Hepatolenticular Degeneration
"Hepatolenticular Degeneration" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
| Descriptor ID |
D006527
|
| MeSH Number(s) |
C06.552.413 C10.228.140.079.493 C10.228.140.163.100.360 C10.228.662.400 C10.574.500.487 C16.320.400.361 C16.320.565.189.360 C16.320.565.618.403 C18.452.132.100.360 C18.452.648.189.360 C18.452.648.618.403
|
| Concept/Terms |
Hepatolenticular Degeneration- Hepatolenticular Degeneration
- Degeneration, Hepatolenticular
- Neurohepatic Degeneration
- Degeneration, Neurohepatic
- Degenerations, Neurohepatic
- Neurohepatic Degenerations
- Hepato-Neurologic Wilson Disease
- Diseases, Hepato-Neurologic Wilson
- Hepato Neurologic Wilson Disease
- Hepato-Neurologic Wilson Diseases
- Wilson Disease, Hepato-Neurologic
- Wilson Diseases, Hepato-Neurologic
- Hepatocerebral Degeneration
- Degeneration, Hepatocerebral
- Degenerations, Hepatocerebral
- Hepatocerebral Degenerations
- Kinnier-Wilson Disease
- Diseases, Kinnier-Wilson
- Kinnier Wilson Disease
- Kinnier-Wilson Diseases
- Wilson's Disease
- Wilsons Disease
- Progressive Lenticular Degeneration
- Degeneration, Progressive Lenticular
- Lenticular Degeneration, Progressive
- Pseudosclerosis
- Wilson Disease
- Cerebral Pseudosclerosis
- Cerebral Pseudoscleroses
- Pseudoscleroses, Cerebral
- Pseudosclerosis, Cerebral
- Westphal-Strumpell Syndrome
- Westphal Strumpell Syndrome
- Westphal-Strumpell Syndromes
|
Below are MeSH descriptors whose meaning is more general than "Hepatolenticular Degeneration".
- Diseases [C]
- Digestive System Diseases [C06]
- Liver Diseases [C06.552]
- Hepatolenticular Degeneration [C06.552.413]
- Nervous System Diseases [C10]
- Central Nervous System Diseases [C10.228]
- Brain Diseases [C10.228.140]
- Basal Ganglia Diseases [C10.228.140.079]
- Hepatolenticular Degeneration [C10.228.140.079.493]
- Brain Diseases, Metabolic [C10.228.140.163]
- Brain Diseases, Metabolic, Inborn [C10.228.140.163.100]
- Hepatolenticular Degeneration [C10.228.140.163.100.360]
- Movement Disorders [C10.228.662]
- Hepatolenticular Degeneration [C10.228.662.400]
- Neurodegenerative Diseases [C10.574]
- Heredodegenerative Disorders, Nervous System [C10.574.500]
- Hepatolenticular Degeneration [C10.574.500.487]
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
- Heredodegenerative Disorders, Nervous System [C16.320.400]
- Hepatolenticular Degeneration [C16.320.400.361]
- Metabolism, Inborn Errors [C16.320.565]
- Brain Diseases, Metabolic, Inborn [C16.320.565.189]
- Hepatolenticular Degeneration [C16.320.565.189.360]
- Metal Metabolism, Inborn Errors [C16.320.565.618]
- Hepatolenticular Degeneration [C16.320.565.618.403]
- Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
- Brain Diseases, Metabolic [C18.452.132]
- Brain Diseases, Metabolic, Inborn [C18.452.132.100]
- Hepatolenticular Degeneration [C18.452.132.100.360]
- Metabolism, Inborn Errors [C18.452.648]
- Brain Diseases, Metabolic, Inborn [C18.452.648.189]
- Hepatolenticular Degeneration [C18.452.648.189.360]
- Metal Metabolism, Inborn Errors [C18.452.648.618]
- Hepatolenticular Degeneration [C18.452.648.618.403]
Below are MeSH descriptors whose meaning is more specific than "Hepatolenticular Degeneration".
This graph shows the total number of publications written about "Hepatolenticular Degeneration" by people in this website by year, and whether "Hepatolenticular Degeneration" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2016 | 1 | 0 | 1 |
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Below are the most recent publications written about "Hepatolenticular Degeneration" by people in Profiles.