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Intermediate Tip Atrioventriküler Septal Defekt ve Polidaktili

Intermediate type atrioventricular septal defect and polydactyly

Journal Name:

  • İnönü Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 1997

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
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. [Journal of Turgut Özal Medical Center 1997;4(2): 209-211]
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Abstract (Original Language): 
Ellis-van Creveld Syndromu deri, tırnak ve dişleri içeren çeşitli tipte ectodermal 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. Postoperatif 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. [Turgut Özal Tıp Merkezi Dergisi 1997;4(2):209-211]
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  • 2
209-211
  • Turkish

REFERENCES

References: 

1. Ellis RWB, van Creveld S. Syndrome characterized by ectodermal dysplasia, polydactyly, chondrodysplasia and congenital morbus cordis. Arch Dis Childhood 1940;15:65-84.
2. Santos JM, Pipa J, Antunes L, et al. The Ellis-Van Creveld syndrome. Apropos 2 clinical cases. Rev Port Cardiol 1994;13:45-50.
3. Weis H, Crossett ADJr. Chondro-ectodermal dysplasia: report of a case and rewiew of the literature. J Pediat 1955;46:268-75.
4. Levin SE, Dansky R, Milner S, Benatar A, Govendrageloo K, du Plessis J. Atrioventricular septal defect and type A postaxial polydactyly without other major associated anomalies: a specific association. Pediatr Cardiol
1995;16:242-6.

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