LC control no. | sh 90001240 |
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LC classification | RJ496.A32 |
Topical heading | Adrenoleukodystrophy |
Variant(s) | ALD (Disease) Neonatal adrenoleukodystrophy Siemerling-Crentzfeldt disease |
See also | Demyelination Lipidoses Nerves, Peripheral--Diseases Peroxisomal disorders X-linked mental retardation |
Found in | Work cat.: International Workshop on Adrenoleukodystrophy and Peroxisomal Disorders (1989 : Rome, Italy). Adrenoleukodystrophy and other peroxisomal disorders : clinical, biochemical, genetic, and therapeutic aspects : proceedings ... Rome, 10-11 November 1989 (ALD) MESH. Merck ("... is a rare, sex-linked recessive metabolic disorder that occurs in boys and is characterized by adrenal atrophy, and widespread, diffuse cerebral demyelination") Merritt's textbk. neurol.: pp. 536-537 ("the basic defect involves the fatty acids β oxydation system in subcellar organelles, called peroxisomes, which break down very long chain fatty acids (VLCFAs). Consequently these acids accumulate in tissue and plasma") Dorland's med. dict. Stedman's med. dict. (Siemerling-Crentzfeldt disease) Gould med. dict. |