THE RADIOLOGICAL SPECTRUM OF FIBRODYSPLASIA OSSIFICANS PROGRESSIVA
THE RADIOLOGICAL SPECTRUM OF FIBRODYSPLASIA OSSIFICANS PROGRESSIVA
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DOI：
10.1016/s0009-9260(82)80159-1
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发表时间：
1982-01-01
影响因子：
2.6
通讯作者：
BEIGHTON, P
中科院分区：
文献类型：
--
作者：
CREMIN, B;CONNOR, JM;BEIGHTON, P
详细分析了9例南非进行性骨化性纤维发育不良（FOP）患者的影像学表现。其中3例患者获得了10-15年的随访。这种情况通常出现在5岁之前，在一个婴儿中，9个月时出现异常钙化。这种疾病是自发进行性的，但手、横膈膜和内脏不会受到影响。韧带与长骨连接处的骨化产生假性外生骨疣外观。长骨干的束缚和近端干骺端的生长可能会导致肩关节和髋关节脱位。在髋关节中，髋臼顶的倾斜构型是由栓系股骨近端干骺端的正常生长产生的。关节周围组织的钙化以及骨性和纤维性关节强直有助于关节固定。类似的生长抑制过程导致颈椎体及其后附件之间的尺寸差异。除了大脚趾的发育异常外，FOP的骨骼变化是软组织骨化的结果，这种情况不是原发性骨发育不良。
The radiographic manifestations of 9 South African patients with fibrodysplasia ossificans progressiva (FOP) were analyzed in detail. In 3 of these individuals, a 10-15 yr follow-up was available. The condition usually presents before the age of 5 yr; in one infant, abnormal calcification was demonstrated at 9 mo. The disorder is remorselessly progressive but the hands, diaphragm and viscera are spared. Ossification in ligaments at the site of their attachment to long bones produces a pseudo-exostosis appearance. Tethering of the shafts of long bones and growth at the proximal metaphyses can result in dislocation of shoulder and hip joints. In the hip joint the sloping configuration of the acetabular roof is produced by normal growth at the proximal metaphyseal end of the tethered femur. Calcification in periarticular tissues, together with bony and fibrous ankylosis, contributed to joint fixation. A similar process of growth inhibition leads to disparity in size between the bodies of the cervical vertebrae and their posterior appendages. Apart from the developmental abnormality in the great toes, the skeletal changes in FOP are the consequence of soft tissue ossification and the condition is not a primary bone dysplasia.