Changes of Some Clotting Factors in Hepatolenticular Degeneration (Wilson’s Disease)

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Abstract
Ten patients suffering from hepatolenticular degeneration (Wilson''s disease) were examined for changes in blood coagulation factors. Five could be considered as the hepatic; 5, the neurologic form. Cases with prevailing hepatic symptomatology showed remarkable lengthening of prothrombin time, decrease of factors II, V, VII, X and antithrombin HI activity, more or less marked hypo-fibrinogenemia and thrombocytopenia. These changes were more pronounced here than in decompensated liver cirrhosis, the difference being mostly of statistical significance. In the neurologic group the activity of blood clotting factors mentioned was normal or only slightly lowered. Hemorrhage occurred in 3 cases of the hepatic form. Penicillamine treatment resulted in improvement of clotting tests in proportion to the clinical and laboratory restoration of hepatic function.