Wilson s disease, called also hepatolenticular degeneration, is a genetic neurodegenerative defect in the metabolism of copper, characterized by a broad clinical spectrum, thus requiring high. Wilson s disease is a genetic disorder in which excess copper builds up in the body. Progressive hepatolenticular degeneration, or wilsons disease, is a genetic disorder of copper metabolism. Wilson, nacio en nueva jersey usa en 1878 y murio en 1937. Brainrelated symptoms include tremors, muscle stiffness, trouble speaking, personality changes, anxiety and seeing or. Wilsons disease is a genetic disorder in which excess copper builds up in the body. Progressive hepatolenticular degeneration, or wilson s disease, is a genetic disorder of copper metabolism. The electroencephalogram in hepatolenticular degeneration. Files are available under licenses specified on their description page. The effect of bal 2,3dimercaptopropanol on hepatolenticular degeneration wilson s disease.
Palabras clave degeneracion hepatolenticular adolescente ceruloplasmina. It is caused by defects in the atp7b gene encoding coppertransporting atpase 2 ec 3. He was a 26 yearsold male who began to show psychological symptoms, and later developed neurological. All structured data from the file and property namespaces is available under the creative commons cc0 license. Hepatolenticular degeneration wilson disease is a rare inherited disease that usually affects the liver, but may present in different forms and. Esta enfermedad fue descrita por primera vez en 1912 por kinnear wilson 1. Symptoms are typically related to the brain and liver. A rare autosomal recessive disease characterized by the deposition of copper in the brain.
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