About The Medical Condition Adrenoleukodystrophies, X-linked
Find the definition of the medical term Adrenoleukodystrophies, X-linked. Adrenoleukodystrophies, X-linked defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Adrenoleukodystrophies, X-linked
| Adrenoleukodystrophies, X-linked Medical Condition |
|---|
What's The Definition Of The Medical Condition Adrenoleukodystrophies, X-linked?
An X-linked recessive leukodystrophy characterized by an abnormal accumulation of saturated very long chain fatty acids in LYSOSOMES. It primarily affects the white matter of the CENTRAL NERVOUS SYSTEM and the ADRENAL CORTEX. This disorder results from defective beta-oxidation and occurs almost exclusively in males and has multiple phenotypes. Relatively common clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; adrenal insufficiency; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. A related condition, adrenomyeloneuropathy, usually has its onset in adult life and is characterized by spastic paraparesis, adrenal insufficiency, neuropathy, and HYPOGONADISM. (From Neuropediatrics 1998 Feb;29(1):3-13; Menkes, Textbook of Child Neurology, 5th ed, p188) The defective gene for this disorder has been localized to the long arm of the X chromosome (Xq28).
|
More Medical Conditions
A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z
Medical Conditions Definitions Of The Day
- Arthroplasties, Replacement, Knee ‐ Replacement of the…
- Soya Oil ‐ Oil from soybean or…
- Tyrosine Aminotransferase ‐ An enzyme that catalyzes the conversion of L-TYROSINE and 2-oxoglutarate…
- Memory Losses, Retrograde ‐ Loss of the ability to recall information that had been previously…
- Deformities, Foot ‐ Alterations or deviations from normal shape or size which result…
- Gas Gangrenes ‐ A severe condition resulting from bacteria invading healthy muscle…
- Neuron, Nitrergic ‐ Nerve cells where transmission is mediated by…
- Teonicol ‐ A vasodilator used in peripheral vascular disorders and insufficiency.…
- Beryllium ‐ Beryllium. An element with the atomic symbol Be, atomic number…
- Hormone Receptors, Nuclear ‐ Proteins in the cytoplasm or nucleus that specifically bind signaling…