{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T06:29:18Z","timestamp":1790231358397,"version":"4.1.0"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2024,1,25]],"date-time":"2024-01-25T00:00:00Z","timestamp":1706140800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Science and Innovation Fund of VieCuri Medical Center"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,4,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Data on bone microarchitecture in osteogenesis imperfecta (OI) are scarce. The aim of this cross-sectional study was to assess bone microarchitecture and strength in a large cohort of adults with OI using high-resolution peripheral quantitative computed tomography (HR-pQCT) and to evaluate challenges of using HR-pQCT in this cohort. Second-generation HR-pQCT scans were obtained at the distal radius and tibia in 118 men and women with Sillence OI type I, III, or IV using an extremity-length-dependent scan protocol. In total, 102 radius and 105 tibia scans of sufficient quality could be obtained, of which 11 radius scans (11%) and 14 tibia scans (13%) had a deviated axial scan angle as compared with axial angle data of 13 young women. In the scans without a deviated axial angle and compared with normative HR-pQCT data, Z-scores at the radius for trabecular bone mineral density (BMD), number, and separation were \u22121.6\u2009\u00b1\u20091.3, \u22122.5\u2009\u00b1\u20091.4, and \u22122.7 (IQR: 2.7), respectively. They were \u22121.4\u2009\u00b1\u20091.5 and \u22121.1\u2009\u00b1\u20091.2 for stiffness and failure load and between \u00b11 for trabecular thickness and cortical bone parameters. Z-scores were significantly lower for total and trabecular BMD, stiffness, failure load, and cortical area and thickness at the tibia. Additionally, local microarchitectural inhomogeneities were observed, most pronounced being trabecular void volumes. In the scans with a deviated axial angle, the proportion of Z-scores &amp;lt;\u22124 or\u2009&amp;gt;4 was significantly higher for trabecular BMD and separation (radius) or most total and trabecular bone parameters (tibia). To conclude, especially trabecular bone microarchitecture and bone strength were impaired in adults with OI. HR-pQCT may be used without challenges in most adults with OI, but approximately 12% of the scans may have a deviated axial angle in OI due to bone deformities or scan positioning limitations. Furthermore, standard HR-pQCT parameters may not always be reliable due to microarchitectural inhomogeneities nor fully reflect all inhomogeneities.<\/jats:p>","DOI":"10.1093\/jbmr\/zjae013","type":"journal-article","created":{"date-parts":[[2024,1,25]],"date-time":"2024-01-25T15:18:42Z","timestamp":1706195922000},"page":"271-286","source":"Crossref","is-referenced-by-count":12,"title":["Bone microarchitecture and strength assessment in adults with osteogenesis imperfecta using HR-pQCT: normative comparison and challenges"],"prefix":"10.1093","volume":"39","author":[{"given":"M S A M","family":"Bevers","sequence":"first","affiliation":[{"name":"Department of Internal Medicine , VieCuri Medical Center, Tegelseweg 210, 5912 BL Venlo , The Netherlands"},{"name":"NUTRIM School for Nutrition and Translational Research In Metabolism, Maastricht University Medical Center , Minderbroedersberg 4-6, 6211 LK, Maastricht , The Netherlands"},{"name":"Department of Biomedical Engineering, Eindhoven University of Technology , Groene Loper 3, 5612 AE, Eindhoven , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A G J","family":"Harsevoort","sequence":"additional","affiliation":[{"name":"Expert Center for adults with Osteogenesis Imperfecta, Isala , Dokter van Heesweg 2, 8025 AB, Zwolle , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K","family":"Gooijer","sequence":"additional","affiliation":[{"name":"Expert Center for adults with Osteogenesis Imperfecta, Isala , Dokter van Heesweg 2, 8025 AB, Zwolle , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C E","family":"Wyers","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine , VieCuri Medical Center, Tegelseweg 210, 5912 BL Venlo , The Netherlands"},{"name":"NUTRIM School for Nutrition and Translational Research In Metabolism, Maastricht University Medical Center , Minderbroedersberg 4-6, 6211 LK, Maastricht , The Netherlands"},{"name":"Department of Internal Medicine , Subdivision of Rheumatology, , P. Debyelaan 25, 6229 HX, Maastricht , The Netherlands"},{"name":"Maastricht University Medical Center , Subdivision of Rheumatology, , P. Debyelaan 25, 6229 HX, Maastricht , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Feenstra","sequence":"additional","affiliation":[{"name":"Expert Center for adults with Osteogenesis Imperfecta, Isala , Dokter van Heesweg 2, 8025 AB, Zwolle , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B","family":"van Rietbergen","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering, Eindhoven University of Technology , Groene Loper 3, 5612 AE, Eindhoven , The Netherlands"},{"name":"Department of Orthopedic Surgery, Maastricht University Medical Center , P. Debyelaan 25, 6229 HX, Maastricht , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M F","family":"Boomsma","sequence":"additional","affiliation":[{"name":"Department of Radiology , Isala, Dokter van Heesweg 2, 8025 AB, Zwolle , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J P","family":"van den Bergh","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine , VieCuri Medical Center, Tegelseweg 210, 5912 BL Venlo , The Netherlands"},{"name":"NUTRIM School for Nutrition and Translational Research In Metabolism, Maastricht University Medical Center , Minderbroedersberg 4-6, 6211 LK, Maastricht , The Netherlands"},{"name":"Department of Internal Medicine , Subdivision of Rheumatology, , P. Debyelaan 25, 6229 HX, Maastricht , The Netherlands"},{"name":"Maastricht University Medical Center , Subdivision of Rheumatology, , P. Debyelaan 25, 6229 HX, Maastricht , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G J M","family":"Janus","sequence":"additional","affiliation":[{"name":"Expert Center for adults with Osteogenesis Imperfecta, Isala , Dokter van Heesweg 2, 8025 AB, Zwolle , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,1,25]]},"reference":[{"issue":"10028","key":"2024041923054689600_ref1","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1016\/S0140-6736(15)00728-X","article-title":"Osteogenesis imperfecta","volume":"387","author":"Forlino","year":"2016","journal-title":"Lancet"},{"issue":"1","key":"2024041923054689600_ref2","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1093\/nar\/26.1.253","article-title":"The human collagen mutation database 1998","volume":"26","author":"Dalgleish","year":"1998","journal-title":"Nucleic Acids Res"},{"issue":"5","key":"2024041923054689600_ref3","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1002\/humu.21438","article-title":"LOVD v. 2.0: the next generation in gene variant databases","volume":"32","author":"Fokkema","year":"2011","journal-title":"Hum Mutat"},{"issue":"6","key":"2024041923054689600_ref4","doi-asserted-by":"crossref","first-page":"1470","DOI":"10.1002\/ajmg.a.36545","article-title":"Osteogenesis imperfecta: clinical diagnosis, nomenclature and severity assessment","volume":"164","author":"Van Dijk","year":"2014","journal-title":"Am J Med Genet A"},{"issue":"1","key":"2024041923054689600_ref5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1302\/1863-2548.13.180190","article-title":"Current concepts in osteogenesis imperfecta: bone structure, biomechanics and medical management","volume":"13","author":"Nijhuis","year":"2019","journal-title":"J Child Orthop"},{"issue":"1","key":"2024041923054689600_ref6","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1002\/jbmr.2920","article-title":"Fracture rates and fracture sites in patients with osteogenesis imperfecta: a nationwide register-based cohort study","volume":"32","author":"Folkestad","year":"2017","journal-title":"J Bone Miner Res"},{"issue":"13-14","key":"2024041923054689600_ref7","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s10354-015-0369-2","article-title":"Bone mass and mineralization in osteogenesis imperfecta","volume":"165","author":"Fratzl-Zelman","year":"2015","journal-title":"Wien Med Wochenschr"},{"issue":"4","key":"2024041923054689600_ref8","first-page":"306","article-title":"The bone behind a low areal bone mineral density: peripheral quantitative computed tomographic analysis in a woman with osteogenesis imperfecta","volume":"2","author":"Rauch","year":"2002","journal-title":"J Musculoskelet Neuronal Interact"},{"issue":"2","key":"2024041923054689600_ref9","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1385\/JCD:6:2:173","article-title":"The volumetric bone density and cortical thickness in adult patients affected by osteogenesis imperfecta","volume":"6","author":"Gatti","year":"2003","journal-title":"J Clin Densitom"},{"issue":"10","key":"2024041923054689600_ref10","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1136\/jcp.37.10.1081","article-title":"Iliac bone histomorphometry in adults and children with osteogenesis imperfecta","volume":"37","author":"Ste-Marie","year":"1984","journal-title":"J Clin Pathol"},{"issue":"336","key":"2024041923054689600_ref11","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1097\/00003086-199703000-00034","article-title":"Bone histomorphometry in adults with type IA osteogenesis imperfecta","volume":"336","author":"McCarthy","year":"1997","journal-title":"Clin Orthop Relat Res"},{"issue":"3","key":"2024041923054689600_ref12","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1203\/00006450-198303000-00007","article-title":"Increased bone turnover with decreased bone formation by osteoblasts in children with osteogenesis imperfecta tarda","volume":"17","author":"Baron","year":"1983","journal-title":"Pediatr Res"},{"issue":"6","key":"2024041923054689600_ref13","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/S8756-3282(00)00269-6","article-title":"Static and dynamic bone histomorphometry in children with osteogenesis imperfecta","volume":"26","author":"Rauch","year":"2000","journal-title":"Bone"},{"issue":"6","key":"2024041923054689600_ref14","doi-asserted-by":"crossref","first-page":"1405","DOI":"10.1002\/jbmr.1592","article-title":"Bone geometry, density, and microarchitecture in the distal radius and tibia in adults with osteogenesis imperfecta type I assessed by high-resolution pQCT","volume":"27","author":"Folkestad","year":"2012","journal-title":"J Bone Miner Res"},{"issue":"10","key":"2024041923054689600_ref15","doi-asserted-by":"crossref","first-page":"2431","DOI":"10.1007\/s00198-015-3156-4","article-title":"Bone structure assessed by HR-pQCT, TBS and DXL in adult patients with different types of osteogenesis imperfecta","volume":"26","author":"Kocijan","year":"2015","journal-title":"Osteoporos Int"},{"issue":"5","key":"2024041923054689600_ref16","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1007\/s00223-018-0447-8","article-title":"Comparison of bone microarchitecture between adult osteogenesis imperfecta and early-onset osteoporosis","volume":"103","author":"Rolvien","year":"2018","journal-title":"Calcif Tissue Int"},{"issue":"12","key":"2024041923054689600_ref17","first-page":"1781","article-title":"High-resolution peripheral quantitative computed tomography in children with osteogenesis imperfecta","volume":"50","author":"Fennimore","year":"2020","journal-title":"Technol Innov"},{"issue":"3","key":"2024041923054689600_ref18","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1016\/j.jocd.2007.12.013","article-title":"Site-specific variation of bone microarchitecture in the distal radius and tibia","volume":"11","author":"Boyd","year":"2008","journal-title":"J Clin Densitom"},{"issue":"1","key":"2024041923054689600_ref19","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/BF02546396","article-title":"The distribution of cortical and trabecular bone mass along the lengths of the radius and ulna and the implications for in vivo bone mass measurements","volume":"20","author":"Schlenker","year":"1976","journal-title":"Calcif Tissue Res"},{"key":"2024041923054689600_ref20","doi-asserted-by":"crossref","DOI":"10.1016\/j.bone.2021.116282","article-title":"Fabric-elasticity relationships of tibial trabecular bone are similar in osteogenesis imperfecta and healthy individuals","volume":"155","author":"Simon","year":"2022","journal-title":"Bone"},{"issue":"2","key":"2024041923054689600_ref21","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1136\/jmg.16.2.101","article-title":"Genetic heterogeneity in osteogenesis imperfecta","volume":"16","author":"Sillence","year":"1979","journal-title":"J Med Genet"},{"issue":"&NA","key":"2024041923054689600_ref22","first-page":"111","article-title":"Nonoperative treatment of osteogenesis imperfecta: orthotic and mobility management","volume":"159","author":"Bleck","year":"1981","journal-title":"Clin Orthop Relat Res"},{"issue":"3","key":"2024041923054689600_ref23","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1067\/mpd.2000.107892","article-title":"Osteogenesis imperfecta in childhood: prognosis for walking","volume":"137","author":"Engelbert","year":"2000","journal-title":"J Pediatr"},{"issue":"7","key":"2024041923054689600_ref24","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1007\/s00198-017-4010-7","article-title":"The comparability of HR-pQCT bone measurements is improved by scanning anatomically standardized regions","volume":"28","author":"Bonaretti","year":"2017","journal-title":"Osteoporos Int"},{"issue":"1","key":"2024041923054689600_ref25","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.bone.2011.10.003","article-title":"Visual grading of motion induced image degradation in high resolution peripheral computed tomography: impact of image quality on measures of bone density and microarchitecture","volume":"50","author":"Pialat","year":"2012","journal-title":"Bone"},{"issue":"3","key":"2024041923054689600_ref26","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1007\/s00223-018-0416-2","article-title":"Reliability of HR-pQCT derived cortical bone structural parameters when using uncorrected instead of corrected automatically generated endocortical contours in a cross-sectional study: the Maastricht study","volume":"103","author":"De Waard","year":"2018","journal-title":"Calcif Tissue Int"},{"issue":"9","key":"2024041923054689600_ref27","doi-asserted-by":"crossref","first-page":"1607","DOI":"10.1007\/s00198-020-05438-5","article-title":"Guidelines for the assessment of bone density and microarchitecture in vivo using high-resolution peripheral quantitative computed tomography","volume":"31","author":"Whittier","year":"2020","journal-title":"Osteoporos Int"},{"issue":"4","key":"2024041923054689600_ref28","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1007\/s00198-021-06164-2","article-title":"Reference data and calculators for second-generation HR-pQCT measures of the radius and tibia at anatomically standardized regions in white adults","volume":"33","author":"Warden","year":"2022","journal-title":"Osteoporos Int"},{"issue":"6","key":"2024041923054689600_ref29","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1016\/S8756-3282(02)00736-6","article-title":"Estimation of distal radius failure load with micro-finite element analysis models based on three-dimensional peripheral quantitative computed tomography images","volume":"30","author":"Pistoia","year":"2002","journal-title":"Bone"},{"issue":"2","key":"2024041923054689600_ref30","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1007\/s00256-012-1512-4","article-title":"Femoral geometric parameters and BMD measurements by DXA in adult patients with different types of osteogenesis imperfecta","volume":"42","author":"Kocijan","year":"2013","journal-title":"Skeletal Radiol"},{"issue":"4","key":"2024041923054689600_ref31","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1002\/jbmr.2835","article-title":"Bone material properties in osteogenesis imperfecta","volume":"31","author":"Bishop","year":"2016","journal-title":"J Bone Miner Res"},{"key":"2024041923054689600_ref32","doi-asserted-by":"crossref","DOI":"10.1016\/j.bone.2020.115785","article-title":"A new approach for quantifying localized bone loss by measuring void spaces","volume":"143","author":"Whittier","year":"2021","journal-title":"Bone"},{"issue":"11","key":"2024041923054689600_ref33","doi-asserted-by":"crossref","first-page":"3331","DOI":"10.1007\/s00198-016-3653-0","article-title":"Skeletal phenotypes in adult patients with osteogenesis imperfecta\u2014correlations with COL1A1\/COL1A2 genotype and collagen structure","volume":"27","author":"Hald","year":"2016","journal-title":"Osteoporos Int"},{"key":"2024041923054689600_ref34","doi-asserted-by":"crossref","DOI":"10.1016\/j.bone.2021.115880","article-title":"Multisite longitudinal calibration of HR-pQCT scanners and precision in osteogenesis imperfecta","volume":"147","author":"Mikolajewicz","year":"2021","journal-title":"Bone"},{"issue":"5","key":"2024041923054689600_ref35","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1002\/jbmr.4541","article-title":"3D image registration marginally improves the precision of HR-pQCT measurements compared to cross-sectional-area registration in adults with osteogenesis imperfecta","volume":"37","author":"Hosseinitabatabaei","year":"2022","journal-title":"J Bone Miner Res"},{"issue":"6","key":"2024041923054689600_ref36","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1007\/s11914-020-00642-y","article-title":"Ethnic differences in bone microarchitecture","volume":"18","author":"Durdin","year":"2020","journal-title":"Curr Osteoporos Rep"}],"container-title":["Journal of Bone and Mineral Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jbmr\/advance-article-pdf\/doi\/10.1093\/jbmr\/zjae013\/57028573\/zjae013.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jbmr\/article-pdf\/39\/3\/271\/57285086\/zjae013.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jbmr\/article-pdf\/39\/3\/271\/57285086\/zjae013.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,19]],"date-time":"2024-04-19T19:06:36Z","timestamp":1713553596000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jbmr\/article\/39\/3\/271\/7589768"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,25]]},"references-count":36,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2024,1,25]]},"published-print":{"date-parts":[[2024,4,19]]}},"URL":"https:\/\/doi.org\/10.1093\/jbmr\/zjae013","relation":{},"ISSN":["0884-0431","1523-4681"],"issn-type":[{"value":"0884-0431","type":"print"},{"value":"1523-4681","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,3]]},"published":{"date-parts":[[2024,1,25]]}}}