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一例先天性幽门闭锁合并营养不良性大疱性表皮松解症。

A case of congenital pyloric atresia with dystrophic epidermolysis bullosa.

作者信息

Short Scott S, Grant Christa N, Merianos Demetri, Haydel Dana, Ford Henri R

机构信息

Department of Pediatric Surgery, Children's Hospital Los Angeles, 4650 Sunset Blvd. MT#100, Los Angeles, CA, 90027, USA.

出版信息

Pediatr Surg Int. 2014 Jun;30(6):681-4. doi: 10.1007/s00383-014-3505-y. Epub 2014 Apr 30.

Abstract

Pyloric atresia with epidermolysis bullosa (EB) dystrophica is a rare entity that may not be immediately recognized. We describe the fourth confirmed case of pyloric atresia associated with the dystrophic subtype of EB diagnosed by standard pathologic measures, and discuss the clinical disease features and recent advances in the pathophysiology.

摘要

伴有营养不良型大疱性表皮松解症(EB)的幽门闭锁是一种罕见的病症,可能无法立即被识别。我们描述了第四例经标准病理方法确诊的与营养不良型EB相关的幽门闭锁病例,并讨论了临床疾病特征和病理生理学的最新进展。

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