背景:Kupffer细胞(KCs)的细胞质中的透明球(HG)已被评估为自身免疫性肝炎(AIH)的典型特征。这项研究旨在确定Kupffer细胞透明球(KCHGs)在诊断AIH与儿科慢性肝病(PCLD)的其他原因。
方法:这项回顾性研究招募了124名儿童;58名患有AIH,50例慢性丙型肝炎病毒(HCV)感染,和16患有威尔逊病(WD)。两名病理学家检索了肝活检的石蜡块,并准备了新的切割切片用于高碘酸-席夫碱(PAS-D)染色。他们在开始治疗前独立检查肝活检。两名儿科医生检查了人口统计的医疗记录,临床,实验室,和血清学发现。
结果:女性占研究儿童的48.6%,中位年龄为5.8(4.9)岁。病理学家确定KCHGs占67.24%,12.5%,AIH的6.0%,WD,和HCV分别影响儿童,P<0.001。与血清阴性AIH患者相比,血清阳性的KCHGs比例明显更高(77.5%vs.50.0%),(P<0.05)。在多变量分析中,KCHGs和延长的凝血酶原时间是区分AIH和其他研究的PCLD的唯一重要预测因子。如果看到KCHGs,AIH的赔率增加了68倍。在患有AIH的儿童中,KCHGs的存在与较高的直接胆红素中位数水平相关2.2(1.3)与1.2(2.2)、免疫球蛋白G3.2(1.9)与2.0(1.7),(P<0.05),但不是组织病理学发现或肝纤维化和活动。
结论:KCHGs是可以区分AIH和其他PCLD的关键指标,在血清阳性和血清阴性AIH之间。
BACKGROUND: Hyaline globules (HGs) in the cytoplasm of Kupffer cells (KCs) have been appraised for being a typical feature of autoimmune hepatitis (AIH). This study aimed to determine how useful Kupffer cell hyaline globules (KCHGs) are in diagnosing AIH vs. other causes of pediatric chronic liver diseases (PCLDs).
METHODS: This retrospective study recruited 124 children; 58 with AIH, 50 with chronic hepatitis C virus (HCV) infection, and 16 with Wilson\'s disease (WD). Two pathologists retrieved paraffin blocks of liver biopsies and prepared new cut sections for Periodic acid-Schiff-Diastase (PAS-D) stain. They independently examined liver biopsies before starting treatment. Two pediatricians reviewed medical records for demographic, clinical, laboratory, and serological findings.
RESULTS: Females represented 48.6% of the studied children with a median age of 5.8 (4.9) years. Pathologists identified KCHGs in 67.24%, 12.5%, and 6.0% of AIH, WD, and HCV affected children respectively, P < 0.001. A significantly higher proportion of seropositive than seronegative AIH patients had KCHGs (77.5% vs. 50.0%), (P < 0.05). In multivariate analysis, KCHGs and prolonged prothrombin time were the only significant predictors that differentiate between AIH and the other studied PCLDs. The odds ratio of having AIH increased 68 times if KCHGs were seen. Among children with AIH, the presence of KCHGs was associated with higher median levels of direct bilirubin 2.2 (1.3) vs. 1.2 (2.2), and immunoglobulin G 3.2 (1.9) vs. 2.0 (1.7), (P < 0.05), but not to histopathological findings or hepatic fibrosis and activity.
CONCLUSIONS: KCHGs are key indicators that can differentiate between AIH and other PCLDs, and between seropositive and seronegative AIH.