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先天性胆道闭锁:一种始于宫内的疾病及其对治疗、诊断和发病机制的影响。

Biliary Atresia as a Disease Starting In Utero: Implications for Treatment, Diagnosis, and Pathogenesis.

机构信息

Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine and Texas Children's Hospital, Houston, TX.

出版信息

J Pediatr Gastroenterol Nutr. 2019 Oct;69(4):396-403. doi: 10.1097/MPG.0000000000002450.

Abstract

Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.

摘要

先天性胆道闭锁(BA)是小儿肝移植最常见的原因。BA 的多种表现、自然病史以及Kasai 门腔分流术的治疗已得到充分描述;然而,BA 相对于出生的起始时间尚未明确界定。在这篇综述中,我们讨论了实验室、影像学和临床数据,这些数据表明,即使不是所有形式的 BA 都可能在出生前开始。这种早期发病提示我们更早地提供治疗,并识别 BA 病因的可能因素。

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