A Case of Adrenoleukodystrophy. |
Kee Sung Yang, Soo Tack Bae, Hong Bae Kim, Ji Sub Oh |
Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea |
Adrenoleukodystrophy 1례 |
양기성, 배수택, 김홍배, 오지섭 |
부산 왈레스기념 침례병원 소아과 |
Received: 15 July 1988 • Accepted: 26 September 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. |
Key Words:
Adrenoleukodystrophy (ALD), Demyelination of brain white matter, Adrenal insufficiency.
Very long chanin fatty acids (VLCFAs) |
|