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Topic: Adrenoleukodystrophy


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In the News (Tue 1 Dec 09)

  
  Adrenoleukodystrophy - Wikipedia, the free encyclopedia
Adrenoleukodystrophy (ALD) is a degenerative disorder of the fatty white sheath covering nerve fibers, known as myelin.
A type of leukodystrophy, the victims of ALD are typically male, as the disease is usually inherited in a sex-linked manner on the X chromosome.
The prevalence of X-linked adrenoleukodystrophy is approximately 1 in 20,000 individuals.
en.wikipedia.org /wiki/Adrenoleukodystrophy   (826 words)

  
 Adrenoleukodystrophy
Adrenoleukodystrophy is a rare inherited metabolic disorder characterized by the loss of the fatty covering (myelin sheath) on nerve fibers within the brain (cerebral demyelination) and the progressive degeneration of the adrenal gland (adrenal atrophy).
Neonatal Adrenoleukodystrophy is inherited as an autosomal recessive genetic trait.
The symptoms of Adrenoleukodystrophy are thought to result from deficient enzyme (e.g., lignoceroyl-CoA ligase) activity, leading to an abnormal accumulation of very long chain fatty acids in certain cells and tissues, particularly in the brain and the outer region of the adrenal glands (adrenal cortex).
hw.healthdialog.com /kbase/nord/nord43.htm   (3285 words)

  
 Adrenoleukodystrophy Encyclopedia Search - Drug Price Search
Adrenoleukodystrophy describes any of several closely related inheritable disorders of the breakdown (metabolism) of certain fats (long chain fatty acids).
A specific treatment for adrenoleukodystrophy is not available, but a diet low in long chain fatty acids and the administration of special oils has been demonstrated to lower the blood levels of the long chain fatty acids.
The childhood form of adrenoleukodystrophy is a progressive disease that leads to a vegetative state in approximately two years after neurologic symptoms develop.
www.drug-price-search.com /encyclopedia/?encyclopedia_name_url=209&level=2   (597 words)

  
 MedlinePlus Medical Encyclopedia: Adrenoleukodystrophy
Adrenoleukodystrophy describes any of several closely related inheritable disorders that involve the breakdown (metabolism) of certain fats (long chain fatty acids).
Adrenoleukodystrophy is transmitted as an X-linked trait (the neonatal form is by autosomal recessive transmission).
Intrauterine diagnosis of adrenoleukodystrophy is available and done by evaluation of cells from chorionic villus sampling or from amniotic cells (amniocentesis).
www.nlm.nih.gov /medlineplus/ency/article/001182.htm   (705 words)

  
 MedFriendly.com: Adrenoleukodystrophy
Adrenoleukodystrophy (abbreviated as ALD) is a rare disorder that is characterized by a buildup of saturated, very long chain fatty acids (abbreviated VLCFA) in the tissues of the body.
Adrenoleukodystrophy is abbreviated as ALD or as X-ALD.
Adrenoleukodystrophy is also known as sex-linked adrenoleukodystrophy, melanodermic leukodystrophy, and x-linked adrenoleukodystrophy.
www.medfriendly.com /adrenoleukodystrophy.html   (6892 words)

  
 Adrenoleukodystrophy: Facts and details from Encyclopedia Topic   (Site not responding. Last check: 2007-10-20)
Adrenoleukodystrophy (ALD) is a degenerative disorder of nerve fiber axon quick summary:
An axon, or "nerve fiber," is a long slender projection of a nerve cell, or "neuron," which conducts electrical impulses away from the neurons cell body...
Lorenzos oil is a 4:1 mixture of oleic and erucic acid used in the preventative treatment of adrenoleukodystrophy (ald)....
www.absoluteastronomy.com /encyclopedia/a/ad/adrenoleukodystrophy.htm   (1216 words)

  
 Lorenzo's oil for AMN and ALD
Adrenoleukodystrophy (ALD) is a rare (incidence about 1 in 45,000), genetic disorder characterized by the breakdown or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland.
ALD is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath, the fatty covering — which acts as an insulator — on nerve fibers in the brain.
X-linked adrenoleukodystrophy: overview and prognosis as a function of age and brain magnetic resonance imaging abnormality.
www.jr2.ox.ac.uk /bandolier/booth/neurol/lorenz.html   (1397 words)

  
 BBC - Health - Conditions - Adrenoleukodystrophy
Adrenoleukodystrophy (ALD) is a rare inherited disorder that leads to progressive brain damage, failure of the adrenal gland, and eventually death.
ALD is one of a group of disorders called the leukodystrophies where the fatty covering of nerve fibres, called the myelin sheath, breaks down.
Adrenoleukodystrophy is one of several inherited disorders where the myelin sheath - the fatty insulating layer that covers many of the neurons - is progressively damaged, because of a faulty gene.
www.bbc.co.uk /health/conditions/adrenoleukodystrophy1.shtml   (908 words)

  
 X-Linked Adrenoleukodystrophy
All of the mutations not previously reported occurred in residues conserved between human and rodent proteins and were within the transmembrane and nucleotide binding fold domains [H Moser, A Moser, S Steinberg; personal observation].
Moser AB and Moser HW (1999) The prenatal diagnosis of X-linked adrenoleukodystrophy.
Moser HW (1997) Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy.
www.geneclinics.org /profiles/x-ald/details.html   (4578 words)

  
 X-linked adrenoleukodystrophy - Genetics Home Reference
X-linked adrenoleukodystrophy is a disorder that mainly affects the nervous system and the adrenal glands (small glands located on top of each kidney).
Mutations in the ABCD1 gene cause X-linked adrenoleukodystrophy.
Mutations in the ABCD1 gene cause a shortage (deficiency) of a cellular transporter known as adrenoleukodystrophy protein.
ghr.nlm.nih.gov /condition=xlinkedadrenoleukodystrophy   (931 words)

  
 Adrenoleukodystrophy   (Site not responding. Last check: 2007-10-20)
Adrenoleukodystrophy (ALD) is a rare, genetic disorder characterized by the breakdown or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland.
ALD is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath, the fatty covering -- which acts as an insulator -- on nerve fibers in the brain.
Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.
healthlink.mcw.edu /article/921176192.html   (354 words)

  
 Facts on X-Linked Adrenoleukodystrophy (X-ALD)   (Site not responding. Last check: 2007-10-20)
Stephan Kemp, Ph.D and Hugo Moser, M.D. Adrenoleukodystrophy (X-ALD) is a serious progressive, genetic disorder, which affects the adrenal glands and the white matter of the nervous system.
In 1971, Dr. Michael Blaw coined the name adrenoleukodystrophy; adreno refers to the adrenal glands; leuko refers to the white matter of the brain, and dystrophy means imperfect growth or development.
X-ALD is a peroxisomal storage disease whereby abnormal function of peroxisomes leads to the accumulation of very long-chain fatty acids (VLCFA) in tissues of the body, especially the brain and the adrenal glands.
www.x-ald.nl /facts.htm   (1067 words)

  
 Adrenoleukodystrophy   (Site not responding. Last check: 2007-10-20)
Adrenoleukodystrophy describes any of several closely related inheritable disorders of the breakdown (metabolism) of certain fats (long chain fatty acid) that affects the adrenal glands, nervous system, and testes.
Its incidence is estimated at 1 out of 20,000 to 1 in 50,000 and affects all races.
The other major component of the childhood form and all other forms of adrenoleukodystrophy is the development of impaired adrenal gland function (similar to Addison disease).
www.shands.org /health/information/article/001182.htm   (632 words)

  
 Adrenoleukodystrophy - Lorenzo's Oil - DrGreene.com
Adrenoleukodystrophy is one of the genetic neurodegenerative disorders.
Adrenoleukodystrophy comes in three types (classic, neonatal, and adrenomyeloneuropathy).
Adrenoleukodystrophy is one of the neurodegenerative diseases of childhood for which bone marrow transplantation is most successful.
www.drgreene.org /body.cfm?id=21&action=detail&ref=13   (453 words)

  
 Help with ALD (adrenoleukodystrophy)
Adrenoleukodystrophy (ALD) is a rare, genetic disease for which there is no effective treatment or cure.
It is best known as the disease portrayed in the 1993 movie "Lorenzos Oil" starring Nick Nolte and Susan Sarandon.
All of the proceeds of the Run For ALD will benefit the Kennedy Krieger Institute, a non-profit hospital dedicated to treating, preventing and ultimately curing ALD and other neurogenetic diseases.
www.run4ald.org   (104 words)

  
 eMedicine - Peroxisomal Disorders : Article by Aziza K Chedrawi, MD   (Site not responding. Last check: 2007-10-20)
MRI of a patient with adrenoleukodystrophy showing the typical pattern of posterior white-matter involvement.
Jaffe R, Crumrine P, Hashida Y, et al: Neonatal adrenoleukodystrophy: clinical, pathologic, and biochemical delineation of a syndrome affecting both males and females.
Knazek RA, Rizzo WB, Schulman JD, et al: Membrane microviscosity is increased in the erythrocytes of patients with adrenoleukodystrophy and adrenomyeloneuropathy.
www.emedicine.com /NEURO/topic309.htm   (5503 words)

  
 Kennedy Krieger Institute: Adrenoleukodystrophy (ALD)   (Site not responding. Last check: 2007-10-20)
ALD is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath, the fatty covering, on nerve fibers in the brain.
Neonatal adrenoleukodystrophy (NALD) is a disorder that is totally distinct from X-linked adrenoleukodystrophy.
NALD and X-linked adrenoleukodystrophy never occur in the same family.
www.kennedykrieger.org /kki_diag.jsp?pid=1069   (388 words)

  
 Museum of Pathology   (Site not responding. Last check: 2007-10-20)
These specimens are from a patient suffering from adrenoleukodystrophy.
There were many giant astrocytes and around the blood vessels many small round cells and compound granular corpuscles.
Adrenoleukodystrophy is a familial progressive demyelinating disorder affecting the cerebral white matter.
museum.med.monash.edu.au /spec/index.cfm?spec=WQ4   (160 words)

  
 Treatment of X-linked adrenoleukodystrophy with Lorenzo's oil -- MOSER 67 (3): 279 -- Journal of Neurology, ...
Progression of abnormalities in adrenomyelopathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil".
The future for treatment by bone marrow transplantation for adrenoleukodystrophy, metachromatic leukodystrophy, globoid leukodystrophy and Hurler syndrome.
Gene redundancy and pharmacologic gene therapy: implications for X-linked adrenoleukodystrophy.
jnnp.bmjjournals.com /cgi/content/full/67/3/279   (602 words)

  
 [No title]
Adrenoleukodystrophy Foundation - Obtain educational material, research, contacts and memorials.
Adrenoleukodystrophy X-Linked - Includes a database of mutations of X-ALD.
Loren's Adrenoleukodystrophy Page - Features information about the disorder and examines the relationships between the condition and a popular film, Lorenzo's Oil.
botw.org /top/Health/Conditions_and_Illness/Genetic_Disorders/Adrenoleukodystrophy   (146 words)

  
 Adrenoleukodystrophy (ALD): A Case Study
Using the Film "Lorenzo's Oil"
This is an interdisciplinary study that could be used on several levels and with a variety of classes, for example: Biology, Chemistry, Genetics, Ethics, Health, Neurology, Nutrition, Pathology, Psychology and Anatomy.
Adrenoleukodystrophy (ALD) is a rare inherited metabolic disorder characterized by the loss of the fatty covering (myelin sheath) on nerve fibers within the brain and progressive degeneration of the adrenal gland.
The basic defect is the impaired capacity to degrade very long chain fatty acids that are found in the blood plasma and tissues of the body.
www.accessexcellence.org /AE/AEPC/WWC/1994/adreno.html   (1217 words)

  
 Adrenoleukodystrophy
This condition is one of a group of genetic disorders called the leukodystrophies, that results in damage to the myelin sheath (the fatty covering on the nerve cells in the brain) which Lorenzo's oil, a mixture of glyceryl trioleate and glyceryl trierucate, attempts to reduce.
This Web resource on adrenoleukodystrophy (a rare genetic disorder) is produced by the National Institute of Neurological Disorders and Stroke (NINDS).
A description of adrenoleukodystrophy is provided, and available treatments, prognosis, and current research activities are all discussed.
omni.ac.uk /browse/mesh/D000326.html   (231 words)

  
 Adrenoleukodystrophy - ALDS   (Site not responding. Last check: 2007-10-20)
The term adrenoleukodystrophy encompasses two distinct genetic disorders; X-linked adrenoleukodystrophy and neonatal ALD.
Adrenoleukodystrophy (ALD) is a serious genetic disorder which progressively affects the adrenal gland, along with the white matter of the nervous system.
In ALD, there is an abnormal accumulation of very long chain fatty acids, which causes tissue damage, however the exact nature of this relationship is not understood.
www.alds.org.au /ald.html   (391 words)

  
 What is Adrenoleukodystrophy?
Adrenoleukodystrophy (ALD) falls into a group of disorders known as leukodystrophies.
This disease is diagnosed with a plasma test to determine the level of very long chain fatty acids, regardless of symptomatology.
The figure above shows the location of the Adrenoleukodystrophy gene on the x chromosome.
www.ikm.jmu.edu /Buttsjl/ISAT493/Adrenoleukodystrophy/aldintro.html   (153 words)

  
 Adrenoleukodystrophy Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
Adrenoleukodystrophy Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
The nation's leading supporter of biomedical research on disorders of the brain and nervous system
Adrenoleukodystrophy (ALD) is one of a group of genetic disorders called the
ninds.nih.gov /disorders/adrenoleukodystrophy/adrenoleukodystrophy.htm   (642 words)

  
 Prognosis of Adrenoleukodystrophy - WrongDiagnosis.com
About prognosis: The 'prognosis' of Adrenoleukodystrophy usually refers to the likely outcome of Adrenoleukodystrophy.
The prognosis of Adrenoleukodystrophy may include the duration of Adrenoleukodystrophy, chances of complications of Adrenoleukodystrophy, probable outcomes, prospects for recovery, recovery period for Adrenoleukodystrophy, survival rates, death rates, and other outcome possibilities in the overall prognosis of Adrenoleukodystrophy.
Prognosis for Adrenoleukodystrophy: Prognosis for patients with ALD is generally poor except after successful bone marrow transplantation.
www.wrongdiagnosis.com /a/adrenoleukodystrophy/prognosis.htm   (173 words)

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