Clinicopathological features of drug-induced liver injury or primary biliary cholangitis with vanishing bile duct syndrome
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摘要:
目的比较药物性肝损伤(DILI)与原发性胆汁性胆管炎(PBC)引起的胆管消失综合征(VBDS)患者的病理学特征,总结临床鉴别特点。方法回顾性选取2003年1月-2018年1月于石家庄市第五医院经肝穿刺病理明确诊断合并VBDS的DILI患者(D-VBDS组) 42例、PBC患者(P-VBDS组) 43例,收集入选患者的一般资料、临床症状体征、血清学指标等数据,观察病理形态学特征。计量资料2组间比较采用Mann-Whitney U秩和检验;计数资料2组间比较采用X2检验。结果 2组患者均以40~50岁女性多见。D-VBDS组发生皮肤巩膜黄染较P-VBDS组多见,差异有统计学意义(X2=5. 683,P=0. 017)。肝生化各指标高峰值比较,D-VBDS组TBil(Z=-2. 020,P=0. 043)、DBil (Z=-2. 910,P=0. 004)明显高于P-VBDS组。病理形态学分析显示,P-VBDS组在汇管区炎症、汇管区纤维化、汇管区肉芽肿病理形态学评分高于D-VBDS组,差异均有统计学意义(Z值分别为-3. 136、-2. 710、-...
Abstract:Objective To investigate the clinical features of patients with drug-induced liver injury ( DILI) and vanishing bile duct syndrome ( VBDS) and patients with primary biliary cholangitis ( PBC) and VBDS. Methods A retrospective analysis was performed for the clinical data of 42 DILI patients and 43 PBC patients who visited Shijiazhuang Fifth Hospital from January 2003 to January 2018 were diagnosed with VBDS by liver biopsy, including general information, clinical symptoms and signs, and serological markers, and pathomorphological features were observed. The Mann-Whitney U rank sum test was used for comparison between two groups; the chi-square test was used for comparison of categorical data between groups. Results Most of the patients in the two groups were female patients aged 40-50 years. The D-VBDS group had a significantly higher incidence rate of yellow staining of skin and sclera than the P-VBDS group ( X2=5. 683, P = 0. 017) . Comparison of the peak values of liver biochemical indices showed that compared with the P-VBDS group, the D-VBDS group had significantly higher total bilirubin ( Z =-2. 020, P = 0. 043) and direct bilirubin ( Z =-2. 910, P = 0. 004) . According to the results of pathomorphological analysis and morphological scores of the portal area, the P-VBDS group had significantly higher morphological scores of portal inflammation, fibrosis, and granuloma than the D-VBDS group ( Z =-3. 136, -2. 710, and-2. 913, P =0. 002, 0. 007, and 0. 005) . The morphological scores of the hepatic lobules showed that compared with the P-VBDS group, the D-VBDS group had significantly higher morphological scores of intralobular inflammation, bile thrombus in bile capillary, and activated phagocytes ( Z =-3. 255, -2. 455, and-4. 398, P = 0. 001, 0. 014, and < 0. 001) . Conclusion Pathomorphological features of the liver are objective and accurate indices for the differentiation between DILI with VBDS and PBS with VBDS and have great significance in making a definite diagnosis, understanding the degree of injury, and predicting prognosis.
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