Endocrine Abstracts

Adrenoleukodystrophy has a prevalence rate of 1:20 000 and is a cause of adrenal insufficiency in association with demyelination within the nervous system due to a failure of β-oxidation of fatty acids within peroxisomes due to reduced activity of very long chain acyl-CoA synthetase. Several forms are recognized; a childhood cerebral form (30 to 40% cases), adult adrenomyeloneuropathy (40% cases), and Addison’s disease only (7% cases). Adrenomyeloneuropathy, by contr...