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Dictionary » Hepatolenticular degeneration
 

Hepatolenticular degeneration

Introduction: Hepatolenticular degeneration

Description of Hepatolenticular degeneration

Hepatolenticular degeneration (medical condition): Wilson disease, or hepatolenticular degeneration, is a neurodegenerative...more »

See also:

Wilson's Disease:
  »Introduction: Wilson's Disease
  »Symptoms of Wilson's Disease
  »Causes of Wilson's Disease
  »Tests for Wilson's Disease
  »Treatments for Wilson's Disease

Hepatolenticular degeneration: a rare inherited disorder of copper metabolism; copper accumulates in the liver and then in the red blood cells and brain.
Source: WordNet 2.1

Hepatolenticular degeneration: 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.
Source: Diseases Database

Hepatolenticular degeneration: rare autosomal recessive disease characterized by the deposition of copper in the brain, liver, cornea and other organs; clinical features include liver cirrhosis, liver failure, splenomegaly, tremor, bradykinesia, dysarthria, chorea, muscle rigidity, Kayser-Fleischer rings (pigmented corneal lesions), ataxia and intellectual deterioration; hepatic dysfunction may precede neurologic dysfunction by several years.
Source: CRISP

Hepatolenticular degeneration: 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.
Source: MeSH 2007

Hepatolenticular degeneration: Related Topics

These medical condition or symptom topics may be relevant to medical information for Hepatolenticular degeneration:

Hepatolenticular degeneration: Rare Disease

Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Hepatolenticular degeneration is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Hepatolenticular degeneration, or a subtype of Hepatolenticular degeneration, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet

Ophanet, a consortium of European partners, currently defines a condition rare when if affects 1 person per 2,000. They list Hepatolenticular degeneration as a "rare disease".

Source - Orphanet

Hepatolenticular degeneration as a Disease

Hepatolenticular degeneration: Another name for Wilson's Disease (or close medical condition association).
  »Introduction: Wilson's Disease
  »Symptoms of Wilson's Disease
  »Causes of Wilson's Disease
  »Treatments for Wilson's Disease

Hepatolenticular degeneration: Related Diseases

Hepatolenticular degeneration: Hepatolenticular degeneration is listed as a type of (or associated with) the following medical conditions in our database:

Causes of Hepatolenticular degeneration

Some of the causes of Hepatolenticular degeneration are included in the list below:

  • Wilson disease is an autosomal recessive inherited condition caused by mutations or deletions of the ATP7B protein encoded by chromosome subbands 13q14.3-q 21.1

Symptoms of Hepatolenticular degeneration (Wilson's Disease)

Some of the symptoms of Hepatolenticular degeneration incude:

Treatments for Hepatolenticular degeneration (Wilson's Disease)

Treatments for Hepatolenticular degeneration (Wilson's Disease) include:

  • Lifelong treatment
  • Low-copper diet - avoid shellfish, chocolate, liver, mushrooms, nuts, and various other foods.
  • Vitamin B6 supplements (pyridoxine)
  • Zinc supplements
  • Anti-copper agents

Treatment of Hepatolenticular degeneration: For more treatment information about Hepatolenticular degeneration, see treatment of Wilson's Disease (Hepatolenticular degeneration)

Hepatolenticular degeneration: Article Excerpts about Wilson's Disease

Genes and Disease by the National Center for Biotechnology (Excerpt)

Wilson's Disease is a rare autosomal recessive disorder of copper transport, resulting in copper accumulation and toxicity to the liver and brain....(Source: Genes and Disease by the National Center for Biotechnology)

Wilson's Disease: NIDDK (Excerpt)

Wilson's disease causes the body to retain copper. The liver of a person who has Wilson's disease does not... (Source: excerpt from Wilson's Disease: NIDDK)

NINDS Wilson's Disease Information Page: NINDS (Excerpt)

Wilson's disease is an inherited disorder in which excessive amounts of copper accumulate in the body. Although... (Source: excerpt from NINDS Wilson's Disease Information Page: NINDS)

Hepatolenticular degeneration: Related Disease Topics

These medical disease topics may be related to Hepatolenticular degeneration:

Terms associated with Hepatolenticular degeneration:

Terms Similar to Hepatolenticular degeneration:

Source: Diseases Database

Source - NIH

Source - CRISP

Source - WordNet 2.1

Source - MeSH 2007

Broader terms for Hepatolenticular degeneration

Source - MeSH 2007

Source - CRISP

Source - WordNet 2.1

The term Hepatolenticular degeneration can be used for:

Source: CRISP

Other terms that may be related to Hepatolenticular degeneration:

Source: CRISP

Hierarchical classifications of Hepatolenticular degeneration

The following list attempts to classify Hepatolenticular degeneration into categories where each line is subset of the next.

WordNet 2.1

  • congenital disease,genetic abnormality,genetic defect,genetic disease,genetic disorder,hereditary condition,hereditary disease,inherited disease,inherited disorder
  • disease
  • illness,malady,sickness,unwellness
  • health problem,ill health,unhealthiness
  • pathological state
  • condition,status
  • state

Source: WordNet 2.1

MeSH 2007 Hierarchy:

MeSH 2007 Hierarchy

MeSH 2007 Hierarchy

MeSH 2007 Hierarchy

MeSH 2007 Hierarchy

External links related to: Hepatolenticular degeneration

Source: Diseases Database

Interesting Medical Articles:

Medical dictionaries:

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