Wilson's disease with liver copper in normal concentration
Odièvre, M.; Benard, J.M.; Debray, H.; Neveu, Y.; Leleu, C.; Yvart, J.; Labrune, P.; Archambaud, F.; Bedossa, P.; Le Stradic, C.
Archives Francaises de Pediatrie 45(8): 565-567
1988
ISSN/ISBN: 0003-9764 PMID: 3214251 Document Number: 323924
The cases of 2 children with Wilson's disease revealed by persistent hypertransaminasemia are reported. Blood ceruloplasmin concentration was low but the liver content of copper was lower than usually seen in presymptomatic forms of the disease. The apparently low liver copper concentration could be explained by an unusually important steatosis.
Document emailed within 1 workday
Related Documents
Lee, H.H.; Hill, G.M.; Sikha, V.K.; Brewer, G.J.; Prasad, A.S.; Owyang, C. 1990: Pancreaticobiliary secretion of zinc and copper in normal persons and patients with Wilson's disease Journal of Laboratory and Clinical Medicine 116(3): 283-288Wawschinek, O.; Beyer, W. 1982: Determination of copper in liver puncture samples in the diagnosis of Wilson's disease Journal of Clinical Chemistry and Clinical Biochemistry 20(12): 929-930
Diaz, J.; Acosta, F.; Cañizares, F.; Bueno, F.S.; Tornel, P.L.; Tovar, I.; Contreras, R.F.; Marquez, M.; Martinez, P.; Parrilla, P. 1995: Does orthotopic liver transplantation normalize copper metabolism in patients with Wilson's disease? Transplantation Proceedings 27(4): 2306
Marecek, Z.; Heyrovský, A.; Volek, V. 1975: The effect of long term treatment with penicillamine on the copper content in the liver in patients with Wilson's disease Acta Hepato-Gastroenterologica 22(5): 292-296
Lee, D.Y.; Brewer, G.J.; Wang, Y.X. 1989: Treatment of Wilson's disease with zinc. VII. Protection of the liver from copper toxicity by zinc-induced metallothionein in a rat model Journal of Laboratory and Clinical Medicine 114(6): 639-645
Kobayashi, S.; Ochiai, T.; Hori, S.; Suzuki, T.; Shimizu, T.; Gunji, Y.; Shimada, H.; Yamamoto, S.; Ogawa, A.; Kohno, Y.; Sunaga, M.; Shimazu, M.; Tanaka, K. 2001: Copper metabolism after living donor liver transplantation for hepatic failure of Wilson's disease from a gene mutated donor Hepato-Gastroenterology 48(41): 1259-1261
McQuaid, A.; Lamand, M.; Mason, J. 1992: The interactions of penicillamine with copper in vivo and the effect on hepatic metallothionein levels and copper/zinc distribution: the implications for Wilson's disease and arthritis therapy Journal of Laboratory and Clinical Medicine 119(6): 744-750
Degli Esposti, A.; Antener, I.; Ambrosioni, G.; Bonfiglioli, G. 1976: Wilson's disease. Summary of studies carried out in a family group with Wilson's disease. Significance of amino-acid balance in homo and heterozygotes for the detection of liver damage Minerva Pediatrica 28(34): 2063-2086
Stapelbroek, J.M.; Ploos van Amstel, J.K.; van Hattum, J.; van den Berg, L.H.; Klomp, L.W.J.; Houwen, R.H.J. 2003: From gene to disease; Wilson disease: copper storage due to mutations in ATP7B Nederlands Tijdschrift Voor Geneeskunde 147(13): 603-605
Guérin, J.M.; Raux, M.; Meresse, S.; Lamotte, M. 1982: Peritoneal dialysis for eliminating copper in patients with Wilson's disease La Semaine des Hopitaux: Organe Fonde Par l'Association d'Enseignement Medical des Hopitaux de Paris 58(10): 613-615
Pawlak, J. 1975: Copper metabolism disorders as an attempt at explaining analogous pathological mechanisms in Wilson's syndrome and severe cases of liver cirrhosis Wiadomosci Lekarskie 28(12): 1041-1044
Suzuki, K.T. 1995: Disordered copper metabolism in LEC rats, an animal model of Wilson disease: roles of metallothionein Research Communications in Molecular Pathology and Pharmacology 89(2): 221-240
Seidel, J.; Caca, K.; Schwab, S.G.; Berr, F.; Wildenauer, D.B.; Mentzel, H.J.; Horn, N.; Kauf, E. 2001: Disturbed copper transport in humans. Part 2: mutations of the ATP7B gene lead to Wilson disease (WD) Cellular and Molecular Biology 47 Online Pub: Ol149-Ol157
Kok, K.F.; Hoevenaars, B.; Waanders, E.; Drenth, J.P.H. 2008: Value of molecular analysis of Wilson's disease in the absence of tissue copper deposits: a novel ATP7B mutation in an adult patient Netherlands Journal of Medicine 66(8): 348-350
Samochowiec, E.; Paprotny, P.; Rzempołuch, J. 1979: Blood plasma copper concentration in women with normal pregnancy and in cases complicated by EPH gestoses Ginekologia Polska 50(1): 41-47
Yüce, A.; Koçak, N.; Ozen, H.; Gürakan, F. 1999: Wilson's disease patients with normal ceruloplasmin levels Turkish Journal of Pediatrics 41(1): 99-102
Yuzbasiyan-Gurkan, V.; Brewer, G.J.; Abrams, G.D.; Main, B.; Giacherio, D. 1989: Treatment of Wilson's disease with zinc. V. Changes in serum levels of lipase, amylase, and alkaline phosphatase in patients with Wilson's disease Journal of Laboratory and Clinical Medicine 114(5): 520-526
Dastych, M. 1999: Serum levels of zinc, copper and selenium in patients with Wilson's disease treated with zinc Vnitrni Lekarstvi 45(4): 217-219
Rahelić, D.; Kujundzić, M.; Romić, Z.; Brkić, K.; Petrovecki, M. 2006: Serum concentration of zinc, copper, manganese and magnesium in patients with liver cirrhosis Collegium Antropologicum 30(3): 523-528
Valmary, J.; Ricordel, I.; Mazière, B.; Vergeau, B.; Fléchaire, A.; Algayres, J.P.; Daly, J.P.; Laverdant, C. 1988: Liver pathology in Wilson's disease Annales de Gastroenterologie et d'Hepatologie 24(4): 197-203