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Revista Médica de Costa Rica y Centroamérica

Colegio de Medicos y Cirujanos República de Costa Rica
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2009, Number 590

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Rev Med Cos Cen 2009; 66 (590)

Enfermedad de Wilson

Duarte TT
Full text How to cite this article

Language: Spanish
References: 8
Page: 373-375
PDF size: 202.06 Kb.


Key words:

chelation, atp asa, penicillamine.

ABSTRACT

Wilson disease is an autosomal recessive disorder of copper metabolism. The Wilson disease protein is a copper transporting P-type ATPasa, ATP7B, whose malfunction results in the toxic acumulation of copper in the liver and brain, causing the hepatic and or neurological symptoms accompanying this disease. Chalation and zinc therapy are two treatments used for Wilson disease.


REFERENCES

  1. Huster D, Finegold M, Morgan C. Consequences of copper accumulation in the livers of the ATP7B. American Journal of Pathology.2006; 168: 423-34.

  2. Marchena M, Rivera A, Castillo M. Anemia hemolítica por déficit de piruvato cinasa como manifestación inicial de enfermedad de Wilson. Revista Médica de Costa Rica y Centroamérica. 2005; 571:79-81.

  3. Mounif El-Youssef. Wilson disease. Mayo Clinic Proc. 2003; 78: 1126-36.

  4. Nazer Hisham, Ede RJ, Mowat AP. Wilson`s disease: clinical presentation and use of prognostic index. Gut .1986; 27: 1377-81.

  5. Prudencio R, Betancourt M, Larraín F. Enfermedad de Wilson. Revista chilena de pediatría. 2000; 83: 422-24.

  6. Roberts E, Shilsky M. A practice guide on Wilson disease. Hapatology. 2003; 37: 1475-92.

  7. Shaefer M, Schellenberg M, Merle Uta et al. Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controls. BMC Gastroenterology. 2008; 29: 1-9.

  8. Sullivan Ch, Chopdar A, Shun–Shin G. Dense Kayser Fleischer ring in asintomatic Wilson disease. Br J Ophthalmol. 2002; 86: 114-123.




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Rev Med Cos Cen. 2009;66