{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:07:52Z","timestamp":1760188072606},"reference-count":21,"publisher":"World Scientific Pub Co Pte Lt","issue":"01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hand Surg."],"published-print":{"date-parts":[[2001,7]]},"abstract":"<jats:p> Leri-Weill syndrome (LWS) is a dominant (pseudoautosomal) skeletal dysplasia with mesomelic short stature and bilateral Madelung deformity, due to dyschondrosteosis of the distal radius. It results from the loss of one copy of the Short Stature Homeobox Gene (SHOX) from the tip of the short arm of the X or Y chromosome. SHOX molecular testing enabled us to evaluate the histopathology of the radial physis in LWS patients with a documented SHOX abnormality. A widespread disorganisation of physeal anatomy was revealed with disruption of the normal parallel columnar arrangement of chondrocytes. Tandem stacking of maturing chondrocytes within columns was replaced by a side-by-side arrangement. The presence of hypertrophic osteoid with micro-enchondromata in the radial metaphysis suggests abnormal endochondral ossification. The Vickers' ligament was confirmed to blend with the triangular fibrocartilage complex (TFCC). This histopathological study demonstrates that the zone of dyschondrosteosis in LWS is characterised by marked disruption of normal physeal chondrocyte processes and that a generalised physeal abnormality is present. <\/jats:p>","DOI":"10.1142\/s0218810401000424","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T06:58:59Z","timestamp":1027753139000},"page":"13-23","source":"Crossref","is-referenced-by-count":41,"title":["HISTOPATHOLOGICAL ANALYSIS OF LERI-WEILL DYSCHONDROSTEOSIS: DISORDERED GROWTH PLATE"],"prefix":"10.1142","volume":"06","author":[{"given":"C. F. J.","family":"Munns","sequence":"first","affiliation":[{"name":"Endocrine Research Unit, Royal Children's Hospital Foundation Research Centre, Royal Children's Hospital, Brisbane, Australia"},{"name":"Department of Paediatrics and Child Health, University of Queensland, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I. A.","family":"Glass","sequence":"additional","affiliation":[{"name":"Queensland Clinical Genetics Service, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"LaBrom","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Hayes","sequence":"additional","affiliation":[{"name":"Endocrine Research Unit, Royal Children's Hospital Foundation Research Centre, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Flanagan","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Child Health, University of Queensland, Royal Children's Hospital, Brisbane, Australia"},{"name":"Department of Surgery, University of Queensland, Royal Brisbane Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Berry","sequence":"additional","affiliation":[{"name":"Mater Hospital Cytogenetics Laboratory, South Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V. J.","family":"Hyland","sequence":"additional","affiliation":[{"name":"Department of Surgery, University of Queensland, Royal Brisbane Hospital, Brisbane, Australia"},{"name":"Molecular Genetics Laboratory, Queensland Health Pathology Service, Royal Brisbane Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. A.","family":"Batch","sequence":"additional","affiliation":[{"name":"Endocrine Research Unit, Royal Children's Hospital Foundation Research Centre, Royal Children's Hospital, Brisbane, Australia"},{"name":"Department of Paediatrics and Child Health, University of Queensland, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G. E.","family":"Philips","sequence":"additional","affiliation":[{"name":"Department of Pathology, Queensland Health Pathology Service, Royal Brisbane Hospitals Campus, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Vickers","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics, Royal Children's Hospital, Brisbane, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"219","published-online":{"date-parts":[[2011,11,20]]},"reference":[{"key":"p_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-193809000-00006"},{"key":"p_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0198-67"},{"key":"p_3","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.5.695"},{"key":"p_4","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.8.1341"},{"key":"p_5","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90292-5"},{"key":"p_6","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1302\/0301-620X.58B3.956255","volume":"58","author":"Golding J","year":"1976","journal-title":"J Bone Joint Surg"},{"key":"p_7","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1302\/0301-620X.49B1.66","volume":"49","author":"Henry A","year":"1967","journal-title":"J Bone Joint Surg"},{"key":"p_8","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(66)80247-0"},{"key":"p_9","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.6.2086"},{"key":"p_10","doi-asserted-by":"publisher","DOI":"10.1210\/jc.84.12.4613"},{"key":"p_11","doi-asserted-by":"publisher","DOI":"10.1097\/00004694-199803000-00008"},{"key":"p_12","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.95.1.178"},{"key":"p_14","first-page":"395","volume":"23","author":"Madelung O","year":"1878","journal-title":"Archiv fur Klinische Chirurgie"},{"key":"p_15","first-page":"152","volume":"2","author":"Matev I","year":"1975","journal-title":"The Hand"},{"key":"p_16","doi-asserted-by":"publisher","DOI":"10.1038\/ng0597-54"},{"key":"p_17","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5200402"},{"key":"p_18","doi-asserted-by":"publisher","DOI":"10.1038\/ng0198-70"},{"key":"p_19","doi-asserted-by":"publisher","DOI":"10.1016\/S0303-7207(99)00037-4"},{"key":"p_20","first-page":"966","author":"Vickers D","year":"1998","journal-title":"St. Louis, Mosby"},{"key":"p_21","doi-asserted-by":"publisher","DOI":"10.1016\/0268-0890(89)90070-4"},{"key":"p_22","doi-asserted-by":"publisher","DOI":"10.1016\/S0266-7681(05)80262-1"}],"container-title":["Hand Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.worldscientific.com\/doi\/pdf\/10.1142\/S0218810401000424","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,8,6]],"date-time":"2019-08-06T17:47:36Z","timestamp":1565113656000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.worldscientific.com\/doi\/abs\/10.1142\/S0218810401000424"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,7]]},"references-count":21,"journal-issue":{"issue":"01","published-online":{"date-parts":[[2011,11,20]]},"published-print":{"date-parts":[[2001,7]]}},"alternative-id":["10.1142\/S0218810401000424"],"URL":"https:\/\/doi.org\/10.1142\/s0218810401000424","relation":{},"ISSN":["0218-8104","1793-6535"],"issn-type":[{"value":"0218-8104","type":"print"},{"value":"1793-6535","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,7]]}}}