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Doctor insights on: Autosomal Adrenoleukodystrophy

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What is adrenoleukodystrophy?

What is adrenoleukodystrophy?

Not a good disorder: Adrenoleukodystrophy describes several closely related inherited disorders that disrupt the breakdown of very long chain fatty acids (vlcfa). These vlcfa then accumulate in the nervous system, especially the brain, and the adrenal glands and disrupt their function. The childhood form is progressive and there is no specific treatment available yet. ...Read more

Dr. Harry Chugani
45 doctors shared insights

Adrenoleukodystrophy (Definition)

Adrenoleukodystrophy - genetic disorder in which myelin sheaths (insulation for neurons) in brain ...Read more


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What is adrenoleukodystrophy?

What is  adrenoleukodystrophy?

Abnormal fatty acids: It is a spectrum of disorders that include adrenal insufficiency along with an abnormal deposition of fatty acids in nerve tissues including the brain. It ranges from mild to fatal. ...Read more

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How common is adrenoleukodystrophy?

Adrenoleukodystrophy: 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. ...Read more

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Can adrenoleukodystrophy be treated?

No real cure: Unfortunately, much of what can be done is symptomatic relief, such as adrenal support and perhaps hormonal treatment for testicular dysfnctn. It has been said that lorenzo's oil is of value, but little proof of success. Contact a major medical school, as research projects may result in a few breakthroughs. ...Read more

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What are the tests for adrenoleukodystrophy?

Tests: Tests by a neurologist can include: vision screening, MRI of the brain, blood tests for (vlcfa) very long-chain fatty acids, genetic testing and skin biopsy. ...Read more

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What sort of problem is adrenoleukodystrophy?

Adrenoleukodystrophy: Adrenoleukodystrophy is passed from parents to their children as an x-linked genetic trait. It affects males, although some women who are carriers can have mild disease. It affects 1 in 20, 000. The condition results in the buildup of very-long-chain fatty acids in nervous system, adrenal gland, and testes, which disturbs normal function. For more see: http://www.Umm.Edu/ency/article/001182.Htm. ...Read more

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What are the symptoms of adrenoleukodystrophy?

Depends on type: The symptoms depend on the type of adrenoluekodystrophy. This site is very educational: http://www.Adrenoleukodystrophy.Net/. ...Read more

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Does anyone know about adrenoleukodystrophy diease?

Complex illness: This is an x-linked recessive disease mostly in young males, but can be seen later in life, due to accumulation of long-chain fatty acids. Causes loss of behavioural control, even hyperactivity, but can lose ability to walk, have adrenal and sexual failure. Predominant damage to central nervous myelin (nerve insulation). Hard to diagnose due to varied presentation. No cure. Lorenzo's oil? ...Read more

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What percentage of the us population have adrenoleukodystrophy?

1 in 20,000: From the ncbi: http://www.Ncbi.Nlm.Nih.Gov/pubmedhealth/pmh0002165/ adrenoleukodystrophy (ALD) affects approximately 1 in 20, 000 people in the usa. Given a total population of 311, 591, 917 (www.Google.Com/publicdata), this means approx. 15, 580 people have ALD or 0.005%. ...Read more

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What is the definition or description of: Adrenoleukodystrophy?

Adrenoleukodystrophy: Adrenoleukodystrophy - genetic disorder in which myelin sheaths (insulation for neurons) in brain are damaged. See: http://www.mayoclinic.org/diseases-conditions/adrenoleukodystrophy/basics/definition/CON-20028957 ...Read more

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I was wondering is adrenoleukodystrophy fatal?

Unfortunately YES: It is unusual for these unfortunate children to live through adolescence. ...Read more

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What treatment is currently used for adrenoleukodystrophy, or ald?

Some treatments: From: http://www.Ncbi.Nlm.Nih.Gov/pubmedhealth/pmh0002165/ adrenal dysfunction is treated with steroids (e.g., cortisol). A diet low in very-long-chain fatty acids (vlcfa) and taking special oils (e.g., lorenzo's oil) can lower the blood levels of vlcfa, but doesn't help all patients. Bone marrow transplant is also being tested. See also: http://www.Aldfoundation.Org/. ...Read more

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Do you know if adrenoleukodystrophy is fatal?

Adrenoleukodystrophy: is a group of related X-linked inherited disorders in 3 categories. In the childhood cerebral form with initial neurological signs < age 10, progression varies, but can be rapid & cause complete disability within a few years. Life expectancy depends on severity & rate of progression + intensity of medical support. See http://ulf.org/adrenoleukodystrophy for information & support for the family. ...Read more

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Can you tell me is adrenoleukodystrophy fatal?

Can you tell me is adrenoleukodystrophy fatal?

Adrenoleukodystrophy: is a group of related X-linked inherited disorders in 3 categories. In the childhood cerebral form with initial neurological signs < age 10, progression varies, but can be rapid & cause complete disability within a few years. Life expectancy depends on severity & rate of progression + intensity of medical support. See http://ulf.org/adrenoleukodystrophy for information & support for the family. ...Read more

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What is the life expectancy with adrenoleukodystrophy?

What is the life expectancy with adrenoleukodystrophy?

Varies: This disorder has such a wide spectrum of severity that it is impossible to predict without knowing the subtype. Some forms are mild, other fatal within 5-10 years. For some, bone marrow transplantation may be indicated. Knowledge of type and severity is crucial. ...Read more

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Is adrenoleukodystrophy chromosome problem?....If so, which one?

Yes: 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. ...Read more

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What are some foods that can be eaten by an adrenoleukodystrophy patient?

Whatever he can: Special diets are available, but more important is what he can swallow. Some have severe adrenal disease with diet restrictions. Some are on special high fat diets. Most often we just have them eat what they can and what they enjoy. It is a horrible disease without cure and progressive. ...Read more

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How would advise a patient that has adrenoleukodystrophy in the family about inheritance risk?

Recessive in males: ALD is a male disease that is passed in a recessive manner. It can be carried by the female. Markers with a pattern of long chain fatty acids can be looked for. Specific hormone level alterations may be found early in affected patients, and mris will show very characteristic features. In utero studies have been tried, but are not reliable. ...Read more

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Is lorenzo's oil a cure for adrenoleukodystrophy?

I wish: There was a lot of press regarding this a while ago. The oil does nothing and represents a false hope to a devastating illness. Contact your local medical school where you live, as they have excellent docs to deal with this problem. ...Read more