Research Information System
SENDROM, vol.15, no.5, pp.68-75, 2003 (Scopus)
X-linked adrenoleukodystrophy (ALD) is a serious and progressive disorder where the adrenal cortex and myeline fibers of the nervous system are affected. The biochemical defect in ALD is an impaired ability to oxidase very long chain fatty acids (VLCFA). This results in accumulation of saturated VLCFA in the brain, adrenal tissue, testes, plasma, liver, erytrocytes and leucocytes. ALD affects only males, although a small number of female carriers may develop a milder form of the disease. The phenotype of X-linked ALD is more varied, at least seven clinical subtypes have been identified in males. In this article, besides some characteristics of subtypes of ALD, possible therapeutical approaches are evaluated and the role of nutritional treatment is emphasized.