{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T02:29:13Z","timestamp":1783477753139,"version":"3.55.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,10,1]]},"abstract":"<jats:sec>\n<jats:title>Purpose<\/jats:title>\n<jats:p>To examine the MRI diagnostic performance in the assessment of therapeutic response to burosumab in children with X-linked hypophosphatemia (XLH).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>Prospective longitudinal open cohort.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Patients<\/jats:title>\n<jats:p>Seventeen children with XLH, average age of 10.2 \u00b1 2.7 years, had a knee MRI at baseline and after 1 year of burosumab.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Intervention<\/jats:title>\n<jats:p>Children received burosumab at an average dose of 1.4 \u00b1 0.5 mg\/kg during 1 year for the treatment of severe rickets (the target serum phosphate \u2265 1.2 mmol\/L (\u22653.7 mg\/dL). The primary endpoint was the change from baseline to 12 months in rachitic lesions on knee MRI. Secondary endpoints were changes in biochemical parameters of phosphate and alkaline phosphatase (ALP).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>One year of treatment with burosumab significantly reduced radiological disease activity on knee MRI (by 44 \u00b1 29% in the transverse extent of widening) which was accompanied by a significant reduction in biochemical activity, namely in serum ALP activity, by 28 \u00b1 17%. Additionally, MRI parameters after 1 year of treatment with burosumab (the maximum width of medial physis at 12 months and the change from baseline in the maximum width of lateral physis) were associated with ALP activity at 12 months.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>We suggest that MRI is a valuable and quantitative tool to evaluate the therapeutic response to burosumab. MRI could be an excellent alternative to standard bone radiographs for evaluation of the rachitic lesions in a clinical setting avoiding repeated exposition to ionizing radiation.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-21-0429","type":"journal-article","created":{"date-parts":[[2021,7,22]],"date-time":"2021-07-22T14:53:44Z","timestamp":1626965624000},"page":"475-484","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":8,"title":["Magnetic resonance imaging is a valuable tool to evaluate the therapeutic efficacy of burosumab in children with X-linked hypophosphatemia"],"prefix":"10.1093","volume":"185","author":[{"given":"Volha V","family":"Zhukouskaya","sequence":"first","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"2Genethon, Paris-Saclay University, Univ Evry, Inserm, Integrare research unit UMR_S951, Evry, France"},{"name":"3Division of Endocrinology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy"},{"name":"8Universit\u00e9 de Paris, Institut des maladies musculo-squelettiques, Laboratory Orofacial Pathologies, Imaging and Biotherapies URP2496 and FHU-DDS-Net, Dental School, and Plateforme d\u2019Imagerie du Vivant (PIV), Montrouge, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00e8s","family":"Mannes","sequence":"additional","affiliation":[{"name":"4AP-HP, Department of Pediatric Radiology, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Chaussain","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"6AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Dental Medicine Department, Bretonneau Hospital, GHN-Universit\u00e9 de Paris, Paris, France"},{"name":"7Universit\u00e9 de Paris, Paris, France"},{"name":"8Universit\u00e9 de Paris, Institut des maladies musculo-squelettiques, Laboratory Orofacial Pathologies, Imaging and Biotherapies URP2496 and FHU-DDS-Net, Dental School, and Plateforme d\u2019Imagerie du Vivant (PIV), Montrouge, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1994-4226","authenticated-orcid":true,"given":"Peter","family":"Kamenick\u00fd","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"9AP-HP, Department of Endocrinology and Reproductive Diseases, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christelle","family":"Audrain","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Sophie","family":"Lambert","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"10AP-HP, Departments of Endocrinology and Diabetology for Children and Adolescent Medicine, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e9r\u00f4me","family":"Nevoux","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"11AP-HP, Department of ORL, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Wicart","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"7Universit\u00e9 de Paris, Paris, France"},{"name":"12AP-HP, Department of Pediatric Orthopaedic Surgery, Necker \u2013 Sick Kids University Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karine","family":"Briot","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"7Universit\u00e9 de Paris, Paris, France"},{"name":"13AP-HP, Department of Rheumatology Hospital Cochin, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Di Rocco","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"14Pediatric Neurosurgery, Hospital Femme Mere Enfant, Hospices Civiles de Lyon and University Claude Bernard Lyon, Bron Cedex, France"},{"name":"15Reference Center for Craniosynostosis, INSERM 1033, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9verine","family":"Trabado","sequence":"additional","affiliation":[{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"16AP-HP, Department of Molecular Genetics, Pharmacogenetics and Hormonology, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Pri\u00e9","sequence":"additional","affiliation":[{"name":"7Universit\u00e9 de Paris, Paris, France"},{"name":"17AP-HP, H\u00f4pital Necker Enfants Malades, INSERM U1151, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Di Somma","sequence":"additional","affiliation":[{"name":"3Division of Endocrinology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Colao","sequence":"additional","affiliation":[{"name":"3Division of Endocrinology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Adamsbaum","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"4AP-HP, Department of Pediatric Radiology, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anya","family":"Rothenbuhler","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"10AP-HP, Departments of Endocrinology and Diabetology for Children and Adolescent Medicine, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agn\u00e8s","family":"Linglart","sequence":"additional","affiliation":[{"name":"1AP-HP, Reference Center for Rare Disorders of the Calcium and Phosphate Metabolism, Fili\u00e8re OSCAR, EndoRare, BOND ERN and Platform of Expertise for Rare Diseases Paris-Saclay, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"5University Paris Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"10AP-HP, Departments of Endocrinology and Diabetology for Children and Adolescent Medicine, Bic\u00eatre Paris-Saclay Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","container-title":["European Journal of 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