Löfgren J, Järnerot G, Danielsson D, Hemdal I
Scand J Gastroenterol. 1985 Jun;20(5):647-50. doi: 10.3109/00365528509089711.
The incidence and prevalence of antimitochondrial antibody-positive primary biliary cirrhosis (PBC) has been studied within a defined area in Sweden served by one hospital. During the period 1976-1983 the yearly incidence of PBC was 1.4/10(5) inhabitants, and on 31 December 1983 the prevalence was 12.8/10(5) inhabitants. The prevalence is the highest reported so far. At the time of diagnosis half of the patients were clinically asymptomatic. Two of the patients also had celiac disease with osteomalacia responding to a gluten-free diet. Gallstone disease occurred in 30% of the patients. Four patients died--two of liver-related complications, one of colonic carcinoma, and one of staphylococcal septicemia and endocarditis. One further patient, who is still alive, developed hypernephroma. Our results indicate that PBC is a fairly benign disease in most patients, with a slow progress during which they lead a fairly normal life.
在瑞典一家医院服务的特定区域内，对线粒体抗体阳性的原发性胆汁性肝硬化（PBC）的发病率和患病率进行了研究。在1976 - 1983年期间，PBC的年发病率为每10万居民中有1.4例，到1983年12月31日，患病率为每10万居民中有12.8例。这是迄今为止报告的最高患病率。在诊断时，一半的患者临床上无症状。其中两名患者还患有乳糜泻伴骨软化症，对无麸质饮食有反应。30%的患者患有胆结石病。有4名患者死亡，两名死于肝脏相关并发症，一名死于结肠癌，一名死于葡萄球菌败血症和心内膜炎。另有一名患者仍在世，患了肾上腺样瘤。我们的结果表明，PBC在大多数患者中是一种相当良性的疾病，进展缓慢，在此期间他们能过上相当正常的生活。
