[Wilson's disease with liver copper in normal concentration].
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.
Odièvre
M
M
Services de Pédiatrie L'Hôpital A.-Béclère, Porte-de-Trivaux.
Benard
J M
JM
Debray
H
H
Neveu
Y
Y
Leleu
C
C
Yvart
J
J
Labrune
P
P
Archambaud
F
F
Bedossa
P
P
Le Stradic
C
C
D002648:Child; D003300:Copper / Q000032:analysis*; D006527:Hepatolenticular Degeneration / Q000201:enzymology / Q000378:metabolism*; D006801:Humans; D008099:Liver / Q000032:analysis*; D008297:Male; D000637:Transaminases / Q000097:blood
D003300:Copper; D000637:Transaminases
Maladie de Wilson avec concentration hépatique en cuivre normale.