Salupere V P, Remmel' Kh A, Uĭbo R M
Ter Arkh. 1987;59(2):112-4.
Altogether 31 cases of primary biliary cirrhosis (PBC) were diagnosed over the period of 1972-1985. All the patients were women (an average age of 51.6). PBC diagnosis was based on the syndrome of chronic intrahepatic cholestasia with prevailing skin itching, mitochondrial antibodies (in 26 patients), an increase in the IgM level (in 23 patients) and specific symptoms in liver biopsy specimens. As a result of late PBC diagnosis most patients were at III-IV morphological stages (17 cases). Immunosuppressive therapy (combination of azathioprine with prednisolone) of the PBC patients insignificantly improved liver parenchymal function though the patients' survival increased. Highly specific methods of PBC diagnosis and treatment necessitate the concentration of PBC patients in specialized centers fitted out with appropriate techniques.
1972年至1985年期间共诊断出31例原发性胆汁性肝硬化（PBC）患者。所有患者均为女性（平均年龄51.6岁）。PBC的诊断基于慢性肝内胆汁淤积综合征，伴有明显的皮肤瘙痒、线粒体抗体（26例患者）、IgM水平升高（23例患者）以及肝活检标本中的特定症状。由于PBC诊断较晚，大多数患者处于形态学III - IV期（17例）。PBC患者的免疫抑制治疗（硫唑嘌呤与泼尼松龙联合使用）虽能提高患者生存率，但对肝实质功能的改善不明显。PBC诊断和治疗的高度特异性方法需要将PBC患者集中在配备适当技术的专业中心。
