Familial osteochondritis dissecans associated with early osteoarthritis and disproportionate short stature
2008 (English)In: Osteoarthritis and Cartilage, ISSN 1063-4584, E-ISSN 1522-9653, Vol. 16, no 8, 890-896 p.Article in journal (Refereed) Published
Objective: Familial osteochondritis dissecans (OCD) is a rare disorder characterised by disturbed chondro-skeletal development, disproportionate growth and deformation of the skeleton.
Design: We identified a five-generation family with 15 living affected members from Northern Sweden. The disorder was diagnosed with a case definition of OCD in at least one joint.
Results: Main clinical findings consisted of OCD in knees and/or hips and/or elbows, disproportionate short stature and early osteoarthritis (OA). There were no radiological indications of epiphyseal dysplasia. Anthropometric measurements of affected individuals showed short stature, a high ratio between sitting height and total height, a relatively normal arm span and head circumference. In 12 of 15 cases, onset was during late childhood or adolescence and OA had developed in seven of those patients.
Conclusions: Our observation suggests that OA is a frequent complication in familial OCD even though the lesions appear before closure of physis.
Place, publisher, year, edition, pages
London: Published for the Society by Baillière Tindall , 2008. Vol. 16, no 8, 890-896 p.
Familial osteochondritis dissecans; Osteoarthritis; Skeletal dysplasia; Autosomal dominant; Cartilage
IdentifiersURN: urn:nbn:se:umu:diva-22382DOI: 10.1016/j.joca.2007.11.009OAI: oai:DiVA.org:umu-22382DiVA: diva2:216193