{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:39:53Z","timestamp":1788305993268,"version":"build-2803163510"},"reference-count":3,"publisher":"S. Karger AG","issue":"2","license":[{"start":{"date-parts":[[2001,1,1]],"date-time":"2001-01-01T00:00:00Z","timestamp":978307200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2001,1,1]],"date-time":"2001-01-01T00:00:00Z","timestamp":978307200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"published-print":{"date-parts":[[2001]]},"abstract":"<jats:p>&lt;i&gt;Background\/Aims: &lt;\/i&gt;Leri-Weill dyschondrosteosis (LWD), a mesomelic short stature syndrome with Madelung deformity, was recently reported to be caused by SHOX (short stature homeobox-containing gene) haploinsufficiency. The loss of SHOX on Xp22.32, also called PHOG (pseudoautosomal homeobox-containing osteogenic gene), through structural aberrations of the X chromosome was also implicated in the short stature phenotype and some additional stigmata of Turner syndrome. The aim of this study was to systematically examine left-hand radiographs from Turner girls for the presence of signs of LWD. &lt;i&gt;Methods:&lt;\/i&gt; We retrospectively studied 168 left-hand radiographs from 54 patients with Turner syndrome (bone age &gt;10.5 years) who were treated with rhGH and seen during the last 10 years in our clinic. For comparison, we analyzed 7 radiographs from 5 patients with LWD and 52 radiographs from 20 patients with GH deficiency. The shape of the distal radial epiphysis (triangularisation index = TI) and the carpal angle were quantitatively measured. In addition, we screened for the presence of a premature cleft fusion or an ulnar deviation of the articular surface of the distal radial epiphysis and for fourth metacarpal shortening. One of 54 Turner girls (2%) was affected with LWD and presented with Madelung deformity. &lt;i&gt;Results:&lt;\/i&gt; No milder forms of Madelung deformity were detected. However, there was a significant trend to a triangular shape of the distal radial epiphysis in Turner syndrome: the median TI was 2.7 in normal controls (range 1.8\u20133.7), 3.1 in Turner girls (range 2.0\u20136.3) (p &lt; 0.001 against controls), and 6.0 in patients with LWD (range 3.5\u201311.0) (p &lt; 0.001 against controls). &lt;i&gt;Conclusions: &lt;\/i&gt;The triangularisation index did not correlate with the carpal angle (median 122.5<\/jats:p>","DOI":"10.1159\/000049973","type":"journal-article","created":{"date-parts":[[2003,4,15]],"date-time":"2003-04-15T10:10:37Z","timestamp":1050401437000},"page":"71-76","source":"Crossref","is-referenced-by-count":22,"title":["Radiological Signs of Leri-Weill Dyschondrosteosis in Turner Syndrome"],"prefix":"10.1159","volume":"55","author":[{"given":"Gerhard","family":"Binder","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Fritsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Schweizer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael B.","family":"Ranke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2001,8,15]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0597-54"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.85.1.245"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-199607000-00001"}],"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/49973","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/49973","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T04:18:58Z","timestamp":1745468338000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000049973"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001]]},"references-count":3,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2001,7,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000049973","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001]]}}}