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Titolo:
Wilson's disease - Copper unfettered
Autore:
Pfeil, SA; Lynn, DJ;
Indirizzi:
Ohioolumbus,niv, Coll Med & Publ Hlth, Dept Internal Med, Div Digest Dis, C Ohio State Univ Columbus OH USA 43210 ept Internal Med, Div Digest Dis, C Ohio State Univ, Coll Med & Publ Hlth, Dept Neurol, Columbus, OH 43210 USAOhio State Univ Columbus OH USA 43210 Dept Neurol, Columbus, OH 43210 USA
Titolo Testata:
JOURNAL OF CLINICAL GASTROENTEROLOGY
fascicolo: 1, volume: 29, anno: 1999,
pagine: 22 - 31
SICI:
0192-0790(199907)29:1<22:WD-CU>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
FULMINANT HEPATIC-FAILURE; CHRONIC ACTIVE HEPATITIS; KAYSER-FLEISCHER RINGS; EVANS CINNAMON RATS; LIVER-TRANSPLANTATION; PENICILLAMINE THERAPY; INITIAL THERAPY; GENE; DIAGNOSIS; MANIFESTATION;
Keywords:
Wilson's disease; cirrhosis; copper metabolism; hepatolenticular degeneration;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
78
Recensione:
Indirizzi per estratti:
Indirizzo: Pfeil, SA Ohio State Univ, Coll Med & Publ Hlth, Dept Internal Med, Div Digest Dis, 2 Ohio State Univ 2 N Doan Hall,410 W 10th Ave Columbus OH USA 43210
Citazione:
S.A. Pfeil e D.J. Lynn, "Wilson's disease - Copper unfettered", J CLIN GAST, 29(1), 1999, pp. 22-31

Abstract

Wilson's disease is a rare autosomal recessive inherited disorder of copper metabolism. Hepatic excretion of copper is impaired due to mutation of the gene for a copper-transporting adenosine triphosphatase, ATP7B. Copper accumulation in liver. brain, and other tissues may cause a wide spectrum of hepatic, neuropsychiatric. and other clinical manifestations. The diagnosismay be supported by measurement of serum ceruloplasmin, urinary copper excretion, and hepatic copper content as well as by detection of Kayser-Fleischer rings. Several treatments are available to increase urinary excretion and decrease intestinal absorption of copper.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/09/20 alle ore 00:19:23