Parelkar Sandesh V, Kapadnis Satish P, Sanghvi Beejal V, Joshi Prashant B, Mundada Dinesh, Shetty Shishira, Oak Sanjay N
Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra, India.
Afr J Paediatr Surg. 2014 Oct-Dec;11(4):362-5. doi: 10.4103/0189-6725.143178.
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.
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。
