Servicio de Pediatría, Hospital Universitario de Santander, Bucaramanga, Colombia.
Biomedica. 2021 Jun 29;41(2):201-207. doi: 10.7705/biomedica.5401.
Pyloric atresia is a rare digestive malformation. It represents about 1% of intestinal atresias and is associated with some other genetic or anatomical alteration in 55% of the cases. In 20% of them, it is associated with epidermolysis bullosa, which is described as an established syndrome with a bad prognosis. We present two cases of consecutive siblings with this condition and fatal outcomes in both of them. We made a review of the literature and discussed the main topics.
幽门闭锁是一种罕见的消化畸形。它占肠闭锁的 1%,且 55%的病例伴有其他一些遗传或解剖异常。其中 20%的病例与大疱性表皮松解症相关,后者被描述为一种具有不良预后的既定综合征。我们报告了两例连续的同胞病例,他们都因此病而死亡。我们对文献进行了回顾,并讨论了主要议题。