{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T23:49:52Z","timestamp":1793836192667,"version":"4.6.1"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2024,8,16]],"date-time":"2024-08-16T00:00:00Z","timestamp":1723766400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100013220","name":"Ultragenyx Pharmaceutical Inc.","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013220","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>In patients with X-linked hypophosphatemia (XLH), conventional therapy with oral phosphate salts and active vitamin D has been associated with nephrocalcinosis. However, the nature of the relationships among XLH, its treatment, nephrocalcinosis, and kidney function remain poorly understood.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Renal ultrasounds were performed and glomerular filtration rates were estimated (eGFR) at baseline in burosumab-na\u00efve patients with XLH who participated in burosumab clinical trials (NCT02181764, NCT02526160, NCT02537431, NCT02163577, NCT02750618, NCT02915705) or enrolled in the XLH Disease Monitoring Program (XLH-DMP; NCT03651505). In this cross-sectional analysis, patient, disease, and treatment characteristics were described among patients with and without nephrocalcinosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>The analysis included 196 children (mean [SD] age 7.6 [4.0] yr) and 318 adults (40.3 [13.1] yr). Mean (SD) height z-score was \u22121.9 (1.2) for children and \u22122.3 (1.7) for adults. Nearly all children (97%) and adults (94%) had previously received conventional therapy. Nephrocalcinosis was detected in 22% of children and 38% of adults. In children, reduced eGFR &amp;lt;90\u00a0mL\/min\/1.73\u00a0m2 was more prevalent in those with nephrocalcinosis (25%) than in those without (11%), a finding that was not observed in adults. Children with nephrocalcinosis had lower mean values of TmP\/GFR (p&amp;lt;.05), serum 1,25(OH)2D (p&amp;lt;.05), and eGFR (p&amp;lt;.001) and higher mean serum calcium concentrations (p&amp;lt;.05) than did those without nephrocalcinosis. Adults with nephrocalcinosis had lower mean serum phosphorus (p&amp;lt;.01) and 1,25(OH)2D (p&amp;lt;.05) concentrations than those without. Exploratory logistic regression analyses revealed no significant associations between the presence of nephrocalcinosis and other described patient or disease characteristics.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Nephrocalcinosis was observed in nearly one-quarter of children and more than one-third of adults with XLH. Further study is needed to better understand the predictors and long-term consequences of nephrocalcinosis, with surveillance for nephrocalcinosis remaining important in the management of XLH.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/jbmr\/zjae127","type":"journal-article","created":{"date-parts":[[2024,8,16]],"date-time":"2024-08-16T18:23:53Z","timestamp":1723832633000},"page":"1493-1502","source":"Crossref","is-referenced-by-count":19,"title":["Nephrocalcinosis and kidney function in children and adults with X-linked hypophosphatemia: baseline results from a large longitudinal study"],"prefix":"10.1093","volume":"39","author":[{"given":"Anthony A","family":"Portale","sequence":"first","affiliation":[{"name":"Division of Pediatric Nephrology, University of California San Francisco Benioff Children\u2019s Hospital , San Francisco, CA 94158,","place":["United 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Santiago,","place":["Chile"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven W","family":"Ing","sequence":"additional","affiliation":[{"name":"Division of Endocrinology , Diabetes, and Metabolism, , Columbus, OH 43210,","place":["United States"]},{"name":"Ohio State University Wexner Medical Center , Diabetes, and Metabolism, , Columbus, OH 43210,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne M","family":"Jan de Beur","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism , Department of Medicine, , Charlotteville, VA 22903,","place":["United States"]},{"name":"University of Virginia School of Medicine , Department of Medicine, , Charlotteville, VA 22903,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Regina M","family":"Martin","sequence":"additional","affiliation":[{"name":"Hospital das Cl\u00ednicas da Faculdade de Medicina da Universidade de S\u00e3o Paulo , Sao Paulo 05403-010,","place":["Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana I","family":"Meza-Martinez","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario de San Jos\u00e9 , 111221 Bogota DC,","place":["Colombia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Paloian","sequence":"additional","affiliation":[{"name":"University of Wisconsin School of Medicine and Public Health , Madison, WI 53726,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ambika","family":"Ashraf","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham , Birmingham, AL 35294,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley P","family":"Dixon","sequence":"additional","affiliation":[{"name":"Renal Section , Department of Pediatrics, , Aurora, CO 80045,","place":["United States"]},{"name":"University of Colorado School of Medicine , Department of Pediatrics, , Aurora, CO 80045,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3733-8956","authenticated-orcid":false,"given":"Aliya","family":"Khan","sequence":"additional","affiliation":[{"name":"McMaster University , Hamilton, Ontario L8S 4L8,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig","family":"Langman","sequence":"additional","affiliation":[{"name":"Emeritus Professor of Pediatrics, Northwestern Feinberg School of Medicine , Chicago, IL 60611,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angel","family":"Chen","sequence":"additional","affiliation":[{"name":"Ultragenyx Pharmaceutical Inc. , Novato, CA 94949,","place":["United 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