A case of lipoid congenital adrenal hyperplasia presenting with cholestasis

A case of lipoid congenital adrenal hyperplasia presenting with cholestasis

BACKGROUNDLipoid congenital adrenal hyperplasia, is the rarest and usually the most severe form of adrenal steroidogenic defect,which may presents as infantile cholestasis. CASE PRESENTATIONHere we present a 45 days old infant who came to our attention with cholestasis and severe intractable vomitin...

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Veröffentlicht in:Iranian journal of pediatrics 2011, Vol.21 (4), p.539-542
Hauptverfasser: Khodadad, Ahmad, Modaresi, Vajiheh, Kiani, Mohammad-Ali, Rabani, Ali, Pakseresht, Bahar
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container_end_page 542
container_issue 4
container_start_page 539
container_title Iranian journal of pediatrics
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creator Khodadad, Ahmad
Modaresi, Vajiheh
Kiani, Mohammad-Ali
Rabani, Ali
Pakseresht, Bahar
description BACKGROUNDLipoid congenital adrenal hyperplasia, is the rarest and usually the most severe form of adrenal steroidogenic defect,which may presents as infantile cholestasis. CASE PRESENTATIONHere 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. CONCLUSIONCongenital adrenal hyperplasia as one of the causes of neonatal cholestasis should be kept in mind, whenever there are also electrolytes abnormalities.
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title A case of lipoid congenital adrenal hyperplasia presenting with cholestasis
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