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具有非典型变异型的小儿急性病毒性肝炎:临床困境与自然病史

Pediatric acute viral hepatitis with atypical variants: Clinical dilemmas and natural history.

作者信息

Sarma Moinak Sen, Ravindranath Aathira

机构信息

Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India.

Division of Pediatric Gastroenterology, Apollo BGS Hospitals, Mysuru 570023, Karnataka, India.

出版信息

World J Hepatol. 2022 May 27;14(5):944-955. doi: 10.4254/wjh.v14.i5.944.

DOI:10.4254/wjh.v14.i5.944
PMID:35721282
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9157701/
Abstract

Classical acute viral hepatitis (AVH) has an uncomplicated outcome. Acute liver failure has a grave prognosis. Atypical manifestations of AVH are a group of disorders that causes significant morbidity and dilemmas in children. These include prolonged cholestasis, relapsing hepatitis, ascitic form of AVH, late-onset hepatic failure (LOHF), intravascular hemolysis, and provoking an autoimmune trigger leading to autoimmune hepatitis. These entities cause significant liver dysfunction or worsening and are often difficult to differentiate from chronic liver disease (CLD). Ascitic form of AVH, LOHF, decompensated CLD and acute-on-chronic liver failure have significant overlapping features that need to be carefully dissected out. In many cases, only on long-term follow-up, these clinical entities can be separately identified. Intravascular hemolysis is usually caused by associated glucose-6-phosphate dehydrogenase deficiency. Rarely CLD such as Wilson disease and autoimmune hepatitis can also present with hemolysis in the initial presentation, which can mimic AVH with hemolysis. Identifying deviations from typical manifestations aid in avoiding unnecessary investigations, allowing focused therapy and alleviating anxiety.

摘要

典型的急性病毒性肝炎(AVH)预后良好。急性肝衰竭预后严重。AVH的非典型表现是一组在儿童中会导致严重发病和困境的病症。这些包括长期胆汁淤积、复发性肝炎、AVH的腹水型、迟发性肝衰竭(LOHF)、血管内溶血以及引发自身免疫触发导致自身免疫性肝炎。这些病症会导致严重的肝功能障碍或恶化,并且常常难以与慢性肝病(CLD)区分开来。AVH的腹水型、LOHF、失代偿性CLD和慢加急性肝衰竭有显著的重叠特征,需要仔细辨别。在许多情况下,只有通过长期随访才能分别识别这些临床病症。血管内溶血通常由相关的葡萄糖-6-磷酸脱氢酶缺乏引起。罕见的CLD如威尔逊病和自身免疫性肝炎在初始表现时也可能出现溶血,这可能会模仿伴有溶血的AVH。识别与典型表现的偏差有助于避免不必要的检查,进行有针对性的治疗并减轻焦虑。

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