Adhami E J, Cullufi P
University Hospital Center of Tirana, Albania.
Panminerva Med. 1995 Mar;37(1):18-21.
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.
在阿尔巴尼亚诊断出40例威尔逊氏病患者；其中男性24例，女性16例，平均年龄18.8 +/- 8.47岁。发病率为1:75,000，患病率为1:148,000。根据临床数据，75%的病例发现有凯泽-弗莱舍尔环，60%有神经系统症状，27.5%有精神障碍。所有急性和慢性肝炎或肝硬化病例的肝脏均有损伤。35%的病例出现脾功能亢进体征。将这些数据与60例伴有脾功能亢进的酒精性或病毒性肝硬化对照组进行比较，发现脾功能亢进在较年轻的年龄段出现（p < 0.0001）。威尔逊氏病患者的血小板低于对照组（p < 0.05），但对照组的白细胞较低（p < 0.001）。42.5%的病例发现有门静脉高压。其中6例（35.3%）并发胃肠道出血。在一对双胞胎中，该病伴有另一种先天性异常：双侧膝外翻。另外两名患者有腮腺肥大，6名患者有急性溶血体征，伴有非球形细胞性贫血，库姆斯试验阴性。
