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先天性胆道闭锁肝组织病理与卡赛术后早期结局的关系。

Biliary atresia liver histopathological determinants of early post-Kasai outcome.

机构信息

National Children Hospital, Hanoi, Vietnam.

University of Rochester School of Medicine and Dentistry, Rochester, NY, Untied States.

出版信息

J Pediatr Surg. 2021 Jul;56(7):1169-1173. doi: 10.1016/j.jpedsurg.2021.03.039. Epub 2021 Mar 26.

DOI:10.1016/j.jpedsurg.2021.03.039
PMID:33838902
Abstract

BACKGROUND

A retrospective chart review of liver histologies in Kasai biliary atresia BA patients operated 1/2017- 7/2019 at our institution was conducted to identify histologic prognostic factors for biliary outcome.

METHODS

Patients with wedge liver biopsies and portal plate biopsies (n = 85) were categorized into unfavorable and favorable outcome, based on a 3-month serum total bilirubin level of <34 μM or mortality. Hepatocellular histologies, presence of ductal plate malformation (DPM) and of large bile duct of ≥ 150 μm diameter size at the portal plate were evaluated.

RESULTS

Total Bilirubin levels> 34 μM correlates with worse 1-year survival. Age at surgery, histologic fibrosis or inflammation does not predict outcome. Potential adverse predictors are severe hepatocellular swelling, severe cholestasis, presence of DPM (n = 24), and portal plate bile duct size < 150 µm (n = 28). In multivariate analyses adjusting for age at Kasai and postop cholangitis, bile duct size and severe hepatocellular swelling remain independent histologic prognosticators (OR 3.25, p = 0.039 and OR 3.26, p = 0.006 respectively), but not DPM.

CONCLUSION

Advanced histologic findings of portal plate bile duct size of <150 µm and severe hepatocellular damage predict poor post-Kasai jaundice clearance and short-term survival outcome, irrespective of Kasai timing.

LEVEL OF EVIDENCE

Level III.

摘要

背景

对本机构于 2017 年 1 月至 2019 年 7 月间行手术治疗的胆道闭锁(BA)患儿的肝组织学进行回顾性图表分析,旨在确定与胆道结局相关的组织学预后因素。

方法

根据术后 3 个月血清总胆红素水平<34μM 或死亡率,将接受楔形肝活检和门脉板活检的患儿(n=85)分为不良结局和良好结局组。评估肝细胞组织学、胆管板畸形(DPM)的存在以及门脉板内胆管直径≥150μm 的大胆管的存在。

结果

总胆红素水平>34μM 与 1 年生存率较差相关。手术时年龄、组织学纤维化或炎症程度不能预测结局。潜在的不利预测因素是严重的肝细胞肿胀、严重的胆汁淤积、DPM 的存在(n=24)和胆管直径<150μm(n=28)。在调整了胆道造口术时年龄和术后胆管炎的多变量分析中,胆管直径和严重的肝细胞肿胀仍然是独立的组织学预后因素(OR 3.25,p=0.039 和 OR 3.26,p=0.006),而 DPM 则不然。

结论

无论胆道造口术时机如何,门脉板胆管直径<150μm 和严重的肝细胞损伤的晚期组织学表现预测了术后黄疸消退不良和短期生存结局不佳。

证据等级

III 级。

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