{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T01:21:11Z","timestamp":1790817671261,"version":"4.1.0"},"reference-count":80,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"},{"start":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T00:00:00Z","timestamp":1660089600000},"content-version":"vor","delay-in-days":617,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100013220","name":"Ultragenyx Pharmaceutical Inc.","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013220","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Kyowa Kirin International plc."},{"name":"Austrian Social Health Insurance Fund"},{"DOI":"10.13039\/501100007146","name":"Austrian Social Health Compensation Board","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007146","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013220","name":"Ultragenyx Pharmaceutical","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013220","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007146","name":"Allgemeine Unfallversicherungsanstalt","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007146","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["asbmr.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,12,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>X-linked hypophosphatemia (XLH) is characterized by excess fibroblast growth factor 23 (FGF23) secretion, renal phosphate wasting, and low 1,25(OH)2D3. Adult patients present with osteomalacia, hypomineralized periosteocytic lesions, bone fragility, and pain. Burosumab is a fully human monoclonal FGF23 antibody approved for XLH treatment. UX023-CL304 was an open-label, phase 3 study investigating the effects of burosumab on osteomalacia in adults with XLH, who remained untreated at least 2\u2009years prior enrollment. Here, we present the effect of burosumab on bone material properties. We analyzed transiliac bone biopsy samples from 11 individuals before and after 48\u2009weeks of subcutaneous burosumab treatment (1.0\u2009mg\/kg administered every 4\u2009weeks). We used quantitative backscattered electron imaging (qBEI) and Fourier transform infrared imaging (FTIRI) to assess bone mineralization density distribution (BMDD), mineralized bone volume, properties of the organic matrix, and size of periosteocytic lesions. The outcomes were compared with reference values from healthy adults and with four XLH patients either untreated or treated by conventional therapy. Prior to burosumab, the average mineralization in cancellous bone was lower than in healthy reference. CaLow, the fraction of lowly mineralized matrix, and CaHigh, the fraction of highly mineralized matrix, were both elevated resulting in a broad heterogeneity in mineralization (CaWidth). Burosumab resulted in a decrease of CaHigh toward normal range, whereas CaLow and CaWidth remained elevated. The mineralized bone volume was notably increased (+35.9%). The size of the periosteocytic lesions was variable but lower than in untreated XLH patients. FTIRI indicated decreased enzymatic collagen crosslink ratio heterogeneity. In summary, matrix mineralization in XLH is very heterogeneous. Highly mineralized regions represent old bone packets, probably protected from osteoclastic resorption by osteoid seams. The concomitant decrease of highly mineralized matrix, persistence of lowly mineralized matrix, and increase in mineralized bone volume after burosumab suggest a boost in mineralization of preexisting unmineralized or very lowly mineralized matrix, providing a potential explanation for previously observed improved osteomalacia. \u00a9 2022 American Society for Bone and Mineral Research (ASBMR).<\/jats:p>","DOI":"10.1002\/jbmr.4641","type":"journal-article","created":{"date-parts":[[2022,7,1]],"date-time":"2022-07-01T08:38:45Z","timestamp":1656664725000},"page":"1665-1678","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Bone Matrix Mineralization and Response to Burosumab in Adult Patients With X-Linked Hypophosphatemia: Results From the Phase 3, Single-Arm International Trial"],"prefix":"10.1093","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1164-1877","authenticated-orcid":false,"given":"Nadja","family":"Fratzl-Zelman","sequence":"first","affiliation":[{"name":"Vienna Bone and Growth Center Vienna Austria"},{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6046-0365","authenticated-orcid":false,"given":"Markus A.","family":"Hartmann","sequence":"additional","affiliation":[{"name":"Vienna Bone and Growth Center Vienna Austria"},{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9543-6530","authenticated-orcid":false,"given":"Sonja","family":"Gamsjaeger","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stamatia","family":"Rokidi","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleftherios P.","family":"Paschalis","sequence":"additional","affiliation":[{"name":"Vienna Bone and Growth Center Vienna Austria"},{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6575-8443","authenticated-orcid":false,"given":"St\u00e9phane","family":"Blouin","sequence":"additional","affiliation":[{"name":"Vienna Bone and Growth Center Vienna Austria"},{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Zwerina","sequence":"additional","affiliation":[{"name":"Vienna Bone and Growth Center Vienna Austria"},{"name":"Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling 1st Med Department Hanusch Hospital Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,7,1]]},"reference":[{"issue":"7","key":"2024012713582243700_jbmr4641-bib-0001","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1002\/jbmr.340","article-title":"A clinician's guide to X-linked hypophosphatemia","volume":"26","author":"Carpenter","year":"2011","journal-title":"J Bone Miner Res"},{"issue":"3","key":"2024012713582243700_jbmr4641-bib-0002","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1002\/jbmr.1766","article-title":"Proteolytic processing of osteopontin by PHEX and accumulation of osteopontin fragments in Hyp mouse bone, the murine model of X-linked hypophosphatemia","volume":"28","author":"Barros","year":"2013","journal-title":"J Bone Miner Res"},{"issue":"Suppl 1","key":"2024012713582243700_jbmr4641-bib-0003","doi-asserted-by":"crossref","first-page":"79","DOI":"10.3109\/03008207.2014.923864","article-title":"Abnormal osteopontin 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