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Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association

Pyloric atresia (PA) is an unusual congenital disorder that accounts for about 1% of all intestinal atresia, with an incidence around 1 in 100,000 live births. PA may occur as an isolated disorder or may be associated with epidermolysis bullosa (EB). EB comprises a heterogeneous group of cutaneous g...

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Autor principal: Pan, Pradyumna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8323568/
https://www.ncbi.nlm.nih.gov/pubmed/34385771
http://dx.doi.org/10.4103/jiaps.JIAPS_125_20
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author Pan, Pradyumna
author_facet Pan, Pradyumna
author_sort Pan, Pradyumna
collection PubMed
description Pyloric atresia (PA) is an unusual congenital disorder that accounts for about 1% of all intestinal atresia, with an incidence around 1 in 100,000 live births. PA may occur as an isolated disorder or may be associated with epidermolysis bullosa (EB). EB comprises a heterogeneous group of cutaneous genetic diseases of inherited blistering and skin fragility disorders. Wound management in EB is complex and influenced by several comorbidities and the fragility of the skin. The prognosis of children with EB depends on the type of mutation inherited. Some types are mild and even improve with age, while others are so severe it is not likely that a child to survive into adulthood. We present three cases of PA in this article and one in conjunction with junctional EB.
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spelling pubmed-83235682021-08-11 Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association Pan, Pradyumna J Indian Assoc Pediatr Surg Case Report Pyloric atresia (PA) is an unusual congenital disorder that accounts for about 1% of all intestinal atresia, with an incidence around 1 in 100,000 live births. PA may occur as an isolated disorder or may be associated with epidermolysis bullosa (EB). EB comprises a heterogeneous group of cutaneous genetic diseases of inherited blistering and skin fragility disorders. Wound management in EB is complex and influenced by several comorbidities and the fragility of the skin. The prognosis of children with EB depends on the type of mutation inherited. Some types are mild and even improve with age, while others are so severe it is not likely that a child to survive into adulthood. We present three cases of PA in this article and one in conjunction with junctional EB. Wolters Kluwer - Medknow 2021 2021-07-12 /pmc/articles/PMC8323568/ /pubmed/34385771 http://dx.doi.org/10.4103/jiaps.JIAPS_125_20 Text en Copyright: © 2021 Journal of Indian Association of Pediatric Surgeons https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Pan, Pradyumna
Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title_full Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title_fullStr Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title_full_unstemmed Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title_short Congenital Pyloric Atresia and Epidermolysis Bullosa: Report of a Rare Association
title_sort congenital pyloric atresia and epidermolysis bullosa: report of a rare association
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8323568/
https://www.ncbi.nlm.nih.gov/pubmed/34385771
http://dx.doi.org/10.4103/jiaps.JIAPS_125_20
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