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   bart syndrome associated with pyloric atresia: an uncommon case report  
   
نویسنده fatemi zohre ,mohammadi pourang ,babaei homa ,ramezani-ali akbari khadijeh ,ramezani-ali akbari soudabeh
منبع journal of pediatric perspectives - 2023 - دوره : 11 - شماره : 5 - صفحه:17830 -17836
چکیده    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.
کلیدواژه absence of skin ,bart's syndrome ,epidermolysis bullosa ,pyloric atresia
آدرس mashhad university of medical sciences, faculty of medicine, pediatric congenital cardiology division, pediatric department, iran, kermanshah university of medical sciences, school of medicine, iran, kermanshah university of medical sciences, school of medicine, imam reza hospital, department of pediatrics, iran, shahid beheshti university of medical sciences, school of medicine, department of immunology, iran, kermanshah university of medical sciences, school of medicine, iran
پست الکترونیکی sraliakbari@yahoo.com
 
     
   
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