Pediatric case
laparotomy - 01-22-08 14:31 Bookmark and Share

A 27 month old girl presents with malodorous (adult type) axillary smell and some strands of pubic hair. She is in the 97th percentile for weight and the 90th percentile for height. CBC, Urea and electrolytes, FSH, LH, 17 OH progresterone and testesterone are normal. However DHEA-S is elevated 2.30 micromole/l (normal range for her age is below 0.3 micromole/l) and androstenedione is 1.34 micromole/l (normal range for her age is below 0.4 micromole/l. Her bone age is normal for her age.

1. What is your diagnosis?
2. What is the cause of this anomaly?
3. What is your next step in management?
4. What is the definitive management of this condition?

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#1
Re: Pediatric case
laparotomy - 01-22-08 15:21

Idea anyone?

#2
Re: Pediatric case
laparotomy - 01-23-08 13:45

Doesnt anyone out there want to do peds? (by the way peds is a part of the USMLE).

Come on guys give it a shot...

#3
Re: Pediatric case
8401glacieres - 01-24-08 05:59

The diagnosisi is PREMATURE ADRENARCHY.
As given in the stem this female child has all features of hyperadrenalism without other associated features of CAH. Therefore it is isolated premature adrenarchy, which can be having a remote chance of progressing to CAH(congenital adrenal hyperplasis).

Cause of this anamoly is deficiency of beta hydroxy steroid.

Next best thing to do is-----------BASAL & STIMULATED estimation of 17-OH Progesteron, DHEA and Andostenedion estimation.

#4
Re: Pediatric case
laparotomy - 01-24-08 06:55

Basal 17 OH progresterone, DHEA and Andronenedione is already done the latter two being elevated

#5
Re: Pediatric case
laparotomy - 01-26-08 13:20

How about non classical 21 OH deficiency or other forms of congenital adrenal hyperplasia? Now this topic is hot in the USMLE

#6
Re: Pediatric case
taghrid - 01-27-08 01:01

It might be delayed type CAH , But we should investigate for tumers, Like adrenal tumers or ovarian teratomas.

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