{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T06:33:06Z","timestamp":1792477986396,"version":"4.5.0"},"reference-count":0,"publisher":"Bioscientifica","issue":"8","license":[{"start":{"date-parts":[[2017,11,1]],"date-time":"2017-11-01T00:00:00Z","timestamp":1509494400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["ec.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,11]]},"abstract":"\n Background<\/jats:title>\n \n X-linked hypophosphatemic rickets (XLHR) is due to mutations in\n PHEX<\/jats:italic>\n leading to unregulated production of FGF23 and hypophosphatemia. XLHR is characterized by leg bowing of variable severity. Phosphate supplements and oral vitamin analogs, partially or, in some cases, fully restore the limb straightness. Surgery is the alternative for severe or residual limb deformities.\n <\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To retrospectively assess the results of surgical limb correction in XLHR (osteotomies and bone alignment except for 3 transient hemiepiphysiodesis).<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n We analyzed the incidence of recurrence and post-surgical complications in 49 XLHR patients (29F, 20M) (mean age at diagnosis 6.0 years (\u00b1\u20097.1)).<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n At first surgery, the mean age was 13.4 years (\u00b1\u20095.0). Recurrence was observed in 14\/49 (29%) patients. The number of additional operations significantly decreased with age (2.0 (\u00b1\u20090.9), 1.7 (\u00b1\u20091.0) and 1.2 (\u00b1\u20090.4) in children <11 years, between 11 and 15, and >15 years;\n P<\/jats:italic>\n \u2009<\u20090.001). Incidence of recurrence seemed to be lower in patients with good metabolic control of the rickets (25% vs 33%). Complications were observed in 57% of patients.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n We report a large series of surgical procedures in XLHR. Our results confirm that phosphate supplements and vitamin D analog therapy is the first line of treatment to correct leg bowing. Surgery before puberty is associated with a high risk of recurrence of the limb deformity. Such procedures should only be recommended, following multidisciplinary discussions, in patients with severe distortion leading to mechanical joint and ligament complications, or for residual deformities once growth plates have fused.<\/jats:p>\n <\/jats:sec>","DOI":"10.1530\/ec-17-0154","type":"journal-article","created":{"date-parts":[[2017,9,27]],"date-time":"2017-09-27T20:25:26Z","timestamp":1506543926000},"page":"566-573","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-10","source":"Crossref","is-referenced-by-count":55,"title":["Outcomes of orthopedic surgery in a cohort of 49 patients with X-linked hypophosphatemic rickets (XLHR)"],"prefix":"10.1530","volume":"6","author":[{"given":"A","family":"Gizard","sequence":"first","affiliation":[{"name":"1Department of Pediatric Orthopedic Surgery, Besan\u00e7on University Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Rothenbuhler","sequence":"additional","affiliation":[{"name":"2APHP, Department of Pediatric Endocrinology, Bic\u00eatre Paris Sud, Le Kremlin Bic\u00eatre, France"},{"name":"3Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, Le Kremlin Bic\u00eatre, France"},{"name":"4Plateforme d\u2019Expertise Paris Sud Maladies Rares and Fili\u00e8re OSCAR, Bic\u00eatre Paris Sud, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z","family":"Pejin","sequence":"additional","affiliation":[{"name":"5APHP, Department of Pediatric Orthopedic Surgery, Necker Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Finidori","sequence":"additional","affiliation":[{"name":"5APHP, Department of Pediatric Orthopedic Surgery, Necker Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C","family":"Glorion","sequence":"additional","affiliation":[{"name":"5APHP, Department of Pediatric Orthopedic Surgery, Necker Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B","family":"de Billy","sequence":"additional","affiliation":[{"name":"1Department of Pediatric Orthopedic Surgery, Besan\u00e7on University Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Linglart","sequence":"additional","affiliation":[{"name":"2APHP, Department of Pediatric Endocrinology, Bic\u00eatre Paris Sud, Le Kremlin Bic\u00eatre, France"},{"name":"3Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, Le Kremlin Bic\u00eatre, France"},{"name":"4Plateforme d\u2019Expertise Paris Sud Maladies Rares and Fili\u00e8re OSCAR, Bic\u00eatre Paris Sud, Le Kremlin Bic\u00eatre, France"},{"name":"6INSERM U1169, H\u00f4pital Bic\u00eatre, Le Kremlin Bic\u00eatre, et Universit\u00e9 Paris-Saclay, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Wicart","sequence":"additional","affiliation":[{"name":"3Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, Le Kremlin Bic\u00eatre, France"},{"name":"5APHP, Department of Pediatric Orthopedic Surgery, Necker Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","container-title":["Endocrine Connections"],"original-title":[],"link":[{"URL":"https:\/\/ec.bioscientifica.com\/view\/journals\/ec\/6\/8\/EC-17-0154.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/ec.bioscientifica.com\/downloadpdf\/journals\/ec\/6\/8\/EC-17-0154.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:42:20Z","timestamp":1784554940000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/ec\/article\/6\/8\/566-573\/35310"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,11]]},"references-count":0,"journal-issue":{"issue":"8"},"URL":"https:\/\/doi.org\/10.1530\/ec-17-0154","relation":{},"ISSN":["2049-3614"],"issn-type":[{"value":"2049-3614","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,11]]}}}