A practice guideline on Wilson disease

@article{Roberts2003APG,
  title={A practice guideline on Wilson disease},
  author={Eve A. Roberts and Michael L. Schilsky},
  journal={Hepatology},
  year={2003},
  volume={37}
}
This guideline is intended for use by physicians. It describes preferable up-to-date approaches to the diagnosis and treatment of patients with Wilson disease. As their purpose is to direct patient care, these guidelines should not be considered inflexible mandates. They have been developed in a manner consistent with the American Association for the Study of Liver Diseases Policy Statement on Development and Use of Practice Guidelines. These guidelines provide data-supported approaches to the… 

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References

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Diagnosis of Wilson's disease: an experience over three decades

The paper by Gow et al discussed the diagnosis of Wilson's disease in 30 patients presenting to two different clinical facilities over 28 years (1971–1998), and it is important that the information be valid.

Mass screening for Wilson’s disease: Results and recommendations

A review of mass screening for Wilson’s disease in Japan using a specific monoclonal antibody to holoceruloplasmin is presented and the age of 3 years is thought to be the best point for Wilson's disease mass screening.

Liver transplantation for Wilson's disease: Indications and outcome

It is demonstrated that liver transplantation is life‐saving but not without risk for patients with wilsonian fulminant hepatitis or chronic severe hepatic insufficiency unresponsive to medical therapy, and neurological or psychiatric symptoms due to Wilson's disease may improve after liver transplants.

Wilson's disease: clinical presentation and use of prognostic index.

Based on the severity of abnormality of serum aspartate aminotransferase, bilirubin, and prothrombin time on admission a prognostic index was derived which enabled complete separation of fatal and nonfatal cases and when used in a further nine index cases correctly predicted the outcome.

Wilson disease: clinical presentation, treatment, and survival.

Treatment with D-penicillamine improved most of the hematologic and neurologic abnormalities but had little effect on hepatomegaly and splenomeGaly and did not reverse cirrhosis in patients with Wilson disease.

Liver transplantation for Wilson's disease: a single-center experience.

  • B. EghtesadN. Nezakatgoo J. Rakela
  • Medicine
    Liver transplantation and surgery : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 1999
The experience shows OLT is a life-saving procedure in patients with end-stage Wilson's disease and is associated with excellent long-term survival, and the neurological manifestation of the disease can improve significantly after OLT.

Treatment of Wilson's disease.

The four anticopper drugs are discussed, then the use of these drugs in various clinical stages of Wilson's disease is discussed, and a section on follow-up, compliance, and prognosis is concluded.

Wilson's disease in patients presenting with liver disease: a diagnostic challenge.

A high index of suspicion: the key to an early diagnosis of Wilson's disease in childhood.

Wilson's disease in childhood is generally detected by maintaining a high suspicion of liver disease in patients who have no or nonspecific hepatic symptoms and drug therapy is effective and well tolerated, even in some cases of hepatic insufficiency.

Transplant livers in wilson's disease for hepatic, not neurologic, indications

  • G. BrewerF. Askari
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2000
The authors infer, and in some places outright claim, that orthoptic liver transplantation (OLT) is an effective, and perhaps advantageous, way to treat the neurological symptoms of Wilson’s disease and are apparently unaware that OLT is simply another anticopper therapy as far as neurological symptoms, other than hepatic encephalopathy, are concerned.
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