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先天性幽门闭锁——三例报告并文献复习

Pyloric atresia--three cases and review of literature.

作者信息

Parelkar Sandesh V, Kapadnis Satish P, Sanghvi Beejal V, Joshi Prashant B, Mundada Dinesh, Shetty Shishira, Oak Sanjay N

机构信息

Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra, India.

出版信息

Afr J Paediatr Surg. 2014 Oct-Dec;11(4):362-5. doi: 10.4103/0189-6725.143178.

Abstract

Pyloric atresia (PA) is a rare congenital anomaly that constitutes approximately 1% of all intestinal atresias, and its incidence is approximately 1 in 100,000 live births. PA may occur as an isolated condition or associated with other abnormalities, the most common being Junctional epidermolysis bullosa (JEB). Evidence suggests that PA-EB (Pyloric Atresia - Epidermolysis Bullosa) Syndrome is a distinct entity. In this report, we present three cases of pyloric atresia, one of which was associated with Junctional epidermolysis bullosa. The literature on the subject is also reviewed.

摘要

幽门闭锁(PA)是一种罕见的先天性异常,约占所有肠道闭锁的1%,其发病率约为每10万例活产中有1例。PA可能作为一种孤立的病症出现,也可能与其他异常相关,最常见的是交界性大疱性表皮松解症(JEB)。有证据表明,PA-EB(幽门闭锁-大疱性表皮松解症)综合征是一种独特的病症。在本报告中,我们介绍了3例幽门闭锁病例,其中1例与交界性大疱性表皮松解症相关。同时也对该主题的文献进行了综述。

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