Hepatolenticular degeneration

Hepatolenticular Degeneration (Wilson's Disease)

A R?eport of five cases, with Commentary WILSON'S disease is a heredo-familial disorder, the essential pathological elements of which consist in a cirrhotic state of the liver comb;ned with degenerative changes in the lenticular nuclei of the brain. The term "hepatolenticular degenera-tion" is therefore aptly precise, but affords little clue to the diversity of the clinical manifestations. A lo...

Wilson's disease; hepatolenticular degeneration.

The inclusion of a rare neurological disease in an issue of this journal devoted to disorders of the liver would seem to warrant some explanation for although cirrhosis of the liver is an integral part of the syndrome of Wilson's disease, it accounts for an insignificant proportion of all patients suffering from hepatic cirrhosis. However, although quantitatively unimportant, its significance r...

Abdominal ultrasonography in hepatolenticular degeneration. A study of 33 patients.

The ultrasonographic study of 33 patients with hepatolenticular degeneration showed the following main changes: disorders of the hepatic echotexture (29 cases), changes of the splenic dimensions (21), liver shrinkage (10), cholelithiasis (8), hepatomegaly and ascites (1). The disorders of liver echotexture exhibited different patterns, from slight to severe changes of the hepatic echogenicity, ...

Amino-aciduria and copper metabolism in hepatolenticular degeneration.

Hepatolenticular degeneration combined with primary antiphospholipid syndrome: a case report.

biochemical parameters were within the normal reference ranges. Thrombocytes were 77 ! 10 9 /l; neither thrombocyte aggregation nor schistocytes were found on peripheral blood smear examination. The autoantibodies against ds-DNA, Sm, Ro/ SS-A, La/SS-B and RNP antigens (ELISA) and ANA titer (1: 40 HEP-2) were negative. The antiphospholipid antibodies – IgG isotype (48 GPL) and IgM isotype (56 GP...