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常染色体显性短肢症:暂时性干骺端条纹征

Autosomal dominant brachyolmia: transient metaphyseal striations.

作者信息

Lim Yun-Jung, Lee Hye-Ran, Kim Ok-Hwa, Cho Tae-Joon, Park Kun-Bo

机构信息

Department of Radiology, Inje University Haeundae Paik Hospital, 875, Haeun-daero, Haeundae-gu, Busan, 48108, South Korea.

Division of Pediatric Orthopaedics, Seoul National University Children's Hospital, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.

出版信息

Skeletal Radiol. 2017 Sep;46(9):1297-1300. doi: 10.1007/s00256-017-2684-8. Epub 2017 Jun 10.

Abstract

We report transient proximal and distal femoral metaphyseal striations that have not previously been described in autosomal dominant brachyolmia. The pelvis/hip radiograph of a 13-year-old boy demonstrated bilaterally symmetrical proximal femoral metaphyseal vertical striations. Additional vertical striations were also observed at the distal femur and proximal tibia metaphysis. Radiography of the thoracolumbar spine demonstrated platyspondyly with irregular endplates and overfaced pedicles. TRPV4 mutations were confirmed in this patient. Similar proximal femoral metaphyseal vertical striations were noted in the patient's sibling. Those streaks disappeared on the follow-up radiographs, and we considered it a unique radiologic finding transiently observed in autosomal dominant brachyolmia.

摘要

我们报告了常染色体显性短肢性侏儒症中此前未被描述过的股骨近端和远端干骺端短暂性条纹。一名13岁男孩的骨盆/髋部X线片显示双侧股骨近端干骺端垂直条纹对称。在股骨远端和胫骨近端干骺端也观察到了额外的垂直条纹。胸腰椎X线片显示椎体扁平,终板不规则,椎弓根重叠。该患者经证实存在TRPV4突变。在患者的兄弟姐妹中也发现了类似的股骨近端干骺端垂直条纹。这些条纹在随访X线片中消失了,我们认为这是常染色体显性短肢性侏儒症中暂时观察到的一种独特的放射学表现。

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