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RISS 인기검색어
- 검색키워드
- 저자 : H. Jagadishchandra (검색결과 2 건)
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Ellis-van Creveld syndrome in an Indian child: a case report
Veena, K.M.,Jagadishchandra, H.,Rao, Prasanna Kumar,Chatra, Laxmikanth Korean Academy of Oral and Maxillofacial Radiology 2011 Imaging Science in Dentistry Vol.41 No.4
Ellis-van Creveld syndrome is a rare congenital genetic disorder having autosomal recessive inheritance. It is a syndrome affecting the Amish population of Pennsylvania in USA with prevalence rate of 1/5,000 live at birth. In non-Amish population, the birth prevalence is 7/1,000,000. The syndrome is characterized by bilateral postaxial polydactyly of the hands, chondrodysplasia of long bones resulting in acromesomelic dwarfism, ectodermal dysplasia affecting nails as well as teeth and congenital heart malformation. There were very rare reports of this syndrome in dentistry. The present case focuses on the striking and constant oral findings of these patients, which are the main diagnostic features of this syndrome. Since the oral manifestations affect the esthetic, speech, and jaw growth of the child, the dentists have an important role to play in proper management of such case.
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Ellis-van Creveld syndrome in an Indian child: a case report
K.M. Veena,H. Jagadishchandra,Prasanna Kumar Rao,Laxmikanth Chatra 대한영상치의학회 2011 Imaging Science in Dentistry Vol.41 No.4
Ellis-van Creveld syndrome is a rare congenital genetic disorder having autosomal recessive inheritance. It is a syndrome affecting the Amish population of Pennsylvania in USA with prevalence rate of 1/5,000 live at birth. In non-Amish population, the birth prevalence is 7/1,000,000. The syndrome is characterized by bilateral postaxial polydactyly of the hands, chondrodysplasia of long bones resulting in acromesomelic dwarfism, ectodermal dysplasia affecting nails as well as teeth and congenital heart malformation. There were very rare reports of this syndrome in dentistry. The present case focuses on the striking and constant oral findings of these patients, which are the main diagnostic features of this syndrome. Since the oral manifestations affect the esthetic, speech, and jaw growth of the child, the dentists have an important role to play in proper management of such case.
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