Hyperplastic callus formation in osteogenesis imperfecta type V: follow-up of three generations over ten years

Hyperplastic callus formation in osteogenesis imperfecta type V: follow-up of three generations over ten years
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DOI:
10.1007/s00256-007-0441-0
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发表时间:
2008-05-01
期刊:
影响因子:
2.1
通讯作者:
Rauch, Frank
Rauch, Frank
中科院分区:
医学4区
文献类型:
--
作者:
Cheung, Moira S.;Azouz, E. Michel;Rauch, Frank

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增生性骨痂(HPC)的形成是V型成骨不全(OI)的一个显著特征;然而,对其长期结果知之甚少。在这个病例报告中,我们描述了一位OI V型患者在10年的随访中股骨HPC的发生、表现和病程。本病例的X线片与其父亲和祖父股骨内的HPC形成进行了对比,后者也患有OI V型。病例报告表明,HPC不仅在急性期,而且由于骨结构的残留改变,可以导致显著的发病率。
Hyperplastic callus (HPC) formation is a prominent feature of osteogenesis imperfecta (OI) type V; however, little is known about its long-term outcome. In this case report we describe the occurrence, appearance and course of a femoral HPC in a patient with OI type V during 10 years of follow-up. Radiographs of HPC in this child were compared and contrasted with HPC formation in the femur of his father and paternal grandfather, who also were affected with OI type V. This case report makes it clear that HPC can lead to significant morbidity, not only in the acute phase but also long term as a result of residual alteration in bone architecture.