Intermediate tip atrioventriküler septal defekt ve polidaktili
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Date
1997
Journal Title
Journal ISSN
Volume Title
Publisher
İnönü Üniversitesi Tıp Fakültesi Dergisi
Access Rights
info:eu-repo/semantics/openAccess
Abstract
Ellis-van Creveld Syndromu deri, tırnak ve dişleri içeren çeşitli tipte ektodermal displazi, polidaktili ve kondrodermal displaziye ilaveten konjenital kalp defektlerini içermektedir. Polidaktili ve intermediate tip atrioventriküler septal defekti olan ve ilave mayor anomalisi olmayan bir olgu sunuldu. Bu spesifik birliktelik oldukça nadir bir durumdur. Olgu atrioventriküler septal defekt sebebiyle opere edildi. Postoperııtif tam düzelme sağlandı. Bu olguda saptanan atrioventriküler septal defekt ve polidaktilin spesifik bir birliktelik olduğu düşünülmüştür. Bu klinik tablo Ellis-van Creveld Syndromuna benzer bazı özellikler taşımasına rağmen bu sendroma uymamaktadır.
Ellis-van creveld syndrome includes several types of ectodermal dysplasia involving skin, nails and teeth, polydactyly and congenital heart defects in addition to chondrodystrophy. A case is described with polydactyly and intermediate type atrioventricular septal defect without other major associated anomalies. This specific association is extremely rare. The patient underwent cardiac operation for atrioventricular septal defect. Complete improvement was seen postoperatively. It is suggested that there is a specific association between polydactyly and the atrioventricular septal defect found in this patient. This picture does not conform to, but bears some resemblance to the ellis-van creveld syndrome.
Ellis-van creveld syndrome includes several types of ectodermal dysplasia involving skin, nails and teeth, polydactyly and congenital heart defects in addition to chondrodystrophy. A case is described with polydactyly and intermediate type atrioventricular septal defect without other major associated anomalies. This specific association is extremely rare. The patient underwent cardiac operation for atrioventricular septal defect. Complete improvement was seen postoperatively. It is suggested that there is a specific association between polydactyly and the atrioventricular septal defect found in this patient. This picture does not conform to, but bears some resemblance to the ellis-van creveld syndrome.
Description
[Journal of Turgut Ozal Medical Center 1997;4(2): 209-211]
Keywords
Ellis-van Creveld Syndromu, Polidaktili, Atrioventriküler septal defekt, Ellis-van Creveld Syndrome, Polydactyly, Atrioventricular septal defect
Journal or Series
Turgut Özal Tıp Merkezi Dergisi
WoS Q Value
Scopus Q Value
Volume
4
Issue
2
Citation
Türköz, R.,Paç, A.,Berat, H.,Paç, M.,(1997). Intermediate tip atrioventriküler septal defekt ve polidaktili.Turgut Özal Tıp Merkezi Dergisi, 4 (2).209-211 ss.
