Abstract
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, brachymetacarpia of the 4th digit, carpal and epiphyseal angle, as well as a new sign the distal radio-ulnar 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 radio-ulnar 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.
Similar content being viewed by others
Abbreviations
- GH :
-
growth hormone
- LWD :
-
Leri-Weill dyschondrosteosis
- TS :
-
Turner syndrome
References
Archibald RM, Finby N, De Vito F (1959) Endocrine significance of short metacarpals. J Clin Endocrinol Metab 19: 1312–1322
Binder G, Fritsch H, Schweizer R, Ranke MB (2001) Radiological signs of Leri-Weill dyschondrosteosis in Turner syndrome. Horm Res 55: 71–76
Kosho T, Muroya K, Nagai T, Fujimoto M, Yokoya S, Sakamoto H, Hirano T, Terasaki H, ohashi M, Nishimura G, Sato S, Matsuo N, Ogata T (1999) Skeletal features and growth patterns in 14 patients with haploinsufficiency of SHOX: implications for the development of Turner syndrome. J Clin Endocrinol Metab 84: 4613–4621
Kosowicz J (1959) Skeletal changes in Turner’s syndrome and their significance in differential diagnosis. Polish Med Hist Sci Bull 2: 23–26
Kosowicz J (1965) The roentgen appearance of the hand and wrist in gonadal dysgenesis. Am J Roentgenol 93: 354–361
Lippe B (1991) Turner syndrome. Endocrinol Metab Clin North Am 20: 121–152
Necic S, Grant DB (1978) Diagnostic value of hand X-rays in Turner’s syndrome. Acta Paediatr Scand 67: 309–312
Park E (1997) Radiological anthropometry of the hand in Turner’s syndrome. J Phys Anthropol 46: 463–470
Rosenfeld RG (1989) Turner syndrome: a guide for physicians. The Turner’s Syndrome Society: Minnetonka, Minnesota
Ross JL, Scott C, Marttila P, Kowal K, Nass A, Papenhausen P, Abboudi J, Osterman L, Kushner H, Carter P, Ezaki R, Elder F, Wei F, Chen H, Zinn AR (2001) Phenotypes associated with SHOX deficiency. J Clin Endocrinol Metab 86: 5674–5680
Schiller S, Spranger S, Schechinger B, Fukami M, Merker S, Drop SL, Troger J, Knoblauch H, Kunze J, Seidel J, Rappold GA (2000) Phenotypic variation and genetic heterogeneity in Leri-Weill syndrome. Eur J Hum Genet 8: 54–62
Shears DJ, Vassal HJ, Goodman FR, Palmer RW, Reardon W, Superti-Furga A, Scambler PJ, Winter RM (1998) Mutation and deletion of the pseudoautosomal gene SHOX cause Leri-Weill dyschondrosteosis. Nat Genet 19: 70–73
Acknowledgements
The authors wish to thank N. Crowte for critical reading of the manuscript, M. White-Koning for statistical advice and F. Conte for technical assistance.
Author information
Authors and Affiliations
Corresponding author
Additional information
Presented in part at the 41st Annual Meeting of the European Society for Paediatric Endocrinology.
Rights and permissions
About this article
Cite this article
Tauber, M., Lounis, N., Coulet, J. et al. Wrist anomalies in Turner syndrome compared with Leri-Weill dyschondrosteosis: a new feature in Turner syndrome. Eur J Pediatr 163, 475–481 (2004). https://doi.org/10.1007/s00431-004-1471-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00431-004-1471-4