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先天性胆道闭锁的时相组织病理学变化:快速纤维化进展的视角。

Temporal histopathological changes in biliary atresia: A perspective for rapid fibrosis progression.

机构信息

Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt.

Department of Pathology, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt.

出版信息

Ann Hepatol. 2021 Mar-Apr;21:100263. doi: 10.1016/j.aohep.2020.09.007. Epub 2020 Sep 29.

Abstract

INTRODUCTION AND OBJECTIVES

Biliary atresia (BA) is characterized by rapid progression of fibrosis with no definite causes. Histopathological findings have been extensively described, but very few studies have assessed temporal changes in BA. Understanding these short-term changes and their relationship with fibrosis progression could have an impact on ameliorating rapid fibrogenesis. We aimed to study the relationship between temporal histopathological changes and fibrosis progression in BA within a short time interval.

PATIENTS AND METHODS

Forty-nine infants with BA who underwent Kasai portoenterostomy, a diagnostic liver biopsy, and an intraoperative liver biopsy were recruited. Histopathological characteristics of the two biopsies were examined. Temporal histopathological changes were assessed by comparing the two types of biopsies. Correlation of temporal changes in fibrosis with age, interval between biopsies, laboratory profiles, and temporal histopathological changes were studied.

RESULTS

In the univariate analysis, bile ductular proliferation (BDP), portal infiltrate, giant cells, hepatocellular swelling, and fibrosis showed significant temporal changes within a short interval (5-31 days). BDP and fibrosis showed the most frequent increase in their grades (32/49 and 31/49 cases, respectively). In the multivariate analysis, BDP was the only independent pathological feature showing a significant temporal increase (p = 0.021, 95% confidence interval: 1.249-16.017). Fibrosis progression was correlated with temporal changes in BDP (r = 0.456, p = 0.001), but not with age (p = 0.283) or the interval between the biopsies (p = 0.309).

CONCLUSIONS

Fibrosis in BA progresses rapidly and is significantly correlated with BDP. Assessment of targeting BDP as an adjuvant medical therapy is recommended.

摘要

简介与目的

胆道闭锁(BA)的特征是纤维化迅速进展,而无明确病因。其组织病理学表现已有广泛描述,但仅有少数研究评估了 BA 的短期变化。了解这些短期变化及其与纤维化进展的关系可能对改善快速纤维化具有重要意义。我们旨在研究 BA 在短时间内的组织病理学变化与纤维化进展之间的关系。

患者与方法

我们招募了 49 名接受葛西(Kasai)胆肠吻合术的 BA 婴儿,对他们进行了诊断性肝活检和术中肝活检。检查了两次活检的组织病理学特征。通过比较两种活检,评估了时间性组织病理学变化。研究了纤维化的时间性变化与年龄、活检间隔、实验室特征和时间性组织病理学变化之间的相关性。

结果

在单变量分析中,胆管增生(BDP)、门脉浸润、巨细胞、肝细胞肿胀和纤维化在短时间内(5-31 天)显示出显著的时间性变化。BDP 和纤维化的分级增加最为频繁(分别为 32/49 和 31/49 例)。在多变量分析中,BDP 是唯一显示出显著时间性增加的独立病理特征(p=0.021,95%置信区间:1.249-16.017)。纤维化进展与 BDP 的时间性变化相关(r=0.456,p=0.001),但与年龄(p=0.283)或活检间隔(p=0.309)无关。

结论

BA 中的纤维化进展迅速,与 BDP 显著相关。建议评估针对 BDP 的辅助药物治疗。

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