.

Her current medications include:
     MVI with Fe 1 po q.d.

Margaret's physical exam was notable for:
     BP 117/62   P 102   R    T    HT 117.4cm   WT 24.5kg
     Mouth was clear. Neck supple, no adenopathy.  Lungs  clear.  CV: regular rate and rhythm without murmur. Abdomen: soft,  nontender, no hepatosplenomegaly or mass.  Extremities: no edema. 
     
     
A urinalysis revealed:
     SPEC GR UA  1.024           RBC/HPF     0-3        
     PH UA       6               WBC/HPF     0-2        
     BLOOD UA    Neg.            SQUAM EPI   0          
     PROTEIN UA  Tr              BACTERIA    Neg.  
     GLUCOSE UA  Neg.       

Laboratory studies at this time include:
          CR          0.4   MG/DL       MCH        25.7      PG (L)
          WBC        6.79   K/MM3       MCHC       31.4    G/DL    
          RBC        4.59   M/MM3       RDW        13.7       %    
          HGB        11.8    G/DL       HDW        2.61    G/DL    
          HCT.       37.5       %       PLT UNC     340   K/MM3    
          MCV        81.8      FL       

     Margaret is doing well.  She has normal blood pressure, normal urinalysis,  and normal renal function.  Her CBC is now normal and she has no need  for further iron supplementation. 

Margaret should be seen on a yearly basis either by you or in the renal clinic here and should have a blood  pressure, urinalysis, and creatinine evaluated at that time.

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

               Sincerely,


               Thilo Beaker, MD            Kinze Brando, MD
               Fellow, Pediatric Nephrology Attending, Pediatric Nephrology


CONTENT:
Torrence Bird, M.D.          RE: Fran Wotton
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/14/87

Dear Dr. Bird:

We had the pleasure of seeing Fran in the Endocrine Program on 9/20/93 for evaluation of premature puberty.

History: Fran is a 6 and 1/12 year old black girl referred for question of premature puberty.  According to her mother since birth she has had some breast development which never receded.  However, since age 3 1/2 the breast have been increasing in size.  Mother also noticed the growth of pubic hair since age 3 1/2.  There has not been any leukorhea or vaginal bleeding.  She has always grown above the 95th percentile.  

She does not take any medications.

Past medical history:  She is the product of a normal pregnancy, delivered by repeat Cesarean section with birth weight of 7 lbs. 14 oz.  She had several seizures at the age of 2 years which did not require hospitalization.  She does not have any illnesses.  She has never been hospitalized.

Review of systems:  Unremarkable.

Family history:  Mother is healthy and had menarche at 9 years of age.  Father is healthy.  Fran has a 16 year old sister who had menarche at the age of 13 and 11 year old brother, who is tall and has already started his puberty.  There is no known endocrine disease in the family.  Mother's height is 5'6" and father's height is approximately 6'.

Physical exam:  
Height: 129.9 cm        Weight: 37 kg           BP: 110/70              
Pulse: 80               Breast: Tanner II       Pubic hair: Tanner II   
Her growth curve replotted on the endocrine curve shows that she has always grown on above the 95th percentile but there has not been any recent acceleration.   General: Very well looking but overweight girl. Skin: Mongolian spots, two hypopigmented macules on her back and one hyperpigmented macule on her left thigh.  She had a few comedons on the forehead.  HEENT: Unremarkable. Neck: No thyromegaly. Chest: Lungs were clear. Heart: Normal S1, S2 no murmurs. Abdomen: Soft, no masses, no organomegaly. Genitourinary: Small amount of glandular tissue palpable on the left breast consistent with early Tanner II.  Most of the breast prominence was composed of fatty tissue.  Normal size clitoris and red vaginal mucosa, nonconsistent with estrogen effect.  The pubic hair was Tanner II covering the labia.  No axillary hair but has been noted to have body odor for some time. Extremities: Unremarkable. Neurological: Unremarkable.

Impression: Fran may have precocious puberty.  The breast examination was difficult as most of the breast prominent seemed to be fat.  

We will doe a bone age today and if the bone age is not advanced, we will see the patient again in three months and reassess her progress.  However, if the bone age if advanced we will then organize a GnRH stimulation test to differentiate between central vs. peripheral puberty.  We will keep you informed of further progress of this patient.

She will also need a screening test for intermediates of cortisol metabolism due to her advanced adrenarche.

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

Sincerely,


Ariel Adler, MD           
Attending in Endocrinology   


Addendum: Katia Melchuk, M.D.
Fellow in Endocrinology

Problem list to date:
IDIOPATHIC-SEXUAL-PRECOCITY  Start: 09/20/93  End:          Invalid: 

Transcribed by Kohane


CONTENT:
Babs Hagen, M.D.          RE: Ty Waterman
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/09/85

Dear Dr. Hagen:

Prince Almed is an eight year old boy brought along with his siblings for evaluation of his growth.  He is reported to be taller than his siblings and has been growing from year to year.  He had an uneventful past medical history with only minor illnesses.  The review of systems was negative.

On physical examination his weight was 131.6 cm and his weight was 30 kg.  He appeared healthy looking and well developed.  Skin and hair were normal HEENT negative.  Thyroid was not enlarged.  He had no lymphadenopathy. Lungs were clear.  Heart sound were normal, no murmurs heard.  Abdomen was soft, no tenderness, no hepatosplenomegaly.  Genitalia were Prepubertal. Both testes were descended.  Extremities were normal.  Neurologic examination was negative.

Impression:  Prince Ahmed is a normal eight year old boy with a normal height on the 75th percentile.

Sincerely yours,





Kendell S. Simpson, M.D.
Clinical Director


CONTENT:
Ibrahima Carerras, M.D.          RE: Naomi Wolsey
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/17/83

Dear Dr. Carerras:

We had the pleasure of seeing Naomi in the Endocrine Program on 10/28/93 for evaluation of precocious puberty.

History: Naomi is 9 years and 10 months old girl with a history of fetal hydantoin syndrome and  of failure to thrive between 10 months and two years.  She has  been growing and developing normally throughout the past years and following her growth curve along the 10th percentile.  Mother noted appearance of breast development and pubic hair approximately 1 1/2 years  ago.  Pubic hair has not increased much over the past year, and she has no axillary hair or body odor, and no menstrual bleeding.  Naomi was see at HCHP on September 23, 1993, and was felt to be in Tanner stage III for breast development and pubic hair.  Bone age was obtained that was read as 8 years 10 months, based on the appearance  of the epiphysis 10 years at a chronological age of 9 years, 9 months.   Naomi herself is not concerned about her height, only  about her short hands and feet.  Mother is not concerned by her height but by the fact that she is gaining more weight than height.

Current medications: None.

Past medical history:  Remarkable for the fetal hydantoin syndrome and failure to thrive which was managed by tube feedings.  The etiology of the failure to thrive was felt to be due to emotional stress brought about by the divorce of her parents.  She also has recurrent otitis media.

Review of systems:  Unremarkable.  She attends regular school and is doing well.

Family history:   Mother's height is 5'2", father's height is 5'8".  Naomi has one older sister who is 11 years old, 5' tall and had her menarche at age 10.  Mother had menarche at age 11.   Negative for any endocrine disorders.

Physical exam:  
Height: 239.4 cm        Weight: 35 kg           BP: 108/70              
Pulse: 78               U/L ratio:  2.52        Arm span: 123           
Breast: Tanner II       
(10th percentile, delayed maturation rate curve)  (above the 50th percentile)
General: Healthy looking young girl, in no acute distress.  Skin: No lesions.   HEENT: Unremarkable.  Neck: No thyromegaly.  Chest: Lungs are clear.  No axillary hair.  Heart: Regular rate and rhythm, normal S21, S2, no murmurs.  Abdomen: Soft, no hepatosplenomegaly.  Genitourinary:  Unremarkable.  Pubic hair is Tanner stage II to III. Vaginal mucosa is pinkish.  Extremities: Short fingers and toes, sausage-like in shape.  Neurological: Intact.  DTR's +2 with no delay in the relaxation phase.

Laboratory studies:  Bone age obtained of 8 years, 10 months at a chronologic age of 9 years and 9 months.

Impression: Naomi is in early puberty and has been following her growth curve.  Based on her present height  and mildly delayed bone age (8 years, 10 months), Naomi's predicted final height is 5'2" which closely corresponds to her mid-parental height of 5'2 1/2".  There is no evidence suggesting any endocrinopathy at the present time.  We will rule out thyroid disease or GH deficiency.

Disposition: We would like to see Naomi back in clinic in six months to reassess her growth.

Laboratory studies pending at this time include: Thyroid function tests and IGF1 levels.
Thank you for letting us share in the care of Naomi.

Sincerely,


Penelope Hagen, MD              
Attending in Endocrinology   


Addendum: Nada Kerouz, M.D.
Fellow in Endocrinology


CONTENT:
Clotilde Rolf, M.D.          RE: Andrew Torricelli
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/28/92

Dear Dr. Rolf:

We had the pleasure of seeing Andrew in the Endocrine Program on 8/3/95 for septo-optic dysplasia.

History: Andrew is a 3-5/12 year old male with bilateral optic nerve hypoplasia, dysgenesis of the corpus  callosum, a seizure disorder, pervasive developmental delay,  hypotonia, and genitourinary abnormalities, which have been  repaired.  He was born after 41 weeks of gestation,  after a fetal ultrasound demonstrated hydronephrosis and bladder distension.  He underwent status post bilateral  ureteral reimplantation in 12/92 and  repeat reimplantation  and megaureter repair in 12/93.  Delayed development was noted at 2 months of age because of  failure of head control and hypotonia.  In 6/94 he presented with a generalized tonic-clonic seizure in the context of an otitis media and  fever, and was felt at that time to have had a febrile seizure.   Two months later he presented in status epilepticus,  which lasted approximately 1  hour.  His work-up included a borderline abnormal EEG, and a head MRI which  revealed dysgenesis of the corpus callosum.  He had a normal karyotype  and routine electrolytes, lactate, pyruvate, and organic acid levels.   An ophthalmologic exam revealed  bilateral optic nerve hypoplasia, and  the patient was referred also for  possible DeMorsier's syndrome, or septo-optic dysplasia to rule out hypothalamic/pituitary dysfunction.

Current medications: Tegretol

Past medical history:  Mrs. Torricelli's pregnancy, labor and delivery were  unremarkable, and Apgar's were 8 at 1 minute and 9 at 5 minutes.  Andrew's growth  and development have been significantly delayed.  He rolled over at 10  months of age, sat up at 15 months of age, stood alone at 2 years, and  walked unassisted at 2-1/2 years of age.  Currently his gross motor skills are in the 12 to 15 month range,  expressive language abilities are at the 9 to 10 month range, and  comprehension is at the 15 to 18 months range.   His hospitalizations include two for his urologic  surgeries and a single hospitalization for croup.  He has sustained  injuries including right tibial fracture and left clavicle fracture,  both of which were repaired by closed reduction.

Review of systems:   History of early dentition, beginning at 2 months of  age.

Family history:  His parents are non-consanguineous and his family  history is significant for multiple family members with seizure  disorders, including maternal great-grandmother, maternal aunt, and mother, who was diagnosed with right temporal lobe seizures and who was  taking Dilantin during the pregnancy.  There is no history of fetal  ulcers, cardiac disease, or endocrine abnormalities.  Mother's height is  6'0", and had menarche at 13  years of age.  The father's height is 6'1/2".  Other significant family  history includes speech delay and a maternal cousin, with a seizure disorder.  Heights are  greater than 6' in all of the maternal aunts and uncles.  A paternal aunt and maternal grandmother have liver cirrhosis.

Physical exam:  
Height: 115.7 cm        Weight: 21.7 kg (height and weight >95%)    
Head circumference 55 cm    
Pubic hair: Tanner I    
Right testis: 1.9 x 1.5 cm     Left testis: 1.8 x 1.5 cm 
Phallus length: 5.8 cm         Mid-shaft diameter: 1.9 cm 
General: A large child who was minimally communicative
HEENT: Dysmorphic features include a prominent forehead and  flattened nasal bridge.  His pupils were equally round and reactive to  light, and extraocular movements were intact.   Tympanic membranes were  normal bilaterally.  His mucous membranes were moist and his palate was  intact.
Chest: Clear to auscultation.
Heart: Regular rate and  rhythm with no murmur.
Abdomen: Soft, nondistended, nontender, and no hepatosplenomegaly or masses.  He had normal active  bowel sounds and a midline umbilical and low transfer scars which were  well healed. 
Genitourinary:   Prepubertal male. 
Neurological: Alert and responsive with decreased tone throughout, proximally   more so than distally, 5/5 strength throughout.  His gait was  mildly ataxic.  His sensation was grossly intact as were his cranial  nerves II through XII.  Deep tendon reflexes were 2+ bilaterally, his  biceps and patella with downgoing toes and 1+ ankle jerks bilaterally.

Laboratory studies:  White count  6.7 with 1 band, 35 polys, 48 lymphs, 5 monos, 3 eos, 8 atypical  lymphs.  Hemoglobin=14.0 and hematocrit=41.7.  Platelet count= 296, and MCV =90.3.  His lactate was 1.4, pyruvate was 0.1, and the organic acid screen was normal.  Electrolytes were within normal limits, with a sodium=139, potassium =4.4, chloride=106,  bicarbonate=22, BUN and creatinine 15/0.4 and glucose=86.  His  calcium=9.4, phosphate=5.6 with a total protein=6.0 and an albumin=2.8.  His uric acid=2.4.  His bilirubin =0.4, alk phos=550, LDH=694, AST=52, ALT=82.  His urinalysis revealed a pH of 7.5 and specific  gravity of 1.015, otherwise dip negative.

Impression:  A 3-1/2 year old male with a seizure disorder, pervasive developmental delay, hypotonia,   a  midline CNS abnormality, bilateral optic nerve  hypoplasia,  GU abnormalities, and a  strong family history of seizure disorders, including maternal Dilantin  use during pregnancy.  History and exam are also significant for his  overall large size, early  dentition, and dysmorphic features.  Given the height of his parents', Andrew seems to have genetic tall stature.  He has no evidence for  hypothalamic pituitary dysfunction.  We recommended the following studies be obtained closer to home in Worcester: growth hormone IGF-1, TFT's, bone age, and a.m. urine specific gravity to rule out diabetes insipidus   We would like to see Andrew for follow-up in the Endocrine Clinic in six months.  Please fax (617-355-7588) or send us the results of the laboratory testing.

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


Sincerely,




Mitsuko Hagen, MD                        Penelope Hagen, MD              
Resident in Pediatrics              Attending in Endocrinology


CONTENT:
Etta Beaker, M.D.          RE: Doris Uhland
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/03/92

Dear Dr. Beaker:

We had the pleasure of seeing Doris in the Endocrine Program on 2/3/94 for failure to thrive.

History: Doris is a 17 month old with failure to thrive who I last saw three months ago.  At that time, she had a normall CBC, normal liver and renal functions and an ESR of 58.  Mom missed an appointment with a dietician but states that she has increased Doris's feedings in frequency and amount.  Developmentally, she is walking well and beginning to say single words.

Physical exam:  
Height: 67.5 cm         Weight: 6.23 kg         
General:   Alert, active child.
Skin: Normal turgor and texture.
HEENT: Head circumference:  44.5 cm.  Positive red reflex.
Neck: Thyroid not palpable.
Chest: Lungs clear.
Heart: Regular rate and rhythm, no murmurs.
Abdomen: Soft, nontender, no hepatosplenomegaly.
Genitourinary: Normal Tanner I female.
Extremities: 
Neurological:  DTR 2-3+/4+ bilaterally.

Impression: Doris is a 17 months old who is very short and thin.  She has grown 3 cm in three months but only gained 0.2 kg in this time.  I am very concerned about her nutritional status.  She may need a GI evaluation.

Disposition: Follow up at 2 years of age.

Laboratory studies pending at this time include: Bone age and ESR.
Thank you for letting us share in the care of Doris.

Sincerely,


Ariel Adler, MD           
Attending in Endocrinology


CONTENT:
Etta Beaker, M.D.          RE: Doris Uhland
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/03/92

Dear Dr. Beaker:

We had the pleasure of seeing Doris in the Endocrine Program on 11/05/93 for failure to thrive.

History: Doris is a 14 month old black girl with a history of failure to thrive.
Pregnancy, labor and delivery were normal; Doris was a full term infant who weighed 5 lb 8 oz and was 17 inches in length.

Her appetite has been poor, her mother discribed her as a "picky eater".  Diet has been as follows: 7am 8 oz juice, 8am cereal/Cheerios small jar of strained fruit/baby food.  6-7 oz whole milk.  Snack: Gerber pretzels, Lunch baby food jar/noodles. 6-7 oz milk Dinner: vegetables/rice/meat 6-7 ox milk.  Snack: Cheerios.

She is on no medications.

Past medical history:  Notable for only for being dropped as an infant without sequalae.

Review of systems:  Negative in detail.

Family history:  Maternal height 5' 1", paternal height 5' 3". A paternal aunt has asthma.  Paternal grandmother is 5'2", paternal grandfather is 5'6"

Physical exam:  
Height: 64.5 cm         Weight: 60.4 kg         Pulse: 88               
Doris was a very alert, interactive child.  She appeared very thin.  Skin: hypopigmented area near her left ear.  HEENT: Normocephalic, atraumatic. Neck:  Thyroid not palpable.  Chest:  Clear. Cor: Regular Rate and Rhythm, no murmur.  Abdomen: soft, non-tender, no hepatosplenomegaly, no masses.  GU: normal prepubertal.  Extremities: normal.  Neurological:  normal.

Laboratory studies:  (06/15/93) TSH 6.0, Pb 3.6,

Impression: Failure to thrive.  The primary problems appears to be a lack of po intake, whether due to a or lack of feeding appropriately or lack of knowledge about appropriate feeders.  I have encouraged parents to increase caloric intake.  we may need to consider diencephalic syndrome, however, she has also a decreased growth rate.

Disposition: Follow up in three months.

Laboratory studies pending at this time include: TSH, T4, and TBGI, SMAC, endomysial antibodies.  A nutrition consult at HCHP is highly recommended.
Thank you for letting us share in the care of Doris.

Sincerely,


Ariel Adler, MD           
Attending in Endocrinology   


Addendum: Laboratory results include: IGF-1 57 (nl), T4 11.8 (nl), TBGI 0.84 (nl), TSH 1.6 (nl), SMAC normal, ESR 58.  The endomyseal antibody is pending.


CONTENT:
Loocie Lear, M.D.          RE: Alexander Bruce
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/01/93

Dear Dr. Lear:

We had the pleasure of seeing Alexander in the Endocrine Program on 6/5/95 for .

History: As per the father, the patient was living with the mother but actually the court went to evaluate how he was doing and because they were concerned because he was malnourished, the court gave the son to the father so now he is under the care.  They also thought there was a problem with growth and therefore, it was suggested that he be evaluated for growth and short stature.  

At the time, he was very skinny and weak when he took care of him, now in the last two months he has gained a couple of pounds.  He is more active and is eating well.

Current medications: None.

Past medical history:  The pregnancy was full term, the delivery was C-section.  He was not breast feeding.  He is eating very well.  Immunizations are up to date.  No hospitalizations or operations.

Family history:  His father had some type of polio problem when he was born, some paralysis that he had surgery for.  Also, he had cleft palate.  Mother has epilepsy.

Physical exam:  
Height: 73.8 cm         Weight: 9.24 kg         BP: 90/60               
Pulse: 130              U/L ratio:  1.42        Arm span: 77            
General: He is a very active boy.  
Skin: Normal. 
HEENT: The anterior fontanelle is still open.  
Neck: There is no goiter.  
Chest: Lungs clear to auscultation.  
Heart: S1, S2, S3.  
Abdomen: Positive for hepatomegaly, 5 cm below the right costal margin.  
Neurological: Nonfocal.

Impression: The patient will be seen in three months for follow up.


Laboratory studies pending at this time include: ALB         3.4    G/DL       LYMPH-A    51.0       %    
ALKP        266     U/L       MONO-A      3.1       %    
CO 2         23   MEQ/L       EOS-A       5.1       %    
CA          9.4   MG/DL       BASO-A       .9       %    
CR          0.3   MG/DL       ATYP LY     3.9       %    
GLU          89   MG/DL       MICRO-A       +    
PHOS        4.8   MG/DL       HYPO-A        +    
AST          34     U/L       ESR          56   MM/HR (H)
ALT          12     U/L       SOURCE-  RANDOM    
BUN          12   MG/DL       APPEAR    CLEAR    
NA          136  MMOL/L       COLOR U   STRAW    
K           4.2  MMOL/L       SPEC GR   1.002 (L)
CL          104  MMOL/L       WBC ENZ     NEG    
WBC       10.42   K/MM3       NITRITE     NEG    
RBC        4.13   M/MM3       PH UA       6.0    
HGB        10.8    G/DL       PROTEIN     NEG    
HCT.       32.0       % (L)   GLUCOSE     NEG    
MCV        77.5      FL       KETONE      NEG    
MCH        26.2      PG       UROBIL      0.2    
MCHC       33.7    G/DL       BILI UA     NEG    
RDW        14.9       %       BLOOD U     NEG    
HDW        3.29    G/DL (H)   NO MICRMACRO OK    
PLT UNC     490   K/MM3 (H)   IGF-I        46   ng/mL    
NEUT AB    3.75   K/MM3       T4         13.2   ug/dL    
LYMPH A    5.31   K/MM3       TBGI        .84 (C)
EOS ABS     .53   K/MM3 (H)   TSH        2.00   uU/mL    
NEUT-A     36.0       %       
Thank you for letting us share in the care of Alexander.

Sincerely,


Maureen Shields, MD             
Attending in Endocrinology   


Addendum: Kenneth Shepard, M.D.


CONTENT:
Loocie Lear, M.D.          RE: Alexander Bruce
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/01/93

Dear Dr. Lear:

We had the pleasure of seeing Alexander in the Endocrine Program on 6/5/95 for failure to thrive.

History: As you know, Alexander is a now 2 year old who has been noted since approximately 14 months of age to exhibit poor weight gain.  Little past history is currently available.  Because of concerns of  inadequate food availability Alexander was recently placed in the care of his  father and over the past one month he has been reported to be gaining  weight increasingly well.  Recent evaluation has included normal thyroid  function tests, though linear growth rate remains attenuated.

Past medical history:  Alexander was the product of a full-term pregnancy,  delivered Caesarean section.  He has had no significant recent illness  or recorded hospitalization.

Family history:  Mother's height 5'0", father's height also 5'0" with a  history in the father of lower limb paralysis of unclear etiology.   Father also had cleft lip repair during childhood.

Physical exam:  
Height: 73.8 cm            Weight: 9.24 kg         
BP: 90/60                  Pulse: 130              
U/L ratio:  1.42           Arm span: 77 cm           
Pubic hair: Tanner I    
Right testis: 1 x 1 cm     Left testis: 1 x 1 cm     
Phallus length: 5.2 cm     Mid-shaft diameter: 1.3 cm 
Alexander was an active, though a quite thin appearing boy.  Skin texture was normal, turgor was good.   HEENT exam revealed small anterior fontanel, no thyromegaly.  There was  no lymphadenopathy.  Chest was clear to auscultation.  Cardiac exam  revealed regular rate and rhythm.  Abdominal exam was notable for  moderate hepatomegaly with liver edge 5 cm below the right costal  margin.  Neurologic exam showed 2+ and equal deep tendon reflexes with  normal relaxation phase.

Labs obtained in clinic today included normal electrolytes, BUN and  creatinine, random glucose was 89 mg/dl, phosphorus 4.8, calcium 9.4,  alkaline phosphatase 266, all within normal for age.  Albumin was 3.4  mg/dl.  Hematocrit was 32%.  Thyroid function tests were normal with TSH 2 mIU/ml.  T4 13.2 mcg/dl.  TBGI 0.84.  Of note:  Erythrocyte  sedimentation rate was markedly elevated at 56 mm/hr.  Liver function  tests were normal.

Impression: Alexander is a 2 year old with a history of malnutrition, by  report, and previous growth data also suggestive of malnutrition, with  weight loss occurring between 14 months of age and 18 months of age and  only recently increasingly gaining weight since in the custody of the  father.  This, no doubt, has contributed to Alexander's poor growth although  it is unclear whether this is the only etiology of his poor growth.   Thyroid function tests being normal and normal somatomedin-C level  obtained today are reassuring and we would like to continue to follow  Alexander to ensure catch-up growth occurs after continued adequate  nutritional replacement.  Of some concern is the elevated erythrocyte  sedimentation rate at 56 mm/hr, as I have discussed with you by phone,  suggesting possibly intercurrent or chronic infection which also may  account for his failure to thrive.  This will certainly require follow-up  in the future.

Disposition: We hope to see him again in approximately 3-4 months time.

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


Sincerely,




Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Loocie Lear, M.D.          RE: Alexander Bruce
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/01/93

Dear Dr. Lear:

I had the pleasure of seeing Alexander in the Endocrine Program on 9/25/95 for short stature and failure to thrive.  As you know, Alexander is a now 27-month-old who since his last visit has been in the continued care of his father.  He has had a sustained adequate nutritional intake by report of the paternal grandmother who reports him drinking 16 oz. of milk daily, taking 3 meals and constantly snacking.  He sleeps through the night without difficulty and exhibits a very good energy level.  The grandmother reports repeat laboratory evaluation since his last visit to Children's Hospital was obtained through the Chelsea office, which were unremarkable.  He has had no fever, vomiting or diarrhea since his last visit.

Current medications: None.

Physical exam:
Ht: 78.4 cm    Wt: 10.3 kg    U/L ratio:  1.18    HC: 48 cm
BP: 94/palp             Pulse: 92               Pubic hair: Tanner I    
Phallus length: 5.4 cm  Mid-shaft diam.: 1.5 cm 
Alexander was a well-appearing active boy with exam notable for normal skin texture and turgor.  HEENT exam revealed intact extraocular movements.  Pupils equal and responsive.  Neck was without thyromegaly.  Chest was clear to auscultation.  Cardiac exam showed regular rate and rhythm.  Abdomen was soft, nontender, nondistended.   Liver edges 1 cm. below the right costal margin.  GU: Tanner stage 1.  Neurologic exam showed 2+ and equal deep tendon reflexes with normal relaxation phase.

Laboratory studies:  Laboratory evaluation at the time of his last visit included a moderately elevated erythrocyte sedimentation rate of  56 mm./hr., an IgF-1 in normal range at 46 ng/ml.  Thyroid function tests were also normal at that visit.

Impression: Alexander exhibits continued short stature though an adequate interval growth rate with evidence of some catch-up growth.  He has also had appropriate weight gain since his last visit.  We will obtain no further studies today assuming follow-up of the elevated erythrocyte sedimentation rate was done through your office.  We would like to see him again in 4 months' time to ensure adequate catch-up growth continues but are obtaining no  studies  currently.



Sincerely,




Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Loocie Lear, M.D.          RE: Alexander Bruce
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/01/93

Dear Dr. Lear:

I saw Alexander in the Endocrine Program on 2/2/96 for failure to thrive short stature.

History: Since his last visit, Alexander has overall been healthy, but did develop a fever two days ago.  He has had no cough, vomiting, or diarrhea with this episode, but his mother reports a similar illness with her the previous week. 

He is currently continuing in the care of his grandmother primarily, though is with his mother on weekends.  His mother reports today that he is speaking more than previously in his Speech Intervention Program.

Current medications: Tylenol p.r.n.

Physical exam:
HT: 82.2 cm    WT: 11.2 kg
BP: 88/        Pulse: 84               R. testis: 1.6 x 0.8 cm 
L. testis: 1.6 x 0.8 cm Phallus length: 5.4 cm  Mid-shaft diam.: 1.5 cm 
On physical examination today Alexander was a generally well appearing boy.  Skin texture was normal.  Turgor was good.  HEENT exam revealed erythematous tonsils and shoddy cervical adenopathy.  Neck was without thyromegaly.  Heart:  regular rate and rhythm.  Abdomen was soft without hepatosplenomegaly or mass.  GU exam was Tanner Stage I.  Neurologic  exam:  2+ and equal deep tendon reflexes.

Impression: Alexander continues to exhibit good catch up in weight gain and  improving linear growth.  On exam today he does have an injected oropharynx, though is currently afebrile.  To this end, I have obtained a throat swab which the mother is bringing to your office to evaluate for possible Strep pharyngitis.  We would like to see Alexander again in 6 month's time to again evaluate his growth and weight gain.   

Thank you for allowing us assist in Alexander's care.



Sincerely,




Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Pavarotti, M.D.          RE: Martha Terborch
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/06/83

Dear Dr. Pavarotti:

     We had the pleasure of seeing Martha in the Renal Program on 11/22/95 for hemolytic uremic syndrome.
     Martha has done well since her last visit with us.  She has had a normal activity level and says she is doing well in school.  She has no new complaints.

Her current medications include:


Martha's physical exam was notable for:
     BP 108/47   P 85   R 20   T    HT 148.8cm   WT 40kg
     General appearance: no acute distress.  
     HEENT:  moist mucous membranes without exudate or masses.  
     Lungs: clear.  
     Heart:  regular rate and rhythm without murmurs, gallops or rubs.  
     Abdomen:  soft, nontender, nondistended without bruits or masses palpable.   Extremities: warm and well-perfused without clubbing, cyanosis or edema.   
     Skin: unremarkable.  
     Neurological: nonfocal.
A urinalysis revealed:
     SPEC GR UA  1.016           WBC/HPF     N          
     PH UA       7               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               WAXY CAST   N          
     RBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:
          CR          0.6   MG/DL       

     Martha has had an excellent recovery from her hemolytic uremic syndrome nearly a year-and-a-half ago.  She continues to have normal renal function and seems to have  no residual effects from her HUS.  The Terborchs inform me that they are planning to move near  Philadelphia over the next summer.  We will arrange for transfer to a pediatric nephrologist at the Children's Hospital in Philadelphia at that time.

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

               Sincerely,


               Conrado Seinfeld, MD         Clotilde Goldwater, MD
               Fellow, Pediatric Nephrology Director, Pediatric Nephrology


CONTENT:
Steve Chef, M.D.          RE: Martha Terborch
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/06/83

Dear Dr. Chef:

We had the pleasure of seeing Martha in the Renal Program on 7/17/96 for hemolytic uremic syndrome.
Martha has done well since her last visit with us in  December 1995.  She has had excellent weight and height gain over that period  of time.  She has had no hematuria.



Martha's physical exam was notable for:
     BP 107/56   P 85   R    T    HT 154.2cm   WT 43.5kg
     General appearance: in no acute distress.  
     HEENT:  moist mucous membranes without exudates or masses.  
     Lungs: clear.  
     Heart:  regular rate and rhythm, without murmurs, gallops or rubs. 
     Abdomen:  soft, nontender, nondistended, without masses or bruits.  
     Extremities:  warm and well perfused without cyanosis or edema.  
     Skin: unremarkable.   
     Neurological: nonfocal.
A urinalysis revealed:
     SPEC GR UA  1.018           WBC/HPF     -          
     WBC ENZ UA  -               RBC/HPF     -          
     PH UA       6               WBC CAST    -          
     PROTEIN UA  tr              RBC CAST    -          
     BLOOD UA    -               GRAN CAST   -          
     GLUCOSE UA  -               WAXY CAST   -     
     BACTERIA    -               SQUAM EPI   -     

Laboratory studies at this time include:
          CR          0.6   MG/DL       

Martha has had an excellent recovery from her hemolytic  uremic syndrome.  She has no sequelae at this point, and her renal  function has remained stable over the past year and a half. 

Martha's family is moving to Pennsylvania over the summer - she will be followed up there.  I am  forwarding a summary of her medical course to Dr. Bernard Kaplan, Chief,  Division of Nephrology at Children's Hospital of Philadelphia.  She should continue to  have yearly follow up visits with a pediatric nephrologist into  adulthood.

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

               Sincerely,


               Conrado Seinfeld, MD         Mitsuko Khan, MD
               Fellow, Pediatric Nephrology Director, Clinical Services


CONTENT:
Elle Steinbeck, M.D.          RE: Andie Gerome
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/93

Dear Dr. Steinbeck:

     We had the pleasure of seeing Andie in the Renal Program on 4/21/94 for evaluation of renal dysplasia, vesico-ureteral reflux and obstructive uropathy.
     Andie is a six month old who was noted to have bilateral hydronephrosis on prenatal ultrasound.  Evaluation at one month of age revealed bilateral ureteral-pelvic junction obstruction, left worse than right, bilateral vesico-ureteral reflux, and right renal dysplasia.  She had renal insufficiency with a creatinine of 1.1 and a nuclear medicine study showed that 77% of her renal function was in her left kidney.  On December 12th, Andie underwent bilateral pyeloplasties with a subsequent improvement in renal function.  Her most recent creatinine on February 2nd, was 0.4.  Follow-up renal ultrasound and IVP in February showed no contrast material in her right kidney and disparate renal sizes with the left kidney measuring 5.8 cm and the right 3.1 cm.  The collecting system of the left kidney was ectatic but the anastomotic site was widely patent.  Andie has had persistent problems with renal tubular acidosis with her most recent bicarbonate level being 15.  She is receiving Amoxicillin prophylaxis as she continues to have vesico-ureteral reflux and is scheduled to have follow-up evaluation of her kidneys by Jim Mandel, M.D. in October.
     On review of her past medical history, Andie was the six pound one ounce product of a full term pregnancy that was complicated by bleeding at seven and a half months.  This bleeding was the basis for the prenatal ultrasound.  She was delivered by normal spontaneous vaginal delivery and had no perinatal problems.  Aside from her urologic surgery, Andie has been doing well.  She currently is eating a combination of fruits, vegetables and formula.  She is babbling and is able to sit for brief periods of time unsupported.  Her immunizations are up to date.  There is no family history of kidney disease, hypertension or diabetes.  Andie has two siblings, a six year old sister and two year old brother.  Her brother has a history of pyeloric stenosis and chronic otitis media requiring PE tubes.  Her mother is five feet tall and her father is five feet eight inches.

Current medications include:
     AMOXICILLIN 2 ml q.d.
     BICITRA 4  ml  t.i.d.

Physical exam:
     BP 53/41   P 114    R 44    HT 62.5cm   WT 6.84kg  HC  42.5cm
     General: Alert, well appearing infant in no apparent distress
     Skin: No lesions
     HEENT: No evidence of infection
     Neck: Supple, no palpable adenopathy
     Chest: Lungs clear with good aeration
     Heart: Regular rhythm without murmur
     Abdomen: Well healed surgical scars.  Soft non-tender, without 
     palpable masses or organomegaly.
     Genitourinary: Normal female external genitalia
     Extremities: Normal form, no edema
     Neurological:No focal findings

Urinalysis:
          SPEC GR UA  1.005                PH UA       9          
          PROTEIN UA  N     mg/dl          GLUCOSE UA  N     mg/dl
          BLOOD UA    N                    RBC/HPF     N     /HPF 
          WBC/HPF     rare  /LPF           CASTS  N
          TUBULAR EPITHELIAL CELLS  positive

     Andie is a six month old with vesicoureteral reflux status post bilateral pyeloplasties with probable non-functioning right kidney.  She has mild renal insufficiency and acidosis.  At this time one cannot accurately predict the course that her renal function will follow.  If the left kidney is normallly formed and grows approriately she will have a fovorable outlook.  I would expect that she also has an inability to maximally concentrate her urine, which would be a potential problem if she develops an intercurrent GI illness.  It is difficult to assess her growth with one point, but at this time her weight is the 40%ile, length 10%ile and head circumference 50%ile for age.  As her bicarbonate has not been above 20 in the last several months this may be contributing to the difference between her length and weight percentiles.
     I asked Mrs. Gerome to increase the Bicitra to 6ml tid.  I would recheck her bicarbonate locally in several weeks and further increase the dose if the level is less than 20.
     I would like to re-evaluate Andie in six months in conjunction with her visit to Dr. Mandel.  Thank you for allowing us to share in the care of Andie.  If you have any questions or concerns, please give me a call.

     Sincerely,


     Kinze Brando, MD               
     Attending, Pediatric Nephrology


CONTENT:
Elle Steinbeck, M.D.          RE: Andie Gerome
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/93

Dear Dr. Steinbeck:

     We had the pleasure of seeing Andie in the Renal Program on 10/05/94 for renal dysplasia, vesicoureteral reflux and obstructive uropathy.

     Andie is a one year old status post bilateral pyeloplasty in December 1993 with right renal dysplasia who is here for follow-up evaluation.  In the interval since her last visit in April she has been doing well.  She has had no urinary tact infections. She is very active and is learning to walk.  She has not been taking Bicitra for at least the last month and took that medication sporadically prior to that.  Her diet currently consists of table food and 2% milk.  She is described as a picky eater.

Current medications include:
     AMOXICILLIN 50 mg q.d.
     BICITRA p.r.n.

Physical exam:
     BP 101/65   P 110   R 34   HT 67cm   WT 7.54kg
     Head Circumference: 45.5 cm
     General: Andie was a small alert, well appearing infant in no distress
     HEENT: No evidence of infection
     Chest: Lungs clear with good aeration
     Heart: Regular rhythm, without murmur
     Abdomen: Well healed surgical scars, soft and non-tender without palpable masses or organomegaly
     Extremities: Normal form, no edema

Urinalysis:
     SPEC GR UA  1.005           PH UA       6          
     PROTEIN UA  N               BLOOD UA    1+         
     GLUCOSE UA  N          
     Insufficient volume for microscopic exam.

Laboratory studies at this time include:
          CO 2         21   MEQ/L       CR          0.5   MG/DL (H)
          BUN          23   MG/DL (H)   NA          139  MMOL/L    
          K           4.0  MMOL/L       CL          106  MMOL/L    
          
          DMSA renal scan: 87% function on left and 13% on right.  Decreased uptake at left upper pole.


     Since the time of her last visit Andie has generally done well.  Her plasma bicarbonate level is acceptable off the Bicitra.  I am concerned however, about her growth.  At this time both her length and weight are <5%ile and she continues to have a head circumference at the 50%ile for age.  Although Andie has had a number of carbon dioxide levels of 19, I do not think her poor growth is solely on the basis of acidosis.  I would expect that to affect her length disproportionally as compared to her weight.  At this time her estimated GFR is 74 ml/min/1.7Msquared.  I would not this expect this degree of renal insufficiency to affect her growth directly.

On speaking with her mother it seems that her caloric intake is insufficient.  The fact that she is an active child exacerbates the problem with inadequate intake.  I suggested to Mrs. Gerome that her intake should be increased.  Some children with this mild renal insufficiency do have anorexia and benefit from caloric supplements.  As we discussed, I referred her to Nancy Spinozzi, the renal dietitian.  She was unable to determine her current intake as Mrs. Gerome was comfortable with her understanding of the appropriate approach.  If you or Mrs. Gerome would like nutritional input in the future, please give me a call.

     Andie will return to Children's Hospital to see Dr. Retik in six months.  I will follow-up with her at that point.  Thank you for allowing us to share in Andie's care.  If you have any questions or concerns, please do not hesitate to contact me.

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Elle Steinbeck, M.D.          RE: Andie Gerome
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/02/93

Dear Dr. Steinbeck:

     We had the pleasure of seeing Andie in the Renal Program on 10/10/95 for renal dysplasia, vesico-ureteral reflux and obstructive uropathy.
     Since the time of her last visit in October 1994, Andie has been feeling well.  She has had no urinary tract infections.  In order to increase her rate of weight gain and growth,  her diet is being supplemented with Pediasure.  She had been on Amoxicillin prophylaxis, however, this has been discontinued for unclear reasons.  Andie is seen at the time of follow-up evaluation by Dr. Retik.

Her current medications include:
     Pediasure 8 oz. qd

Andie's physical exam was notable for:
     BP /   P 102   R 28   T    HT 79cm   WT 9.5kg
     In general, Andie was a small well-appearing toddler with height and weight both less than the 5th percentile.   
     HEENT: no evidence of infection.  
     Chest and lungs: clear with good  aeration.  
     Heart:  regular rhythm without murmur.  
     Abdomen:  benign.  
     Extremities:  no edema.
A urinalysis revealed:


Laboratory studies at this time include:
          K           3.8  MMOL/L       BUN          18   MG/DL    
          CO 2         21   MEQ/L (L)   NA          140  MMOL/L    
          CR          0.5   MG/DL       CL          103  MMOL/L    

     RADIOLOGIC EVALUATION:  Renal ultrasound showed a right kidney that was echogenic and measured 3.1 cm., which is unchanged from previous evaluation.  The left kidney had increased in size to 7.5 cm. with no change in the mild pelvic dilatation.  The Mag 3 renal scan showed no evident function on the right.  The left kidney showed a slight  flattening to the upper pole.  Lasix wash-out showed no evidence of obstruction.  Radionuclide cystogram showed grade I reflux on the left and grade II reflux on the right. 

In general, Andie is doing well.  Her genetic height potential is between the 5-10th percentile, so a proportion of her small size is likely due to her potential.  She has had a history of poor intake that is being treated with caloric supplementation.  At this time  there is no renal reason for inhibition of weight gain or growth given her normal electrolytes, bicarbonate level, and minimally depressed renal function.  Given the fact that Andie has a single functioning kidney, she should continue prophylactic antibiotics.  Dr. Retik initiated treatment with Bactrim.

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

               Sincerely,


               Kinze Brando, MD
               Attending, Pediatric Nephrology


CONTENT:
Giacinto Ibsen, M.D.          RE: Milt Fenelon
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/24/93

Dear Dr. Ibsen:

We had the pleasure of seeing Milt in the Endocrine Program on 11/15/93 for undervirilization.

History: Fenelon is a three week old boy born at 33 weeks gestation at Brigham & Women's Hospital.  Endocrine was consulted  after birth to aid in the evaluation of undervirilized genitalia.  Electrolytes were stable throughout that admission.  Since his  discharge at two weeks of age, he has been well.  He is followed by the Urology Division.

Current medications: None.

Physical exam:  
Height: 42.4 cm         Weight: 1.97 kg         Pulse: 120              
Breast: Tanner I        Pubic hair: Tanner I    Phallus length: 2.2 cm  
Mid-shaft diam.: 0.8 cm 
Head circumference:  32.5 cm

Healthy looking infant, eating avidly, in no distress. Cor S1S2 with III/VI systolic murmur at the RUSB,  radiating to the back and axillae.  I/VI at LLSB. GU hooded foreskin, bifid scrotum, chordee, testes palpable.

Laboratory studies:  10/25/93: chromosomes 46XY; 10/28/93: testosterone 70 ng/dl, dihydrotestosterone 21 ng/dl, dehydroepiandrosterone (DHEA) 454 ngd/l, androstenedione 55 ng/dl;  11/15/93: DHEA 249 ng/dL, androstenedione 71 ng/dL, 17-hydroxyprogesterone 120 ng/dL, 17-hydroxypregnenolone  447 ng/dL, progesterone 51 ng/dL.

Impression: Milt is gaining weight and looks well.  The hormonal results are all within normal limits and do not point to the etiology of his undervirilization.  Dr. Lew Holmes, Genetics, has suggested the possibility of Smith-Lemli-Opitz Syndrome and forwarded information on testing for this condition (see enclosed pages).

Disposition: We would like to see Milt again at six to nine months of age to assess his genital development and the need for hormonal therapy.
Thank you for letting us share in the care of Milt.

Sincerely,


Rene Daley, MD         Lancelot Blue, MD             
                             Attending in Endocrinology


CONTENT:
Ilya Green, M.D.          RE: Jan Baring
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/19/86

Dear Dr. Green:

We had the pleasure of seeing Jan in the Endocrine Program on 12/08/93 for evaluation of her pubertal development.

History: Jan is a 7 4 /12 year old girl who was noted in August 1993 to have breast bud on the left.  This was not associated with any rapid growth but she did have some whitish vaginal discharge.  The parents denied ingestion or application of estrogen containing compounds.  There is no history of headache, polyuria, or polydipsia.  She does occasionally complain of abdominal pain.

Past medical history:  Significant for failure to thrive in infancy which resolved spontaneously and chicken pox

Review of systems:  negative.

Family history:  Mother is 5'7"; her parents are 6' and 5'8", she has three sister who are 5'4, 5'2" and 5'1".  Father is 5'8"; his parent are 5'9" and 5'.  He has three sisters who are 4'11", 5' and 5'3".  Mother had menarche at 13, there is no history of precocious puberty.

Physical exam:  
Height: 117.0 cm        Weight: 19.8 kg         BP: 104/62              
Breast: Tanner I        
Jan was healthy looking, pleasant, interactive and cooperative.  Skin:  No abnormal pigmentation.  HEENT: EOM intact, normal fundi, Benign oropharynx.  She had four permanent incisors.  Neck:  Thyroid not enlarged.  Chest:  there is pouting of both nipples but only few strands of glandular breast tissue, more on the left.  Cor:  Lungs are clear.  Normal S12 and S2, no murmurs.  Abdomen:  Soft, no hepatosplenomegaly.  GU minimally estrogenized vagina mucosa.  Extremities:  Normal.  Neurologic:  DTR 2+ symmetrical.  Cranial nerves II-XII.

Laboratory studies:  I had the opportunity to review to films of the left hand and wrist taken in September and on 12/07/93.  The September plate is more consistent with a bone age of 5 9/12 rather than 6 10/12 and the plate of 12/07/93 revealed some maturation of the bones as compared to that of September but still less than 6 10/12.

Impression: Precocious puberty. The regression of the breast tissue and the fact that the bone age is not advanced suggest that Jan has most probably what has been termed "non-sustained" or "transient" precocious puberty.  At present there is no need for further testing or for any therapeutic intervention; however she needs to be monitored closely for further pubertal development.


Laboratory studies pending at this time include: None.
Thank you for letting us share in the care of Jan.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Cavinder Hammer, M.D.          RE: Corinna Drake
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/07/92

Dear Dr. Hammer:

We had the pleasure of seeing Corinna in the Endocrine Program on 4/4/94 for evaluation of premature thelarche.

History: As you know, Corinna is a healthy 21 month old who has had premature thelarche since birth and a question of a recent growth spurt.  

Corinna was a former full term 6 lb 12 oz baby who has always been tall for her age, growing at between the 75th and 90th percentile for her age.  Her weight has followed the normal curve along the 75h percentile for age .  As mentioned, she has had breast tissue since infancy but her mother reports that the breast tissue has not grown since birth, and if anything, has regressed.  In November 1993, on office measurement she seems to have experienced a slight growth spurt with one measurement placing her at greater than the 95th percentile for her age.  At that time she had a work-up which included normal thyroid function tests, estradiol, prolactin, all normal.  A LH .2 and FSH 2.4, a renal and pelvic ultrasound were also obtained which showed a normal prepubertal uterus and normal right ovary, the left ovary was not seen and there were no pelvic masses noted.  On follow-up approximately one month ago, further studies included a bone age and repeat FSH.  At 20 months of age her bone age was read as 30 months, her FSH level was 4.9.

Past medical history:  Notable for normal development, a large healthy appetite since birth and mild eczema.  She has no known drug allergies.  Her immunzations are up to date.

Review of systems:  Her mother has not noted a rapid growth spurt.  She has had no change in hand size or facial features.  She has no axillary or pubic hair, nor any abnormal body odor.  She takes no medications.  There is no history of ingestion of an medications.

Family history:  Notable for many members on the paternal side who are tall.  Her father is 6'2" and has a brother who is 6'3" and paternal grandmother is 6'.  On the maternal side, members are of average size.  Maternal grandfather is 6'5".  Her father has kidneys problems of unknown etiology.

Physical exam:  
Height: 87.7 cm         Weight: 12.3 kg         
Ht: which is just below the 90th percentile for her age  Wt: which is just above the 75th percentile for age 
General: Well-appearing girl with normal facies.
Skin: One cafe-au-lait spot on her right thigh.  No axillary or pubic hair.
HEENT: Unremarkable.
Neck: Supple with a normal thyroid.  No lymph adenopathy.
Chest: Lungs clear to auscultation.  Breasts buds bilaterally.  Her areolae measured 1.5 cm bilaterally.  Her breast soft tissue measured 4 cm. bilaterally.  Breast are soft and non-tender to palpation with no palpable masses.
Heart: Unremarkable.
Abdomen: Soft.  No hepatosplenomegaly or masses.
Genitourinary:  Normal immature female with normal external genitalia.
Extremities:  No clubbing. 
Neurological: Nonfocal.

Impression: Corinna is a 21 month old girl who appears to be growing along approximately the 90th percentile for height and 75th percentile for weight.  Upon review of the records that you kindly sent us, we did notice that her growth point at 16 months of age and at 20 months of age were identical suggesting that one of these may be an error.  With family history of tall stature, she is likely expressing her genetic potential.  She does have premature thelarche, but at present, is experiencing no other signs or symptoms consistent with precocious puberty.  Her bone age of 30 months is slightly greater than her height age of 27 months.    We suggest that you have the film reviewed by your Radiology Department or feel free to mail a copy to us for a second opinion.  If upon review it remains slightly advanced for height, we suggest that you check the bone age again in approximately six months. 

The FSH level of 4.9 U/L is normal for her sex and age.  We would not recommend further work-up at this time.  We should be happy to check her in the future if you find further acceleration in her growth and development.

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

Sincerely,


Owen Stone, MD              Mitsuko Simpson, MD             
                             Chief Emeritus               


Addendum: Resident in Pedatrics

Problem list to date:

Transcribed by Sullivan_ma


CONTENT:
Cavinder Hammer, M.D.          RE: Corinna Drake
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/07/92

Dear Dr. Hammer,

Thanks for forwarding the hand and wrist x-ray of Corinna done on 3/5/94.  There is slight scatter of epiphyseal development.  On review, we would read her bone age about the same as reported by the HCHP Department of Radiology or, perhaps, slightly less (i.e. 28 months).  This is essentially the same as her height age but ahead of her chronological age.  This should be checked in six months if there is progression of breast development.

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


Sincerely yours,




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


CONTENT:
Cavinder Hammer, M.D.          RE: Corinna Drake
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/07/92

Dear Dr. Hammer:

We had the pleasure of seeing Corinna in the Endocrine Program on 9/22/94 for premature thelarche.

History: Corinna was first noted to have premature thelarche shortly after birth.  Initial work up at HCHP in the fall of 1993 included random gonadotrophin sampling and a pelvic ultrasound, which demonstrated no uterine or ovarian pathology.   She was seen in endocrine clinic by Dr. Crigler last April, who noted breast enlargement and an advanced bone age, but no other signs of progressive precocious puberty.

Interval history is notable for unchanged breast size, and no galactorrhea,pubic or axillary hair development, vaginal discharge, body odor,  acne or developmental abnormalities.   There are no birth control pills or estrogen medications at home.

Current medications: None.

Physical exam:  
Height: 93.4 cm         Weight: 14 kg           
General: Well appearing and healthy. 
Skin: One hypopigmented plaque above the right knee measuring 4.5 cm in largest dimension.   
Neck: Without thyromegaly.  
Chest: Right breast 4.5 cm in greatest diameter, left 4.0 cm.  
Abdomen: Without masses.   
Genitourinary: Vaginal mucosa is slightly pink without discharge.

Laboratory studies:  Repeat bone age obtained at this visit, 3 years 6 months, by our interpretation.

Impression: Corinna is a 26 and a half month old girl with persistent premature thelarche, and advanced bone age, but no other evidence of progressive precocious puberty.   We still believe that the most likely diagnosis is idiopathic, self-limited premature thelarche.   However, the advanced bone age remains concerning, and the possibility of central or peripheral precosity remains.

Disposition: We would like to see Corinna back in clinic in six months or sooner should symptoms of precocious puberty progress.   At that time, we will obtain a bone age and consider GnRH stimulation testing and repeat pelvic ultrasound.
Thank you for letting us share in the care of Corinna.

Sincerely,


Gleide Crane, MD             Penelope Hagen, MD              
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Cavinder Hammer, M.D.          RE: Corinna Drake
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/07/92

Dear Dr. Hammer:

I saw Corinna in the Endocrine Program on 10/30/95 for premature thelarche.

History: Corinna has been followed in our clinic for several years for breast bud development.  Workup has included a normal pelvic ultrasound, normal random gonadotropin sampling, and an advanced bone age.  Interval history has been unremarkable; breast size remains unchanged or slightly decreased in size, no body odor, no acne, no vaginal discharge, no abnormal or labile behavior, and reportedly normal linear growth.

Current medications: None.

Physical exam:   Length: 103.9 cm    Wt: 17.6 kg  Breast: Tanner III      
On physical examination,  she is well-appearing.  HEENT:  normal.  Neck: without thyromegaly.  Lungs:  clear.  Cardiac:  regular rate and rhythm, no murmurs.  Chest:  right breast measuring 3.5 cm, left breast measuring 3.0 cm, consistency soft.  Abdomen: without hepatosplenomegaly or masses.  GU: normal prepubertal girl, no clitoromegaly, vaginal mucosa unestrogenized.

Laboratory studies:  Bone age at this visit 4 years.

Impression: Corinna is a 3-4/12 year old girl with breast bud development of longstanding duration, a bit smaller today, and normal growth along the accelerated curve.  The absence of new symptoms suggests an idiopathic process which has now resolved.   The bone age advancing by only 6 months over the past year supports this assessment.

Disposition: Return to clinic in 1 year, or sooner for symptoms of renewed precocious puberty, including enlarging breasts, development of pubic hair, body odor, acne, or accelerating growth.


Sincerely,




Gleide Crane, MD             
Attending in Endocrinology


CONTENT:
Biki Capulet, M.D.          RE: Franklin Baez
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/03/93

Dear Dr. Capulet:

We had the pleasure of seeing Franklin in the Endocrine Program on 12/6/93 for ambiguous genitalia.

History: Max has been followed by us since his birth at Beth Israel
          Hospital.  He was born by full-term spontaneous vaginal delivery, weighing 3.9 kg.  The pregnancy was uncomplicated.  The only medications taken by his mother during gestation were TUMS and Tylenol.  She denies hirsutism and any use of oral contraceptives.  Ambiguity of the genitalia was noted at birth, and the preliminary evaluation by both Urology and Endocrinology was performed by a few hours of life.  The baby was thought to be a male pseudohermaphrodite, and a male sex assignment was recommended.  He went home by 24 hours and came back at three days to our clinic for reassessment and blood testing.

Family history:  The mother is Asian-American, 22 years old, and in good health.  The father is African-American, also healthy.  They have a three-year-old daughter who is healthy.

Physical exam:  
Height: 51.8 cm         Weight: 3.71 kg         BP: 74/34               
Pulse: 132              Breast: Tanner I        Pubic hair: Tanner I    
Phallus length: 3.3 cm  Mid-shaft diam.: 1.1 cm 
Head circumference:  36 cm.

Healthy looking boy in no distress.  Skin without birthmarks.  Cor without murmur.  Abdomen soft, without organomegaly.   GU:  Phallus with chordee and hooded prepuce.  Incompletely descended scrotal folds with bifid scrotum posteriorly, well  rugated.  Third-degree hypospadias with meatus on the perineum at the base of the phallus.  No uterus palpated by rectal exam.

Laboratory studies:  12/4/93 - Chromosomes (Beth Israel Hospital)  46 XY

12/6/93 - testosterone 37 ng/dl, dihydrotestosterone 20 ng/dl, LH <0.12 IU/l, FSH 0.1 IU/l, ACTH 38 pg/ml, cortisol 1.1 mcg/dl, androstenedione 72 ng/dl, dehydroepiandrosterone 177 ng/dl, pregnenolone 445 ng/dl, progesterone 164 ng/dl, 17-hydroxypregnenolone 156 ng/dl, 17-hydroxyprogesterone 55 ng/dl
          
12/22/93 - pelvic ultrasound showed no uterus or cervix;  voiding  cystourogram, no reflux into utricle noted, no vesicoureteral reflux

Impression: The laboratory studies confirm male sex, but they do not  reveal a specific etiology.  Causes of incomplete virilization  include 5-alpha-reductase deficiency and certain forms of congenital adrenal hyperplasia.  These are unlikely given the studies performed thus far.  Partial androgen insensitivity, gonadotropin deficiency, and partial defects in the steroid synthetic pathway have not yet been completely excluded.  A teratogen exposure early in the first trimester is also a possibility.

Disposition: We would like to see Max again before four months of life to repeat the LH and FSH and testosterone levels, during the window of time in which they should be measurable.  At that time, it may be desirable to treat him with a short course of hCG (or testosterone if hCG does not induce endogenous productions of testostereon) to increase the size of his phallus before surgical correction of his abnormal genitalia.  Before carrying this out we shall discuss this with Dr. Atala in the Division of Urology.
Thank you for letting us share in the care of Franklin.

Sincerely,


Rene Daley, MD         Mitsuko Simpson, MD             
                             Chief Emeritus


CONTENT:
Sabie Daley, M.D.          RE: Imogene Haggard
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/21/92

Dear Dr. Daley:

We saw Imogene in the Endocrine Program on 11/20/95 for slow growth.

History: Imogene is a former full-term, 6 pound, 15 ounce product of a normal pregnancy, labor, and delivery, whose history up until now has been marked by poor growth and multiple hospitalizations.  Her mother thinks that Imogene has been in the hospital at least five times for fever and wheezing; each admission being five days to a week in length.  Her last admission was a year ago.  In between these admissions Imogene is alert, active, and has a very good energy level, but intermittently wheezes and is treated with inhaled beta agonists.  Her mother says that Imogene is a very picky eater, and she has seen a nutritionist  in the past, and Pediasure supplements have been prescribed.  Her mother says that she gives around 16 to 20 ounces of Pediasure supplementation a day. 

Imogene is developmentally normal.

Current medications: None.

Review of systems:  A review of system reveals no other  concerning features, specifically, she had no diarrhea, abdominal pain, cough, or shortness of breath, at times when she is not wheezy.

Family history:  Imogene's mother is a healthy woman who is 5'1".  Her father was 6'1".  Her mother has never been tested for HIV disease, but denies any risk factors.  There is no family history of thyroid disease.

Physical exam:
Height: 86.4 cm        Weight: 10.1 kg
Breast: Tanner I       Pubic hair: Tanner I    
She is a smiling and alert young child, who was happily playing in the room.  Her oropharynx was without lesions and her neck was supple without thyromegaly.  Auscultation of her chest revealed clear breadth sounds and a soft Still's murmur.  Her abdomen was soft and nontender with no  hepatosplenomegaly.  Neurologically, she was grossly intact.

Laboratory studies:  I note that a bone age performed at an outside hospital when she was 34 months of age was normal (that is to say 31 to 37 months).  A sweat tests  was also normal.

From today:  Ca 9.3 mg/dL, CO2 24 mEq/L, Na 141 mmol/L, Cl 105 mmol/L, Cr 0.3 mg/dL, BUN 8 mg/dL, glucose 60 mg/dL, total protein 6.9 mg/dL, ALT 13 U/L,  WBC 9.92 K/MM3, Hct 36.7%, MCV 81.7 FL, platelets 435 K/MM3, neutr-A 34.4%, lymph-A 53.0%, mono-A 5.7%, eos-A 3.1%, baso-A 0.7%, atyp ly 3.2%, ESR 14 mm/hr, TSH 3.00 uU/mL.  Antiendomysial antibodies - negative.

Impression: Imogene is a 3 and 1/12 year old girl with short stature and a low weight-for-height ratio.  Her short stature is most likely a combination of genetic short stature, poor nutrition and recurrent illness.  We see no evidence at the present time of an endocrinologic disease.

Disposition: We recommend a repeat visit to the nutritionist, with close attention to total calories consumed.  In addition, we would like to see Imogene back in Clinic in 6 months to assess interval growth.



Sincerely,




Clotilde Kaelin, MD             Gleide Crane, MD             
Resident in Pediatrics          Attending in Endocrinology


CONTENT:
Octavia Montague, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Montague:

We had the pleasure of seeing Jan in the Endocrine Program on 1/20/94 for optic glioma.

History: Jan is a 5 and 8/12 year old Caucasian female with optic glioma and neurofibromatosis, diagnosed in December of 1993.  Jan has been known to have cafe au lait spots since infancy without any manifestation of neurofibromatosis until recently when she failed a school age eye exam in the Summer of 1993.   A visual field test revealed temporal superior field defect on he right side.   An MRI in 12/93 showed an optic chiasm mass with some possible enlargement of the left optic nerve and enlargement of the right brain stem.   Jan has begun radiation treatment.   She is on the 12th out of 30 treatments.   Her complete radiation dose will reach 5,400 rads.

Jan currently attends kindergarten.   She has been growing and eating  well.   Mom states that recently she has been a little irritable while  undergoing radiation therapy.   Jan now is at more than the 95th percentile for both height and weight.   She has not had any recent change in diet or appetite.   Mom denies any evidence of adult perspiration odor or any evidence of pubic hair growth.   Mom states that Jan has always had evidence of a left breast bud since infancy and has recently noticed evidence of a right breast bud.

There is no family history of neurofibromatosis in Jan's two siblings, age 8 and 3 or other relatives family.

Past medical history:  Significant for growth spurt noted at approximately age 3 from the 10th percentile to the 95th percentile for height and weight.

Review of systems:  Significant for cafe au lait spots around the abdominal area since infancy.   Jan has not had any cardiac, respiratory, GI or neurologic problems.

Physical exam:  
Height: 122.1 cm        Weight: 25.7 kg         BP: 80/60               
Pulse: 85               U/L ratio:  1           Arm span: 119           
Breast: Tanner III      Pubic hair: Tanner I    
General: Alert and oriented.   Well developed and well nourished, active child.
Skin: Multiple cafe-au-lait spots on abdomen, skin folds with freckles.  No fibroma.  Vague patch of increased pigmentation on back in lumbar region.
HEENT: Normocephalic, atraumatic, extraocular motions intact.   Oropharynx is within normal limits.
Neck: No thyromegaly.
Chest: Clear to auscultation bilaterally.  Right breat tissue: 7x6 cm (HxV); left breast tissue: 5x5 cm.
Heart: Regular rate and rhythm.   Normal S1, S2 without murmurs.
Abdomen: Nontender, nondistended.   Normal active bowel sounds.   No organomegaly.   Numerous cafe-au-lait spots visualized on the belly.
Genitourinary: Tanner stage I for pubic hair.   The vaginal mucosa is red.
Extremities: No clubbing, cyanosis or edema.   Full range of motion.
Neurological: Within normal limits.

Laboratory studies:  MRI from 12/17/93 showed a mass in the optic chasm as stated above.

Impression: Jan is a 5 and 8/12 year old girl with neurofibromatosis, optic glioma evidence of premature thelarche, and growth acceleration, with possible precocious puberty.


Laboratory studies pending at this time include: Bone age was 7 4/12 years,  LH and estadiol were undetectable and  FSH was 0.9.
Disposition: Jan will be scheduled for a GnRH stimulation test to evaluate the presence of precoicou s pubrerty.  Jan will return to clinic in six months.
Thank you for letting us share in the care of Jan.

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


Addendum: Sherry Huang
Harvard Medical Student


CONTENT:
Vashti McPherson, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. McPherson:

We had the pleasure of seeing Jan in the Endocrine Program on 08/18/94 for follow up evaluation of precocious puberty.

History: This is a return visit for Jan, who has an optic glioma associated with neurofibromatosis.  MRI 4/94 was without change.  Mother notes no change in behavior, no body odor, no vaginal discharge.  Breast size probably unchanged.

Current medications: None.

Physical exam:  
Height: 125.1 cm        Weight: 26.8 kg         Breast: Tanner III      
Pubic hair: Tanner I    
Skin: Cafe-au-lait spots as noted previously.  Chest:  Breasts:  (L) 6 x 5.5 cm, (R) 6 x 5 cm [essentially no change from 01/94].  GU: Red vaginal mucosa.  No pubic hair.

Impression: Jan continues to have increased growth velocity (10 cm/yr).  Her predicted adult height based on the bone age obtained in January is 5'3" to 5'4".  She may have early precocious puberty, although her initial GnRH stimulation tests on 04/05/94 was prepubertal.

Disposition: We will assess in six months, along with an evaluation of the growth hormone axis, in our outpatient testing center (CAT/CR) after her next visit to consider Lupron therapy.  No laboratory studies were performed at the time of this visit.
Thank you for letting us share in the care of Jan.

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Vashti McPherson, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. McPherson:

We had the pleasure of seeing Jan in the Endocrine Program on 5/18/95 for optic glioma.

History: Jan is a 7 year old female last seen 3/3/95.  She is doing well in school.  Mother feels breast are slightly larger.

Current medications: none.

Physical exam:  
Height: 127.3 cm        Weight: 30.2 kg         BP: 100/80              
Pulse: 85               Breast: Tanner III      Pubic hair: Tanner I    

Neck: No palpable thyroid.
Chest:  Brast 7x7 cm. bilaterally, slightly larger than at last visit. 

Genitourinary: Vagina red, unestrogenized.

Impression: 1.  Her growth velocity is falling at a time when breast development is proceeding.  She may have combined precocious puberty and GH deficiency.  I have discussed with mother and we ahve reviewed possible complications of GH treatment, tumors or regrowth of glioma. 
2  With bone age of 7 yr 10 month with with CA of 6 yr 9 mo on 3/3/95, predicted height = 5'3".


Laboratory studies pending at this time include: ITT/GnRH test is scheduled in CAT/CR.
Disposition: ITT/GnRH for GH/gonadotropin evaluation.
Thank you for letting us share in the care of Jan.

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Sanford Benise, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Benise:

We had the pleasure of seeing Jan in the Endocrine Program on 2/23/95 for optic glioma.

History: Jan is a 6 and 10/12 year old girl with an optic glioma, now 1 year following cranial irradiation.  Breast development has progressed.  She has no body odor, pubic or axillary hair, or vaginal discharge.  She is doing well in school except for reading.

Current medications: none.

Physical exam:  
Height: 126.4 cm        Weight: 29.5 kg         BP: 90/60               
Pulse: 80               Breast: Tanner III      Pubic hair: Tanner I    

Chest:  Right breast 7x6 cm, increase from 8/94.  Left breast 5x6 cm, no change.
Genitourinary: Vagina red, not estrogenized.

Impression: 1.  Precocious puberty.  Breast enlargement has continued.  Height velocity has slowed, which could be a sign of post-XRT state or possible GH deficiency.

We plan to repeat GnRH stimulation test within the next month, and bone age and reassess.

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

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Sanford Benise, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Benise:

We had the pleasure of seeing Jan in the Endocrine Program on 7/13/95 for optic glioma and gh deficiency.

History: She was last seen on 5/18/95 for optic glioma secondary to neurofibromatosis.  She has had progressive breast development for the past 6 months, and has had growth deceleration for the past 6 months as well.  Insulin tolerance/glucagon testing on 6/7/95 disclosed growth hormone deficiency, and GnRH testing disclosed early puberty.  Thyroid function and adrenal function were normal.  I have discussed at length with the mother my advice to begin both growth hormone therapy (Genentech Nutropin, 0.025 mg/kg/d) and Lupron Depot (7.5 mg IM/4 weeks).  We discussed the risks of GH treatment, including possible development of second tumors such as leukemia and regrowth of tumor.  I have sent a review of the cost/benefits of GH therapy to the Malin's home.

Current medications: None.

Physical exam:  
Height: 127.4 cm        Weight: 30.4 kg         BP: 80/60               
Pulse: 100              Breast: Tanner III      Pubic hair: Tanner I    
General: Well appearing girl; many cafe-au-lait lesions.
Chest:  Left breast 8x7 cm, 2 cm. areola; right breast 7x2 cm, 2 cm areola.  Both larger than last visit.   
Abdomen:  No masses.

Laboratory studies:  See above.

Impression: 1.  Growth hormone deficiency, 1.5 years following radiation therapy.  We discussed the benefits and risks of treatment, and mother agrees to proceed.  We will start Genetech Nutropin.
2.  Precocious puberty.  Increasing breast size, early pubertal gonadotropin pattern following GnRH.  We discussed starting Lupron with mother. 

As you know, you and I also spoke about these issues.

Disposition: 1. Begin Lupron 7.5 mg IM q 4 weeks.
2.  Begin rhGH, 0.025 mg/kg/d SQ.
Thank you for letting us share in the care of Jan.

Sincerely,


Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Sanford Benise, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Benise:

I saw Jan in the Endocrine Program on 12/28/95 for Neurofibromatosis/optic glioma.

History: Since last being seen on 07/13/95, Jan has started on Nutropin  0.025 mg. per kilogram per day and Lupron 7.5 mg. IM q.4 weeks, both started 08/29/95.  She had an MRI of her head at the Jimmy Fund in 10/95, which is reportedly without change from prior studies.  She is now in second grade and doing well except for needing some assistance in reading with fairly frequent letter reversals.  She does complain of occasional headaches, but none in the morning or at night.  She has no vomiting.  Her appetite appears good.  Her mother has noted a decrease in her breast size since starting Lupron.  She has not had any pubic hair or menstrual flow.

Current medications: Nutropin, 0.025 mg/kg/d.  Lupron, 7.5 mg IM q 4 weeks

Physical exam:
HT: 131.5 cm            WT: 33.1 kg    
Arm span: 132 cm        U/L ratio:  0.96
BP: 90/70               Pulse: 80                    
Breast: Tanner III      Pubic hair: Tanner I    
Cafe au lait spots as  before.  The exam is unchanged from before, except that her breasts both appear smaller than before, approximately 8 x 6 cm., but still with a 2 cm. areola bilaterally.

Impression: 
1.   Growth hormone deficiency:  Jan is now on growth hormone treatment and it appears as if her decline in her height has halted, although  we need more points to definitely assess this. 

2.   Precocious puberty:  With the onset of Lupron therapy, breast size appears diminished.  We should recheck a growth stimulation test sometime this year, perhaps after her next visit to me to make sure she remains suppressed.

3.  Thyroid.  Euthyroid by history and physical exam. We will get thyroid function tests on the next visit.

Disposition: Maintain current medications.  Return to Clinic in six months.


Sincerely,






Bernardo Red, MD           
Chief, Division of Endocrinology


CONTENT:
Sanford Benise, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Benise:

I saw Jan in the Endocrine Program on 8/8/96 for optic glioma.

History: Jan was last seen on 12/20/95.  As you know, she was started on growth hormone and Lupron therapy in August of 1995.  Since that time, she has been doing very well and she has a good appetite.  She has done well in school though is staying back in the second grade for next year.  She has had no vaginal discharge.  Her mother feels that her breast size has decreased since Lupron was started.

Current medications: Nutropin 0.8 mg (0.022 mg per kg) per day, Lupron 7.5 mg IM every 4 to 5 weeks, the latter is administered by the mother.

Physical exam:
HT: 136 cm              WT: 36.2 kg    
Arm span: 135 cm        U/L ratio:  0.89
BP: 106/65              Pulse: 115                   
Breast: Tanner II       Pubic hair: Tanner I    
General appearance; well.  Breasts; 4 cm diameter breast tissue bilaterally, flatter than last exam.  Areolae still 2 cm bilaterally.  Lungs clear.  Heart without murmurs.  Abdomen without masses.

Impression: 

1.  Growth hormone deficiency.  She is growing well on growth hormone  replacement.  Weight and height are commensurate.  We will increase growth hormone to 0.9 mg per day to maintain her at 0.025 mg per kg per day.  

2.  Precocious puberty.  On 7.5 mg of Lupron every 4 weeks, she appears to be suppressed.  However, to ensure this we will repeat a GnRH stimulation test within the next couple of months.  She will call Regina Schofield to arrange this.  

3.  Thyroid function.  Thyroid function tests should be attained at the time of the GnRH test.  If you agree, I would like to see Jan back in 6 months.

Sincerely,





Bernardo Red, MD           
Chief, Division of Endocrinology

cc: Mrs. Tracey Christy   15 Acorn Dr   Andover , MA  01810
    Nancy Tarbell, M.D.  Radiation Oncology  Children's Hospital, Bader One  
    Anne Fulton, MD  Opthalmology, Children's Hospital


CONTENT:
Sanford Benise, M.D.          RE: Jan Christy
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/03/88

Dear Dr. Benise:

Jan Christy had a GnRH stimulation test performed on 10-10-96 to treatment of precocious puberty.  In response to GnRH, her LH changed from 0.98 to 1.16 IU/ml, and FSH form 1.03 to 1.56 IU/ml. This demonstrates excellent suppression of gonadotropins by Lupron, indicating that she should stay on the same dose.  This result is also consistent with her physicial exam, which shows no advancement of her breast development.

Thyroid function tests reveal a borderline low T4 of 6.6 ug/dl, with a low TBGI (equivalent to T3 resin uptake) of 0.88.  The TSH of 2.3 uU/ml is not helpful given Jan's hypothalamic-pituitary deficits.  Given that Jan's T4 was above 10 ug/dl on the last two measurements, I believe she is now slightly hypothyroid and should be treated with L-thyroxine (Levoxyl), 50 micrograms per day. I have spoken with her mother and have initiated her on this therapy.

Thank you for referring Jan to us.


Sincerely,



Bernardo A. Red, M.D.
Chief, Division of Endocrinology


CONTENT:
Daley, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Daley:

We had the pleasure of seeing Florence in the Endocrine Program on 1/10/94 for premature thelarche                                                                                                                                                                                                                                                                                                                                                                                                           thelarche.

History: Florence is a 4 and 11/12 year old, normal healthy child who has been followed since age 1 for enlarged breasts.   She is now transferred from Massachusetts General Hospital because of insurance coverage.   Medical records from Mass. General shows that the breasts were 5.5 cm with  an areolarof 1.5 cm at 23 months and 6.5 cm at 31 months.   There is no history of any hair growth, body odor or vaginal discharge.   The breasts have grown but not out of proportion to the body growth.   

Of note, she had a pelvic ultrasound at Mass. General at 3 years and 4 months which showed infantile uterus and small normal prepubertal ovaries with a few follicles with diameters of 0.5 cm.

Past medical history:  Normal pregnancy.   Growth and development of the child was normal with no hospitalizations, injuries or operations.

Review of systems:  Normal.

Family history:  Mother's height is 5'1".   Father height is 5'6.5".

Physical exam:  
Height: 113.6 cm        Weight: 16.3 kg         BP: 86/52               
Pulse: 85               U/L ratio:  1.1         Arm span: 107           
General:   Alert and healthy.  Breasts:  Left breast Tanner 3, right breast Tanner 3+.  Skin:   No rash or pigmentation.     HEENT:  No fundi.   Small palpable cervical lymph nodes with no supraclavicular lymph nodes.     GU:  No clitoromegaly, vaginal mucosa shows no signs of estrogenization.  The vaginal mucosa is red.  Heart:  Normal S1 adn S2, no murmur.  Abdomen:  Soft, no tnederness, no hepatosplenomegaly.  Extremities: Normal.

Laboratory studies:  Bone age of 2 years at 23 months.   Bone age of 4 years at 3 years and four months.   At 31 months she had an LH of 2.1 and an FSH of 8.4, prolactin of 11, estrodial of <5..

Impression: Although relatively old for the diagnosis of premature thelarche, the normal pelvic ultrasound, the normal growth rate, the lack of significant acceleration of bone maturation, and the non-estrogenized vaginal mucosa, are reassuring and this diagnosis remains the most plausible.

Disposition: Reassess in six months.
Thank you for letting us share in the care of Florence.

Sincerely,


Kendell Simpson, MD             
Clinical Director            


Addendum: Judith Carroll, M.D.
Fellow in Endocrinology


CONTENT:
Daley, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Daley:

We had the pleasure of seeing Florence in the Endocrine Program on 8/17/94 for .

History: I saw Florence at age 5 years 6 months for evaluation of premature thelarche.

She was last seen by my colleague, Dr. Najjar, on January 10, 1994.  Her parents reports that they suspect there has been some increase in the size of her breasts.  There has been no vaginal discharge, spotting, or bleeding.  

She has enjoyed good health and is active and energetic.  She has a good appetite and normal bowel movements.  She sleeps well without disturbance.  She reports no headaches, vision or hearing disturbance.  

She is concerned about her physical appearance and the fact that she is different from her peers.  

The family live in Saudi Arabia and return to the United States for approximately one month at six month intervals.  

Maternal height 155 cm., paternal height 167.7 cm.  

Medications:  Flintstones (multivitamins) intermittently.  No other medications.

Physical exam:  
Height: 119 cm          Weight: 22.1 kg         BP: 92/50               
Pulse: 80               Pubic hair: Tanner I    
Breast:  Tanner stage III-IV
General: Well developed, well nourished, healthy looking child.
Skin: One small area of cafe-au-lait pigmentation on the lower anterior abdominal wall.  
HEENT, neck and lymph nodes:  Unremarkable.  No thyromegaly.
Chest: Clear.  Areolae right 2.1 cm. left 2.5 cm.; breast diameter right and left 7 cm.
Heart:  Normal.
Abdomen: No organ enlargement or masses.
Genitourinary: Normal female external genitalia; no clitoromegaly.  Moist and reddish vaginal mucosa.
Extremities: Normal.
Neurological: Deep tendon reflexes were 1+ in the lower extremities.  PERRL; EOMI; normal fundi with sharp discs and vessels.

Laboratory studies:  Bone age was 5 years at chronologic age 5 years 6 months (SD=9 months).  Results of a GnRH test were as follows:  baseline LH and FSH were <0.1 and 1.7 IU/L; serum estradiol <5 pg/mL, DS 29 5g/dL, 17-hydroxy progesterone 19 png/dL, '-HCG 2.5 U/L.  In response to GnRH, her LH and FSH levels increased: a peak LH level of 3.9 was reached at 30 minutes and a peak FSH level of 14.1 U/L was reached at 45 minutes.  The response suggests early puberty of central origin with FSH predominance.

Impression: She has shown an increased growth velocity and a marked increase in weight since her last visit.  Her current weight is, however, appropriate for her height.  

The increased size of her breasts, increased growth rate and rate of weight gain, and results of the GnRH test all indicate precocious puberty of central origin.  However, her bone age is not advanced at this time.  I cannot explain this discrepancy.  Nevertheless, in view of the psychosocial problems that precocious puberty presents for this girl, I would be in favor of her commencing treatment with a GnRH analog (Lupron Depot) in a dose of 7.5 mg at 4 weekly intervals.  Prior to starting therapy, I would recommend that she have a cranial MRI to rule out the unlikely possibility of a central abnormality that is causing the precocity.

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

Sincerely,


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


CONTENT:
Carter, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Carter:

We had the pleasure of seeing Florence in the Endocrine Program on 12/21/94 for .

History: I saw Florence, age 5 years 10.5 months, in follow-up for premature breast development.  

She has enjoyed good health since I last saw her.  She reports no headaches or visual disturbance.   Her mother stated that she was a happy child and is doing well in school.   There has been no vaginal discharge or staining of the underwear.

Current medications: Flintstone vitamins i daily.

Physical exam:  
Height: 121.7 cm        Weight: 21.5 kg         BP: 85/49               
Pulse: 82               
Tanner breasts stage III-IV; stage I Tanner pubic hair.
She appeared well developed and well nourished.   She looked well.   Skin was unremarkable.  HEENT was normal; she had no thyromegaly and no cervical lymphadenopathy.   She had scattered rhonchi on chest examination.   Heart was normal and abdomen was unremarkable.   She has normal appearing external female genitalia with reddish vaginal mucosa and no leukorrhea.   Right areola was  2.0 cm, left 2.1 cm with mounding; right breast diameter was 6.0, left breast 5.0 cm.  Neurologic exam was unremarkable.

Impression: Growth rate is minimally accelerated; weight has actually decreased since her last visit.   

Cranial MRI was normal.   I have recommended deferring treatment at this time because the clinical examination indicates that her precocious puberty  currently is inactive.   I plan to repeat her bone age and gonadotropins and possibly repeat the GnRH test at her next visit in 6 months.


Laboratory studies pending at this time include: Cranial MRI showed no pituitary or hypothalamic abnormality.
Thank you for letting us share in the care of Florence.

Sincerely,


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


CONTENT:
Yale Carter, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Carter:

We had the pleasure of seeing Florence in the Endocrine Program on 7/31/95 for .

History: I saw Florence, age 6 years 5-1/2 months, for follow-up of premature breast development. 

She has been healthy since her last visit except for an accidental  fracture of her right wrist. 

Parents report no evidence of acne, body odor, vaginal secretions or  bleeding or the appearance of sexual hair. 

She is a good student.  She reports no headaches or visual disturbance.   She is currently on no medications.

Physical exam:  
Height: 124.8 cm        Weight: 24.1 kg         BP: 86/40               
Pulse: 96               Arm span: 119.6         Breast: Tanner IV       
Pubic hair: Tanner I    
General Appearance:  Well developed, well nourished,  good color and hydration.  Skin smooth and warm.  Physical exam was  entirely normal except for the Tanner 4 breats.  Her right areola measured 2.3 cm and the left areola was 2.2 cm.  The transverse diameter of the base of her breats was ~7 cm bilaterally.  She has normal  appearing external genitalia.  There was no leukorrhea; the vaginal  mucosa was red.  Neurologic exam was unremarkable.

Impression: There has been no increase in growth velocity but her breasts have increased in size.  Her bone age is appropriate for her chronologic age and does not reflect exposure to sex steroids.  The clinical picture is extremely unusual and raises the suspicion that her breasts are exquisitely sensitive to the very low level of estrogen that is normally present in the circulation of a prepubertal child.


Laboratory studies pending at this time include: Bone age was 6 10/12 (SD=10.2 months). A GnRH test has been scheduled.  Results are pending.
Disposition: Depending on the results of her GnRH test, one may elect to use Lupron depot in an effort to completely suppress estrogen secretion and diminish the size of her breasts.  An alternative approach might be to block aromatase, the enzyme that converts testosterone to estrogen, using testolactone, and thereby decrease the formation of estrogen.
Thank you for letting us share in the care of Florence.

Sincerely,


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


CONTENT:
Tinisha Benise, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Benise:

I saw Florence, age six years 5-1/2 months, for follow up of premature  breast development.   

She has been healthy with the exception of an injury to her right wrist  resulting in a fracture. 

Mother reported no acne or body odor, no vaginal secretions or  bleeding, and no sexual hair. 

She has normal vision and hearing.  She is a good student.

Current medications:  None.

Physical exam:  
Height: 124.8 cm        Weight: 24.1 kg       Arm span: 118.2 cm.        
BP: 86/40               Pulse: 96                  
Breast: Tanner IV       Pubic hair: Tanner I    
Well-developed, well-nourished health appearing  girl.  Skin was smooth and warm.  She had a small area of pigmentation on the lower anterior abdomen.  HEENT and neck were unremarkable.  Chest was clear and heart was normal.  Abdomen revealed no organ enlargement or masses.  She has normal appearing external genitalia.  Neurologic exam was normal.  Areolae measured 2.3 cm. on the right and 2.2 cm. on  the left.  Breast diameter was approximately 7 cm.

Laboratory studies:  Bone age was six years 10 months (standard deviation 10.2 months). A  GnRH test was performed on 8-4-95.  Unfortunately, for technical reasons  samples were not obtained at 30 and 45 minutes.  Nevertheless her basal serum LH and FSH were 0.16 and 2.5 IU/L, respectively; serum estradiol was less than 10 pg/ml.  Peak LH was 4.4 IU/L at 60 minutes and the peak FSH was 15 IU/L at 60 minutes. 

I interpret these data as evidence that Florence's hypothalamo-pituitary axis is active.  The results are consistent with an early pubertal response.

Impression: Although she has not shown clear evidence of accelerated growth velocity, there has been progressive enlargement of the breasts unaccompanied by other signs of secondary sexual development.  Given theabove GnRH test results, a trial of GnRH agonist therapy to suppress puberty would seem appropriate.  

I would strongly recommend that Florence start treatment with Lupron Depo pediatric 7.5 mg. intramuscularly at four week intervals.  A repeat GnRH test should be obtained in three months to demonstrate that this dose is suppressing LH and FSH secretion.



Sincerely,




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


CONTENT:
Tinisha Benise, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Benise:

I saw Florence, nearly 7 years old, for follow-up of premature breast  development. 

Medications:  Lupron Depo Pediatric 7.5 mg (0.3 mg/kg) at 4 week intervals; the last dose was given January 15, 1996. 

She has enjoyed good health.  She reported no headaches or vomiting.  There has been no obvious change in the size of her breasts.  There has been no vaginal secretion or bleeding. 

Her mother stated that she has been more moody and emotional since starting treatment with Lupron.  To date, she has received only two doses.

Current medications: None.

Physical exam:
HT: 127.9 cm            WT: 25.1 kg
BP: 84/60               Pulse: 72               Pubic hair: Tanner I    
She looked well, had good color and hydration.  Skin was smooth and warm.  The breasts measured approximately 6-7 cm diameter on the right, 6 cm on the left and the areola diameter was 2 cm bilaterally.  There were no other features of note on the physical exam and she does not have pubic hair.

Laboratory studies: Urinalysis was normal.  Bone age was 7 10/12 years.  Baseline LH and FSH 0.90 and 1.60 IU/L; 45 minutes after GnRH the values were unchanged.  The test was done 24 days after the last dose of Lupron.
Compared to the pre-therapy GnRH test, the stimulated values are lower, but baseline LH is still slightly increased.

Impression: Inspection of her growth chart indicates no acceleration in statural  growth or weight gain over the past several months.  According to my measurements, there has been no increase in breast size.

She should continue to receive Lupron Pediatric Depot 7.5 mg IM, but I would suggest increasing the frequency of its administration to once every three weeks in an effort to more completely suppress LH secretion. 

I will see her again when she returns to the United States of America in 6 months.


Sincerely,







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


CONTENT:
Tinisha Benise, M.D.          RE: Florence Killwitz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/89

Dear Dr. Benise:

I saw Florence, age 7 years 5 months, for follow-up of precocious puberty. 

Medications:  Lupron depo pediatric 7.5 mg IM q. monthly (0.28 mg/kg). 

Her parents report diminution of breast size; no vaginal secretions, bleeding or spotting.  She is top of her class, reports no headaches or visual changes.  She is physically active.

Physical exam:
HT: 131.8 cm            WT: 27.1 kg
BP: 98/57               Pulse: 68                      
Breast: Tanner IV       Pubic hair: Tanner I    
She looked well, had good color and hydration.  Skin was tanned.  HEENT was unremarkable.  The diameter of the breasts was approximately 6 cm; areolae 2 cm in diameter.  The right breast was more prominent than the left and was more "mushy" than on her previous exam.  The remainder of the exam was normal.

Laboratory studies: Serum LH .90, FSH 1.30 IU/L (prepubertal LH is <0.90 IU/L); estradiol pending.

Impression: There appears to have been slight growth acceleration with a modest weight gain in the past six months.  By history and physical exam, my impression is that her breast size has decreased, and her breasts are less firm.   

Her serum LH is not completely suppressed on the current dose of Lupron.

Disposition: My intention is to increase her dose of Lupron depo to 11.25 mg IM q. 4 weeks.  I will see her again in six months.

Sincerely,





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

cc: Mr. & Mrs. Richard Killwitz  41 Rice Spring Lane  Wayland, MA  01778


CONTENT:
Kendell Simpson, M.D.          RE: Agatha Barres
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/23/83

Dear Dr. :

We had the pleasure of seeing Agatha in the Endocrine Program on 1/13/94 for evaluation of her height and pubertal development.

History: Agatha is a 10 and 4/12 years old, born at term, birth weight was 3200 mg, pregnancy, delivery and neonatal period were uneventful.  She has been healthy in general except for recurrent tonsillitis which was successfully treated with a  T and A.  She also had chicken pox.  She has no history of headaches, polydipsia, polyuria, nocturia or visual disturbances.

Agatha has always been relatively shorter than her siblings but had been growing.  Late in spring, her mother noted that she had breast budding which had developed further with time.  She had no vaginal discharge or vaginal bleeding.

She had the following investigations performed in Riyadh: T4 67 nmol/L, TSH 1 U/L, on one occasion, and Free T4 of 12 pmol/L, and TSH 0.7 U/L.  With positive anti-thyroglobulin antibodies on another.  Bone age at 9 years of age was interpreted at 12.5 yers.  MRI of the brain in October 1993 was normal.

Family history:  Father is 5'4", mother is 154 cm.  Father is the shortest among his brothers, his two sisters are taller than his wife, both are on thyroid medication.  Mother had her menarche at age 11, her brothers and sisters are reported as "tall".  She has two sisters who are 164 and 160 cm. respectively, and a brother who is 170 cm tall.

Physical exam:  
Height: 134.5 cm        Weight: 38 kg           BP: 90/60               
Pulse: 82               Breast: Tanner IV       Pubic hair: Tanner III  
Agatha was healthy looking and coopertive.  Skin: No acne, no abnormal pigmentation.  HEENT: Normal fundi.  EOM intact.  Benign oropharynx.  Neck: Thyroid not enlarged.  No lymphadenopathy.  Chest: Clear lungs.  Heart: Normal S1 and S2, no murmurs.  Abdomen: Soft, no tenderness, no hepatosplenomegaly.  Extremities: Normal.  Neurological: DTR 2+, symmetrical.  Cranial nerves II-XII intact.

Impression: Agatha most likely has familial short stature and early puberty, and  Hashimoto thyroiditis.  She has normal thyroid function at present and no evidence of growth hormone deficiency.

Disposition: Thyroid function tests need to be monitored regularly not to miss an evolving hypothyroid state.  Otherwise no treatments for her Hashimoto thyroiditis, her stature or her pubertal development are indicated.

Laboratory studies pending at this time include: T4 6.7 mcg/dl, TBGI 0.90, T3 154 ng/dl, TSH 1.8 mcU/ml.  Thyroglobulin antibodies positive, TPO antibodies negative, IGF1 493 ng/ml.  Bone age: Officially read as 12 1/2 years.  My reading is between 11 and 12, nerer to 11 than 12.
Thank you for letting us share in the care of Agatha.

Sincerely,


Kendell Simpson, MD             
Clinical Director


CONTENT:
Tuba Pavarotti, M.D.          RE: Cecile Antigonus
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  11/19/92

Dear Dr. Pavarotti:

We had the pleasure of seeing Cecile in the Endocrine Program on 2/3/94 for .

History: Cecile is a  14 month old girl who was noted to have slight growth of dark hair in the labial region in the past six weeks.  There was no excess hair noted elsewhere although she has some fine growth of slightly pigmented hair in the upper back which is longstanding.

Past medical history:  Pregnancy was normal with delivery at 37 weeks.   Mother took Terbutalene for 10 days until one week before baby's birth.   No other medications taken at pregnancy.  Cecile was breastfed for 7 months.  Mother was on an antibiotic for a while at that time.   Cecile has taken antibiotics for otitis at various times consisting of several courses of Amoxicillin and one course of Ceclor.    
 
The patient's mother has noted no unusual body odor and no acne or other skin changes.   She has had no vaginal discharge.   Growth has been normal.

Family history:  Mother's height is 5'1.5".   Father's height is 5'6.5".

Physical exam:  
Height: 76.6 cm         Weight: 9.58 kg         Breast: Tanner I        
General Appearance:  She is healthy appearing and active.   
Skin:  Warm and dry with no rashes.   There is a small amount of hair, which is very fine and slightly pigmented on the upper back.   There is approximately 1x3 cm birth mark in the left upper back.  
Lungs:  Clear to auscultation. 
Cardiac:  Regular without murmurs.
Abdomen:  Soft with no masses.   
GU:  Revealed very few fine dark hairs which and did not have the appearance of terminal pubic hair.   She had red vaginal mucosa.
Extremities:  Within normal limits.

Impression: She is at the 50 percentile for height and weight at this time.    The mother and grandmother were instructed to bring her back to medical attention if the more typical appearance of pubic hair would be seen.  At that time further evaluation would be initiated.

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

Sincerely,


Ariel Adler, MD           
Attending in Endocrinology   


Addendum: Christopher K. Balkany, M.D.
Fellow in Endocrinology


CONTENT:
Fakhri Welby, M.D.          RE: Maude Hegel
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/24/93

Dear Dr. Welby:

We had the pleasure of seeing Maude in the Endocrine Program on 4/17/95 for short stature/growth failure.

History: This is an almost 16 month old who is a former 4 lb 4 oz product of a 36 week gestation.  Mother was inducted at 36 weeks for IUGR, first noted at 7 months gestation.  Mom reports the IUGR was secondary to placental insufficiency.  Pregnancy was otherwise complicated by maternal low platelets throughout pregnancy.  The mother reports finding of an asymptomatic CMV infection.  Maude was tested negative for CMV at three months.  Maude's growth has been significant for height developing along the 5%, head circumference also developing along the 5%.  Weight falling off of the 5% curve since six months of age and is now 50% for a five month old.  Mom reports a dietary history of 3 oz of cereal in the morning, 8 oz of spaghetti at lunch and a dinner of vegetables and meats.  She drinks about 24 oz of milk per day and several bottles of juice.  Has no history of vomiting or spitting.  She has 2-3 formed stools a day, these are not greasy and do not float.  She has a history of normal development.  She walked at ten months and now says about ten words.   No history of frequent infections or past hospitalizations.  Her work-up so far at Center Pediatrics has included normal CBC, electrolytes, LFT's, TFT's, sed rate and a bone age done at nine months read as about 7 months.

Current medications: None.  No allergies to any medications.  Has an allergy to oranges and develops a skin rash.

Review of systems:  Noncontributory.

Family history:  Significant for healthy parents.  Mother is currently expecting another child.   Maude's grandfather died at age 44 of a heart attack and father's uncle has had triple bypass at an early age.  Father's mother died of ovarian cancer at age 65 and maternal grandmother is still living with heart disease and diabetes.  

Mother's height is 5'5" and father is 5'9 1/2".  Paternal grandmother 5'3", grandfather 6'1".  Maternal grandmother 5'3", grandfather 5'2".  Mother reports short stature siblings in her family.

Physical exam:  
Height: 71.8 cm         Weight: 7.37 kg         Pulse: 92               
Pubic hair: Tanner I    
General: Alert, vigorous, well appearing child who cried vigorously during exam.
Head circumference = 44.5.  Height was 5% for age and weight was 50% for a five month old.  Respiratory rate was 20.  She is a markedly small child for age, muscular in appearance.
HEENT: Significant for a closed anterior fontanelle.  Nondysmorphic facies.  She had no adenopathy on exam.
Chest: Clear to auscultation.
Heart: Regular RR, without murmur.
Abdomen: Positive bowel sounds, soft, nontender, nondistended, no hepatosplenomegaly.
Extremities: Appeared normal with normal appearing digits.
Neurological: Alert, appropriate, DTRs were 2+, cranial nerves 2-12 intact.  She walks well, has good tone, and said ten words.

No labs were done at this time.

Impression: This is a 16 month old former IUGR infant who has been growing steadily along the 5%centile  for height and head circumference, but has fallen off the weight curve.  She appears to have a good dietary history and no history consistent with malabsorption.  When assessed at ten months her bone age appeared approximately equal to her chronologic age, which mitigates for normal homeostasis.  This patient's small stature seems most likely due to genetic small stature.  We would recommend a karyotype to rule out Turner's syndrome or other genetic causes of short stature  We would recommend repeating a bone age as the six month interval since the previous bone age would be an appropriate time for reassessment.

Disposition: Neither test was accomplished at this time.  A return appointment has been made to see Maude back in three months.  If you have not obtained them before that time, we can do so.
Thank you for letting us share in the care of Maude.

Sincerely,


Fozzie Green, MD         Maritza Brezny, MD             
                             Attending in Endocrinology


CONTENT:
Cavinder Khan, M.D.          RE: Art Macleod
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/05/89

Dear Dr. Khan:

We had the pleasure of seeing Art in the Endocrine Program on 3/14/94 for short stature.

History: Art is a 4 and 7/12 year old white male with a history of poor growth and poor weight gain since birth.   

The patient was a full term infant born by normal standard vaginal delivery without complications.  His birth weight was 6 pounds 13 ounces.  His parents first noted poor weight gain early in his life and do not report a period of weight loss.  He does not have a history of diarrhea or constipation.   There is no history of polyuria or polydipsia.  The parents are not aware of any genitourinary anomalies.  He is a well child who has been developing appropriately without any symptoms of regression.  The parents report Art to be a little hyperactive.   The patient was initially started on breast milk.  Solids were started at around 6 months of age.    His diet includes plenty of fluids in the form of juices.   His parents report that he eats a little bit of everything and does not avoid particular foods.

Current medications: None.

Past medical history:  Significant for recurrent ear infections.  He had bilateral ear tube placement at 8 months of age.

Family history:  Significant for the absence of gastrointestinal illnesses, cystic fibrosis or renal disease.   Mother's height is 5'4 3/4" and father's height is 5'9.5".  Art has a 2 year old and a 3 month old brother.   The 2 year old apparently is growing along the 50th percentile for both height and weight.  The maternal growth was delayed.  She had menarche at age 18.

Physical exam:  
Height: 97.8 cm         Weight: 13 kg           BP: 100/65              
Pulse: 82               Breast: Tanner I        Pubic hair: Tanner I    
General: Well appearing boy, very active in no apparent distress.
Skin: No rashes.  
HEENT: Clear oropharynx.  Tympanic membranes were not visualized. Extraocular muscles were intact.  Pupils were equal, round and reactive to light.
Neck: Supple without thyromegaly.  No lymphadenopathy. 
Chest: Clear to percussion and auscultation with pectus excavatum.  No rosary bead appearance of the costo-vertebral joints.   
Heart:  Normal rhythm without a murmur.
Abdomen: Soft, nontender, nondistended without masses.  
Genitourinary: Descended testes, 2x1 cm bilaterally, circumcised. No pubic hair was noted.  
Extremities: No clubbing, cyanosis or edema.  
Neurological: Revealed intact cranial nerves with good motor function; deep tendon reflexes were 2+ bilaterally.  

Bone age was 3.5 years.

Laboratory studies:  IGF-1 55 ng/mL, T4 7.7 mcg/dL, TSH 3.9 mcU/mL, sodium 138 mEq/L, potassium 3.5 mEq/L, chloride 104 mEq/L, bicarbonate 19 mEq/L, BUN 11 and creatinine 0.4, glucose 109, hematocrit 31.2%, white blood cell count 7.9k,  platelet count of 279,000, AST 35, ALT 9, ESR of 5mm/hr.  Urinalysis: pH 7.0 with a negative urine dip.

Impression: Art is a 4 and 7/12 year old white boy with short stature and low weight,  who is otherwise developing well.   He has apparently fallen off his growth track over the last two years.  His weight has always been below the 5th percentile.  In addition to endocrinologic reasons for poor growth, other organic causes of growth and weight gain should be explored.  Our initial laboratory screen is significant for a mildly decreased bicarbonate level in the setting of a normal urinary pH.   This finding could be due to renal tubular acidosis.    Cystic fibrosis or celiac disease are other common causes of poor growth that are not excluded.

Endocrinologic reasons for poor growth include deficiencies along the growth hormone axis, thyroid disease and adrenal disorders.   The latter two diagnoses are less likely in Art given that he has continued to grow and weight gain has not been excessive.  Thyroid function tests were within normal limits.   Of note, the IGF-1 level is also within normal limits for Art's age.   

We recommend close monitoring of Art's growth.   As part of his organic work up, repeat electrolyte measurements, a sweat test and anti-endomyseal antibody screen for  gluten enteropathy will prove useful to rule out metabolic acidosis, cystic fibrosis and celiac disease, respectively.    It is possible that the mild metabolic acidosis noted on our initial screen is secondary to blood drawing technique.   We will be happy to arrange for this test during the next clinic visit.

Disposition: We would like to see Art in six months to further evaluate his growth.
Thank you for letting us share in the care of Art.

Sincerely,


Brett Meisner, MD               Kendell Simpson, MD             
Fellow in Endocrinology      Clinical Director


CONTENT:
Cavinder Khan, M.D.          RE: Art Macleod
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  08/05/89

Dear Dr. Khan:

We had the pleasure of seeing Art in the Endocrine Program on 9/26/94 for short stature.

History: Art is a 5 1/12 year old boy with a history of poor growth and poor weight gain since birth.   

He has been feeling well in the past six months.  There have been no intercurrent illnesses.  He is a very active, sociable boy with good attention span.  His appetite has markedly improved in the last month.  His mother is concerned about weight gain; he has gained one pound in the last six months.  Review of systems is negative.

Current medications: None.

Physical exam:  
Height: 101.4 cm        Weight: 13.4 kg         BP: 92/63               
Pulse: 73               Breast: Tanner I        Pubic hair: Tanner I    
R. testis: 2 x 1 cm     L. testis: 2 x 1 cm     Phallus length: 3.5 cm  
Mid-shaft diam.: 1.3 cm 
General: Well appearing boy, very active in no apparent distress.
Skin: No rashes.  
HEENT: Clear oropharynx.  Extraocular muscles were intact.  Pupils were equal, round and reactive to light.
Neck: Supple without thyromegaly.  No lymphadenopathy. 
Chest: Clear to and auscultation with pectus excavatum. 
Heart:  Normal rhythm without a murmur.
Abdomen: Soft, nontender, nondistended without masses.  
Genitourinary: Descended testes, circumcised.  
Extremities: No clubbing, cyanosis or edema.  
Neurological: Normal

Laboratory studies:  On 8/31/94 normal electrolytes, bicarbonate 29.7 mg/dL.  Sweat test today was normal.

Impression: Art is growing at rate of 7 cm/year, adequate for his age.  His presentation is most consistent with constitutional delay since both his endocrine screen and systemic work up have been normal to date.  Of note, celiac disease has not been fully ruled out.  Yearly height measurements will be adequate to follow his growth.  A significant decrease in growth velocity will warrant further endocrine evaluation.  We will be happy to follow Art in our clinic if you deem our evaluation is necessary.

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

Sincerely,


Brett Meisner, MD               Maritza Brezny, MD             
Fellow in Endocrinology      Attending in Endocrinology
  


Problem list to date:
SHORT-STATURE                                09/28/94  
CONSTITUTIONAL-DELAY                         09/28/94  
Transcribed by Diaz_r


CONTENT:
Uta Chef, M.D.          RE: Natalie Francis
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/05/92

Dear Dr. Chef:

We had the pleasure of seeing Natalie in the Endocrine Program on 3/10/94 for breast tissue development.

History: Natalie is a 19 month old girl whose mother noted breast tissue enlargement on the left side for several months.  There have been no changes since then.  There is no history of galactorrhea, fevers or tenderness.   There is no axillary or pubic hair or vaginal bleeding or discharge noted by the mother.

Current medications: None.

Past medical history:  Pregnancy was complicated by premature labor treated with Trabutalene.  She was  a full-term baby delivered vaginally.  She has not had any other medical problems.   She, however,  has been persistently low weight for her height and age.

Review of systems:  Negative.

Family history:  She has a 15 year old brother with a a horseshoe kidney without problems, a 13 year old sister who is healthy.  Both parents are healthy.

Physical exam:  
Height: 81.5 cm         Weight: 9.31 kg         
Head circumference is 46.5 cm.   
General: Well appearing.   
Skin: Unremarkable.
HEENT: EOMI, PERRL.  
Neck: There are no lymph nodes noted.   
Chest: Clear.  There are quarter sized, soft breast tissue bilaterally, nontender.  Cardiac: Regular rate and rhythm without murmur.
Abdomen: Soft, nontender.  No hepatosplenomegaly, no masses noted.
GU:  Tanner I, non-estrogenized, normal appearing vagina and genitalia.  
Neurological: Normal.

Impression: Natalie is a 19 month old female with small bilateral breast development, no other indications of estrogen effect.  Breast size is stable at this point.  

Her other issues are that she is low weight for height and this is likely unrelated to breast development.


Laboratory studies pending at this time include: We have ordered a pelvic ultrasound to rule out ovarian mass or cyst.
Disposition: Follow up as indicated.
Thank you for letting us share in the care of Natalie.

Sincerely,


Eldre Crane, MD            Elmo Domingo, MD                 
                             Attending in Endocrinology   


Addendum: Resident in Pediatrics


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

We had the pleasure of seeing Heidi in the Endocrine Program on 4/25/94 for .

History: I saw Heidi, age 2 years 2 months, for evaluaton of short stature.  

As you know, she had a consultation with Dr. Crawford at MGH in June 1993.  A clear cut diagnosis was not possible at that time.  The possibility of a mild form of Cornelia deLange syndrome was entertained.   Heidi also saw Dr. Holmes.  Mild dysmorphic feature were noted in addition to mild pulmonic valve stenosis, but the karotype was normal and a specific diagnosis was not possible.   

Heidi recently had a follow up visit with Dr. Lang who was of the opinion that the pulmonic valve stenosis is less severe and the foramen ovale is no longer patent.   

Heidi is currently receiving no medications, vitamins or mineral supplements.  She has three meals and three snacks daily.  She has a varied and nutritious diet but does not eat meat or chicken and has fruit only occasionally.  She has daily bowel movements; stools were described as "mushy".   She has delayed speech and walking was delayed.  She has never had seizures or loss of consciousness.  She has had intermittent otitis media.  Her hearing was described as being normal.  She has not had urinary tract infections.  

She began to walk at 14 months and is now beginning to run.  Her parents stated that her balance was not quite normal.   She has good language comprehension.

She was the product of a full term pregnancy and was born by normal vaginal delivery.   Mother had anemia during pregnancy and was treated with vitamins and iron.   Labor lasted approximately one and a half hours;  strong labor was brief.   Birth weight was 6 pounds 11 ounces and length was 18 inches.   She was nursed for two months but had a poor suck and was changed to formula (Isomil).   She never had a large appetite.   The only significant postnatal problem was a clogged lacrimal duct which required manipulation to open the duct.

Family history:  Mother is 35 years old and 5'1", father is 36 years old and is 5'10".   Both are healthy.  Maternal grandfather 5'10", maternal grandmother 5'0" and has had hypothyroidism since age ? 20 years.   Maternal greatgrandmother also has hypothyroidism.  Maternal grandfather is 5'10"; paternal grandmother 5'6" and paternal granfather is 6'1".   Maternal uncle is 5'8", paternal uncles 6'0" and 5'7".   Heidi has a 6 year old brother who is in the 85th percentile.

Physical exam:  
Height: 77.6 cm         Weight: 10.2 kg         U/L ratio:  1.16        
Arm span: 75            
Head circumference 45.3 cm.  Upper to lower segment ratio: 1.12.  
General: Examination was difficult because she was fearful and crying. 
Skin: Smooth, warm and there were no rashes.  
HEENT: Hair texture was normal and she had normal nails.  Palate was normal; there were 12 erupted teeth.  Anterior fontanelle admitted the tip of a finger.   
Neck: There was no neck webbing.  Thyroid gland was not palpable.
Chest: Clear to percussion and auscultation.   
Heart: There was no heart murmur audible at this time. 
Abdomen:   Liver, spleen and kidneys were not palapble and there were no abdominal masses.  
Genitourinary: She had normal female external genitalia.   
Extremities: Normal.  
Neurological: Deep tendon reflexes were 2+ and symmetric in the lower extremities.

Laboratory studies:  Hemoglobin 10.8 g/dL, MCV 72, white cell count 18.67k, platelets 295,000, sedimentation rate 9 mm/hr.  Total protein 7.2 g/dL, albumin g/dL.  Serum AST, ALT and LDH were 23, 11 and 136 IU/L.    Venous PH 7.35, PCO2 44 and TCO2 meq/L, serum calcium 9.8, magnesium 1.8, phosphorous 4.9 mg/dL.  Sodium 139, potassium 4.2, chloride 105, CO2 22, BUN 18, creatinine 0.1, blood glucose 108 mg/dL.   Serum thyroxine 9.6 mg/dL, TBGI 0.95, TSH 2.2 mcU/mL, all normal.   Cholesterol 149, triglyceride 195 mg/dL, serum IGF-1 30 ng/mL (normal range 17 to 248, mean 66).   Bone age was 18 months (1 standard deviation 4.6 months).

Impression: Heidi's growth rate is very slow.  It appears that she has grown 1.5 cm in five months.   She has the average length of a 15 month old; whereas her length is well below the 5th percentile, her weigth is at the 5th percentile. 

Concerning possible causes for her slow growth, I considered the possibilty of Noonan's syndrome because of the presence of pulmonic valve stenosis.  However, she lacks the physical stigmata usually associated with this syndrome.   The normal chromosones rule out Turner syndrome.   One has to consider the possibility of growth hormone deficiency and I would recommend pituitary functon tests at this time to evaluate her growth hormone response to provocative stimuli.

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

Sincerely,


Bernardo Bird, MD         
Clinical Director            


Addendum: Associate Professor of Pediatrics, HMS


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

We had the pleasure of seeing Heidi in the Endocrine Program on 10/31/94 for follow-up of short stature.

History: I saw Heidi, age 2 years 8 months, in follow-up for short stature.

She had neuroendocrine testing performed in May 1994, at which time growth hormone reached a peak level of 13.8 ng/ml in response to insulin-induced hypoglycemia and a peak level of 9.2 ng/ml after administration of glucagon.  Her serum cortisol level increased to 29.1 5g/dl, prolactin to 37 ng/ml, normal responses.  Her baseline serum thyroxine was 9.7 5g/dl, TBGI 0.88, TT3 162 ng/dl, and TSH 2.3 5U/ml, all normal; serum GHBP was 101 pmol/L, all within the normal range for her age.  These results indicate normal growth hormone and cortisol secretion.  Thyroid function was normal and her prolactin response did not suggest any abnormality of hypothalamic-pituitary dysfunction.  In the interim, Heidi has enjoyed good health except for a few days prior to this visit during which she appears to have had a viral syndrome.  Her appetite was unchanged but her mother stated that there is more variety in her diet.  She has bowel movements at least once daily and they are well-formed.  She still wears diapers.  She sleeps undisturbed from 7:00 p.m. until 7:00 a.m.  She does not nap during the day.   She has made progress with respect to her speech.  She now has 4-5 words and her mother stated that her developmental status is close to age-appropriate.  She was described as being a very social child who is happy and has abundant energy.  

She does have an astigmatism in both eyes.  

Medications: none.

Physical exam:  
Height: 80.2 cm         Weight: 10.8 kg         U/L ratio:  1.08        
Arm span: 78            Breast: Tanner I        
Length:  80.8 cm.
Head circumference: 46 cm.
General: Physical exam was difficult because she was terrified and unco-operative.  She was petite, but appeared well-developed and well-nourished.  She has no obvious dysmorphic features.  Skin was smooth and warm.  No nevi; normal nails and hair.  HEENT was unremarkable; she has a normal posterior hairline.  Thyroid gland was not palpable.  Lymph nodes were not enlarged.  Chest was clear to percussion and auscultation.  I did not hear a heart murmur.  Abdomen: no organ enlargement or masses.  She has normal appearing female external genitalia.  Extremities were normal with ? mild genu valgus.  Neurologic: 2+ and symmetric DTRs in the lower extremities.

Laboratory studies:  Karyotype 46, XX; bone age 18 months (SD 4 months); IGF-1 21 ng/ml (normal 17-248), IGFBP3 1.0  mg/L (normal 1.4-3.0) and GHBP 456 pmol/L (normal 95-762).

Impression: Heidi's height is well below the 5th percentile; weight is just below the 5th percentile.  Her weight for height is in the 50th centile.  Head circumference is <5th centile.  Her annualized growth velocity was 6.6 cm/yr based on her growth during the last six months.  Allsion's IGF-1 is at the extreme low end of the normal range; IGFBP3 is low for her age, but she does have normal GHBP levels.  Her growth hormone levels in response to provocative stimuli rule out classic growth hormone deficiency.   The chromosome analysis rules out Turner syndrome.  Her weight for height does not support a diagnosis of nutritional deficiency or a gastrointestinal cause for poor growth.  Her body proportions are not those of a child with hypochondroplasia.  Her growth pattern, delayed bone age, low IGF-1 and IGF BP3 levels, are consistent with GH deficiency.  One wonders, therefore, if she has a neurosecretory defect that is not evident from classical testing.   She does have a small head circumference and there has been mild developmental delay and, therefore, it is possible that I am overlooking a mild form of a genetic syndrome characterized by growth failure.   I would recommend that Heidi see a geneticist for a second opinion .

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

Sincerely,


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


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

We had the pleasure of seeing Heidi in the Endocrine Program on 4/24/95 for .

History: I saw Heidi, age 3 years 2 months, for follow up  of short stature.

On the advice of Dr. Feingold, her parents have been encouraging her to consume additional calories to rule out the possibility of a nutritional cause for her slow growth.  She has enjoyed good general health.  She was recently seen by Dr. Lang whose impression was that Heidi's pulmonic valve stenosis is mild.  Heidi has been physically active.  She is making normal developmental progress.

Although I have not seen the written report, by the parent's report a  skeletal survey showed no evidence of skeletal dysplasia; and the teleconference arranged by Dr. Feingold did not help to make a specific diagnosis that would help to explain Heidi'd slow growth.

Current medications: None.

Physical exam:  
Height: 82.3 cm         Weight: 11.8 kg         U/L ratio:  1.03        
Arm span: 82.5          Breast: Tanner I        Pubic hair: Tanner I    
Head circumference was 46 cm.  Blood pressure was difficult to obtain because she was crying.  She looked well, had good color and hydration.  Skin was smooth and warm.  HEENT was unremarkable.  She has twenty erupted teeth.  The palate was normal.  Chest was clear to percussion and auscultation.  With her crying, I was unable to adequately evaluate her heart.  Abdomen revealed no organ enlargement or masses.  She has normal female external genitalia.  Neurologic exam revealed 2-3+ symmetric DTR's in the lower extremities.

Bone age was 2 years (standard deviation = 6 months); serum IGF-1 51 (normal 17-248) mg/ml, IGFBP-3 1.3 (normal 1.4-3.0) mg/L, GHBP 81 (normal 66-306) pg/L.

Heidi has grown 2.1 cm in the past six months, an annualized growth velocity of 4.2 cm/year, which is significantly less than the 3rd percentile.  During this interval, her weight has increased 1.0 kg.  Her serum IGF-1 has increased from a value of 21 ng/ml in October, 1994, and her IGFBP-3 has increased from 1.0 to 1.3 mg/L.  This is still just below the normal range (1.4-3.0).  Her GHBP is within the normal range (66-306).

Impression: Heidi's bone age has advanced.  Her serum IGF-1 level has increased, but her IGFBP-3 remains just below the lower limit of normal.  There has not been a dramatic response to calories; therefore, I would recommend resumption of a normal diet for age so as not to cause obesity.  I would be in favor of an additional six months of careful follow up to monitor her growth.  If her growth rate does not increase during the next six months, I would be willing to consider a trial of growth hormone therapy.

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

Sincerely,


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


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

I saw Heidi, age 3 years 7 months, for follow-up of short stature.   

She has enjoyed good health, appetite has increased, and a dietary  review indicates that she has a balanced and nutritious diet. 

She began wearing glasses in July 1995.   

She is currently not taking any medications.

Current medications: None.

Physical exam:
HT: 85.3 cm    WT: 11.95 cm    
Arm span: 84 cm    U/L ratio:  1.08
Pulse: 100              
Physical examination revealed a well-developed, well-nourished petite girl.  Skin was smooth and warm.  She wore glasses.  The remainder of  the examination was unremarkable, except to note that she has genu valgus.

Laboratory studies: IGF-1 44 ng/ml (17-248) and IGFBP-3 1.4 mg/L (1.4-3.0), both within the normal range.

Impression: In the interval since October 31, 1994, Allsion has grown 5.1 cm, an  annualized growth velocity of 5.8 cm. per year, which is approximately 3rd percentile.  Compared to her height on April 24, 1995, she  has grown 3 cm. (in five months), an annualized velocity of 7.2 cm. per year.  The screening tests for GH insufficiency are within the normal range.  My impression is that her pattern of growth is consistent with constitutional growth delay.  Heidi's growth rate is at the low end of the normal range.

Disposition: Provided that she  continues to grow at a normal rate, I would not be in favor of treating her with growth hormone. 

I reviewed the results with both her parents, and answered numerous questions concerning her growth and growth potential.  We discussed the psychosocial implications of short stature and the  importance of treating the child in an age appropriate manner and not "babying" her.


Sincerely,





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


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

I saw Heidi, age 4 years, for follow up of short stature. 

Medications:  Flinstone multivitamin once daily. 

There has been no change in her appetite or bowel function.  She sleeps well.  She is an active child who attends a preschool program three days per week.  She sleeps undisturbed from 7:30 p.m. until approximately 6:30 a.m.  General health has been good.

Current medications: None.

Physical exam:
HT: 86.6 cm             WT: 12.8 kg   
Arm span: 85 cm         U/L ratio:  1.09        HC: 47.4 cm
BP: 80/52               Pulse: 100                    
Breast: Tanner I        Pubic hair: Tanner I    
She appeared well, had good color and hydration.  Skin was smooth and warm.  She wore glasses.  There was no thyromegaly.  Chest was clear and heart was normal.  Abdomen was "chubby".  The liver edge was deep and blunt  approximately 3 cm. below the right costal margin; no splenomegaly.  She has genu valgus.  Deep tendon reflexes were 2+ and symmetric.  She has normal sensation.
Bone age: 3 years

Laboratory studies: Serum IGF-I 69 ng/mL (normal 17-248), T4 10.0 5g/dL, TBGI .85, TSH 3.9 5U/mL, all normal.  IGFBP-3 pending.  Bone age 3 years (SD = 9/12 months).  Bone age has advanced one year in 11 months.

Impression: Her growth velocity over the past 10.5 months is 4.9 cm/yr (less than 3rd  percentile).  Her current "height age" is 2 years.

Inspection of her growth curve reveals that her height has deviated further from the lowest percentile on the height curve.  Weight is normal.  She is relatively overweight for height.  Her TFTs were normal; bone age has matured by one year in 11 months; her IGF-1 level has increased and is now close to the normal mean for her age.  

Frankly, Heidi's pattern of growth continues to perplex me.  I discussed with her parents my suspicion that Heidi may have non-classical growth hormone insufficiency.  However, the most recent IGF-1 level and progression of skeletal maturation argues against that diagnosis.  Once again, we raised the question of a trial of growth hormone therapy in the future.  Mr. Hefner was in favor of a period of further observation.  I think he's right!


Sincerely,






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


CONTENT:
Etta Swartzenegger, M.D.          RE: Heidi Hefner
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/24/92

Dear Dr. Swartzenegger:

I saw Heidi, age four years seven months, for follow-up of short stature. 

Medications:  None. 

She has enjoyed good health in the interval since I last saw her.  She attends nursery school from 9 a.m. to noon.  Apparently, she is happy at school and very sociable.  Her speech has progressed appropriately.  She sleeps well from approximately 7:30 p.m. until 7 a.m. without disturbance.  According to her mother, "she seems to be eating more."  She has normal bowel function.  Vision has not changed.

Physical exam:
HT: 90.0 cm             WT: 13.7 kg    
Arm span: 90 cm         U/L ratio:  1.09
BP: 105/57              Pulse: 125              
She was a petite child withglasses.  She looked well.  Skin was smooth and warm.  HEENT was unremarkable.  There was no thyromegaly or lymphadenopathy.  Chest was clear.  She had normal heart sounds and no murmur.  She had a moderately protuberant abdomen; liver edge was soft and palpable approximately 1-2 finger- breadths below the costal margin.  She had normal appearing female genitalia.  She has bilateral genu valgus.  Neurological exam was grossly normal.

Impression: In the interval since March 4, 1996, she has grown 3.4 cm., an annualized growth velocity of 6.3 cm. per year.  She has gained 0.9 kg. in this interval.  Over a longer period, from September 19, 1995, her annualized growth velocity is 4.7 cm/yr.   

Plotted on the Tanner growth chart for North American children, she is growing significantly below the curve for late maturing children.  Indeed, she is appreciably below the -2 SD late maturers and her current "height age" is approximately 2.5 years.  Her recent growth velocity (over the past six months) is within the normal range.  As you know from my previous correspondence, we have repeatedly measured IGFBP3, and IGF-1.  The mosts recent tests were within the normal range.  Formal pituitary stimulation testing revealed normal growth hormone levels, and her growth hormone binding protein (GHBP) has been repeatedly normal.     

Some of the clinical features; namely, her relative extreme short stature, chubbiness, and slow growth velocity are suggestive of growth hormone insufficiency.  However, the biochemical support for that diagnosis is lacking.  I am still uncertain about the diagnosis.  Either she has severe constitutional growth delay or she has a form of growth hormone insufficiency.  I have not re-measured IGF-1, IGFBP3, or bone age at this time.  I suggested this be done again in approximately six months.  If Heidi falls further and further below the fifth percentile, I would strongly advocate for a trial of growth hormone  

Her parents are appropriately concerned and frustrated because I have not been able to provide them with a definitive answer concerning the cause of Heidi's slow growth.  Therefore, I have suggested that they seek yet another opinion, perhaps from Dr. Jack Crawford at the Massachusetts General Hospital, who saw her early in life.


Sincerely,





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

cc: Mr. & Mrs. Hefner   21 Major Hale Dr   Framingham, MA  01701
    Peter Lang, M.D.  Dept. of Cardiology  Children's Hospital
    Murray Finegold, M.D.  40 2nd Avenue Suite 460  Waltham, MA 02157


CONTENT:
Yale DeNiro, M.D.          RE: Vasco Teasdale
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/20/91

Dear Dr. DeNiro:

     We had the pleasure of evaluating Vasco in the Renal Program on 6/8/95 for Suppressive Pain.
     3-year old boy with recent onset of  suprapubic discomfort resulting in a painful and cold sensation after  voiding.  There does not appear to be any difficulty in initiating  voiding, nor significant dysuria at the time of voiding, but Vasco is  a poor historian and it is difficult to be sure of the exact timing  during the voiding cycle.   

The symptoms have persisted on and off since the end of April 1995.   Vasco feels that there may be some improvement after voiding, and  that there is occasional penile tip pain prevoiding, although this is  not a consistent finding during questioning.

His current medications include:
     Motrin (Ibuprofen 150 mg. t.i.d. x 24 hrs.)
     Benadryl prn
     Cedar Bactrin

Past medical history:
     Normal milestones and no serious illnesses.

Review of systems:
     No nocturia, anuresis, or hematuria, or urinary  tract infection.  He shows a bug bite on the left arm with significant  hardening and reddening and fever today despite a clear urinalysis.  It  is likely the fever is associated with the bug bite and he is already  planned to be reviewed by Dr. DeNiro later this morning.

Vasco's physical exam was notable for:
     BP 105/63   P 114   R    T    HT 104.4cm   WT 18.2kg
     General:  A well hydrated, well covered, cooperative 3-year  old.  Head, ears, nose, and throat:  Pupils and reactive. 
     Skin: No rashes 
     Neck: Supple
     Lymph Nodes: Not palpable.  
     Heart:  S1, S2, rate rhythm regular.  
     Abdomen: Mild suprapubic tenderness, otherwise no abnormal findings.   
     Genitourinary:   Testes descended.  No meatal or penile lesions. 
     Extremities:  No edema or rash apart from the localized swelling around  the bug bite.  
     Neurological: No localizing signs.
A urinalysis revealed:
     SPEC GR UA  1025            GLUCOSE UA  Neg.       
     PH UA       5               RBC/HPF     0          
     BLOOD UA    Neg.            WBC/HPF     0-1        
     PROTEIN UA        mg/dl     BACTERIA    Neg.  
     Bladder and Transitional- Cells some multinucested.

Laboratory studies at this time include:
          CAU         2.4   MG/DL       CRU       145.4   MG/DL    

     Likely Trigono-urethritis, possibly of a viral nature since there  are a significant number of bladder transitional white cells, some of  which are multinucleated.   

If there were persistent symptoms over a further a 4-6 week period,  a  voiding study and ultrasound would be appropriate, but in the interim a  check to exclude increased calcium excretion and symptomatic measures  using Pyridium for the pain may be reasonable.

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

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Yale DeNiro, M.D.          RE: Vasco Teasdale
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/20/91

Dear Dr. DeNiro:

     We had the pleasure of seeing Vasco in the Renal Program on 10/19/95 for dysuria/frequency.
     This is a 4 year-old boy with history of occasional suprapubic pain who returns for follow-up.  He basically is doing better.  Mother mentioned that there was only one occasion of bladder pain in 6 weeks.  He also had occasional frequency but most of the time it was difficult to assess his real pain appreciation.

His current medications include:


Vasco's physical exam was notable for:
     BP 90/60   P 100   R 22   T    HT 107.9cm   WT 20kg
     General appearance:  he appeared comfortable, in no  acute distress.  
     HEENT revealed clear sclerae, oropharyngeal mucosa  moist, 
     Neck: supple, nonpalpable lymph nodes.  
     Chest: revealed  bilaterally clear to auscultation.
     COR: revealed S1, S2, sinus regular rhythm, there is no murmur appreciated.  Abdomen:  soft, nontender, no flank pain on percussion.   
     GU: normal male external genitalia.  
     Extremities:  no edema, range of motion full.  
     Skin:  warm, well perfused.  
     Neurological exam:  grossly  intact.
A urinalysis revealed:
     SPEC GR UA  1.030           WBC/HPF     N          
     PH UA       8               SQUAM EPI   1          
     WBC ENZ UA  N               RBC CAST    N          
     BLOOD UA    N               WBC CAST    N          
     PROTEIN UA  N               GRAN CAST   N          
     GLUCOSE UA  N               WAXY CAST   N          
     RBC/HPF     N               BACTERIA    N     

Laboratory studies at this time include:


     Vasco is a 4 year-old boy with history of intermittent suprapubic pain for the last 6 months.  Most times the pain is resolved by void, accompanied by occasional frequency but without burning or fever.  A renal ultrasound was performed today and both kidneys and bladder within normal limits.  The recommendation is to encourage voiding by the clock.  There is no further imaging study indicated at this time.

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

               Sincerely,


               Mitsuko Khan, MD
               Director, Clinical Services


CONTENT:
Yan Plato, M.D.          RE: Margaret Ederle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/18/92

Dear Dr. Plato:

We had the pleasure of seeing Margaret in the Endocrine Program on 4/11/94 for breast and pubic hair development.

History: As you well know, she is a 15 3/4 month old infant referred here for further evaluation of persistent breast enlargement and pubic hair development.   She was first noted to have breast development at her first medical check up.   She was then seen reportedly by different doctors who did not mention anything to the parents until her first year exam when you saw her again and noted 2-3 cm palpable breast tissue bilaterally.   At her 15 month follow up, breasts were unchanged but sparse, light pubic hair was noted.   

Other pertinent history includes labial adhesions first noted at age 4 months.  Premarin cream was used for two weeks and then manual separation was done at age 6 months followed by Vaseline applications.  No other symptoms are noted except for hair growth over her lower back, present since birth and actually slowly resolving.  The patient has no vaginal bleeding or discharge.   She has no access to birth control pills or other estrogen or androgen containing medications.   

Her growth has been normal and she is following the same height and weight curves.   No accelerated growth is noted.  Her development is normal as well.   

There is no family history of premature thelarche or precocious puberty. 
Her maternal cousin has lymphoma and developed pubic hair after he received high dose ACTH and the pubic hair subsided after ACTH discontinuation.

Current medications: None at the present time.

Past medical history:  Recurrent otitis, status post tube placement.   No other diseases are reported.

Review of systems:  Only positive for recurrent ear infections that are being treated more easily after the tube placement.  The patient also has chronic upper respiratory infections and a chronic cough.   She also had a past history of heart murmur that apparently resolved.  No other abnormalities are noted.

Family history:  Negative similar complaints as mentioned.   Mother's height is 5'6".  Father's height is 5'11".   Mother has a goiter for which she is on Synthroid and reports a history of hypoglycemia (unclear whether fasting or post prandial hypoglycemia).   Episodes of hypotension after her delivery and two carpal tunnel surgeries.

Physical exam:  
Height: 77.7 cm         Weight: 9.39 kg         BP: 100/36              
Pulse: 126              
General: Healthy looking female infant in no acute distress. 
Skin: Unremarkable.  On care au lait spot is noted over her forehead. 
HEENT: Unremarkable.   
Neck: Thyroid is normal.  
Chest: Reveals bilateral palpable breast tissue.  On the right 4 cm, on the left 3.5 cm.   
Heart: Regular rate and rhythm.   Normal S1 and S2.  No systolic murmur. 
Abdomen: No masses. 
Genitourinary: Remarkable for persistent posterior labial adhesion.  Vaginal mucosa is nonestrogenized.   
Extremities: Normal.
Neurological: Normal.

Impression: 15 3/4 month old female with premature thelarche and labial adhesions.   Her premature thelarche is probably not true precocious puberty as there is no accelerated growth.  We ordered an ultrasound of the ovaries and adrenals to assess her ovarian function expecting to probably see either normal ovaries or normal ovaries containing multiple follicles.    A tumor will hopefully be ruled out.  

The patient will need to be followed up.   The labial adhesions on the other hand, are to be observed and further management with Vaseline or lanolin to try to improve the separation.  If the adhesions persist and start being associated with excessive pubic hair development, excess androgen will be ruled out and ACTH stimulation test would be done then.

Disposition: Return in six months for follow up.
Thank you for letting us share in the care of Margaret.

Sincerely,


Maureen Shields, MD             
Attending in Endocrinology   


Addendum: Nada Kerouz, M.D.
Fellow in Endocrinology

Ultrasound was normal.


CONTENT:
Yan Plato, M.D.          RE: Margaret Ederle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/18/92

Dear Dr. Plato:

We had the pleasure of seeing Margaret in the Endocrine Program on 10/3/94 for premature thelarche.

History: As you know, Margaret is now 22 months old who, since her last visit, has had a slight increase in breast size according to her mother, though no vaginal discharge, bleeding or pubic hair development.  The labial adhesions Margaret exhibited previously improved transiently while the parents were applying a lubricant to the vaginal area however, they have not done so recently.   Of note, she was treated with Estrogen cream for one week in July although the parents do not report an associated change in breast size with this therapy.

Physical exam:  
Height: 84.5 cm         Weight: 10.6 kg         BP: 90/                 
Pulse: 88               Breast: Tanner III      Pubic hair: Tanner I    
General: Margaret was a well appearing girl.  Skin: Skin texture was normal, turgor was good.    HEENT: Intact extraocular movements.   Equally responsive pupils.  Moist oropharynx.  Neck: No thyromegaly.   Chest: Clear to auscultation.  Examination of the chest showed breast tissue measuring 4.6 cm in diameter on the left, 4.2 cm on the right.    Heart: Regular rate and rhythm.    Abdomen: Soft without hepatosplenomegaly or masses.   Genitourinary: Tanner stage I. There was no clitoromegaly.   Labial adhesions were prominent.    Neurological: Nonfocal.

Impression: Since her last visit, Margaret has exhibited a mild increase in the size of her breast tissue.   Her evaluation is otherwise unremarkable except for the presence of continued labial adhesions.   Her previously normal pelvic and adrenal ultrasound is reassuring in evaluation of ovarian pathology, and with continued activation of the hypothalamic pituitary gonadal axis present in prepubertal girls before three years of age we are electing to do no studies today but follow her in six months time to repeat her examination.

Disposition: We hope to see Margaret again in six months time.
Thank you for letting us share in the care of Margaret.

Sincerely,


Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Yan Plato, M.D.          RE: Margaret Ederle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/18/92

Dear Dr. Plato:

We had the pleasure of seeing Margaret in the Endocrine Program on 5/15/95 for  premature thelarche.

History: As you know, Margaret is now 29 months old and parents report there has been no significant change in breast size since her last visit.  She has had no development of pubic hair, no growth acceleration, oracne development though she does have intermittent mood swings.  She wakes up approximately once during the evening at 11 pm then sleeps through the night without difficulty.  Margaret's parents report her having less frequent otitis since placement of new myringotomy tubes in January of this year at which time labial adhesions were also lysed with good result.

Current medications: Benadryl prn.

Physical exam:  
Height: 89.1 cm         Weight: 12.3 kg         BP: 90/                 
Pulse: 88               Breast: Tanner III      Pubic hair: Tanner I    
General: Well appearing.  
Skin: Texture was normal, turgor was good.  
HEENT: Revealed intact extraocular movements.  Equally responsive pupils. Moist oropharynx.  
Neck: Without thyromegaly.  
Chest: Revealed 4.6 cm breast buds bilaterally.   
Heart: Revealed regular rate and rhythm.  
Abdomen: Soft without hepatosplenomegaly or masses.  
Neurological: 2+ and equal deep tendon reflexes with normal relaxation phase.

Impression: Since her last visit, Margaret has had little progression in signs of estrogen production.  She has had no growth acceleration and no evidence of continued pubertal development.  She has no CNS symptoms suggestive of pathology.  All these findings are consistent with premature thelarche.   

Today, as part of her ongoing assessment, will obtain an x-ray of the left hand and wrist for bone age assessment to define the impact of her low level estrogen production on overall skeletal maturation and have elected to see her again in six months time.

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

Sincerely,


Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Yan Plato, M.D.          RE: Margaret Ederle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/18/92

Dear Dr. Plato:

I saw Margaret in the Endocrine Program on 11/27/95 for premature thelarche.

History: As you know, Margaret is a now 35 month old who has been healthy since her last visit.  She has had no significant change in breast size since that time and no pubic hair development, vaginal discharge, headache or vomiting.  The mother does report frequent temper tantrums which resolve and appear quickly.  Her bone age at the time of her last visit was 3 and 6/12 years and a chronologic age of 2 and 6/12 years.

Current medications: None.

Physical exam:
Height: 94.5 cm   Weight: 13.6 kg  Breast: Tanner III   Pubic hair: Tanner I  
BP: 94/72         Pulse: 80               
On examination today, Margaret was a well-appearing girl.  Skin texture: normal, turgor good.  HEENT exam revealed intact extraocular movements, equally responsive pupils, moist oropharynx.  Neck was without thyromegaly.  Chest was clear to auscultation.  Examination of the breasts revealed 4 cm. breast bud on the left, 4 cm. on the right.  Examination of the heart revealed a regular rate and rhythm.  Abdomen was soft.  Neurologic exam showed 2+ and equal deep tendon reflexes with normal relaxation phase.

Impression: Margaret has exhibited stable or slight regression in breast size and no acceleration in linear growth.  She exhibits no signs of estrogen excess currently.  Because of this, we are obtaining no studies today but would like to continue to follow reduction in Margaret's thelarche in six months at which time we will repeat another bone age to ensure excessive maturation is not occurring.



Sincerely,




Maureen Shields, MD             
Attending in Endocrinology


CONTENT:
Yan Plato, M.D.          RE: Margaret Ederle
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/18/92

Dear Dr. Plato:

We had the pleasure of seeing Margaret Ederle in the Endocrine Program on May 28, 1996 in follow up of premature thelarche.  As you know, Margaret  is now a 3 and 5/12 year old who since her last visit has had no significant  change in breast size according to her mother, though has had no  noticeable reduction as well.  She has had no pubic hair or axillary  hair development, no apocrine odor through her sweater and no acne.  She  denies headaches and sleeps through the night without difficulty.  Her  only medical problems have been relatively frequent ear infections since  dislodging of her myringotomy tubes earlier this year.  She continues to  have relatively frequent temper tantrums, unchanged in nature from  previous episodes.  She is currently on no medication.

Physical exam:
HT: 98.6 cm    WT: 14.8 kg      BP: 98/45      Pulse: 120              
Breast: Tanner III      Pubic hair: Tanner I    
On exam today Margaret is a well appearing girl.  Skin texture was normal.   Turgor was normal.  HEENT:  Pupils equal, round, and reactive to light.   Responsive pupils.  There was no thyromegaly.  Chest is clear to  auscultation.  Examination of the breasts revealed 4 cm. breast buds  bilaterally.  Cardiac exam reveals a regular rate and rhythm.  Abdomen  was soft without hepatosplenomegaly or masses.  GU exam:  Normal Tanner  Stage I pubic hair.  Neurologic exam:  2+ and equal deep tendon reflexes  with normal relaxation phase.

Our impression is that since her last visit Margaret has had no  progression in signs of estrogen access, specifically no acceleration of linear growth, and no increase in  breast size.  She does continue to have breast bud maintenance,  suggesting some small amount of residual estrogen production.  To ensure  this not resulting in advancing skeletal age, we are checking a bone age  today and would like to see her again in 6-12 month's time to follow up  her rate of linear growth and progress depending on the results of this  bone age. 

Thank you for allowing us to participate in Margaret's care.


Sincerely,




Maureen Shields, MD             
Attending in Endocrinology   

Addendum: Bone age was 3 6/12 yrs; consistent with chronologic age.


CONTENT:
Tuba Domingo, M.D.          RE: Marcy Feke
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/22/89

Dear Dr. Domingo:

We had the pleasure of seeing Marcy in the Endocrine Program on 5/19/94 for childhood acne.

History: I saw Marcy on 5/19/94 for evaluation of facial acne in a relatively young child.   She is now 4 and 11/12 and was noted to have acne beginning at about 3 and a half years of age.  There has been no growth rate increase,  apocrine odor, body hair changes or body habitus changes.   She is behaviorally normal and is scheduled to start kindergarten in September.   She has been seen by two dermatologists in the Harvard Community Health Plan who suggested endocrine testing because of the unusual finding of acne at this time.   She has not yet had any blood or urine testing.   She has been treated with Clindomycin topically, Salcyic acid washes after treatment with Benzoyl Peroxide and Retin A for this acne.  

Of note, she has had several blood pressure readings in the 110 systolic range with diastolics in the 50-60 range.   She has usually been a little bit upset or agitated when these have been done as she was today even with the Dynmap device.

Past medical history:  Unremarkable.   She did not have neonatal acne.  She has never had salt craving.  Mother is not aware of her having any genital changes, specifically clitoromegaly.  No evidence of vaginal mucous staining or breast budding.

Family history:  Significant for mother with severe acne which developed at age 9 and continues to the present.   She still requires treatment.  She had menarche at age 13.  None of her three siblings had an acne problem and father did not have any problem like this either.   Marcy's now 2 year old brother had transient neonatal acne.  Mother's height is 62 inches, father's height is 71 inches.

Physical exam:  
BP: 107/73              Pulse: 80               Breast: Tanner I        
Pubic hair: Tanner I    
Height 116 cm.  Weight 22.7, which makes her taller than average but growing at a completely normal rate over the past five years according to the excellent growth data that accompanied her.
General: She is a robust girl who looks approximately age 6.   She has hypertrichosis of her arms, back and even vellus type hairs over her mons but mother insists that this is even less evident than had been seen earlier in childhood.    
Skin: She has papular facial acne with some whiteheads.  
Neck: No thyromegaly.   
Abdomen: Unremarkable.   
Genitourinary: External genitals show a normal prepubertal red vulva and introital area mucosa without any signs of clitoromegaly.

Laboratory studies:  Bone age is normal at 5 years.  24 hour urine: 17-ketosteroids 2.1 mg/24 hours (normal), free cortisol 17 mcg/24 hours (normal).  Serum estradiol <5 pg/ml (prepubertal), serum testosterone <10 ng/dL (normal), serum DHEA sulfate 33 mcg/dL (norma), serum 17OH pregnenelone 29 ng/dL (norma), serum androstenedione 11 ng/dL (normal).  T4 9.9 mcg/dL, TBGI 0.85, TSH 2.3 mcU/ml (all normal).

Impression: Idiopathic acne of childhood without evidence of increased androgenization or increased cortisol effect.   This is probably a familial acne and unlikely to be on an Endocrine basis.   It is possible that there could be abnormalities in terms of increased androgen receptor activation in the skin but it is unlikely that she has elevated androgen levels.  The slightly borderline blood pressure needs continued follow up.

Disposition: She need not return for further Endocrine evaluation or follow up.   
Thanks for referring her.
Thank you for letting us share in the care of Marcy.

Sincerely,


Nilto Carter, MD             
Attending in Endocrinology


CONTENT:
Niall Kaelin, M.D.          RE: Agatha Bombeck
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/22/93

Dear Dr. Kaelin:

     We had the pleasure of seeing Agatha in the Renal Program on 7/12/95 for urinary tract infections and vesicoureteral reflux.
     Agatha is a 2-1/2 year old with a history of urinary tract infections and vesicoureteral reflux.  The patient was first diagnosed with a urinary tract infection in April of 1994 when she  presented with fever, vomiting and foul smelling urine.  A cystoscopy and  VCUG were performed which showed normal anatomy but bilateral grade 2 vesicoureteral reflux in May of 1994.  The patient has since been followed with monthly bag urine cultures.  She has had a single urinary tract infection in November of 1994 during which time she was asymptomatic but  had a positive bag urine culture at that time.  She was treated with  Bactrim and has remained on Bactrim prophylaxis since that time.  The  patient had a repeat VCUG in June of this year which showed grade 2 to 3  vesicoureteral reflux bilaterally and was referred to the nephrology  clinic.

Her current medications include:
     BACTRIM 1 tsp p.o.q.d.
     AMOXICILLIN 1 tsp. p.o.t.i.d. x 10 days for OM

Agatha's physical exam was notable for:
     BP 105/65   P    R    T    HT 86.25cm   WT 13.1kg
     On physical examination, Agatha is approximately 50th percentile for weight  and 20th percentile for height.  In general she is a well-developed girl in  no acute distress.  Her HEENT exam was notable only for an erythematous and  dull left tympanic membrane.  Her oropharynx was benign.  Her neck was  supple.  She had no significant lymphadenopathy.  Her lungs were clear to  auscultation bilaterally.  Her cardiac exam was benign with regular rate  and rhythm, normal S1 and S2 and no murmur.  She had normoactive bowel sounds.  Her abdomen was soft, non-distended, non-tender with no  hepatosplenomegaly or mass.  No costovertebral angle tenderness was  elicited.  She had normal female external genitalia.  Her extremity exam  was benign and her neurological exam was non-focal.
A urinalysis revealed:


Laboratory studies at this time include:
          CR          0.4   MG/DL       

     Agatha is a young girl with reflux who has been doing well on urinary tract antibiotic prophylaxis.  She has normal renal function and no evidence of any other underlying renal disease.  We recommend that she continue on prophylaxis and have annual radionuclear cystograms (RNCs) to document if her reflux has resolved.   We suggest follow-up with an RNC rather than a VCUG because of reduced radiation exposure.  If she develops signs or symptoms of a urinary tract infection, a urine culture should be obtained.  If Agatha is not toilet trained, a catheterized specimen should be obtained for culture since a bagged specimen will tend to be contaminated.

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

               Sincerely,


               Antonio Khan, MD
               Instructor in Pediatrics


CONTENT:
Niall Kaelin, M.D.          RE: Agatha Bombeck
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/22/93

Dear Dr. Kaelin:

     We had the pleasure of seeing Agatha in the Renal Program on 4/10/96 for vesicoureteral reflux.
     Agatha has done well since her last visit with us. She continues on Bactrim prophylaxis for her vesicoureteral reflux.  She has had no recurrence of urinary tract infection upon screening in your office.   Agatha as not other complaints.

Her current medications include:
     BACTRIM 1 tsp po q.d.

Agatha's physical exam was notable for:
     BP 92/42   P 86   R 24   T    HT 94.7cm   WT 15.5kg
     General appearance:  no acute distress.  
     HEENT:   moist mucous membranes without exudates or masses.  TMs are clear.   Neck:  supple.  
     Lungs:  clear.  
     Heart:  regular rate and rhythm without murmurs, gallops or rubs.  
     Abdomen:  soft, non-tender, non-distended  without masses or bruits.  
     Extremities:  warm and well-perfused without clubbing, cyanosis or edema.  Skin:  unremarkable.  
     Neurologic:   non-focal.
A urinalysis revealed:
     SPEC GR UA  1.025           WBC/HPF     -          
     PH UA       6.0             SQUAM EPI   -          
     WBC ENZ UA  -               RBC CAST    -          
     BLOOD UA    -               WBC CAST    -          
     PROTEIN UA  -               GRAN CAST   -          
     GLUCOSE UA  -               WAXY CAST   -          
     RBC/HPF     -               BACTERIA    -     

Laboratory studies at this time include:
          CR          0.2   MG/DL (L)   

     Assessment:  Agatha has grade II-III vesicoureteral reflux.  She had an elevated blood pressure in your office last month.  Her blood pressure was normal here.  In light of Agatha's excellent growth and kidney  function, we believe that a blood pressure reading obtained in your  office was transient and not indicative of a significant renal problem.   

We would recommend continuing Bactrim prophylaxis at this time.  Agatha  is scheduled to have a repeat VCUG over the summer.  If her reflux has not improved, or has worsened, surgical reimplantation of her ureters, in consultation with her urologist,  Dr. Craig Peters, should be considered.

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

               Sincerely,


               Conrado Seinfeld, MD         Clotilde Goldwater, MD
               Fellow, Pediatric Nephrology Director, Pediatric Nephrology


CONTENT:
Niall Kaelin, M.D.          RE: Agatha Bombeck
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/22/93

Dear Dr. Kaelin:

We had the pleasure of seeing Agatha in the Renal Program on 10/2/96 for vesicoureteral reflux.
Agatha has done well since her last visit with us.  She has had some  intermittent discomfort on urination over the past two weeks.  However,  she has had two negative urine cultures performed in your office.

Her current medications include:
     BACTRIM 1 tsp q.d.


Agatha's physical exam was notable for:
     BP 98/62   P 131   R    T    HT 99cm   WT 16.4kg
     General appearance:  no acute distress.  
     HEENT:   moist mucous membranes without exudate or masses.  
     Lungs:  clear.   
     Heart:  regular rate and rhythm, without murmur, rub, or gallop.   
     Abdomen:  soft, non-tender, non-distended without masses or bruits.   
     Extremities:  warm and well-perfused without clubbing, cyanosis or  edema.  Neurologic:  non-focal.
A urinalysis revealed:
     SPEC GR UA  1.016           WBC/HPF     0          
     WBC ENZ UA  -               RBC/HPF     0          
     PH UA       8               WBC CAST    0          
     PROTEIN UA  -               RBC CAST    0          
     BLOOD UA    -               GRAN CAST   0          
     GLUCOSE UA  -               WAXY CAST   0     
     BACTERIA    -               SQUAM EPI   0     

Agatha has stable grade II-III vesicourteral reflux.  She is  currently being treated for candidal vaginitis as per your  recommendations.  Agatha had a normal DMSA scan over the summer and had  normal renal function when checked at last visit in April.  Our plan would be to manage Agatha conservatively with prophylactic Bactrim.  We  will repeat a serum creatinine level at the time of the next visit.  I know that  Dr. Peters is planning to repeat her VCUG at the beginning of next  summer.  Once again, more aggressive management with surgery would be an  option if Agatha's vesicoureteral reflux does not improve or worsen.

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

               Sincerely,


               Conrado Seinfeld, MD         Xiajo Bonaparte, MD
               Fellow, Pediatric Nephrology Attending, Pediatric Nephrology


cc:
Craig A. Peters, M.D.
Department of Urology
Children's Hospital


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

We had the pleasure of seeing David in the Endocrine Program on 6/23/94 for .

History: Thanks for referring David, who as you know, is a 15 and 10/12 year old with insulin dependent diabetes diagnosed at age 13 and followed at the Mass. General Program by Dr. Lynn Levinsky.   He takes remarkably good care of himself in terms of adjustment of his diet and maintenance of his insulin regimen.   He has a slightly unusual regimen in that his Humulin Regular is weighted toward the evening because of his dietary pattern.   He is currently taking 4 units of Regular and 19 of NPH in the morning, 14 of Regular and 16 of NPH at night giving him a total of 53 units or 0.85 U/kg/day.    He self injects primarily in his arms and legs which does produce a little bit of hypertrophy in his legs and he may need to increase his site rotation.   He has been gaining weight and is currently 173.7 cm and 62.2 kg.   He has had no significant insulin reactions.   

His diet consists of a fairly normal diet with the exception of avoidance of concentrated sweets.  He is not using an exchange or measured diet program.

He doesn't get as much exercise as he might and has yet to spend time with other young people his age with this condition.   I would hope that someone as responsible as he could become involved in the Joslin Diabetes Camp where he could be a good influence on others.  

He has been testing with his One Touch II monitor tid and his blood sugars are almost invariably between 80 and 220 mg/dL.

Physical exam:  
BP: 110/64              Pulse: 62               
On physical, he shows no signs of diabetic difficulties whatsoever.   He has no signs of retinopathy, thyromegaly and reflexes are normal.  Evidently he sees an ophthalmologist annually and this certainly needs to be continued especially now that he is pubertal even though he has less than 5 years of this condition.   

I understand that his asthma exacerbations with Prednisone treatment have thrown his diabetes for a loop and that is understandable considering the insulin resistant state that the Prednisone produces.   Unfortunately, the asthma comes on for him too quickly for him to benefit by inhaled steroids but, if that were possible, it would certainly be preferable.

Impression: Well controlled insulin dependent diabetes.


Laboratory studies pending at this time include: Glycohemoglobin, T4, thyroid antibodies, cholesterol and HDL.
Disposition: I would like to see him in approximately three months.   Please let me know what lab tests you would want me to order or not order here.   The thyroid function tests and cholesterol probably only need to be done annually and the latter only if he shows elevations.   A glycohemoglobin should be done ever three months.
Thank you for letting us share in the care of David.

Sincerely,


Nilto Carter, MD             
Attending in Endocrinology   


Addendum: Anti-thyroid peroxidase antibodies negative.  T4 9.3 mcg/dL, cholesterol 146 mg/dL, HDL cholesterol 38 mg/dL, glycohemoglobin 9.1%.  All of these are excellent.


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

We had the pleasure of seeing David in the Endocrine Program on 12/8/94 for .

History: I had the pleasure of seeing this 17 and 4/12 year old with insulin dependent diabetes today.   He is a junior in high school and his previous control had been quite excellent as measured by glycohemoglobin assay.  He is now 173.9 cm, 62.2 kg, taking 3 of Regular and 19 of NPH in the morning, 13 of Regular and 16 of NPH before supper, giving him a total of 51 units or 0.82 U/kg/day.   He has not been that physically active and he has not had any significant reactions.   On reviewing his blood sugar results by uploading his meter into my portable computer I note that his control has really deteriorated over the past several months compared to the pre-summer.   He is simply not getting enough exercise and his average blood sugar in the morning is running 185 and he is typically between 185 and 280.   

I urged him to give his night time NPH at bed time rather than at supper and giving only Regular insulin at supper.   He may also require an increase in insulin dose as I advised him, and he is definitely going to have to increase his physical activity and curb some of his extra eating unless it is at appropriate times.   It is really not an eating problem so much because he has gained no weight.   He is not ketonuric and his blood pressure is 118/62 and I did a glycohemoglobin again today which was 11.5%, confirms his poorer control. 
 
I would like to see him again in three months and I am pleased to see that he is both wearing his medical alert necklace and has received the flu vaccine .

Physical exam:  




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

Sincerely,


Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear David,

At the time of your visit on 6/8/95 your glycohemoglobin was 10.5% which is acceptable, although it is worth getting it less than 9% if you can do it without insulin reactions. Your thyroid tests were fine.

Have a good summer.



Sincerely,





Nilto Carter MD


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

We had the pleasure of seeing David in the Endocrine Program on 6/8/95 for evaluation of his insulin dependent diabetes for which he has been treated for 4 1/2 years..

History: He is now 17 10/12 years he is entering his last year of high school in September and hopes to enter a program in the culinary arts.  He is working part time at a local country club as a preparation chef.  

His glycemia seems to have been better over the last couple months, perhaps benefited by giving his PM dose of insulin at bedtime and by some increased physical activity particularly working with a horse.

His blood sugars generally run between 95-180 and they usually around 120 when he checks it each day at lunch during school.  He is not having problematic reactions.  His diet sounds appropriate.

Current medications: Humulin Regular 3 units and 21 NPH in the morning, 15 Regular and 21 NPH at supper giving him 60 units/day or 0.94/kg/day.

Physical exam:  
Height: 174.2 cm        Weight: 64 kg                       
BP: 107/56              Pulse: 85               

Impression: I have drawn a glycohemoglobin, cholesterol, HDL, TFT's.  I think he is doing quite well.  The last glycohemoglobin checked in January was somewhat high at 11.5% and I would hope that, based on the readings that we receive today, they would actually be closer to 9%.

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

Sincerely,




Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

David was back for a follow-up visit with me on 10/05/95 for the insulin  dependent diabetes for which he has been treated now for five years.  At 18-2/12 years in the 12th grade, he is hoping to go to Essex Community College for culinary arts.

He is still having a.m. fasting blood sugars that are in the range of 150 to 200 and in spite of this when he is physically active, even 3 units of regular will drop him to the 50's right before lunch.  His dose of insulin (Humulin Lilly), 21 of NPH with 3 to 4 regular pre-breakfast, 14 or regular pre-supper and 0 to 2 of regular with 22 of NPH at bedtime, giving him a total of 0.96 u/kg/day.  He is over injecting the same sites, particularly his thighs, and it is most evident on his left anterior thigh.

Physical exam:   Ht: 175 cm    Wt: 64.5 kg    BP: 109/57              
He has no retinopathy and no thyromegaly.

Laboratory studies:  At the time of his last visit in June, his glycosylated hemoglobin had lowered a bit to 10.5%, his total cholesterol was 169 mg/dl with an HDL of 40 mg/dl.  Normal thyroid functions with a T4 of 10.5 mcg/dl and a TSH of 0.8 mc/ml.

Impression: GHb was rechecked today (8.9% = excellent).  I recommend that he begin using his buttocks for bedtime injections and, if necessary, increase his NPH to try to get his fasting blood sugar less than 130.  He also has to adjust his dose of regular based on his a.m. activity as he is having lows when he has gym in the morning.

Disposition: I will be seeing him again in three months.



Sincerely,




Nilto Carter, MD             
Associate in Endocrinology   


cc:  Kathleen Mott  448 Dunstable Rd   Tyngsboro,  MA  01879


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

Jake was back to see me on 1/11/96 in follow up for insulin dependent diabetes for which he is now almost five years into treatment.  He continues to do beautifully but did have a significant episode of illness in December, a gastroenteritis with vomiting unaccompanied by acidosis but with profound low blood sugars for which he required several days of hospitalization at Lowell General Hospital for  dehydration.  He has returned to his usual state of function and now continues in his last year of high school, considering the possibility of a culinary program following the finishing of high school. 

He continues to take Humulin insulin NPH 21 units in the morning with 3-4 units of Regular, 12-14 units of Regular pre-supper, and 0-2 Regular with 22 of NPH at 10:30pm, giving him approximately a unit/kg/day of insulin.  He has had no significant loads.  He has received flu vaccine and feels quite well. 

Reviewing his testing book shows that his blood sugars are probably averaging in the range of 130-160 and he often is higher than this in the morning but the reasons for this are not always entirely clear.

Physical exam:
HT: 175.8 cm    WT: 63.3 kg      BP: 126/71      Pulse: 77               
His urine is free of ketones or protein and his last glycosylated hemoglobin of 8.9% in October was excellent.

Impression: I have urged him to try to figure out what prompts his blood sugar to go up or down first thing in the morning and to increase or decrease his NPH the night based on his predictions.   

I did do a glycosylating hemoglobin today and want to note that the assay at Children's has switched to the hemoglobin A1C method, so that the upper limit of normal which had previously been 7.5% for a hemoglobin is now only 6% for an A1C.

Disposition: Thanks for allowing me to share in his care.  I look forward to seeing him again in four months.


Sincerely,




Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

I was pleased to see Jake back again on 5/16/96 at 18 and 9/12 years of age  for assessment of his diabetes for which he has been treated for five  years.  He is now in the 12th grade and has not made definite plans for  next year, although he still continues to consider culinary arts.   

At the time of his last visit, his A1C hemoglobin of 7.3% on 1/11/96 was  excellent.  He is maintaining a dose of 3 of regular and 21 of Humulin  NPH each morning, with 13 of regular at supper because of the increased  eating later in the day, and 2 of Regular and 21 of NPH at bed, giving  him a total of 60 u/day, or 0.95 u/kg/day.  He has  dropped a couple of pounds since I saw him in January, but attributes  this to increased physical activity, not to nocturia or heavy  glycosuria.  He is basically asymptomatic.   

He does not have any significant insulin reactions.  His testing book  shows that his blood sugars in the morning and evening are quite  variable and may be as low as 90 or often as high as 220-240 mg/dL.   

I think some of the reason for this may be his injection sites.  He uses  his arms, legs, and buttocks, but there is an obvious hypertrophy over  his anterior thighs which may be suppressing the absorption of the  insulin administered.  He has no evidence of retinopathy.

Physical exam:
HT: 175.2 cm    WT: 63.2 kg      BP: 120/55         Pulse: 83               
He is 175.2  cm., 63.2 kg., blood pressure of 120/55, pulse 83 and regular. His fingers  do not look overly punctured and he has no evidence of hand contracture.   

I have strongly suggested that Jake not use the areas of his thighs that are hypertrophied as he may have much better absorption if he avoids  them.  If he has no success in getting his sugars consistently under 140  in the morning by rotation of sites, then he should increase his NPH  doses twice a day to approximately 23 units b.i.d.

Laboratory studies:  urine creatinine 53.4 mg/dL, microalbumin 45  mg/gCr, HGB A1C 9.2% (a bit high).   His  urine is free of ketones or protein, but has glucose in it.

Impression: I suggested  that based on some preliminary evidence of protection of glycosulation  of proteins with antioxidants, that he take 400 units of vitamin-E  daily. 

Thank you for allowing me to share in his care.  I look forward to  seeing him in approximately three months.



Sincerely,




Nilto Carter, MD             
Associate in Endocrinology   


Addendum: Sorry for the delay.  This was dictated in May but never got out of the hospital computer.  I saw Jake again on 8/15/96.


cc:  Kathleen Mott,   448 Dunstable Rd   Tyngsboro , MA  01879


CONTENT:
Emerson Bird, M.D.          RE: David Mott
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/28/77

Dear Dr. Bird:

History: Jake was back to see me on 08/15/96, following up on five years' treatment with insulin.  He evidentially had a normal ophthalmologic evaluation last January.  When I last saw him in May, he had dropped a little bit of weight and his A1C hemoglobin had increased to 9.2%.  He needed some encouragement to stop using his over-injected sites on his thighs and to move his sites around, as well as to make some insulin adjustments. 

He has finished high school and is looking forward to working this year,  hopefully, in the culinary arts.  He began working this summer as truck driver for an auto parts store, but found their demands excessive and terminated his employment. 

Current medications: His current regimen includes NPH and regular Humulin insulin, taking 20-23 of NPH in the morning, with 3 of regular; 10-14 at supper, because he eats so much more later in the day; and 20-23 of night time NPH-- giving him between 53 and 63 units/day or .84 to 1.0 units/kg./day.

Physical exam:
HT: 174.9 cm            WT: 62.6 kg
BP: 127/67              Pulse: 95               
He had lost .6 kg. since his last visit in May, and approximately 1 kg. since his visit in January.  His exam was unremarkable with respect to thyromegaly.  He had no retinopathy.  His injection sites looked better.

Laboratory studies: HGB A1C 8.7% (H) (reasonable)   

Impression: In addition to the glycohemoglobin elevation last time, his urine protein excretion was--by the micro-albumin method--was slightly elevated at 45 mg. of micro-albumin/gram of creatine, whereas the upper limits of normal are approximately 15-20.  These tests were not done on an overnight specimen, so I have asked to bring in a pooled overnight specimen for similar review at the time of his next visit.

Disposition: I advised Jake that he probably is under-dosing himself with regular insulin to deal with his post breakfast rise in blood sugar.  In that regard, I have told him to check occasionally at lunch to see how far the excursions go.  He has been reluctant to take more regular in the morning--fearful that as he moves about during the day he may have some insulin reactions.  I believe he is probably going to need somewhere between 5-9 units of regular to cover his breakfast, rather than the current 3 units. 

I look forward to seeing him again in three months.  Thanks for allowing me to share in his care.


Sincerely,





Nilto Carter, MD             
Associate in Endocrinology 

cc:  Jake Mott, 448 Dunstable Rd, PO Box 347, Tyngsboro, MA  01879


CONTENT:
Livia Neptune, M.D.          RE: Aggie Daudet
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/25/85

Dear Dr. Neptune:

We had the pleasure of seeing Aggie in the Endocrine Program on 2/23/95 for .

History: Thanks for referring Aggie, who I saw on 2/23/95 at 9 and 2/12 years of age for evaluation of early puberty.   This third grader was first noted to have some breast budding at around age 8 and a half and over the past several months has noted some pubic hair.   She has also had some growth acceleration and increase in clothing size and shoe size.   She denies any changes in blood spotting on her underpants nor evidence of mucous discharge (see physical noting the appearance of such secretory  changes).   She has had no past history of central nervous system trauma, radiation or infection.  

She is only in the third grade because that is policy in Lexington, and she is the tallest child in the class and certainly the earliest in terms of pubertal development.   She is socially very much age 9 and not at all prepared for early menstruation.  

Her only medications are Albuterol for asthma and some topical creams for eczema.   She has never been treated with any estrogen containing substances nor is she exposed to inhaled or ingested estrogens.

Past medical history:  She was a vertex full term easy delivery.   There was no perinatal insults or head trauma of significance or difficulties in the nursery.   She was hospitalized only once for asthma at age 2.

Review of systems:  Entirely negative except for the findings noted above.   She has no headaches, visual changes, heat or cold intolerance.

Family history:  Mother had menarche at age 11 and 8/12.   She had an elevated prolactin before this pregnancy and she was briefly treated with Bromocriptine which enabled her to conceive.   She stopped the Bromocriptine within a week of conception.   Mother is of Northern European ancestry.  Mother is 65 inches, father 71 inches.   Father is of Greek ancestry, his mother is 62 inches, his father is 70 inches inches by report.   No particular family history of known early menarche or endocrine problems other than as noted.   Aggie's 14 year old brother is at an appropriate stage of puberty.

Physical exam:  
Height: 146.3 cm        Weight: 40.7 kg         BP: 121/72              
Pulse: 98               U/L ratio:  0.93        Arm span: 150           
Breast: Tanner III      Pubic hair: Tanner II   
Bone age is11 years, 2 years advanced.   This gives her a predicted adult height of 165.7 cm. (65.2 inches). 

On physical she looks approximately age 11.   She has no acne or significant apocrine changes.   There is some eczema on her elbows.  Her thyroid is palpable but without nodularity.   Chest is clear and there are no cardiac murmurs.   There are no abdominal masses.   She has Tanner stage II axillary hair, very early, and genitals are normal female genitals without clitoromegaly.   The vaginal mucosa looks estrogenized and there is mucous present at the entroitus.

Laboratory studies:  T4 7.7 ug/dL, TBGI .97, TSH 2.90 uU/mL, FSH 4.20 IU/L, PRL 3.7 ng/mL, E2 27.0 pg/mL

Impression: Early puberty at the extreme end of normal range, with a bone age approximately two years advanced.   Based on the current state of progression she is likely to have menarche in approximately 12-18 months.   Since the process of pubertal progression appears normal and not accompanied by headache, visual changes, etc., it is not likely that there is a central lesion here nor is it likely that this is a peripherally induced puberty (without central stimulation.)  

For social reasons the possibility of using Lupron to suppress puberty after doing an GnRH test should be strongly considered.   This would be given for approximately a year in the hope of gaining some time and a somewhat more appropriate menarcheal timing.

Thanks for allowing me to share in her care.

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

Sincerely,


Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Livia Neptune, M.D.          RE: Aggie Daudet
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/25/85

Dear Dr. Neptune:

We had the pleasure of seeing Aggie in the Endocrine Program on 2/23/95 for evaluation of early puberty.

History: This 9 2/12 year old third grader was first noted to have some breast budding at around age eight and a half and over the past several months has noted some pubic hair.  She has also had some growth acceleration, an increase in clothing and shoe size.  She denies any blood spotting on her underwear nor evidence of mucous or discharge (but see physical exam noting the evidence of such secretory changes).

She has had no past history of central nervous system trauma, irradiation or infection.

She's only in the third grade because that is policy in Lexington and she is the tallest child in the class, certainly the earliest in terms of pubertal development.  She is socially very much age nine and is not at all prepared for the concept of early menstruation.

Current medications: Albuterol for asthma and topical creams for eczema.  She has never been treated with any estrogen containing substances nor is she exposed to inhaled or ingested estrogens.

Past medical history:  She was a full term easy delivery, no perinatal insults or head trauma of significance, no difficulties in the nursery.  She was hospitalized only once for asthma at age 2.

Review of systems:  Entirely negative except for findings noted above.  She has no headaches, visual changes, heat or cold intolerance, constipation.

Family history:  Reveals mom having menarche at age 11 8/12.  She is 65" tall.  She had an elevated prolactin before this pregnancy and was briefly treated with Bromocryptine, which enabled her to conceive.  She stopped the Bromocryptine within a week of conception.  Mother is of northern European ancestry.  Her mother is 64" and father 70".  Father is of Greek ancestry; his mother is 62", his father 70" and his height is 71" by report.  No particular family history of known early menarche or endocrine problems other than as noted.  Aggie's 14 year old brother is at an appropriate pubertal state.

Physical exam:  
Height: 146.3 cm        Weight: 40.7 kg         BP: 121/72              
Pulse: 98               U/L ratio:  0.93        Arm span: 150           
Breast: Tanner III      Pubic hair: Tanner II   
Upper/Lower Segment Ratio:  0.925
Bone age:  2 years advanced.
General:   Looks approximately age 11.  No significant apocrine changes.
Skin:   No acne.  Some eczema on her elbows.
Neck:   Thyroid palpable, but without nodularity.
Chest:  Clear.
Heart:   No murmurs.
Abdomen:  No masses.
Genitourinary:  Normal female without clitoromegaly.   Vagina mucosa looks estrogenized and there is mucous present at the introitus.
Extremities:   Stage 2 axillary hair, very early.
Neurological:
Bone age: 11 years


Impression: Early puberty, at the extreme end of the normal range, with a bone age approximately two years advanced.  Based on the current state of progression, she is likely to have menarche in approximately 12-18 months.  Since the process of pubertal progression appears normal and unaccompanied by headaches, visual changes, etc., it is not likely that there is a central lesion here nor is it likely that this is a peripheral (ovarian) induced puberty without central stimulation.

Disposition: Laboratory test:  Thyroid functions normal (T4 7.7 mcg/dL, TSH 2.9 mcU/mL), estradiol 27 pg/mL - (early pubertal).

For social reasons, the possibility of using Lupron to suppress puberty after doing a GNRH test will be done.  This would be given for approximately a year in the hope of gaining some time until menarche.
Thank you for letting us share in the care of Aggie.

Sincerely,


Nilto Carter, MD             
Associate in Endocrinology   


Addendum: Her 3/20/95 GnRH tests was pubertal.   She should begin on Lupron-Depot Ped. 7.5 mg intramuscularly monthly and I should see her again in 3 months.   Since you may wish to order the prescription through the HCHP pharmacy, I will leave it to you.


CONTENT:
Livia Neptune, M.D.          RE: Aggie Daudet
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/25/85

Dear Dr. Neptune:

Aggie was back for assessment of her response to Depot Lupron therapy to suppress puberty, a therapy that was begun in 3/95 after she was confirmed to show early puberty centrally by virtue of GNRH test.  She has been on 7.5 mg of Depot Lupron monthly and she has had some further growth but no remarkable increased breast or pubertal development and she has not had menses, fortunately. 

Now at 10 and 2/12 in a grade 4-5 combination, she would like to defer menses as long as possible but there is really no reason to continue this treatment.  In fact the dose may be slightly low for her current weight and she may be going forward in linear growth due to the slight reduction in relative Lupron effect.

Physical exam:
HT: 152.2 cm    WT: 48.6 kg    Arm span: 158 cm
It is impossible to do a lower segment ratio due to her recent foot fracture and cast.   She has no galactorrhea.  A bone age to my review today is approximately 11 and 1/2-12 years which gives a final height prediction of between 66-68".
Bone age: 11.5 years

Impression: Although she would like to defer menses and would prefer to stay on the  medication, it is in the interest of her bone mass acquisition that she stop at this point knowing that menses could occur soon.  It could also take at least a year for it to occur and this would be entirely within a normal range regardless.

Disposition: I have drawn an estradiol today and I do not need to see her for further follow up unless you consider it necessary.   

Thanks for allowing me to share in her care.



Sincerely,




Nilto Carter, MD             
Associate in Endocrinology


CONTENT:
Luetta Simpson, M.D.          RE: Margie Dell
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/26/82

Dear Dr. Simpson:

Your patient, Margie Dell, age 12 years, was brought for a consultation concerning her short stature diagnosed as ? partial growth hormone deficiency.

She is on human growth hormone (Genotropin) 4 units three times per week.  The records indicate that she has grown 6 cm. in the last 10 months.  Treatment was started February 1993.  

She first was evaluated in 1986 at which time she was 4 1/2 years and height was 92.5 cm., weight 13.3 kg. and bone age was 2 1/2 years; chromosomes were 46, XX.  

In December 1992 she had an exercise stimulated growth hormone level of 1.4 ng/ml at baseline, 4.4 ng/ml after 20 minutes, 3.1 after 40 minutes.  A Clonidine test (100 mcg po) was performed.  The growth hormone values were 0.84, 0.54, 14, 7.6, 4.8 ng/ml.  The notes indicate that the 60 minutes rise in growth hormone was "close to normal".  The serum IGF-1 was 81 ng/ml (normal 117-771).  Height age at this time was 6 1/2 years; the notes indicate that a bone age was "significantly delayed".  Her serum free thyroxine was 100 nmol/L, TSH 2.2 mcU/mL, both normal.  On the basis of these results she was started on growth hormone, 4 units three times per week.

In April 1994 she had a screening biochemical profile which was normal; serum cortisol 220 nmol/L (normal for a random sample); total thyroxine 120 nmol/L; free thyroxine 19 pmol/L; TSH 0.68 mcU/mL; bone age between 7 years 10 months and 8 years 10 months.

She has a small appetite.  Breakfast is at 6:30 a.m., she has a snack at  10:00 a.m.; her main meal is at 3:00 p.m. (a large meal); dinner is at 8:00 p.m.  She does not eat a wide variety of foods but does not have specific food aversions.  

She sleeps from approximately 10:00 p.m. until 6:00 a.m.; sleep is undisturbed except for occasional nocturia.

She is in the sixth grade and does well in school and obtains good grades.

Current medications: Genotropin (KABI Pharmacia brand of growth hormone) in the dose of 4 IU  per injection three times per week.

Past medical history:  She was the product of a healthy pregnancy, born by normal vaginal delivery; birth weight ~ 3 kg. and length 49 cm.  She may have had rubella but the parents were not clear on this point  

She has been hospitalized once for work-up of her growth failure.  At approximately 7 months of age she fell from her bed and injured the left side of her head with loss of  consciousness for ~ 20 minutes.  She has never had surgery or other injuries.

Review of systems:  Her skin is intermittently dry especially on the dorsum of her hands.  There is no impairment of hearing, vision or motor coordination.  She denies headaches.  She has no symptoms of cardiac dysfunction, no dyspnea.  She was constipated until approximately one year ago but no has daily or every other day bowel movements since increasing her consumption of vegetables.  There are no genitourinary symptoms nor were there neurologic symptoms, and certainly no loss of consciousness or seizures.

Family history:  Mother's height 64", father's height 68 1/2".  The parents are not related.  The maternal grandmother was <5' and a maternal aunt was also <5'.  There is no family history of delayed puberty.  The mother has a half-brother (on her father's side of the family) who has a ? delayed growth pattern.

Physical exam:  
Height: 128.6 cm        Weight: 22.9 kg         BP: 87/55               
Pulse: 80               U/L ratio:  0.84        Arm span: 129           
Breast: Tanner II       Pubic hair: Tanner I    
General: Short, healthy appearing, but petite girl. 
Skin: Smooth and warm.
HEENT: Wax in the external auditory canal.
Neck: No thyromegaly, lymph nodes were unremarkable.
Chest: Clear to percussion and auscultation.
Heart: Regular rate and rhythm and no murmur.
Abdomen: No organ enlargement, no masses.
Genitourinary: Normal.
Extremities: Normal.  No axillary hair.
Neurological:  Head circumference 49 cm.  DTRs 1+ and symmetric; intact touch and vibration sense.  EOMI; PERRL; fundi showed normal discs and vessels.

Laboratory studies:  Serum LH 0.12, FSH 2.4 IU/L, serum estradiol <5 pg/ml, DS 9 mcg/dl, serum IGF-1 164 ng/ml.  Bone age 7 years 10 months (SD 10.2 months).

Impression: I re-plotted her growth data on the Bayer and Bayley longitudinal growth chart.  I am not convinced that she has classic growth hormone deficiency;  rather her pattern appears to be that of a child with constitutional growth delay.  It is puzzling that she shows evidence of early puberty despite a retarded bone age.  The apparent increase in growth rate attributed to growth hormone therapy could be due to secretion of sex steroids.  Because she was here for a limited period of time, we did not pursue further investigations but a GnRH test would certainly be valuable to determine whether she has, indeed, entered early puberty.  The low serum estradiol and measurable FSH does not suggest a primary ovarian source of estrogen.  A pelvic ultrasound, nevertheless, may be helpful to rule out an ovarian cyst or tumor.  I did not recommended discontinuing growth hormone, but whether this will favorably impact her final adult height is not at all clear.

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


Sincerely,




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


CONTENT:
Pluto, M.D.          RE: Margie Dell
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  06/26/82

Dear Dr. Pluto:

I saw Margie, age 13 years 10 months, for follow-up evaluation of growth. 

Medications:  None at the present time. 

According to the father's report, Margie saw Dr. Sakati at King Faisal Specialist Hospital approximately 6 weeks ago.  At that time, a repeat bone age was obtained and, according to father's report, Dr. Sakati recommended stopping growth hormone therapy until she was seen again after an interval of several weeks.  Unfortunately, Mr. Dell did not know the bone age and did not have a report for my review. 

Margie is an 8th grade student and does well in school.  She reported no headaches or visual disturbance.  She sleeps without interruption from 11 p.m. until 6 a.m.  She rarely has nocturia.  She is pre-menarcheal 

Her only complaint was that her skin is dry and "sensitive to the cold."

Current medications: None.

Physical exam:

HT: 140.9 cm            WT: 29.3 kg
BP: 91/51               Pulse: 84               Breast: Tanner IV       
She looked well, skin was smooth and extremities were cool.  She had irregularly shaped front teeth with loss of the outer angle of her central incisors.  She had perleche.  There was no thyromegaly or lymphadenopathy.  Chest was clear.  She had Tanner stage IV breast development.  Heart sounds were normal.  Abdomen unremarkable.  Deep tendon reflexes were 1 to 2+ in her lower extremities.

Laboratory studies:  Serum thyroxine 8.3 5g/dL, TBGI 0.96, TSH 1.2 5U/mL, all normal.  Her serum IGFBP-3 2.7 mg/L (normal range 2.2-5.9), IGF-1 222 ng/mL (normal range for girls 12-15.9 years is 261-1,096).

Impression: According to my records, she has grown since I last saw her approximately 2 years ago.  Her "height age" is approximately 10 years 6 months.  Puberty has progressed.  Her IGFBP-3 is normal, whereas the IGF-1 concentration is modestly below the normal range.  These data do not indicate classical GH insufficiency. 

I do not know the cause of her brittle teeth.  I suggested that she consult a pediatric dentist.  I also recommended daily multivitamins for treatment of perleche.


Sincerely,





Bernardo Bird, MD         
Clinical Director of Endocrinology
Associate Profesor of Pediatrics, HMS


CONTENT:
Placido Green, M.D.          RE: Justin Poinsett
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/11/89

Dear Dr. Green:

We had the pleasure of seeing Justin in the Endocrine Program on 8/11/94 for evaluation of gynecomastia.

History: Justin is 5 and 1/12 years of age.  He was first noted to have gynecomastia in November 1993.  He was seen six months later and it seem to have increased in growth.  There is no history of any tenderness or discharge from either breast.  There is no history of any axillary or pubic hair growth, headaches, polyuria, polydipsia, diarrhea or constipation.   He takes no medications.  His mother is on oral contraceptives, but there is no possibility that Justin could have taken any.  There are no other drugs available in the house.  Of interest is the fact that Mrs. Poinsett is a worker at a pharmaceutical company and is exposed various types of drugs.  She is unsure of the types of chemicals she works with but thinks there may be chemical residue on her clothes when she leaves work.

Past medical history:  Justin is the product of Cesarean section for BPD.  He has had normal growth and development.  He will start kindergarten in September.

Review of systems:  History of recurrent otitis media during the first year of life.  He had myringotomy tubes placed and has had no problems since.

Family history:  Type II diabetes in both grandmothers.  Maternal grandfather died of pancreatic CA and maternal uncle with situs inverses.  Mother's height 5'5", father is 5'9".

Physical exam:  
Height: 112.9 cm        Weight: 21.1 kg         R. testis: 2.0 x 0.8 cm 
L. testis: 1.8 x 0.7 cm Phallus length: 7.0 cm  Mid-shaft diam.: 2.0 cm 
General: Well developed, well nourished boy.  there has been some acceleration of his linear growth over the past 18 months.  Skin: No lesions.  No axillary or pubic hairs.  Neck: Supple, thyroid not palpable.  Chest:  Palpable breast tissue, right diameter ~2.5-3.0 cm., left 1.5-2.0 cm.  The areolae measured 1.4 cm. bilaterally, the nipple 0.3 cm. bilaterally.    Heart: Regular rate and rhythm.  No murmurs, gallops or rubs.  S1,S2 normal.  Abdomen: Soft, nontender.  Positive bowel sounds.  No abdominal masses.  Genitourinary:   prepubertal, no signs of virilization noted.   Extremities: Free range of motion, good tone.  Neurological:  Alert and active.  Nonfocal.

Laboratory studies:  DHA  57   ng/dL;  E2 <5   pg/mL. Hand and wrist x-ray showed a bone age of six years.

Impression: Bilateral, palpable breast tissue without change in areolae or nipples.  His mother states that there was some breast tissue in infancy.  Since there is little evidence of estrogen activity now, perhaps it is residual tissue from earlier changes.  On the other hand, there has been slight acceleration of his growth so we should like to check again in six months or sooner if there is further breast development.

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

Sincerely,


Mitsuko Leary, MD           Mitsuko Simpson, MD             
Fellow in Endocrinology      Chief Emeritus


CONTENT:
Ludovica Gonzo, M.D.          RE: Cesar Ward
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  05/30/78

Dear Dr. Gonzo:

We had the pleasure of seeing Cesar in the Endocrine Program on 6/23/94 for obesity, question small phallus and poor height growth.

History: Cesar is a now 16 year old white male who you have been following for some time with obesity.   He is sent to Endocrine at this time for evaluation of his small phallus and a particular concern of a lack of pubertal growth spurt.  Cesar expresses concern regarding his obesity as well as concerns of a small phallus.   

He states that he exercises fairly regularly enjoying multiple sports including football, basketball, baseball and swimming.   He was seen with his father who feels that Cesar is not as active as he might be.  He reports a fairly small diet in which he doesn't take breakfast generally, has a bagel for lunch and eats a regular dinner.   He does state that he frequently snacks on cookies.   Father at the same time suggested that his intake is significantly more than Cesar initially admits to.   As for his past medical history, he has been followed for reactive airways disease for the past two years.   This is usually triggered by exercise and he has been taking a metered dose inhaler, presumably Ventolin on a prn basis for this problem.   He also takes Bromfed for environmental allergies.   

As for his sexual history, Cesar denies ever having been sexually active.  He is able to achieve and maintain an erection and has normal ejaculations.   He entered puberty approximately one and a half years ago by his report.   He denies alcohol, tobacco or other drug use.   Specifically, he denies anabolic steroid use.   He denies headache, visual changes, constipation or diarrhea as well.

Family history:  Notable for a 45 year old father who admits to alcoholism.  There is a history of cancer and coronary artery disease in the grandparents.   Mother's height is 5'5", father's height is 5'8.5", giving a mid parental height of 5'9".     

Social History:  Remarkable for divorced parents.   He lives with his mother most of the week but sees his father frequently.

Physical exam:  
Height: 172.7 cm        Weight: 125.3 kg        BP: 122/92              
Pulse: 76               
General: Obese, well developed white male in no acute distress.  
Skin: Reveals healing poison ivy over his legs bilaterally.   He also has striae present over his back, abdomen and shoulders.   
HEENT: Unremarkable.  
Neck: Supple without thyromegaly.  There is no lymphadenopathy.  
Chest: Lungs are clear bilaterally.   There is a large amount of adipose tissue in his anterior chest.   
Heart: His heart sounds are distant, there is no audible murmur.   
Abdomen: Belly is soft, nontender and nondistended without hepatosplenomegaly.   It is markedly obese.   
Genitourinary: He is a Tanner IV male who is circumcised.   Testes are palpable bilaterally measuring 4.2x2.5 cm on the right and 4x1.8 cm on the left.  His penile stretch length is 8 cm and mid shaft diameter is 2.5 cm.  
Extremities: Without cyanosis, clubbing or edema.
Neurological: Nonfocal.

Impression: Cesar is a 16 year old obese male.   As we discussed by telephone, at present there does not seem to be an endocrinopathy responsible for his obesity.   Likewise, his phallus does seem to be within acceptable size.   The  large amount of suprapubic fat does obscure his phallus, but his stretched  length is within normal limits.   

With regards to his growth, he is within an inch of obtaining his genetic potential using a mid-parental height of 5'9".   His bone age is also consistent with this chronological age which suggests normal skeletal maturation.  It seem then that his growth is acceptable.    

Given his normal height, we can feel fairly comfortable that his obesity is not on the basis of endocrine abnormalities such as elevated cortisol excretion.   We discussed at length with Cesar and his father the importance of ongoing attention to his weight.  We recommended increasing his aerobic activity on a daily basis and continuing to modify his diet with follow up at HCHP.   We also counselled him on avoiding added salt in his diet given his borderline blood pressure.   He did appear somewhat anxious with his examination which may explain in part the elevated blood pressure.

Thank you very much for allowing us to participate in Cesar's care.   Your endocrine evaluation was complete and reassuring.    We don't need to see Cesar back in the Endocrine program unless you have further concerns.


Laboratory studies pending at this time include: No new laboratory tests were obtained and we appreciated you sending your evaluation which included normal thyroid function tests, testosterone levels, normal random cortisol level and cholesterol screening.
Thank you for letting us share in the care of Cesar.

Sincerely,


Yale Lear, MD            Elmo Domingo, MD                 
                                                          


Addendum: Resident in Pediatrics


CONTENT:
Brezny, M.D.          RE: Leontyne Perutz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/01/85

Dear Dr. Brezny:

     We had the pleasure of seeing Leontyne in the Renal Program on 12/07/95 for HUS.
     

Present Illness:  Leontyne was hospitalized at Children's Hospital in  July, 1994, for hemolytic uremic syndrome.  At that time, she had acute  renal failure with oliguria and a creatinine that peaked at about 2.2  mg/dl.  She did not require dialysis, however, and recovered renal  function.  She was subsequently followed as an outpatient and did well.   She regained normal hematocrit and platelet count.  She had a urinalysis  performed in 8-94 which demonstrated trace protein and no blood.  She  had a subsequent urinalysis performed in 12-94 which showed 1+ protein  and no blood.   

Her mother is concerned about progressive renal disease as a result of  hemolytic uremic syndrome.  She has read a significant amount of  information about this and is concerned about the long-term studies that  have been published from both Yutook and France.  She specifically had  questions about what sorts of tests should be done to evaluate Leontyne in  the long-term and what were her chances of developing chronic renal  failure and possibly diabetes.

Her current medications include:


Leontyne's physical exam was notable for:
     BP 111/51   P 83   R    T    HT 132.4cm   WT 29.6kg
     
     
     Physical exam:  Appears well and in no obvious distress.  Happy, active,  alert and cooperative.  HEENT: negative, fundi are benign, discs are  flat and vessels appear normal.  Lymph nodes: no adenopathy.  Chest: no  rales or wheezes. Cor: S1 normal, S2 split, no S3, S4 or murmur.  Abdomen: soft, flat, no masses, tenderness or organomegaly.   Extremities: no edema.
A urinalysis revealed:
     SPEC GR UA  1.020           WBC/HPF     0          
     PH UA       6.0             SQUAM EPI   0          
     WBC ENZ UA  negat           RBC CAST    0          
     BLOOD UA                    WBC CAST    0          
     PROTEIN UA  2+              GRAN CAST              
     GLUCOSE UA  negat           WAXY CAST              
     RBC/HPF                     BACTERIA    negat 

Laboratory studies at this time include:
          CRU       150.6   MG/DL       U PROT      105   MG/DL (H)

      

Assessment:  Leontyne looks quite well and the degree of renal impairment  associated with her hemolytic uremic syndrome was moderate at worst.   However, I am concerned about the amount of proteinuria on her random  urinalysis.  Since hypertension and proteinuria may be the signs of more  extensive renal damage, I think that is worth following up on this and  to assess whether there is any progression in the amount of it.  We will  do a urine protein to creatinine ratio here in order to quantitate it.   Based upon those results, as well as renal function tests which will be  performed at HCHP, we can plan a course of tests to be followed.  I  would think that annual urine protein creatinine ratios and serum BUNs  and creatinines would be appropriate.  However, I will reserve judgment  on the frequency of that until after I have seen those results. 

I did review most of the long-term results of HUS with Leontyne and her  parents.  Importantly, most of those studies show continuing proteinuria  and mild hypertension in the group as a whole without serious impairment  of any of the individual treatment.  That is, those who have the most  severe disease and who are anuric and on dialysis for several weeks had  the highest likelihood of developing continuing renal disease and  possibly end stage renal disease.  On the other hand, those who had the  more mild disease may have continuing proteinuria as evidence of old  injury but not as evidence of ongoing disease.  Thus, I think that it is  generally worth following these children but, once again, based upon  both those series and our own experience, it is fair to conclude that  the vast majority of children with HUS do not develop serious ongoing  renal disease.

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

               Sincerely,


               Clotilde Goldwater, MD
               Director, Pediatric Nephrology


CONTENT:
Ghada Seinfeld, M.D.          RE: Leontyne Perutz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/01/85

Dear Dr. Seinfeld:

     We had the pleasure of seeing Leontyne in the Renal Program on 12/07/95 for HUS.
     
Leontyne was hospitalized at Children's Hospital in  July, 1994, for hemolytic uremic syndrome.  At that time, she had acute  renal failure with oliguria and a creatinine that peaked at about 2.2  mg/dl.  She did not require dialysis, however, and recovered renal  function.  She was subsequently followed as an outpatient and did well.   She regained normal hematocrit and platelet count.  She had a urinalysis  performed in 8-94 which demonstrated trace protein and no blood.  She  had a subsequent urinalysis performed in 12-94 which showed 1+ protein  and no blood.   

Her mother is concerned about progressive renal disease as a result of  hemolytic uremic syndrome.  She has read a significant amount of  information about this and is concerned about the long-term studies that  have been published from both Utah and France.  She specifically had  questions about what sorts of tests should be done to evaluate Leontyne in  the long-term and what were her chances of developing chronic renal  failure and possibly diabetes.

Her current medications include:


Leontyne's physical exam was notable for:
     BP 111/51   P 83   R    T    HT 132.4cm   WT 29.6kg
     
     Appears well and in no obvious distress.  Happy, active,  alert and cooperative.  HEENT: negative, fundi are benign, discs are  flat and vessels appear normal.  Lymph nodes: no adenopathy.  Chest: no  rales or wheezes. Cor: S1 normal, S2 split, no S3, S4 or murmur.  Abdomen: soft, flat, no masses, tenderness or organomegaly.   Extremities: no edema.
A urinalysis revealed:
     SPEC GR UA  1.020           WBC/HPF     0          
     PH UA       6.0             SQUAM EPI   0          
     WBC ENZ UA  negat           RBC CAST    0          
     BLOOD UA                    WBC CAST    0          
     PROTEIN UA  2+              GRAN CAST              
     GLUCOSE UA  negat           WAXY CAST              
     RBC/HPF                     BACTERIA    negat 

Laboratory studies at this time include:
          CRU       150.6   MG/DL       U PROT      105   MG/DL (H)

      
Assessment:  Leontyne looks quite well and the degree of renal impairment  associated with her hemolytic uremic syndrome was moderate at worst.   However, I am concerned about the amount of proteinuria on her random  urinalysis.  Since hypertension and proteinuria may be the signs of more  extensive renal damage, I think that is worth following up on this and  to assess whether there is any progression in the amount of it.  We will  do a urine protein to creatinine ratio here in order to quantitate it.   Based upon those results, as well as renal function tests which will be  performed at HCHP, we can plan a course of tests to be followed.  I  would think that annual urine protein creatinine ratios and serum BUNs  and creatinines would be appropriate.  However, I will reserve judgment  on the frequency of that until after I have seen those results. 

I did review most of the long-term results of HUS with Leontyne and her  parents.  Importantly, most of those studies show continuing proteinuria  and mild hypertension in the group as a whole without serious impairment  of any of the individual treatment.  That is, those who have the most  severe disease and who are anuric and on dialysis for several weeks had  the highest likelihood of developing continuing renal disease and  possibly end stage renal disease.  On the other hand, those who had the  more mild disease may have continuing proteinuria as evidence of old  injury but not as evidence of ongoing disease.  Thus, I think that it is  generally worth following these children but, once again, based upon  both those series and our own experience, it is fair to conclude that  the vast majority of children with HUS do not develop serious ongoing  renal disease.

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

               Sincerely,


               Clotilde Goldwater, MD
               Director, Pediatric Nephrology


CONTENT:
Ghada Seinfeld, M.D.          RE: Leontyne Perutz
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  12/01/85

Dear Dr. Seinfeld:

We had the pleasure of seeing Leontyne in the Renal Program on 12/19/96 for HUS.
Leontyne continues to do well without significant problems.  She has had  no intercurrent illnesses.  Her mother said that she has been testing her blood pressure and that it has generally remained normal.



Leontyne's physical exam was notable for:
     BP 109/66   P 77   R 12   T    HT 138.3cm   WT 33.5kg
     She appears well, in no acute distress.  
     HEENT:   fundi be benign, disks are flat.  Vessels show no abnormality.  Lymph  nodes:  no adenopathy.  
     Chest:  no rales or wheezes.  
     COR:  S1 normal,  S2 split, no S3, S4 or murmur.  
     Abdomen:  soft and flat, no masses,  tenderness or organomegaly.  
     Extremities:  no edema.
A urinalysis revealed:
     SPEC GR UA  1.020           WBC/HPF     2-5        
     WBC ENZ UA  neg             RBC/HPF     0-2        
     PH UA       6.5             WBC CAST    none       
     PROTEIN UA  2+              RBC CAST    none       
     BLOOD UA    neg             GRAN CAST   none       
     GLUCOSE UA  neg             WAXY CAST   none  
     BACTERIA    neg        

Laboratory studies at this time include:
          CRU       170.6   MG/DL       U PROT      108   MG/DL (H)

Overall, Leontyne is doing quite well.  She continues to have mild  proteinuria.  It was quantitated today with a protein to creatinine  ratio.  At her visit one year ago, that ratio was about 0.7.  Today's  value was 0.63, which is essentially the same. 

Given the lack of hypertension and given that the proteinuria continues to be at about the same level, I do not think that we need to do anything  at this point.  Hopefully, the proteinuria is just the manifestation of  an old injury and is not an indication of ongoing damage.  I do not see any reason for further testing at this time, but I should continue to  follow her on an annual basis.

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

               Sincerely,


               Clotilde Goldwater, MD
               Director, Pediatric Nephrology


CONTENT:
Benise, M.D.          RE: Alice Culpeper
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/06/87

Dear Dr. Benise:

We had the pleasure of seeing Alice in the Endocrine Program on 8/29/94 for high cholesterol/short stature.

History: Dear primary provider for Alice Culpeper:

Alice is a 7 year 7 month old female with hypercholesterolemia and short stature.   By history, her mother has noted that Alice's growth has fallen behind that of her peers.   She has also been noted to have hypercholesterolemia at least twice in Venezuela.  Her mother notes that particularly at night her legs and feet frequently tingle.  She has a good appetite and follows a well balanced diet.   She does not consume butter, mayonnaise, eggs or chocolate as she develops an allergic rash.   

In January, 1994, her mother notes that she developed a gastroenteritis and she had an anaphylactic reaction to Flagyl.   She then developed a severe lymphadenitis for which she was hospitalized for several days.   This infection has completely resolved.   She has one normal bowel movement per day and denies diarrhea or constipation.   There is no change in her energy level.   She is a somewhat sedentary child.  

There is no history of vomiting, heat intolerance or palpitations.   She has mild scoliosis that was noted in 7/94.

Current medications: None except vitamins.

Past medical history:  She has an innocent heart  murmur that was noted when she was a child and has had both a normal Holter and echo.  She was delivered at term via C-section.   She had frequent tonsillitis and otitis as a young child.  There is no thyroid disease, diabetes mellitus or other endocrinopathies.

Family history:  There is hypercholesterolemia in her father, paternal grandmother and half brother.  There is no history of early sudden death.   Maternal height 167 cm.  Paternal height 165 cm.

Physical exam:  
Height: 115 cm          Weight: 23.3 kg         BP: 100/65              
Pulse: 80               U/L ratio:  0.93        Arm span: 118           
Breast: Tanner I        Pubic hair: Tanner I    
General:Well nourished, well developed, in no acute distress.    
Skin: Clear with no cafe au lait spots or other lesions.  
HEENT: PERRL, EOMI, normal fundoscopic exam.  Oropharynx clear with moist mucous membranes.  
Neck: Supple without thyromegaly.   Lymph nodes within normal limits. 
Chest: Clear to auscultation.   Tanner I breasts.   
Heart: Mild sinus arrhythmia with a I/VI systolic ejection murmur noted at the left sternal border.  
Abdomen: Soft with no hepatosplenomegaly or masses.   
Genitourinary: Normal Tanner I female.  
Extremities: No clubbing, cyanosis or edema.   Mild right lower thoracic scoliosis noted.  
Neurological: Nonfocal with reflexes 2+ and toes downgoing.

Laboratory studies:  5/94; cholesterol was 253, HDL 46.2 (normal 39-96), LDL 189 (normal less than 150), triglycerides 89 (normal 30 to 200), total lipids 722 (normal 400-800), P02 23, bicarb 20.1, creatinine 0.5 (normal 0.6-1), urinalysis within normal limits.  Growth hormone level post exercise 15.7 ng/cc.

Labs obtained today included: normal TFTs, normal electrolytes, Ca, Mg, serum bicarbonate.  Phosphorus was slightly low at 4.1 mg/dL.  IGF-1 was normal at 210 ng/mL.  ADLC was normal.  Total cholesterol was elevated at 239 mg/dL.  Triglycerides were normal.

Impression: 7 year 7 month old well appearing female with a documented hypercholesterolemia and recent fall off in growth velocity.   The patient now sits at the 5th percentile on the height curve, while her weight has been maintained at the 50th percentile.  Normal post exercise growth hormone.  Bone age obtained in Venezuela was within normal limits (within a 1 year standard deviation).   Also notable in the history is recurrent tingling of the lower extremities.   Given this constellation of features, we attempted to rule out hypothyroidism, pseudohypoparathyroidism, systemic illness and rescreened for growth hormone deficiency.  Our laboratory screening was remarkable only for hypercholesterolemia.

Disposition: We will forward the results of the these studies to the patient's pediatrician in Venezuela via her brother here in Benise.
Thank you for letting us share in the care of Alice.

Sincerely,


Sotiria Bonaparte, MD         Lancelot Blue, MD             
                             Attending in Endocrinology


CONTENT:
Leary, M.D.          RE: Giulio Betterton
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/05/82

Dear Giulio:  

Insulin dose:  4-5R/13N pre-breakfast; 4-6R pre-dinner; 13-14N bedtime 	

Interval  history:   Your blood glucose readings following the clinic visit were right on target.  I hope you are doing well ith dietary choices and insulin injections.

Height:   166.4 cm      Weight:  58.2 kg  
BP:  110/75             Pulse:  86	            Tanner stage:  2

Laboratory test results:
Blood glucose 	468 mg/dl (normal fasting 70-110)	
Glycosylated hemoglobin*  14.1 % (normal 5.2-7.8)
Urinalysis	+++ glucose, - ketones, - protein
  
Comments:  The glycosylated hemoglobin is high as expected.  I expect that good diet control and insulin adjustments as needed will substantially decrease this value.


Sincerely yours,





Rubin Meisner, M.D., Ph.D.	
Fellow in Endocrinology

*Glycosylated hemoglobin reflects average blood glucose levels during the previous 8-12 weeks.  Most people with diabetes have values above normal.  Below is a guide to assess diabetes control in the past 8-12 weeks:  
Glycohemoglobin	Average Blood Glucose
5.2 -7.8%		70 - 110 mg/dl (normal range )
8.0-10.0%		120 - 200 mg/dl
10.0 -12.0%		200 - 260 mg/dl
12% or above		260 mg/dl or higher


CONTENT:
Tucker Hagen, M.D.          RE: Giulio Betterton
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/05/82

Dear Dr. Hagen:

We saw Giulio in the Diabetes Program on 10/24/95 for IDDM.

History: Giulio is a 13 1/12 year old boy who has had IDDM since 7/94.  He receives three insulin injections per day and follows an ADA diet.  

He has had difficulty adjusting to the changes in his diet and the routine imposed by the insulin injections.  In the last few weeks, he has shown more difficulty coping with the demands of diabetes management.  His family has observed physical difficulty with sports activity , erratic BG measurements that do not correlate well with food intake.  He  admits that he periodically eats high sugar content items.  He is fully responsible for insulin administration and denies missing any injections.  Giulio reports concern about his parent's reaction to high glucose values and often records fabricated lower blood sugar values.  His health has, otherwise, been good.  He continues to be an outstanding student and maintains a full schedule of activities.

Current medications: Insulin dose:   4-5R and 13NPH pre-breakfast;  4-6R pre-dinner;  13-14NPH at bedtime

Physical exam:
Ht: 166.4 cm    Wt: 58.2 kg
BP: 110/75              Pulse: 86               Breast: Tanner II       
Well appearing boy in no apparent distress.  General exam notable for increase in testicular size and pubic hair consistent with the onset of puberty.  No thyromegaly.  Injection sites, including the abdomen, do not show hypertrophy.

Laboratory studies:  Plasma glucose 468 mg/dl (normal fasting 70-110); total glycosylated hemoglobin 14.1 % (normal 5.2-7.8); urinalysis: 3+ glucose, -ve ketones and protein.

Impression: GiulioUs difficulty in coping with diabetes management appears to be aggravated by lack of parental supervision.  In addition, faithful recording of blood glucose readings are essential for daily adjustments of insulin therapy.  It is also likely that his insulin requirement has increased since he is in early puberty.  The glycosylated hemoglobin is elevated and consistent with the history.  His growth has remained satisfactory.

Disposition: His parents are aware of the need for more supervision of his diabetes care.  Giulio is willing to share with his parents the responsibility for monitoring blood glucose levels and insulin injections.  By involving his parents, we hope this will lead to appropriate adjustment of insulin doses as the need arises.  We will arrange for a consultation with the clinicUs nutritionist for additional dietary counseling and with our social worker to provide further support and anticipatory guidance.   Giulio will return to clinic in three months.



Sincerely,




Brett Meisner, MD                Bernardo Bird, MD         
Fellow in Endocrinology       Clinical Director of Endocrinology
                              Associate Professor of Pediatrics, HMS


CONTENT:
Tucker Hagen, M.D.          RE: Giulio Betterton
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  09/05/82

Dear  Dr. Hagen:  

Giulio is a 13 10/12 year old boy diagnosed with IDDM  in 7/94.  He receives three insulin injections per day and follows a ADA diet.  He reports very good control of blood glucose despite extraneous exercise in summer hockey camp.  He reports only occasional episodes of hypoglycemia around periods of exercise, otherwise his readings range between 80 and 180 mg/dL. He is purposefully trying to gain muscle mass this summer to enter the varsity hockey team.  His relationship with his family has markedly improved.  He feels comfortable managing diabetes openly with his family now.

Insulin dose:   3R/13N pre-breakfast;  4R pre-dinner;  13N bedtime 	

Height:   172.9 cm  Weight:  70.8 kg 	BP  120/85 Pulse  86	Tanner stage  2

Physical Exam: Well appearing boy in no apparent distress.  Normal general exam.  No thyromegaly.  Injection sites, including the abdomen, do not show hypertrophy.

Laboratory test results:  HbA1c	7.4% (normal 4-6%)
  
Comments:   IDDM is under excellent control.  Giulio is knowledgeable about diabetes and does all his management.  We advised him about the risk of hypoglycemia with increased activity.  We did not recommend a change in insulin dose.

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


Sincerely yours,





Rubin Meisner, M.D., Ph.D.		       Bernardo Bird, MD
Fellow in Endocrinology         Clinical Director of Endocrinology
                                Associate Professor of Pediatrics, HMS


CONTENT:
Mireya Pulanski, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear Dr. Pulanski:

We had the pleasure of seeing Cassie in the Endocrine Program on 12/1/94 for .

History: Cassie is a 7 year old female who was seen in the Endocrine Program on 12/1/94 for evaluation of precocious puberty.   Her mother notes that the patient has had increased height and weight over the past year and is now developing axillary and pubic hair and breast. The patient had been previously seen by Dr. Craighill.  Mother also noted that labs have been done in the past week.   She denies any history of heat or cold intolerance, diarrhea or constipation.  The patient has not had any menses and denies any galactorrhea.  The patient also denies any neurologic symptoms, specifically change in vision.

Current medications: None.

Allergies:  No known drug allergies.

Past medical history:  Negative.

Review of systems:  Normal.

Family history:  The patient lives with mother, father and 3 year old brother.   Mother had the onset of menses at 13-14 years of age.  Mother's height is 5'8", father's height is 5'8".  There was no familial disorders noted.

Physical exam:  
Height: 137.4 cm        Weight: 37.5 kg         BP: 110/78              
Pulse: 80               Breast: Tanner II       
Height: 137.4 cm.   Weight: 37.5 kg.
Blood pressure 100/78.   Pulse:  80.
Breast Tanner II  Pubic Hair: Tanner III sparse.
General: The patient is an alert and oriented female whose growth data shows acceleration in both height and weight.  Skin: Clear with no pigmented areas.  EENT: negative including fundi.
Neck: Supple.
Lymph nodes: Negative.
Chest:  Breasts: The right areolar measures 2 cm with the nipple being 3 mm and the breast bud being about 3.3 cm.  The left areolar measures 1.6 cm with a nipple of 3 mm and a breast bud of 3.5 cm.  The left is more prominent than the right.  Lungs: clear to P & A.
Heart: Regular rate and rhythm without murmur.
Abdomen: Soft and nontender.   Positive bowel sounds.  No organomegaly.
GU: Normal female without much evidence of estrogenization. Tanner III sparse pubic hair with small labia minora.
Extremities: Free range of motion.
Neurologic: Cranial nerve 2-12 intact.   Visual fields are intact with normal reflexes.

Laboratory studies:  8/29/94 11:26 Growth hormone 2.9 ng/ml, TSH 3.2 U/L, FSH 4.1 IU/L, LH 0.20 IU/L, prolactin 7.2 ng/ml, estradiol less than 5 pg/ml.  11/11/94 pelvic ultrasound normal prepubertal uterus and adnexae.  12/6/94  brain MRI normal.  12/12/94 GnRH test see enclosed report.

Impression: Central precocious puberty.   She had a bone age at 6 and 3/4 year which was 8 10/12 years (HA the same) giving her a predicted adult height of 170 cm (66.9", mid-parental height 166 cm).  The mid-parental height is probably a better estimate of final height at this time since she clearly seems to be pubertal.  After a discussion of probably cause and possible treatment, the mother wishes to have treatment if subsequent studies confirm the diagnosis.

Disposition: Since the above studies confirm the clinical impression of idiopathic central precocious puberty, treatment with Lupron (GnRHa capable of reversibly suppressing the neuroendocrine- gonadal axis) will be begun.  Return visit schedule for 4 weeks at which time treatment will be started.
Thank you for letting us share in the care of Cassie.

Sincerely,


Gleide Swartzenegger, MD              Mitsuko Simpson, MD             
                             Chief Emeritus               


Addendum: Resident


Enclosure:  Report of GnRH test


CONTENT:
Mireya Pulanski, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear Dr. Pulanski:

We had the pleasure of seeing Cassie in the Endocrine Program on 12/29/94 for central precocious puberty.

History: The patient is a 7 year old girl who was seen here on December 1, 1994 for precocious puberty and seen again on December 19, 1994 with a history of increasing height and weight over the past year and newly developing axillary and pubic hair and breast development.   In the interim, the patient has not had any illnesses and denies any heat or cold intolerance, neurologic symptos, diarrhea, or constipation.   She has not had any menses.

Current medications: None.

Review of systems:  Positive for occasional snoring and noisy breathing, which does not seem to bother the patient or awaken her from sleep.   She is doing well in school, and interacts reasonably well with her peers.   In general, she does not understand much about her diagnosis.

Physical exam:  
Height: 138.3 cm        Weight: 37.5 kg         BP: 100/60              
Pulse: 80               Breast: Tanner II       Pubic hair: Tanner IV   
General: She is well appearing, mildly obese and tall.   She is alert, oriented and conversive.  
Skin: She has some excoriations in her right antecubital fossa and some well healed chicken pox lesions on her trunk and lower extremities.  She has one isolated, well circumscribed cafe au lait spot in her right axilla. 
HEENT: Pupils were equal, round and reactive to light.  Her extraocular muscles were intact.    Her tonsils were large bilaterally, right greater than left.   There were no significant adenopathy or thyromegaly.   
Neck: 
Chest: Breasts were asymmetrical with more breast tissue apparent on the left compared to the right.  Her left areola measured 2.3 cm in diameter and her nipple was 5.0 cm and on the right her areolar was 2.0 cm in diameter with 3.5 cm of breast tissue.   There was coarse, Tanner stage IV axillary hair bilaterally.   
Lungs were clear to auscultation.    
Heart: Regular rhythm and rate.  S1, S2 were normal.  No S3, S4 or murmurs were audible.   
Abdomen: Soft, mildly obese with good bowel sounds and no evidence of organomegaly.   
Genitourinary: She had sparse pubic hair, Tanner stage IV in distribution.   Some evidence of estrogenization.   Small labia minora and no evidence of clitoromegaly.  
Extremities: Well perfused with free range of motion.  
Neurological: The patient was alert and oriented x3.   Cranial nerves 2-12 were intact and DTR's were 2+ throughout.   Tone and strength were symmetrical bilaterally 5/5.

Impression: The patient is a 7 and 2/12 year old girl with central precocious puberty here for follow up visit.  She shows advanced pubertal changes during today's visit evident in her left breast tissue development, right breast tissue development beginning and further pubic and axillary hair development.   

Studies ordered today are a bone age, left wrist film.

Disposition: Will begin Depo Lupron 11.25 mg q month IMx3 doses.   The patient is to return to clinic in three months.   She will have a GnRH stimulation test before the fourth dose of her Depo Lupron and will return after the result of the GnRH stimulation test are back.
Thank you for letting us share in the care of Cassie.

Sincerely,


Mitsuko Simpson, MD             
Chief Emeritus               


Addendum: Bat-Sheva Levine, M.D.

Problem list to date:
SEXUAL-PRECOCITY                             12/19/94  
Transcribed by Fahey


CONTENT:
Mireya Pulanski, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear Dr. Pulanski:

We had the pleasure of seeing Cassie in the Endocrine Program on 12/29/94 for follow-up of central precocious puberty.

History: General health good since her visit 1 month ago.

Current medications: None.

Review of systems:  Positive for occasional snoring and noisy breathing, which does not seem to bother the patient or awaken her from sleep.   No significant change in pubertal development. No menarche.  She is doing well in school, and interacts reasonably well with her peers.

Physical exam:  
Height: 138.3 cm        Weight: 37.5 kg         BP: 100/60              
Pulse: 80               Breast: Tanner II       Pubic hair: Tanner IV   
General: She is well appearing, mildly obese and tall.   She is alert, oriented and conversive.
Skin: She has some excoriations in her right antecubital fossa and  some well healed chicken pox lesions on her trunk and lower extremities.  She has one isolated, well circumscribed cafe au lait spot in her right axilla.
HEENT: Pupils were equal, round and reactive to light.  Her extraocular muscles were intact.    Her tonsils were large bilaterally, right greater than left.   There were no significant adenopathy or thyromegaly.
Neck: No thyroid enlargement.  No GGE.
Chest: Breasts were asymmetrical with more breast tissue apparent on the left compared to the right.  Her left areola measured 2.3 cm in diameter and her breast was 5.0 cm and on the right her areolar was 2.0 cm in diameter with 3.5 cm of breast tissue.   There was coarse, Tanner stage IV axillary hair bilaterally.
Lungs were clear to auscultation.
Heart: Regular rhythm and rate.  S1, S2 were normal.  No S3, S4 or murmurs were audible.
Abdomen: Soft, mildly obese with good bowel sounds and no evidence of organomegaly.
Genitourinary: She had sparse pubic hair, Tanner stage IV in distribution.   Some evidence of estrogenization.   Small labia minora and no evidence of clitoromegaly.
Extremities: Well perfused with free range of motion.
Neurological: The patient was alert and oriented x3.   Cranial nerves 2-12 were intact and DTR's were 2+ throughout.   Tone and strength were symmetrical bilaterally 5/5.

Laboratory studies:  12/29/94 X-ray hand and wrist: bone age 9.5 yearswhich gives her a predicted adult height of 171 cms(67.3").

Impression: The patient is a 7 and 2/12 year old girl with central precocious puberty here for follow up visit.  She shows advanced pubertal changes during today's visit evident in her left breast tissue development, right breast tissue development beginning and further pubic and axillary hair development.

Disposition: Depo Lupron 11.25 mg q month IM begun.  She will have a GnRH stimulation test before the fourth dose of her Depo Lupron and will return after the results of the GnRH stimulation test are available for adjustment of therapy as indicated.  Subsequent follow-up will be at 3-6 month intervals.
Thank you for letting us share in the care of Cassie.

Sincerely,


Janel Meisner, MD         Mitsuko Simpson, MD             
                             Chief Emeritus               


Addendum: Intern


CONTENT:
Ariel Adler, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear


CONTENT:
Mireya Pulanski, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear Dr. Pulanski:

I saw Cassie in the Endocrine Program on 3/11/96 for follow-up of central precocious puberty.

History: She has been in good health since her last visit several months ago.  She has had significant problems with injections of Lupron, which have decreased significantly recently.  Currently, she experiences only some redness and minimal pain at the injection site with no drainage, for one to two days after injection.  However, she continues to be very upset about getting the injections.  I discussed with Mom the alternatives of daily nasal or subq. injections of GnRH antagonists, and we decided it was best to probably continue with our current regimen since things were improving.  Cassie has had no vaginal bleeding and no vaginal discharge.

Current medications: Lupron 11.25 mg. IM q.3 weeks.

Physical exam:
Height: 146 cm        Weight: 41.2 cm     Bone age: 11 years
BP: 110/60            Pulse: 90               
Breast: Tanner III      Pubic hair: Tanner IV   
General appearance:  She is alert and cooperative.  Her skin has normal turgor and texture.  HEENT:  Pupils are equally reactive to light and accommodation.  Extraocular movements are intact.  Fundi are normal.  Neck:  Thyroid is normal size and consistency.  Lungs are clear.  Breasts are Tanner III bilaterally.  Heart has regular rate and  rhythm.  Abdomen:  Soft, nontender, no hepatosplenomegaly.  GU:  She has Tanner IV pubic hair and pink vaginal mucosa.  Extremities:  Sites of previous injections scars are noted.  Most recent two injection sites are not evident.  

Impression: The problem with Lupron injections has improved significantly.  There is no change in Cassie's pubertal status.  In terms of growth, Cassie continues to grow, however, her acceleration pattern has decreased.

Disposition: Continue the Lupron as previously and I will see her again in 4-6 months.   

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


Sincerely,




Ariel Adler, MD           
Attending in Endocrinology


CONTENT:
Mireya Pulanski, M.D.          RE: Cassie Nizer
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  10/30/87

Dear Dr. Pulanski:

I saw Cassie in the Endocrine Program on 12/16/96 for precocious puberty.

History: As you know, Cassie is a now 9 and 2/12 year old who had been treated with Depot Lupron for precocious puberty.  Most disturbing is that the injection sites for the Depot Lupron have left Cassie scarred.  Also, they have been very painful and have resulted in suppuration.  We have discussed this at length with Cassie and her mom and discussed the options.  These include daily injections of a daily preparation which Mrs. Nizer does not want to do.  Intranasal  Lupron is also not considered.  However, Mrs. Nizer remains very concerned about Cassie beginning her menstrual periods before she is emotionally ready.  Thus, we decided that if Cassie does begin her menses at a point in time when her emotional preparedness is not appropriate, we will begin progesterone therapy to stop menses until she is ready.  Cassie's height is also an issue, but currently, she is 4'11" and mom feels that this is an acceptable height.  Hence, the decision was made to stop Lupron today.

Current medications: Lupron 15 mg IM q. month.

Physical exam:
Height: 149.6 cm        Weight: 42.2 kg
BP: 117/52              Pulse: 86               
Breast: Tanner III      Pubic hair: Tanner IV   
Physical examination is noted for the scars from previous Lupron injections.  General appearance:  she is alert and cooperative.  Skin:  normal turgor and texture.  Neck:  the thyroid has normal size and consistence:  Abdomen:  soft, non-tender no hepatosplenomegaly.

Impression: Cassie has precocious puberty, but continues to have severe reactions to Lupron injections.  Since she is now 4'11" and mom and Cassie are comfortable with her stature and very uncomfortable with continuing the injections, we will discontinue them.

Disposition: If the menses occur, we will begin Cassie on progesterone therapy to stop the periods until Cassie is ready to take care of her herself.  I have asked Cassie to return to clinic in six months.  

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


Sincerely,



Ariel Adler, MD      
Attending in Endocrinology


CONTENT:
Duncan Hugo, M.D.          RE: Julie Bradley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/80

Dear Dr. Hugo:

We had the pleasure of seeing Julie in the Endocrine Program on 8/30/94 for .

History: I saw Julie, age 14 years 7 months, in follow-up for insulin-dependent diabetes mellitus diagnosed in January 1990.  I had previously seen her at the Joslin Clinic until approximately 18 months ago.  I understand that there has been no follow-up for her diabetes because of an interruption in insurance coverage.

She is currently on Lilly pure pork insulin, 11 units Regular and 18 units NPH before breakfast, 10-12 units Regular  and 16-18 units NPH before supper (total daily dose 55-59 units; 0.92-0.97 u/kg/day). 

Julie refuses to do her own insulin injections and, accordingly, her parents are involved in mixing and injecting insulin.  

She does not follow a diabetic meal plan but does have three meals, and  usually has a snack in the afternoon and a snack in the evening "most of the time."  There have been no problems with severe hypoglycemia and she seldom has mild symptomatic hypoglycemia.  She reports no symptoms of uncontrolled diabetes and appears to sleep well without disturbance.

She does monitor blood glucose levels before supper from time-to-time (2-4 times per week).  Blood glucose levels range from 51-325 mg/dL.  They are similar before breakfast, lunch and at bedtime.  She is not physically active.  

Julie stated emphatically that she hates diabetes and does not want anything to do with diabetes.

Physical exam:  
Height: 157.1 cm        Weight: 60.9 kg         BP: 114/82              
Pulse: 72               
She appears healthy, well-developed, well nourished.  She is an attractive young lady with a flat affect.  Tanner breast stage V.  No evidence of diabetic retinopathy.  Thyroid edges palpable but the gland was not obviously enlarged.  Chest was clear.  Heart was normal.  No organ enlargement or masses.  Neurological exam revealed 1+ symmetric reflexes in the lower extremities.  Vibration sense was intact.  Skin was smooth and warm and there was no acne.  There was minimal lipohypertrophy at the injection sites in the upper extremities.

Laboratory studies:  Urine had 1% glucose, no ketones or protein; plasma glucose was 328 mg/dl.  Hemoglobin A1 was 8.7% (normal <7.4%); non-fasting total cholesterol 178, HDL 65, triglycerides 79, calculated LDL 89 mg/dL, all normal; serum TSH 2.9 mcU/mL; spot urine microalbumin 4 5g/mg creatinine.

Impression: She appears healthy and by history does not have symptoms of either hyperglycemia or hypoglycemia.  The hemoglobin A1 indicates well controlled diabetes.  
My main concern is that she is obviously depressed and has not yet accepted diabetes and has made no effort to become independent with respect to diabetes self-care.  I encouraged her to meet with Ms. Ellen Goodman, social worker in the Diabetes Program, to begin an evaluation and develop a treatment plan to help resolve these important problems.   Ms. Goodman has recommended more frequent follow-up and continued psychosocial assessment.  At her next visit, I shall introduce her to our Diabetes Nurse Specialist. Ms. Kathleen Walsh, in the hope that we can begin to enhance Julie's  skills in diabetes management and help her to become more independent.

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

Sincerely,


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


CONTENT:
Duncan Hugo, M.D.          RE: Julie Bradley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/80

Dear Dr. Hugo:

We had the pleasure of seeing Julie in the Endocrine Program on 1/3/95 for insulin-dependent diabetes mellitus.

History: Julie is a 15 year old girl who was seen in the Diabetes program on 1/5/95 for follow up of insulin dependent diabetes mellitus diagnosed in January 1990.   

She denies any major intercurrent illnesses since we last saw her four months ago. 

She is on Lily pure pork insulin, 14 units Regular and 20 units of NPH before breakfast, and 12 units Regular and 18 units NPH before supper.  The total daily dose of insulin is 64 units or 1.05 U/kg/day.   

Julie is not interested in self-administering insulin because she dislikes  needles.  Injections are given by her parents, alternating sites between the upper arms and buttocks.  She reports occasional mild hypoglycemic  episodes secondary to inadequate food intake.  She denies severe hypoglycemic reactions and symptoms of hyperglycemia.  She is on a three meal and three snack diet, but is not consistent with the bedtime snack.

Blood glucose monitoring using the One Touch meter, is done before supper every other day.  The log book was not available at this visit, but by report, blood sugar ranges between 230-380 mg/dL, with the lowest being 50 and the highest 400 mg/dL.

She is not physically active.   She also denies sexual activity, alcohol and tobacco use.  She needs a new Medic Alert tag.  Her last ophthalmology visit was in August 1994.

Physical exam:  
Height: 158 cm          Weight: 60.5 kg         BP: 120/75              
Pulse: 78               Breast: Tanner V        Pubic hair: Tanner V    
General: pleasant girl with a sad and solemn affect.   
Skin: Smooth and warm with no acne and no lipohypertrophy at the injection sites. 
HEENT: Extraocular movements intact.   Pupils equal and reactive to light.  Lenses were clear.   There was no evidence of diabetic retinopathy.   
Neck: The thyroid edges were palpable.  
Chest: Lungs clear. 
Heart: Normal S1, S2, no murmur.  
Abdomen: Soft with no organomegaly.   
Genitourinary: Normal female genitalia.  
Neurological: No tremors.  Knee and ankle reflexes were symmetrical and normal.

Impression: Julie has had IDDM for five years and is still dependent on her parents for insulin injections. Her lack of independence, in addition to a sad and depressed affect, is concerning.   We recommended further meetings with Ellen Goodman, the social worker in the diabetes program. We also encouraged Julie to assume responsibility for insulin injections at least once a week.  The use of an "Injectease" may alleviate her needle apprehension.

The hemoglobin A1 indicates satisfactory diabetes control.  We encourage patients to reduce their hemoglobin A1 to 29.5%.  We are unable to assess her daily blood glucose profile since monitoring is infrequent and the log book is unavailable.  The occasional mild hypoglycemic episodes occur as a result of inadequate food intake.  We cannot recommend alterations in her current insulin regimen due to the unavailability of objective information.


Laboratory studies pending at this time include: Plasma glucose in the mid-afternoon 77 mg/dL; hemoglobin A1 9.7% (normal 5.2-7.8).
Disposition: We would like to see Julie back in three months for a follow up visit.
Thank you for letting us share in the care of Julie.

Sincerely,


Zulma Montague, MD         Bernardo Bird, MD         
Fellow in Endocrinology      Clinical Director
Associate Professor  of Pediatrics, HMS


CONTENT:
Duncan Hugo, M.D.          RE: Julie Bradley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/80

Dear Dr. Hugo:

We had the pleasure of seeing Julie in the Endocrine Program on 4/11/95 for insulin dependent diabetes mellitus.

History: Julie is currently a 15 and 3/12 year old girl with type I diabetes mellitus of 5 years duration.  

She is on Pork insulin, 66 units per day (1.1 U/kg/day).  The insulin dose is divided as follows: 
pre-breakfast Regular insulin 14-15 units, NPH 20-22 units.  
pre-dinner Regular insulin 12-14 units, NPH 18 units.  

Injections are still administered by both parents rotating sides between the upper extremities and buttocks.  Julie is still reluctant to do any injections.   

She denies any symptoms of hyperglycemia.  Occasional symptoms compatible with hypoglycemia occur secondary to inadequate food intake. 

She is on a three meal plan, with occasional snack intake in the afternoon and at night time.  

She goes to the gym twice a week.  She works at Boston Chicken three to four times a week.  

Blood glucose monitoring is done very infrequently, once a week at dinner time.   Blood glucose values range from 148-403.

Physical exam:  
Height: 156.6 cm        Weight: 60 kg           BP: 117/80              
Pulse: 80               Breast: Tanner V        Pubic hair: Tanner IV   
General: Sullen affect, not very communicative.  
Skin: No atrophy/hypertrophy at the injection sites.   
HEENT: Eyes EOMI, PERRLA.  Lens clear.  Thyroid palpable edges, smooth and soft. 
Neck: No thyromegaly.    
Chest: Lungs clear.   
Heart: No murmur.  
Abdomen: No organomegaly, not obese. 
Neurological: No tremor.  DTR's physiologic.

Laboratory studies:  GLY HGB  10.3% (goal <10%)

Impression: Insulin dependent diabetes mellitus in poor control.  reflected by a progressive increase in the HBA1 level.  Blood glucose monitoring is very infrequent, thus, rational adjustment in the insulin dose cannot be made.

Julie has not accepted her diagnosis yet and does not show any interest in independent management.

Disposition: We recommend:

1.  Maintain the same insulin regimen.

2.  We emphasized the importance of careful blood glucose monitoring and compliance with the meal plan in order to achieve better control of her diabetes.   

3.  Again, we encoruaged Julie to do an insulin injection at least once a week.

We would like to see Julie back in three months.
Thank you for letting us share in the care of Julie.

Sincerely,


Zulma Montague, MD         Maritza Brezny, MD             
Fellow in Endocrinology      Attending in Endocrinology


CONTENT:
Magdalena Goldwater, M.D.          RE: Julie Bradley
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  01/17/80

Dear Dr. Goldwater:

We saw Julie in the Diabetes Program on 9/10/96 for follow-up of insulin-dependent diabetes mellitus diagnosed in January, 1990.

History: Julie is 16 and 8/12 years old.  She was lost to follow-up as she never scheduled a return appointment to the diabetes clinic since April, 1995.  Julie stated that she did not comply with follow-ups because she "dislikes hospitals, clinics, and physicians in general."  The reason she scheduled an appointment today is that pork insulin is being discontinued from the market, and she needs prescriptions for another form of insulin.  

She remains on Lilly pork insulin:  14 units of Regular and 21 units of NPH before breakfast, 13 units of Regular and 19 units of NPH before dinner.  The total daily dose is 67 units, or 1 u/kg/day.  Julie denies insulin omission as she is conscious of omission-associated complications such as ketoacidosis, ICU admission and possible coma.  However, she remains noncompliant with blood glucose monitoring, and checks blood glucose levels only every couple of days, usually before dinner.  She reports a range from 90-100 mg/dl.  She also has finally assumed responsibility for insulin self-administration.  Up until April 1995, she adamantly refused either mixing or administering insulin and remained solely dependent on her parents for diabetes management.  

She denies symptoms of uncontrolled diabetes such as polyuria, nocturia, enuresis or polydipsia.  She has a good appetite and denies vomiting or weight loss.  She is concerned about being overweight for her height, and asked advice for weight control.  She is following a meal exchange plan that was last updated in April, 1995.  In addition, she denies vaginal itching, or discharge.  The last yeast infection was in January 1996 and responded to topical antifungal treatment.  She denies symptomatic hypoglycemic episodes.  

Review of Systems:  Julie had menarche at 11-12 years.  She then reports a 7-month period of amenorrhea in 1995, followed by spontaneous resumption of menses in January or February, 1996.  Since then, she has had a regular 4-week cycle, with 5-day periods of regular menstrual flow.  She was evaluated by Dr. Goldwater in January for the secondary amenorrhea, and had a negative pregnancy test.  Further work-up was aborted as menses resumed spontaneously.  

Psychosocial:  Julie met with Ellen Goodman, the diabetes team social worker.  She reportedly went through a difficult time at school and had conflicts with her parents.  She was sad and stressed.  However, she is feeling much better now and is looking forward to starting school again.

Physical exam:
HT: 158.4 cm    WT: 65.9 kg      BP: 131/62        Pulse: 87               
Thyroid: enlarged one time normal, smooth, symmetrical, no nodules felt.  Lungs:  Clear.  Heart:  No murmur.  Abdomen:  No hepatosplenomegaly.  Skin:  Acne scars on the chin.  Injection sites:  resolution of the upper extremity hypertrophy.  No atrophy/hypertrophy over the abdomen.  Fingertips:  Few prick marks seen.

Laboratory studies:  HGB A1C - 7.1 %, glucose - 282 mg/dL.
T4 - 6.0 ug/dL, TSH - 2.00 uU/mL, TT3 - 89 ng/dL, TBGI - 1.05

Impression: Insulin-dependent diabetes mellitus with adequate control as indicated by a glycohemoglobin level in the target range.  Growth records indicate a steady weight gain along the 80th percentile and steady linear growth along the 25th percentile.  Julie has most likely reached her final adult height as she only gained 0.4 cm in 1.5 years.  The thyroid function tests are normal. 

The etiology of the transient secondary amenorrhea remains unclear.  It may be secondary to stress-related psychogenic amenorrhea.  No work-up is necessary at present given the spontaneous resumption of a regular menstrual cycle.

Disposition: 1.  Julie will be changed to Humulin (recombinant human insulin) instead of pork insulin.  This change may cause a more significant effect of the Regular insulin, and a shorter duration of action of the NPH.  Therefore, the insulin dose was modified to 11 units of Regular and 21 units of NPH before breakfast, and 10 units of Regular and 18 units of NPH before dinner.  Julie was instructed about the possibility of hypoglycemic episodes on Humulin, and about the measures taken to prevent them.  

2.  Regular exercise for weight control was recommended, and a follow-up appointment with the dietitian was scheduled 

We would like to see Julie in 3 months.



Sincerely,




Zulma Montague, MD           Nilto Carter, MD             
Fellow in Endocrinology        Associate in Endocrinology


CONTENT:
Vivianne Brando, M.D.          RE: Remy Melbourne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/25/85

Dear Dr. Brando:

We had the pleasure of seeing Remy in the Endocrine Program on 10/27/94 for short stature.

History: Remy is a 9 and 3/12 year old male referred to our program for evaluation of his growth.  To briefly review, Remy was born prematurely after 27 weeks of gestation, delivered early for maternal pre-eclampsia.  He weighted 1 pound 9 ounces at birth.   He had multiple medical problems in the NICU including ventilator dependency for approximately 6 weeks, then oxygen dependency for an additional several months, two episodes of sepsis and hyperbilirubinemia of several weeks duration.   He did not have intraventricular hemorrhage.    He initially had difficulty with growth and was fed by TPN through a central line and then after his second episode of sepsis the central line was removed and he was started on Enteral feeds.  Since the neonatal period  he has had multiple pneumonias, mostly in the first couple of years of life, requiring hospitalizations.  He also had a right middle lobe syndrome in which his right middle lobe was collapsed.   He has reactive airway disease.   Earlier this year he was seen by a neurologist for evaluation of attention deficit.  He was not started on medications at that time.  He was found to have difficulty with fine motor abilities, in particular handwriting.   CT and EEG in June of this year were within normal limits.   

As terms of, his growth, Remy has always been much less than the 5th percentile for both height and weight.  Recently his velocity of weight, gain has increased, and is now at about the 5th percentile.   He was initially followed by a pediatrician in Virginia.   After moving to Massachusetts are over a year ago, Remy was seen by his new pediatrician who was concerned that his height was well below the 5th percentile.  Bone age films were obtained at an outlying hospital.   

Social History:  He is in the second grade and was held back two years because of his medical problems, in particular, his respiratory issues and his inability to attend school for a full day.

Current medications: Pediazole for an otitis, (now day 4 out of 10.)  He is Atrohist for sinusitis, diagnosed in August.   He is also on Ventolin nebulizer which he takes every morning.

Past medical history:  Allergies to dairy products, resulting in vomiting and wheezing.

Review of systems:  Skin; pruritus of his scalp and face.  HEENT; farsighted; enamel hypoplasia.  Respiratory; reactive airways disease and hayfever.  He has approximately 60 allergies to environmental stimulants.  GU; testicles initial undescended testicles but have since descended.

Family history:  The patient has a 13 year old sister who was also born prematurely after 27 weeks of gestation.    She is otherwise healthy and was measured at 163.1 cm.   She has growth delay, but is now at the 50th percentile for height and weight.    The patient's mother is 163.1 cm.  She has a brother who is 6', a 5'9" brother, and a 5'2" sister.  The patient's father was 6 feet tall at his highest however, had two ruptured discs is now approximately 2 inches shorter.  His measured height is 175.8 cm.   He has a 6' brother and a 5'9" sister.  The mother's menarche occurred at 13 years of age and her growth spurt happened between 12 and 13 years of age.   The father's growth spurt took place between 12-13 years of age.   Adult onset diabetes runs in both sides of the family, in the maternal great grandfather and the paternal grandfather.   There are no thyroid or other endocrine problems that run in the family.

Physical exam:  
Height: 115.9 cm        Weight: 23.6 kg         BP: 103/72              
Pulse: 85               R. testis: 1.7 x 1.5 cm Phallus length: 4.5 cm  
Mid-shaft diam.: 1.7 cm 
General: He is an extremely active, well nourished, prepubertal boy.  
Skin: Hypopigmented macular patchy areas on his left lower extremity and cheeks.  
HEENT: Multiple dental caries and dull tympanic membranes.  
Neck: Supple.  He had a nonpalpable thyroid.   
Chest: Clear to auscultation with no wheezing.   
Heart: Regular rate and rhythm.  
Abdomen: Soft and nontender and nondistended.   No hepatosplenomegaly.   
Genitourinary: Normal male with testes descended bilaterally.  However, the left testes was high in the canal.
Neurological: Nonfocal.

Laboratory studies:  alk phos 236=U/L, CO 2=24 mEq/L, BUN 11 mg/dl, creatinine=0.6 mg/dl,      glucose=96 mg/dl, AST=27 U/L, ALT=5 U/L, NA=139 mmol/L, K=4.5 mmol/L,        chloride=100 mmol/L, total bilirubin=0.3 mg/dl, platelets=276K/MM3,    WBC=10.16 K/MM3, RBC=4.71M/MM3 hemoglobin=3.6 G/DL, hematocrit=40.7%        MCV=86.4 FL, PLT UNC=276 K/MM3, neutrophils=49%, lymphs=34%,  monos=5%        
eos= 2%,  baso=1%,   ATYP LY=7 % (H),   BAND=2 % , TBGI=0.94, T4=8.2  ug/dL,     
TT3=157  ng/dL , TSH =1.60   uU/mL, IGF-1=96 ng/ml (range for 6-9 yr male is 88-974 ng/ml, 9-12 yr male is 110-565).
Bone age: 7 years


Impression: Remy has no evidence of an underlying systemic disease on laboratory screening or on examination.   Thyroid function is normal.   Most likely, his delayed growth is secondary to medical complications in his first two years of life as well as a genetic predisposition.  He has a low IGF-1 for his age, but it is in the low-normal range for his bone age of 7 years.

Disposition: We would like to see Remy back in our clinic in three months to go over test results and see how he is growing.  We will repeat the IGF-1, and if still low, recommend a GH stimulation test to rule out GH deficiency.
Thank you for letting us share in the care of Remy.

Sincerely,


Pericles Beaker, MD                Penelope Hagen, MD              
                             Attending in Endocrinology   


Addendum: Senior Resident


CONTENT:
Vivianne Brando, M.D.          RE: Remy Melbourne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/25/85

Dear Dr. Brando:

We had the pleasure of seeing Remy in the Endocrine Program on 2/2/95 for short stature.

History: Remy in now 9 years and 6 months old.  He has been quite healthy since I last saw him. He seems to be growing according to the family and has increased in height and weight.  As a note,  I would like to mention a correction in his initial letter pointed out to me by Mrs. Keight.  KJames was in the NICU for approximately four months after birth, and  oxygen dependent for 6 month period.

Current medications: Ventolin for occasional wheezing.

Physical exam:  
Height: 116.9 cm        Weight: 25.3 kg         BP: 110/80              
Pulse: 90               Pubic hair: Tanner I    
Ht (below the delayed case)     tw:  (at the delayed case)

General:   Well developed and well nourished in no apparent distress.
Skin:  Several erythmeatous papules on the face (felt be due to allergies)
HEENT: Normocephalic atraumatic, pupils equally round and reactive to light.  Teeth are somewhat irregularly spaced.
Neck:   No thyromegaly.
Chest: Clear
Heart:   Regular rate and rhythm without murmur.
Abdomen:  Benign
Genitourinary:  Prepubertal male
Extremities: 
Neurological:  Grossly intact.

Impression: Remy'shad  a low IGF 1 for his age on his lst visit,  though it was within the range of normal for his bone age.  Given the fact that he is growing well and his growth rate is normal, he does not appear to have growth hormone deficiency.  We repeated his IGF 1 today, and if it is low, will do an insulin tolerance test to rule out growth hormone deficiency.

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

Sincerely,


Penelope Hagen, MD              
Attending in Endocrinology


CONTENT:
Vivianne Brando, M.D.          RE: Remy Melbourne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/25/85

Dear Dr. Brando:

We had the pleasure of seeing Remy in the Endocrine Program on 2/2/95 for short stature.

Remy had an IGF-I level of 129 ng/ml on his visit to the Endocrinology clinic on 2/2/95.  This level is within the normal range for his age (110-565 ng/ml), and within the normal range for his bone age of 7 years (88-474 ng/ml).  We would like to continue to follow Melbourne carefully to see how he grows.

Laboratory studies:  IGF-I=129 ng/mL


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

Sincerely,


Penelope Hagen, MD              
Attending in Endocrinology


CONTENT:
Vivianne Brando, M.D.          RE: Remy Melbourne
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  07/25/85

Dear Dr. Brando:

I saw Remy in the Endocrine Program on 10/10/96 for short stature.

History: Remy is an 11 year 2 month old male who is being followed for short stature.  His past medical history is significant for him being on a ventilator for 20 months (not 6 as noted in a previous letter), and he has chronic obstructive pulmonary disease as well as asthma.  However, his asthma and pulmonary disease seem to have been somewhat improved recently.  He is growing, although at a slow rate.  Remy is in the 4th grade and is quite physically active.  He has no evidence for puberty.

Current medications: Serovant and Vanceril.

Physical exam:
Height: 126.4 cm        Weight: 34.5 kg
BP: 119/65              Pulse: 106              
Breast: Tanner I        Pubic hair: Tanner I    
Height is just below the delayed case curve, weight is just above the delayed case curve.  Physical examination is within normal limits, and is notable for some scabs from falls on his extremities.  He has no thyromegaly.  His testes and penile exam reveal that he his prepubertal.

Additional studies:  10/21/96 (at HCHP):  IGFBP3=2.6 mg/l (normal is 2.0-4.8); IGF-1=157 ng/ml (normal for a 9-10 year old is 136-308)

Impression: We have not suspected growth hormone deficiency given that Remy has had a normal IgF-I.  His IGF-I and IGF BP-3, done just after this visit, are in the normal range for his age.  Remy' growth velocity is within the normal range, at 5.7 cm/year over the past 20 months.  I also requested a bone age, and don't yet have the results.  I expect that it is delayed.

Disposition: We would like to see Remy back in our clinic in six months to follow his growth.



Sincerely,




Penelope Hagen, MD              
Attending in Endocrinology   

cc:  Mr. & Mrs. Remy Melbourne   28 Vine St   Andover , MA  01810




Problem list to date:
SHORT-STATURE                                11/30/94  
Transcribed by Sullivan_ma


CONTENT:
Nathan Leary, M.D.          RE: Hobart Miles
123 Santa Clause Lane                                   CH# XX XX XX
Death Valley, CA  90210                                 DOB:  02/16/79

Dear Dr. Leary:

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

History: He notes that his problem began about two years ago and has been seen by an endocrinologist previously and notes a weight loss of 10 pounds over the past months that has been achieved by diet alone.    He has decreased total calories.   The patient does not have any aerobic exercise except for occasionally in gym at school.  He has no discharge from the nipples.   He is in the 10th grade.

Current medications: None.

Past medical history:  Negative except for pressure equalizing tubes at 1 years of age.

Review of systems:  Negative.

Family history:  Negative for gynecomastia.   He has a 4 and a 25 year old sister, both of whom are doing well.  There is a maternal history of migraine headaches.   Mom is on Thyroxine for "low thyroid" and is also on Prozac.  There is a maternal aunt with a thyroid history.  Father has reactive airways disease.  Mother's height is 5'2".  Father's height 5'11".

Physical exam:  
Height: 180.5 cm        Weight: 108 kg          BP: 110/65              
Pulse: 80               Breast: Tanner IV       Pubic hair: Tanner III  
R. testis: 4 x 3 cm     L. testis: 4 x 3 cm     
General: The patient is an obese male with exam notable for bilateral breast tissue at 5x5 cm bilaterally.   The rest of his exam is normal including normal male genitalia with bilateral descended testes of normal size.   The patient's neurologic exam is normal including normal visual fields.

Laboratory studies:  SGOT of 30, SGPT of 22, bilirubin of 0.2, prolactin 7.7, beta HCG l <10, estradiol of 49, free testosterone 5.9, FSH 4.8 and LH 2.7.

Impression: Patient with bilateral gynecomastia with breast tissue plus fatty deposition secondary to obesity with a normally functioning endocrinologic axis per recent labs.

Disposition: At this point, no studies have been ordered and we would suggest weight loss to approximately 20 pounds and then reduction surgery.
Thank you for letting us share in the care of Hobart.

Sincerely,


Gleide Swartzenegger, MD              Nilto Carter, MD             
                             Associate in Endocrinology   


Addendum: Resident in Pediatrics


