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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 1018-4406
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

 en 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

© Copyright 2011 Iran Journal of Pediatrics
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