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肝脏肿物患者及阿伯内西畸形患者的肝脏组织病理学

Liver histopathology in patients with hepatic masses and the Abernethy malformation.

作者信息

Lemoine Caroline, Nilsen Annika, Brandt Katherine, Mohammad Saeed, Melin-Aldana Hector, Superina Riccardo

机构信息

Division of Transplant Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, IL, USA.

Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, IL, USA.

出版信息

J Pediatr Surg. 2019 Feb;54(2):266-271. doi: 10.1016/j.jpedsurg.2018.10.083. Epub 2018 Nov 7.

Abstract

BACKGROUND/PURPOSE: The Abernethy malformation (AM) is a congenital venous malformation in which the splanchnic venous return bypasses the liver and drains directly into the systemic circulation. This deprives the liver of hepatotrophic growth factors and allows metabolic products of digestion to enter the systemic veins without the benefit of passing through the liver. The histologic features of liver biopsies in children with an AM were reviewed.

METHODS

A retrospective review of liver biopsies in patients with AM between 1997 and 2017 was performed. Patients were divided into two groups for comparison of histologic features: presence (M+) or absence (M-) of a coexistent liver mass on imaging. Biopsies were reviewed by a pediatric pathologist. Chi-square test was used for statistical analysis between groups. Significance was assigned to p values <0.05.

RESULTS

Eighteen liver biopsies were reviewed. Masses were present in only 6 patients who had a liver biopsy. Masses were observed with similar frequencies in either type of the Abernethy malformation (I or II). Nine of 12 M- patients and 3/6 M+ patients had the type I AM. Histologically, all patients were noted to have small or absent portal veins. Isolated capillaries were seen more frequently in patients with a known liver mass (p = 0.045), while crowding of portal tracts was more commonly seen in patients without a liver mass (p = 0.019).

CONCLUSION

Liver biopsies in patients with AM demonstrate abnormal vascular and parenchymal histologic features. Livers with coexistent masses were more commonly found to have features suggesting an increased dependence on arterial blood supply.

LEVEL OF EVIDENCE

III.

摘要

背景/目的:阿伯内西畸形(AM)是一种先天性静脉畸形,其中内脏静脉回流绕过肝脏,直接引流到体循环中。这使肝脏无法获得肝营养生长因子,并使消化代谢产物未经肝脏处理就进入体循环静脉。对患有AM的儿童肝脏活检的组织学特征进行了回顾。

方法

对1997年至2017年间患有AM的患者的肝脏活检进行回顾性研究。根据影像学上是否存在共存肝脏肿块(M+)或不存在(M-)将患者分为两组,以比较组织学特征。活检由一名儿科病理学家进行评估。采用卡方检验进行组间统计学分析。p值<0.05具有统计学意义。

结果

共回顾了18例肝脏活检病例。仅6例进行肝脏活检的患者存在肿块。在两种类型的阿伯内西畸形(I型或II型)中观察到肿块的频率相似。12例M-患者中有9例和6例M+患者中有3例为I型AM。组织学上,所有患者均可见门静脉细小或缺如。孤立的毛细血管在已知有肝脏肿块的患者中更常见(p = 0.045),而门管区拥挤在无肝脏肿块的患者中更常见(p = 0.019)。

结论

AM患者的肝脏活检显示血管和实质组织学特征异常。共存肿块的肝脏更常表现出提示对动脉血供依赖性增加的特征。

证据级别

III级。

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