KMID : 0358420080510111337
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Korean Journal of Obstetrics and Gynecology 2008 Volume.51 No. 11 p.1337 ~ p.1341
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A case of Larsen syndrome
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Kim Jin-Sub
Kim Seung-Hyun Kim Jeong-Hyun Jin Chan-Hee Kim Hyun-Young Song Young-Rae Oh Kwan-Young
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Abstract
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Larsen syndrome is a rare congenital skeletal malformation (1 in 100,000 births) caused by a generalized mesenchymal connective tissue disorder. This disorder leads to a broad spectrum of anomalies. Major diagnostic criteria are multiple dislocations of large joints (especially knees), short metacarpals with cylindrical nontapering fingers and craniofacial abnormalities. Clinical variations range from mild clinical expression to lethal forms. Sporadic occurrence as well as autosomal dorminant and recessive inheritance are described.
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KEYWORD
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Larsen syndrome
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