Speiciene Danute, Irnius Algimantas, Leuschner Ulrich, Liakina Valentina, Semuchiniene Teresa, Barakauskiene Ausrine
Center of Hepatology, Gastroenterology, and Dietetics of Vilnius University, Vilnius, Lithuania.
Med Sci Monit. 2007 Jul;13(7):CR299-306.
The rising detection and considerable geographical variation of primary biliary cirrhosis (PBC) in some regions demand increased awareness of the disease. The aim was to analyze the clinical, biochemical, immunological, and histological criteria of PBC patients in Lithuania and evaluate the patterns of disease presentation and histological features.
MATERIAL/METHODS: One hundred thirty-one PBC patients were examined and followed in the Center of Hepatology, Gastroenterology, and Dietetics, Vilnius University Hospital. Their case records were evaluated in this retrospective record-review study.
Most of the patients were women (94.6%) older than 50 years with late stages of PBC. Men were significantly older and had a threefold shorter duration from disease presentation to diagnosis (4.0+/-0.4 vs. 1.4+/-0.4 years). 29.8% of patients had asymptomatic disease at presentation and at diagnosis, were older than the symptomatic ones, and presented with significantly lower prevalence of jaundice, skin signs, and lower alkaline phosphatase (ALP) activity, but higher frequency of sicca syndrome. Antimitochondrial antibody (AMA) positivity was found in 91.7%, bile duct lesions in all patients, while the frequency of histological signs of cholestasis (except copper accumulation) was lower. No significant differences in these parameters in asymptomatic and symptomatic patients were found.
Most PBC patients in Lithuania were at late histological stages, with a predominance of females older than 50 years and long duration from disease presentation to diagnosis. One third of these PBC patients initially had asymptomatic course, with some differences in clinical signs and their prevalence compared with initially symptomatic patients.
在某些地区，原发性胆汁性肝硬化（PBC）的检出率不断上升且存在显著的地域差异，这就需要提高对该疾病的认识。目的是分析立陶宛PBC患者的临床、生化、免疫和组织学标准，并评估疾病表现模式和组织学特征。
材料/方法：维尔纽斯大学医院肝病、胃肠病和饮食学中心对131例PBC患者进行了检查和随访。在这项回顾性病历审查研究中对他们的病历进行了评估。
大多数患者为50岁以上的女性（94.6%），处于PBC晚期。男性年龄明显更大，从疾病出现到诊断的时间缩短了三倍（4.0±0.4年对1.4±0.4年）。29.8%的患者在就诊和诊断时无症状，年龄比有症状的患者大，黄疸、皮肤症状的发生率明显较低，碱性磷酸酶（ALP）活性较低，但干燥综合征的发生率较高。抗线粒体抗体（AMA）阳性率为91.7%，所有患者均有胆管病变，而胆汁淤积的组织学征象（铜蓄积除外）发生率较低。无症状和有症状患者在这些参数上无显著差异。
立陶宛的大多数PBC患者处于组织学晚期，以50岁以上女性为主，从疾病出现到诊断的时间较长。这些PBC患者中有三分之一最初病程无症状，与最初有症状的患者相比，临床体征及其发生率存在一些差异。
