X-linked adrenoleukodystrophy describes several closely related inherited disorders that disrupt the breakdown (metabolism) of certain fats (very-long-chain fatty acids).
Adrenoleukodystrophy is passed down from parents to their children as an X-linked genetic trait. It therefore affects mostly males, although some women who are carriers can have milder forms of the disease. It affects approximately 1 in 20,000 people from all races. The condition results in the buildup of very-long-chain fatty acids in the nervous system, adrenal gland, and testes, which disrupts normal activity. There are three major categories of disease:
Childhood cerebral type: Adrenomyelopathy: Adrenal gland failure (Addison type):
Adrenal dysfunction is treated with steroids (such as cortisol). A specific treatment for X-linked adrenoleukodystrophy is not available, but eating a diet low in very-long-chain fatty acids and taking special oils can lower the blood levels of very-long-chain fatty acids. These oils are called Lorenzo's oil, after the son of the family who discovered the treatment. This treatment is being tested for X-linked adrenoleukodystrophy, but it does not cure the disease and may not help all patients. Bone marrow transplant is also being tested as an experimental treatment.
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Reviewer Info: Rachel A. Lewis, M.D., F.A.A.P., Columbia University Pediatric Faculty Practice, New York, NY. Review provided by VeriMed Healthcare Network.; ADAM Health Illustrated Encyclopedia, 11/12/2007 |