ADRENOLEUKODYSTROPHIES Meaning and
Definition
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Adrenoleukodystrophies (ALD) are a group of rare, genetically inherited disorders that primarily affect the adrenal glands and the white matter of the brain and spinal cord. These conditions are characterized by the accumulation of very long-chain fatty acids (VLCFAs) in various tissues throughout the body.
ALD is typically caused by mutations in the ABCD1 gene, which encodes for a protein involved in the transport of VLCFAs into the peroxisomes – small structures within cells responsible for the breakdown of fatty acids. The impaired function of this protein leads to the buildup of VLCFAs, particularly in the adrenal glands and white matter of the nervous system.
The symptoms and severity of ALD can vary widely, even within affected individuals from the same family. They commonly include adrenal insufficiency (resulting in hormonal imbalances), progressive loss of myelin (demyelination) in the brain and spinal cord leading to neurological impairment, and potentially fatal cerebral demyelination. Childhood cerebral ALD, the most severe form, typically manifests between the ages of 4 and 10 and often leads to progressive neurological deterioration and reduced life expectancy.
While there is currently no cure for ALD, management focuses on treating symptoms and preventing complications. Regular monitoring, hormonal replacement therapy, dietary interventions, physical and occupational therapy, and stem cell transplantation may be used to alleviate symptoms and slow disease progression.
Overall, adrenoleukodystrophies refer to a group of inherited disorders characterized by the abnormal accumulation of very long-chain fatty acids, primarily affecting the adrenal glands and the white matter of the brain and spinal cord.
Common Misspellings for ADRENOLEUKODYSTROPHIES
- zdrenoleukodystrophies
- sdrenoleukodystrophies
- wdrenoleukodystrophies
- qdrenoleukodystrophies
- asrenoleukodystrophies
- axrenoleukodystrophies
- acrenoleukodystrophies
- afrenoleukodystrophies
- arrenoleukodystrophies
- aerenoleukodystrophies
- adeenoleukodystrophies
- addenoleukodystrophies
- adfenoleukodystrophies
- adtenoleukodystrophies
- ad5enoleukodystrophies
- ad4enoleukodystrophies
- adrwnoleukodystrophies
- adrsnoleukodystrophies
- adrdnoleukodystrophies
- adrrnoleukodystrophies
Etymology of ADRENOLEUKODYSTROPHIES
The word "Adrenoleukodystrophies" is derived from combining three different terms: "adreno", "leuko", and "dystrophies".
1. Adreno: This term stems from the word "adrenal", which refers to the adrenal glands, small triangular-shaped endocrine glands located on top of the kidneys. The word "adrenal" itself is derived from the Latin word "ad-", meaning "next to", and "renes", meaning "kidneys".
2. Leuko: This term comes from the Greek word "leukos", meaning "white". In medical terminology, it refers to white matter, which is a type of brain tissue consisting of myelinated neural cells.
3. Dystrophies: This term is derived from the Greek word "dystrophē", meaning "abnormal nourishment" or "degeneration".
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