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Clinical and radiological manifestations of osteogenesis imperfecta type V

Cited 22 time in Web of Science Cited 27 time in Scopus
Authors
Lee, Dong Yeon; Cho, Tae-Joon; Choi, In Ho; Chung, Chin Youb; Yoo, Won Joon; Kim, Ji Hyung; Park, Yong Koo
Issue Date
2006
Publisher
대한의학회 = Korean Academy of Medical Science
Citation
J Korean Med Sci 2006; 21: 709-14
Keywords
Osteogenesis ImperfectaDislocationsRadial Head DislocationOssificationHeterotopicInterosseous Membrane Ossification
Abstract
We reviewed clinical manifestation of 12 patients from three Korean families. They showed mild to moderate bone fragility, and suggested an autosomal dominant inheritance pattern. Significant intrafamilial phenotype variability was obvious. Clinical, radiological, and histopathologic characteristics that distinguished this subtype from others include ossification of interosseous membrane of the forearm with radial head dislocation, hyperplastic callus formation, no evidence of type I collagenopathy and an abnormal histopathologic pattern. Severity of the interosseous membrane ossification was correlated with increasing age (p<0.01) and the radial head dislocation was thought to be a developmental problem rather than a congenital problem. Four children who had bisphosphonate treatment showed improved bone mineral density, radiological changes, and biochemical responses. Osteogenesis imperfecta type V was a distinctive subtype of osteogenesis imperfecta, which caused mild to moderate disability clinically.
ISSN
1011-8934 (Print)
Language
English
URI
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=16891817

http://hdl.handle.net/10371/10868
DOI
https://doi.org/10.3346/jkms.2006.21.4.709
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College of Medicine/School of Medicine (의과대학/대학원)Orthopedic Surgery (정형외과학전공)Journal Papers (저널논문_정형외과학전공)
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