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Clinical and Radiological Manifestations of Osteogenesis Imperfecta Type V

2006; Korean Academy of Medical Sciences; Volume: 21; Issue: 4 Linguagem: Inglês

10.3346/jkms.2006.21.4.709

1598-6357

Dong Yeon Lee, Tae‐Joon Cho, In Ho Choi, Chin Youb Chung, Won Joon Yoo, Ji Hyung Kim, Yong Koo Park,

Bone health and treatments

Clinical and Radiological Manifestations of Osteogenesis Imperfecta Type VWe 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.