Nur Rochmah, - and Muhammad Faizi, - and Neurinda Permata Kusumastuti, - and Wika Yuli Deakandi, - and Leonardo Ferryanto Mak Samadhi, - (2022) Case Report: Diagnosis and Management of Adrenal Crisis in 46XX Congenital Adrenal Hyperplasia Infant. Fol Med Indones, 58 (2). pp. 273-279. ISSN 2338-476X

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Abstract

Adrenal crisis is the acute complication of the patient with congenital adrenal hyperplasia. Congenital adrenal hyperplasia (CAH) is a rare condition, Children with CAH commonly come to the emergency room due to acute complications. This condition has high mortality and thus needs early recognition. Newborn screening for CAH in Indonesia is not routinely performed and has not been suggested yet. The purpose of this case report was to report a case of adrenal crisis in a congenital adrenal hyperplasia patients focused on diagnosis and therapy. A female, 10 months old infant, w as admitted to the emergency department with a chief complaint of a decrease of consciousness for 3 hours before admission and frequent vomiting since born. On physical examination, there was clitoromegaly. Laboratory showed 17-OH progesterone: 173 ng/dL (7-77 ng/dL) and karyotyping: 46 XX. Management of adrenal crisis is a stress dose of hydrocortisone and rehydration. Education is the key to optimal outcomes and and normal growth and development.

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