Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report

Background: Aplasia cutis congenital type VI (Bart’s syndrome) has been known as an extremely rare genetic disorder in which there is localized absence of skin, epidermolysis bullosa and nail deformities. Case report: Here, we present a rare case of Bart’s syndrome in a female newborn diagnosed with...

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Main Authors: Homa Babaei, Pourang Mohammadi, khadijeh Ramezani-Ali Akbari, Soudabeh Ramezani-Ali Akbari, Zohre Fatemi
Format: Article
Language:English
Published: Mashhad University of Medical Sciences 2023-05-01
Series:International Journal of Pediatrics
Subjects:
Online Access:https://ijp.mums.ac.ir/article_22272_d056a16d4c83f7ceda95c27681471ea6.pdf
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author Homa Babaei
Pourang Mohammadi
khadijeh Ramezani-Ali Akbari
Soudabeh Ramezani-Ali Akbari
Zohre Fatemi
author_facet Homa Babaei
Pourang Mohammadi
khadijeh Ramezani-Ali Akbari
Soudabeh Ramezani-Ali Akbari
Zohre Fatemi
author_sort Homa Babaei
collection DOAJ
description Background: Aplasia cutis congenital type VI (Bart’s syndrome) has been known as an extremely rare genetic disorder in which there is localized absence of skin, epidermolysis bullosa and nail deformities. Case report: Here, we present a rare case of Bart’s syndrome in a female newborn diagnosed with congenital loss of skin over upper and lower limbs, trunk, neck and face as well as some bullae on them. Moreover, a dilated stomach was observed in radiographic examination. We treated the baby with TPN, systemic antibiotics, and also her wounds were covered by topical ointments. Laboratory tests, along with liver and renal function analyses were normal, and also serologic tests for infection were negative but she died at the age of 4 days. Conclusion: The association between Bart’s syndrome and pyloric atresia is a highly fatal combination and there is no treatment option to rescue the patients.
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spelling doaj.art-27373901ca0643a9bb88b5ee492567562023-09-03T06:32:42ZengMashhad University of Medical SciencesInternational Journal of Pediatrics2345-50472345-50552023-05-01115178301783610.22038/ijp.2023.70131.516522272Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case ReportHoma Babaei0Pourang Mohammadi1khadijeh Ramezani-Ali Akbari2Soudabeh Ramezani-Ali Akbari3Zohre Fatemi4Professor of Neonatal-Perinatal Medicine , Department of Pediatrics, School of Medicine, Imam Reza Hospital , Kermanshah University of Medical Sciences, Kermanshah, IranDoctor of Medicine, School of Medicine , Kermanshah University of Medical Sciences, Kermanshah, IranDepartment of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, IranDoctor of Medicine, School of Medicine , Kermanshah University of Medical Sciences, Kermanshah, Iranpediatric &congenital cardiology Division,Pediatric Department,Factulty of Medicine , Mashhad University of Medical Sciences, Mashhad,IranBackground: Aplasia cutis congenital type VI (Bart’s syndrome) has been known as an extremely rare genetic disorder in which there is localized absence of skin, epidermolysis bullosa and nail deformities. Case report: Here, we present a rare case of Bart’s syndrome in a female newborn diagnosed with congenital loss of skin over upper and lower limbs, trunk, neck and face as well as some bullae on them. Moreover, a dilated stomach was observed in radiographic examination. We treated the baby with TPN, systemic antibiotics, and also her wounds were covered by topical ointments. Laboratory tests, along with liver and renal function analyses were normal, and also serologic tests for infection were negative but she died at the age of 4 days. Conclusion: The association between Bart’s syndrome and pyloric atresia is a highly fatal combination and there is no treatment option to rescue the patients.https://ijp.mums.ac.ir/article_22272_d056a16d4c83f7ceda95c27681471ea6.pdfbart' s syndrome,,,،absence of skin,,,،epidermolysis bullosa,,,،pyloric atresia
spellingShingle Homa Babaei
Pourang Mohammadi
khadijeh Ramezani-Ali Akbari
Soudabeh Ramezani-Ali Akbari
Zohre Fatemi
Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
International Journal of Pediatrics
bart' s syndrome,,
,،absence of skin,,
,،epidermolysis bullosa,,
,،pyloric atresia
title Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
title_full Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
title_fullStr Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
title_full_unstemmed Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
title_short Bart Syndrome Associated with Pyloric Atresia: An Uncommon Case Report
title_sort bart syndrome associated with pyloric atresia an uncommon case report
topic bart' s syndrome,,
,،absence of skin,,
,،epidermolysis bullosa,,
,،pyloric atresia
url https://ijp.mums.ac.ir/article_22272_d056a16d4c83f7ceda95c27681471ea6.pdf
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AT pourangmohammadi bartsyndromeassociatedwithpyloricatresiaanuncommoncasereport
AT khadijehramezanialiakbari bartsyndromeassociatedwithpyloricatresiaanuncommoncasereport
AT soudabehramezanialiakbari bartsyndromeassociatedwithpyloricatresiaanuncommoncasereport
AT zohrefatemi bartsyndromeassociatedwithpyloricatresiaanuncommoncasereport