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KMID : 0381219790110030077
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Journal of RIMSK
1979 Volume.11 No. 3 p.77 ~ p.80
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Congenital Contactural Arachnodactyly
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Abstract
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Congenital contractural arachnodactyly is a newly delineated disease and characterized by multiple joint contracture, arachnodactyly, crumpled ear and kyphoscoliosis.
The joint contractures are severe at birth and maximal at the knee and usually improved
spontaneously, whereas the kyphoscoliosis usually worsen with age.
There is no ocular and cadiovascular involvement which is found in Marfan´s syndrome Transmission is an autosomal dominant trait.
In view of its different prognosis from Marfan´s syndrome it is important that patientwith this new syndrome be recognized and excluded from Marfan´s syndrome in order to provide correct treatment and genetic counselling for the patient and family.
This report describes one case of congenital contractural arachnodactyly exper ienced in our orthopedic surgery department.
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