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Published on: November 16, 2011
Hypoglycemia due to 3β-Hydroxysteroid Dehydrogenase type II Deficiency in a Newborn
M C Konar1, S Goswami, B G Babu
1Departments of Pediatrics and *Endocrinology, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India. Correspondence to: Dr Mithun Chandra Konar, DE- 290 -1, Giridhari V Apartment, Flat No 3B, 3rd floor, Narayantala (East), Baguiati, Kolkata 700 159, West Bengal, India. dr_mithun60589@yahoo.com.
Background:
3β-hydroxysteroid dehydrogenase type II deficiency results in decreased production of all three groups of adrenal steroids. Recurrent hypoglycemia as a presenting feature of this disorder has not been reported earlier.
Case Characteristics:
A genotypically and phenotypically normal female newborn delivered by in-vitro fertilization presenting with recurrent hypoglycemia. Primary adrenal insufficiency with insignificant mineralocorticoid deficiency and slightly elevated levels of 17-hydro-xyprogesterone, dehydroepian-drosterone sulphate and testosterone.
Outcome:
Successfully managed only with corticosteroid replacement.
Message:
Congenital adrenal hyperplasia can rarely cause recurrent hypoglycemia in newborns.
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