肾上腺脑白质营养不良伴白癜风和溃疡性结肠炎。

Adrenoleukodystrophy associated with vitiligo and ulcerative colitis.

作者信息

Sawaya R A, Mourad F H, Najjar S, Mikati M, Raymond G V

机构信息

Department of Internal Medicine, American University Medical Center, Beirut, Lebanon. rasawaya@cyberia,net.lb

出版信息

Eur Neurol. 1999;42(3):169-72. doi: 10.1159/000008093.

DOI:10.1159/000008093

PMID:10529544

Abstract

Adrenoleukodystrophy (ALD) is an X-linked inherited disorder of lipid metabolism usually presenting in childhood or early adolescence. It is a progressive disease with symptoms of adrenal insufficiency and central nervous system demyelination. The pathology results from the accumulation of very long-chain fatty acids and an inflammatory reaction in the brain white matter. We report a case of ALD associated with adrenal insufficiency and two autoimmune diseases: vitiligo and ulcerative colitis.

摘要

肾上腺脑白质营养不良（ALD）是一种X连锁隐性遗传的脂质代谢紊乱疾病，通常在儿童期或青春期早期发病。它是一种进行性疾病，具有肾上腺功能不全和中枢神经系统脱髓鞘的症状。其病理是由于极长链脂肪酸在脑白质中蓄积以及炎症反应所致。我们报告1例伴有肾上腺功能不全以及两种自身免疫性疾病（白癜风和溃疡性结肠炎）的肾上腺脑白质营养不良病例。