Cargando…

Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests

Hypophosphatasia (HPP) is an inborn disease that causes a rare form of osteomalacia, a mineralization disorder affecting mineralized tissues. Identification of patients at high risk for fractures or other skeletal manifestations (such as insufficiency fractures or excessive bone marrow edema) by bon...

Descripción completa

Detalles Bibliográficos
Autores principales: Schmidt, Felix N., Schmidt, Constantin, Delsmann, Julian, Amling, Michael, Barvencik, Florian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10198909/
https://www.ncbi.nlm.nih.gov/pubmed/37147467
http://dx.doi.org/10.1007/s00223-023-01082-3
_version_ 1785044825242861568
author Schmidt, Felix N.
Schmidt, Constantin
Delsmann, Julian
Amling, Michael
Barvencik, Florian
author_facet Schmidt, Felix N.
Schmidt, Constantin
Delsmann, Julian
Amling, Michael
Barvencik, Florian
author_sort Schmidt, Felix N.
collection PubMed
description Hypophosphatasia (HPP) is an inborn disease that causes a rare form of osteomalacia, a mineralization disorder affecting mineralized tissues. Identification of patients at high risk for fractures or other skeletal manifestations (such as insufficiency fractures or excessive bone marrow edema) by bone densitometry and laboratory tests remains clinically challenging. Therefore, we examined two cohorts of patients with variants in the ALPL gene grouped by bone manifestations. These groups were compared by means of bone microarchitecture using high-resolution peripheral quantitative computed tomography (HR-pQCT) and simulated mechanical performance utilizing finite element analysis (FEA). Whereas the incidence of skeletal manifestations among the patients could not be determined by dual energy X-ray absorptiometry (DXA) or laboratory assessment, HR-pQCT evaluation showed a distinct pattern of HPP patients with such manifestations. Specifically, these patients had a pronounced loss of trabecular bone mineral density, increased trabecular spacing, and decreased ultimate force at the distal radius. Interestingly, the derived results indicate that the non-weight-bearing radius is superior to the weight-bearing tibia in identifying deteriorated skeletal patterns. Overall, the assessment by HR-pQCT appears to be of high clinical relevance due to the improved identification of HPP patients with an increased risk for fractures or other skeletal manifestations, especially at the distal radius. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00223-023-01082-3.
format Online
Article
Text
id pubmed-10198909
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Springer US
record_format MEDLINE/PubMed
spelling pubmed-101989092023-05-21 Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests Schmidt, Felix N. Schmidt, Constantin Delsmann, Julian Amling, Michael Barvencik, Florian Calcif Tissue Int Original Research Hypophosphatasia (HPP) is an inborn disease that causes a rare form of osteomalacia, a mineralization disorder affecting mineralized tissues. Identification of patients at high risk for fractures or other skeletal manifestations (such as insufficiency fractures or excessive bone marrow edema) by bone densitometry and laboratory tests remains clinically challenging. Therefore, we examined two cohorts of patients with variants in the ALPL gene grouped by bone manifestations. These groups were compared by means of bone microarchitecture using high-resolution peripheral quantitative computed tomography (HR-pQCT) and simulated mechanical performance utilizing finite element analysis (FEA). Whereas the incidence of skeletal manifestations among the patients could not be determined by dual energy X-ray absorptiometry (DXA) or laboratory assessment, HR-pQCT evaluation showed a distinct pattern of HPP patients with such manifestations. Specifically, these patients had a pronounced loss of trabecular bone mineral density, increased trabecular spacing, and decreased ultimate force at the distal radius. Interestingly, the derived results indicate that the non-weight-bearing radius is superior to the weight-bearing tibia in identifying deteriorated skeletal patterns. Overall, the assessment by HR-pQCT appears to be of high clinical relevance due to the improved identification of HPP patients with an increased risk for fractures or other skeletal manifestations, especially at the distal radius. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00223-023-01082-3. Springer US 2023-05-06 2023 /pmc/articles/PMC10198909/ /pubmed/37147467 http://dx.doi.org/10.1007/s00223-023-01082-3 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Research
Schmidt, Felix N.
Schmidt, Constantin
Delsmann, Julian
Amling, Michael
Barvencik, Florian
Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title_full Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title_fullStr Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title_full_unstemmed Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title_short Radial HR-pQCT and Finite Element Analysis in HPP Patients are Superior in Identifying Susceptibility to Fracture-Associated Skeletal Affections Compared to DXA and Laboratory Tests
title_sort radial hr-pqct and finite element analysis in hpp patients are superior in identifying susceptibility to fracture-associated skeletal affections compared to dxa and laboratory tests
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10198909/
https://www.ncbi.nlm.nih.gov/pubmed/37147467
http://dx.doi.org/10.1007/s00223-023-01082-3
work_keys_str_mv AT schmidtfelixn radialhrpqctandfiniteelementanalysisinhpppatientsaresuperiorinidentifyingsusceptibilitytofractureassociatedskeletalaffectionscomparedtodxaandlaboratorytests
AT schmidtconstantin radialhrpqctandfiniteelementanalysisinhpppatientsaresuperiorinidentifyingsusceptibilitytofractureassociatedskeletalaffectionscomparedtodxaandlaboratorytests
AT delsmannjulian radialhrpqctandfiniteelementanalysisinhpppatientsaresuperiorinidentifyingsusceptibilitytofractureassociatedskeletalaffectionscomparedtodxaandlaboratorytests
AT amlingmichael radialhrpqctandfiniteelementanalysisinhpppatientsaresuperiorinidentifyingsusceptibilitytofractureassociatedskeletalaffectionscomparedtodxaandlaboratorytests
AT barvencikflorian radialhrpqctandfiniteelementanalysisinhpppatientsaresuperiorinidentifyingsusceptibilitytofractureassociatedskeletalaffectionscomparedtodxaandlaboratorytests