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[胆道闭锁——体征与症状、诊断、临床管理]

[Biliary atresia - signs and symptoms, diagnosis, clinical management].

作者信息

Orłowska Ewa, Czubkowski Piotr, Socha Piotr

机构信息

Klinika Gastroenterologii, Hepatologii, Zaburzeń Odżywiania I Pediatrii Instytut Pomnik Centrum Zdrowia Dziecka, Warszawa, Polska.

出版信息

Wiad Lek. 2017;70(1):112-117.

Abstract

Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.

摘要

胆道闭锁是一种慢性胆管病,可导致肝内和肝外胆管进行性纤维化。该病病因不明。胆道闭锁的基本治疗方法是手术干预,治疗效果取决于患儿年龄,若在出生后2个月内进行手术,效果最佳。因此,儿科医疗保健的主要作用是进行紧急鉴别诊断并迅速确定手术资格,进行最佳的术后管理,以及对持续性胆汁淤积患者尽早确定肝移植资格。作者讨论了胆道闭锁的临床表现、诊断和治疗。

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