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儿童自身免疫性肝炎

Autoimmune Hepatitis in Children.

作者信息

Pathak Saumya, Kamat Deepak

出版信息

Pediatr Ann. 2018 Feb 1;47(2):e81-e86. doi: 10.3928/19382359-20180126-01.

Abstract

Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease. The diagnosis of AIH is made by a combination of clinical manifestations, laboratory evaluation, histopathology, and the exclusion of other more common liver diseases. The best outcome for AIH is dependent on early diagnosis as well as early initiation of immunosuppressant therapy. [Pediatr Ann. 2018;47(2):e81-e86.].

摘要

自身免疫性肝炎(AIH)是一种免疫介导的炎症性肝病。儿童AIH的临床表现高度多变。它可以急性、慢性或隐匿性发病。AIH主要有两种类型——1型和2型,通过特定自身抗体的存在进行区分和定义。AIH若不治疗最终会发展为肝硬化,即使治疗偶尔也会如此。所有出现急性、迁延性或严重肝病体征的儿童都必须怀疑并排除AIH。AIH的诊断是通过临床表现、实验室评估、组织病理学以及排除其他更常见的肝病综合做出的。AIH的最佳治疗效果取决于早期诊断以及尽早开始免疫抑制治疗。[《儿科年鉴》。2018年;47(2):e81 - e86。]

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