Kuiper Edith M M, den Ouden-Muller Jannie W, van Buuren Henk R
Erasmus MC, afd. Maag-, Darm- en Leverziekten, Rotterdam, The Netherlands.
Ned Tijdschr Geneeskd. 2009;153:A483.
In the Netherlands there are probably several thousands of patients with primary biliary cirrhosis (PBC), a slowly progressive liver disease mainly affecting middle-aged women. PBC has characteristics of an autoimmune disease but its precise aetiology remains unknown. Fatigue and pruritus are the main symptoms but patients may also be asymptomatic. The diagnosis can be established through the presence of cholestatic liver test abnormalities, antimitochondrial antibodies and diagnostic or compatible findings upon liver biopsy. Currently most patients are diagnosed with early disease. When treated with ursodeoxycholic acid these patients have a normal prognosis.
在荷兰，可能有数千名原发性胆汁性肝硬化（PBC）患者，这是一种主要影响中年女性的缓慢进展性肝病。PBC具有自身免疫性疾病的特征，但其确切病因尚不清楚。疲劳和瘙痒是主要症状，但患者也可能无症状。通过胆汁淤积性肝功能检查异常、抗线粒体抗体以及肝活检的诊断性或相符性发现可确立诊断。目前，大多数患者被诊断为早期疾病。用熊去氧胆酸治疗时，这些患者预后正常。
