case discussion 1

[3abeer]

You are in the pediatrics ER, & you received 10 days old female, with history of copious vomiting,started 1 day back. otherwise history is negative , she was born as full term normal vaginal delivery
physical exam:
afebile
severly dehydrated,
not indistress
no skin rash
while you are examining her, you could find cliteromegaly

try 2 answer the following questions :
1- what is your diagnosis?

2- what should be your initial management?

[dr_b.e.s.m]

A case of female? pseudohermaphroditism. There are a lot of causes , but i think the most common is CAH [ congenital adrenal hyperplasia] . This patient has dehydration , which goes with salt losing CAH.

Treatment is Hydrocortisone and/or miniralocorticoid adminstration. In this case patient already has severe dehydration , so may be the first step is correction of the hyponatremic dehydration, I don't remember how to correct it by the way , so I hope you can write to us more about it.?

These are some guidelines I found , they represent conditions when we should suspect CAH :

1-Pseudohermaphroditism in females, with urogenital sinus, enlargement of the clitoris, or other evidence of virilization.
2-Salt-losing crisis in infant males or isosexual precocity in older males with infantile testes.
3-Increased linear growth in young children; advancement of skeletal maturation.
4-Urinary and plasma androgen elevation; plasma 17-hydroxyprogesterone and urinary pregnanetriol concentrations increased in the common form; may be associated with electrolyte and water disturbances (hyponatremia, hyperkalemia, and metabolic acidosis), particularly in the newborn period.

[3abeer]

asalamo 3alaikom wa ra7amato ALLAH wabarakato:
correct dr besm 
it is CAH
i will be back with the detailed management inshalla
ramadan kareem