A Case of Lipoid Congenital Adrenal Hyperplasia Presenting with Cholestasis期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

A Case of Lipoid Congenital Adrenal Hyperplasia Presenting with Cholestasis

Authors:	Ahmad Khodadad Vajiheh Modaresi Mohammad-Ali Kiani Ali Rabani Bahar Pakseresht

Affiliation:	1.Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran;2.Center of Excellence for pediatrics, Children''s Medical Center, Tehran, Iran;3.Ali-ebne Abitaleb Medical School, Islamic Azad University, Branch of Yazd, Iran;4.Growth and Development research Center, Tehran University of Medical Sciences, Tehran, Iran;5.Faculty of Pharmacy,Shahid Sadoughi University of Medical Sciences, Yazd, Iran

Abstract:	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.

Keywords:	Cholestasis Lipoid Congenital Adrenal Hyperplasia Neonate Adrenal Hyperplasia