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TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease
Background and objective: Bone involvement occurs in 75% of patients with Gaucher disease (GD), and comprises structural changes, debilitating pain, and bone density abnormalities. Osteoporosis is a silent manifestation of GD until a pathologic fracture occurs. Thus, early diagnosis is crucial for i...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8160801/ https://www.ncbi.nlm.nih.gov/pubmed/34065531 http://dx.doi.org/10.3390/jcm10102217 |
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author | Ivanova, Margarita Dao, Julia Noll, Lauren Fikry, Jacqueline Goker-Alpan, Ozlem |
author_facet | Ivanova, Margarita Dao, Julia Noll, Lauren Fikry, Jacqueline Goker-Alpan, Ozlem |
author_sort | Ivanova, Margarita |
collection | PubMed |
description | Background and objective: Bone involvement occurs in 75% of patients with Gaucher disease (GD), and comprises structural changes, debilitating pain, and bone density abnormalities. Osteoporosis is a silent manifestation of GD until a pathologic fracture occurs. Thus, early diagnosis is crucial for identifying high-risk patients in order to prevent irreversible complications. Methods: Thirty-three patients with GD were assessed prospectively to identify predictive markers associated with bone density abnormalities, osteopenia (OSN), and osteoporosis (OSR). Subjects were categorized into three cohorts based on T- or Z-scores of bone mineral density (BMD). The first GD cohort consisted of those with no bone complications (Z-score ≥ −0.9; T-scores ≥ −1), the second was the OSN group (−1.8 ≥ Z-score ≥ −1; −2.5 ≥ T-score ≥ −1), and the third was the OSR group (Z-score ≤ −1.9; T-scores ≤ −2.5). Serum levels of TRAP5b, RANKL, OPG, and RANK were quantified by enzyme-linked immunosorbent assays. Results: TRAP5b levels were increased in GD patients, and showed a positive correlation with GD biomarkers, including plasma glucosylsphingosine (lyso-Gb1) and macrophage activation markers CCL18 and chitotriosidase. The highest level of TRAP5b was measured in patients with osteoporosis. The elevation of RANKL and RANKL/OPG ratio correlated with osteopenia in GD. Conclusion: TRAP5b, RANKL, and RANKL/OPG elevation indicate osteoclast activation in GD. TRAP5b is a potential bone biomarker for GD with the ability to predict the progression of bone density abnormalities. |
format | Online Article Text |
id | pubmed-8160801 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-81608012021-05-29 TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease Ivanova, Margarita Dao, Julia Noll, Lauren Fikry, Jacqueline Goker-Alpan, Ozlem J Clin Med Article Background and objective: Bone involvement occurs in 75% of patients with Gaucher disease (GD), and comprises structural changes, debilitating pain, and bone density abnormalities. Osteoporosis is a silent manifestation of GD until a pathologic fracture occurs. Thus, early diagnosis is crucial for identifying high-risk patients in order to prevent irreversible complications. Methods: Thirty-three patients with GD were assessed prospectively to identify predictive markers associated with bone density abnormalities, osteopenia (OSN), and osteoporosis (OSR). Subjects were categorized into three cohorts based on T- or Z-scores of bone mineral density (BMD). The first GD cohort consisted of those with no bone complications (Z-score ≥ −0.9; T-scores ≥ −1), the second was the OSN group (−1.8 ≥ Z-score ≥ −1; −2.5 ≥ T-score ≥ −1), and the third was the OSR group (Z-score ≤ −1.9; T-scores ≤ −2.5). Serum levels of TRAP5b, RANKL, OPG, and RANK were quantified by enzyme-linked immunosorbent assays. Results: TRAP5b levels were increased in GD patients, and showed a positive correlation with GD biomarkers, including plasma glucosylsphingosine (lyso-Gb1) and macrophage activation markers CCL18 and chitotriosidase. The highest level of TRAP5b was measured in patients with osteoporosis. The elevation of RANKL and RANKL/OPG ratio correlated with osteopenia in GD. Conclusion: TRAP5b, RANKL, and RANKL/OPG elevation indicate osteoclast activation in GD. TRAP5b is a potential bone biomarker for GD with the ability to predict the progression of bone density abnormalities. MDPI 2021-05-20 /pmc/articles/PMC8160801/ /pubmed/34065531 http://dx.doi.org/10.3390/jcm10102217 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Ivanova, Margarita Dao, Julia Noll, Lauren Fikry, Jacqueline Goker-Alpan, Ozlem TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title | TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title_full | TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title_fullStr | TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title_full_unstemmed | TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title_short | TRAP5b and RANKL/OPG Predict Bone Pathology in Patients with Gaucher Disease |
title_sort | trap5b and rankl/opg predict bone pathology in patients with gaucher disease |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8160801/ https://www.ncbi.nlm.nih.gov/pubmed/34065531 http://dx.doi.org/10.3390/jcm10102217 |
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