All issues > Volume 32(3); 1989

Original Article

J Korean Pediatr Soc. 1989;32(3):430-437. Published online March 31, 1989.

A Case of Adrenoleukodystrophy.

Kee Sung Yang¹, Soo Tack Bae¹, Hong Bae Kim¹, Ji Sub Oh¹

¹Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea

Received: July 15, 1988;  Accepted: September 26, 1988.

Abstract

We experienced a case of Adrenoleukodystrophy (ALD), which is a genetically determined metabolic disorder associated with progressive demyelination of brain white matter and adrenal insuficiency. An 11 year old male revealed rapidly evolving neuologic progression, decreased adrenal reserve, increased plasma content of very long chain fatty acids (VLCFAs) and typical radiological findings. We report here the clinical findings of this patient with brief review of literature.

Keywords :Adrenoleukodystrophy (ALD), Demyelination of brain white matter, Adrenal insufficiency. Very long chanin fatty acids (VLCFAs)