首页> 中文期刊> 《世界核心医学期刊文摘:儿科学分册》 >特纳综合征的腕关节畸形与Leri-Weil软骨骨生成障碍相比较:特纳综合征的一个新特征

特纳综合征的腕关节畸形与Leri-Weil软骨骨生成障碍相比较:特纳综合征的一个新特征

         

摘要

We analysed bone age radiographs in 102 girls with Turner syndrome and compared the findings with 93 control girls and nine girls with Leri-Weill syndrome. Various signs were analysed: radial bowing or Madelung deformity,maximal/minimal height of the radial epiphysis, brachymeta-carpia of the 4th digit, carpal and epiphyseal angle, as well as a new sign the distal radioulnar physeal disparity. Two values differed significantly between the Turner group and the control group, the first being the epiphyseal angle which has already been reported to be greater in Turner syndrome, and the second being the new sign we have been able to describe. Turner patients had an increased distance between the ulnar and radial metaphysis, or “ distal radio-ulnar physeal disparity” , the ulnar being shorter. Furthermore, in 27% of cases the medial extremity of the ulnar epiphysis was flattened and passed below the distal extremity of the radius, whose medial part projected over the distal extremity of the ulna, thus reproducing in reverse the characteristic feature of Leri-Weill syndrome. In the growth hormone-treated Turner patients, we found a significant correlation between distal radioulnar physeal disparity and growth velocity expressed in cm/year (r = 0. 28; P < 0. 002) or in SDS/bone age (r = 0. 21; P < 0. 03) during the first year of treatment. Conclusion: the value of this new sign requires further investigation.

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