Wilson's disease in Albania.

There are 40 patients with Wilson's disease diagnosed in Albania; 24 were males and 16 females with an average age 18.8 +/- 8.47 years. The incidence rate was 1:75.000 and prevalence rate 1:148.000. According to clinical data, the Kayser-Fleischer ring was found in 75% of the cases, the neurologic in 60% and mental disturbances in 27.5%. The liver was damaged in all the cases of acute and chronic hepatitis or liver cirrhosis. In 35% signs of hypersplenism were seen. Comparing these data with a control group of 60 alcoholic or viral cirrhosis with hypersplenism, it was found that hypersplenism is encountered in younger ages (p < 0.0001). The platelets were lower in WD than in the control group (p < 0.05), but leucocytes were lower in the control group (p < 0.001). Portal hypertension was found in 42.5% of the cases. Six of these (35.3%) were complicated by gastrointestinal hemorrhage. In two twins the disease was accompanied with another congenital anomaly: genu valgum bilateralis. Two other patients had hypertrophy of the parotis gland, six patients had signs of acute haemolysis, accompanied by nonspherocytic anemia, Coombs negative.