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Biomédica
versão impressa ISSN 0120-4157versão On-line ISSN 2590-7379
Resumo
MARQUEZ, Katherine et al. Epidermolysis bullosa with pyloric atresia: Report of two cases in consecutive siblings. Biomed. [online]. 2021, vol.41, n.2, pp.201-207. Epub 15-Jun-2021. ISSN 0120-4157. https://doi.org/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.
Palavras-chave : Epidermolysis bullosa; infant, newborn; intestinal obstruction.