原发性胆汁性肝硬化153例分析

目的　探讨原发性胆汁性肝硬化 (PBC)的临床、免疫学及病理学特点。方法　观察 15 3例PBC的临床表现 ,并分析有关实验室检查资料 ,35例行肝组织学检查。结果　有典型的皮肤瘙痒者占 43 79% ,肝肿大占 46 41% ,脾肿大者占 39 87% ,有皮肤、巩膜黄染者占 84 30 % ,随病情进展血清胆红素 (SB)逐步上升 ,而胆碱酯酶 (CHE)逐步下降 (P <0 0 1) ;丙氨酸转氨酶 (ALT)、人血白蛋白在病程晚期明显下降 (P <0 0 5 ) ,ALT、CHE与SB呈酶 胆分离现象 ;天冬氨酸转氨酶 (AST)在病情晚期反而上升 (P <0 0 5 ) ;碱性磷酸酶 (ALP)、γ 谷氨酰转移酶 (GGT)在病情早期即明显升高 ,随病情进展到中期ALP明显下降 (P <0 0 5 ) ,到晚期GGT明显下降 (P <0 0 1)。抗线粒体抗体 (AMA)阳性者占83 7% ,抗线粒体抗体M2亚型 (M2 )阳性者占 6 9 3%。 35例经肝组织学检查确诊病例中 ,10例为AMA阳性者 ,2 5例为血清学检测全阴者。结论　 5 0 %以上的PBC无典型临床症状 ;80 %以上的PBC可以根据临床表现结合血清自身抗体检测确诊 ;部分依临床资料不能确诊病例 ,应行肝脏组织学检查确诊。

Primary biliary cirrhosis: an analysis of 153 cases

OBJECTIVE: To study the clinical, immunological and pathological features of primary biliary cirrhosis (PBC). METHODS: Clinical and laboratory data of 153 patients with PBC treated in our hospital were retrospectively analyzed. Of these patients, 35 underwent hepatic biopsy. RESULTS: Typical manifestations such as itch of skin and hepatosplenomegaly etc. were found in only 39.2% to 46.4% of the patients and jaundice was seen in 84.3%. The serum level of SB was gradually increased while that of CHE decreased along with progression of PBC (P < 0.01). The level of serum Albumin and ALT was significantly lower in late stage than in the early stages (P < 0.05). ALT and CHE were not correlated with SB. AST level was markedly higher in the late stage than in the early stage (P < 0.05). The levels of ALP and GGT rose at the early stage but remarkably decreased at the middle stage (ALP, P < 0.05) and at the late stage (GGT, P < 0.01). The positive rate of AMA was 83.7% and that of M2 69.3%. As for the 35 patients receiving hepatic biopsy, 10 were AMA positive and the other 25 AMA negative. CONCLUSION: More than half of the patients with PBC do not exhibit typical clinical symptoms and over 80% of the cases of PBC can be diagnosed by investigation of clinical features and detection of AMA and M2. For some cases that can not be definitely diagnosed by observation of clinical features, the final diagnosis should be determined by hepatic biopsy.