Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
Vol. 21, No. 4, 2011, pp. 539-542
Bioline Code: pe11093
Full paper language: English
Document type: Case Report
Document available free of charge
Iranian Journal of Pediatrics, Vol. 21, No. 4, 2011, pp. 539-542
© Copyright 2011 Iran Journal of Pediatrics
A Case of Lipoid Congenital Adrenal Hyperplasia Presenting with Cholestasis|
Khodadad, Ahmad; Modaresi, Vajiheh; Kiani, Mohammad-ALi; Rabani, Ali & Pakseresht, Bahar
Background:Lipoid congenital adrenal hyperplasia, is the rarest and usually the most severe form
of adrenal steroidogenic defect,which may presents as infantile cholestasis.
Case Presentation: Here we present a 45 days old infant who came to our attention with cholestasis and severe intractable vomiting and electrolyte disturbances. Evaluation resulted in diagnosis of congenital adrenal hyperplasia. Hydrocortisone and flodrocortisone improved the symptoms including jaundice and vomiting. Hyponatremia and hyperkalemia also resolved with above mentioned treatment.
Conclusion: Congenital adrenal hyperplasia as one of the causes of neonatal cholestasis should be kept in mind, whenever there are also electrolytes abnormalities.
Cholestasis; Lipoid Congenital Adrenal Hyperplasia; Neonate; Adrenal Hyperplasia
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