Orthopaedic manifestations in de Barsy syndrome.
de Barsy syndrome is a rare, genetically transmitted condition characterized by severe cutis laxa, joint hypermobility, growth retardation, mental retardation, and characteristic facies. Affected individuals have many orthopaedic manifestations, including developmental dysplasia of the hip, scoliosis, multiple joint dislocations and subluxations, and congenital vertical talus. The management of the orthopaedic manifestations of this syndrome is presented in two typical cases.
Stanton
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Alfred I. duPont Institute, Wilmington, Delaware 19899.
Rao
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Scott
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D001847:Bone Diseases / Q000151:congenital* / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy; D003483:Cutis Laxa / Q000235:genetics; D005260:Female; D005532:Foot Deformities, Congenital / Q000000981:diagnostic imaging / Q000628:therapy; D006228:Hand Deformities, Congenital / Q000000981:diagnostic imaging / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000235:genetics; D008297:Male; D011859:Radiography; D013577:Syndrome