Folia Medica Indonesiana
Vol. 58 No. 3 (2022): September

Diagnosis and Management of Adrenal Crisis in 46XX Congenital Adrenal Hyperplasia Infant

Nur Rochmah (Department of Child Health, Dr. Soetomo General Hospital / Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia)
Muhammad Faizi (Department of Child Health, Dr. Soetomo General Hospital / Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia)
Neurinda Permata Kusumastuti (Department of Child Health, Dr. Soetomo General Hospital / Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia)
Leonardo Ferryanto Mak Samadhi (Department of Child Health, Dr. Soetomo General Hospital / Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia)
Wika Yuli Deakandi (Faculty of Medicine, Universitas Islam Malang, Malang, East Java, Indonesia)



Article Info

Publish Date
05 Sep 2022

Abstract

Highlight: The diagnosis and therapy of Congenital Adrenal Hyperplasia (CAH) children with Adrenal crisis (AC) case report. Adrenal crisis (AC) is a life-threatening emergency that contributes to the high death rate of children with adrenal insufficiency. The early detection and prompt treatment can improve the outcomes of patients with CAH and AC.   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. The 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 patient focused on diagnosis and therapy. A female, 10 months old infant, was 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 normal growth and development.

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Journal Info

Abbrev

FMI

Publisher

Subject

Other

Description

Folia Medica Indonesiana publishes articles in the field of medical science in particular and health sciences in general, as well as a variety of other fields related to those two disciplines. Most of the articles are research article, and others are articles on case reports and literature review. ...