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A Case of Adrenoleukodystrophy.

Journal of the Korean Pediatric Society 1989;32(3):430-437.
Published online March 31, 1989.
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)


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