Brailski Kh, Damianov B
Vutr Boles. 1983;22(5):27-37.
The results from the complex study on 26 patients with primary biliary cirrhosis (PBC), 20 females and 6 males, an average age of 46 years, are reported. The most frequent symptoms of PBC are itching, jaundice, hepatosplenomegaly; from the laboratory tests--most characteristic is the increase of serum 5'-nucleotidase, AP, LAP, gamma GTP, GOT, cholesterol, cholic acid and antimitochondrial antibodies and IgM (AP, 5'-nucleotidase and antimitochondrial antibodies, being most significant in making the early diagnosis). The laboratory results in PBC are compared with those of the chronic active hepatitis, cirrhosis of the liver, liver cancer, extrahepatic cholestasis, with outlining the characteristic differences, depending on the diagnosis. The diagnostic advantages of the various methods are discussed (mainly laparoscopy and liver biopsy) and the histologic and electron microscopic changes of percutaneous transhepatic cholangiography, via echography--81 per cent, laparoscopy--73 per cent, scintigraphy--61.53 per cent and liver biopsy--50 per cent. The results from the treatment with cholestrimine, corticosteroids and azathioprine and surgical treatment, observing a temporary improvement and progressing of PBC, are reported. With the follow-up care of 20 patients, it was established, that 9 had died 5 years, on the average, after making the diagnosis, 11 survived after the 5 years and they are still followed up. The longest survival was reported in two females--11 and 15 years after the onset of PBC.
报告了对26例原发性胆汁性肝硬化(PBC)患者(20例女性,6例男性,平均年龄46岁)进行综合研究的结果。PBC最常见的症状是瘙痒、黄疸、肝脾肿大;实验室检查方面,最具特征性的是血清5'-核苷酸酶、碱性磷酸酶(AP)、亮氨酸氨基肽酶(LAP)、γ-谷氨酰转肽酶(γGTP)、谷草转氨酶(GOT)、胆固醇、胆酸以及抗线粒体抗体和IgM升高(AP、5'-核苷酸酶和抗线粒体抗体在早期诊断中最为重要)。将PBC的实验室检查结果与慢性活动性肝炎、肝硬化、肝癌、肝外胆汁淤积的结果进行比较,根据诊断概述其特征性差异。讨论了各种诊断方法(主要是腹腔镜检查和肝活检)的优势,以及经皮肝穿刺胆管造影、超声检查、腹腔镜检查、闪烁扫描和肝活检的组织学和电子显微镜变化,其中超声检查诊断准确率为81%,腹腔镜检查为73%,闪烁扫描为61.53%,肝活检为50%。报告了使用消胆胺、皮质类固醇和硫唑嘌呤治疗以及手术治疗的结果,观察到PBC有暂时改善和进展情况。对20例患者进行随访后发现,平均诊断后5年有9例死亡,11例存活且仍在接受随访。报告中存活时间最长的是两名女性,分别在PBC发病后11年和15年。