A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism
Introduction: Osteopenia and osteoporosis are well-known hemophilia A comorbidities. The pathogenesis of bone turnover alteration resulting in reduced bone mass includes impaired osteoblastic differentiation and disinhibition of RANKL-induced osteoclastogenesis as a result of a low FVIII level.Aim:...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Pensoft Publishers
2023-02-01
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| Series: | Folia Medica |
| Subjects: | |
| Online Access: | https://foliamedica.bg/article/75414/download/pdf/ |
| _version_ | 1811161474900426752 |
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| author | Hristina A. Ivanova Zhanet Grudeva-Popova Tanya Deneva Silvia Tsvetkova Nonka Mateva |
| author_facet | Hristina A. Ivanova Zhanet Grudeva-Popova Tanya Deneva Silvia Tsvetkova Nonka Mateva |
| author_sort | Hristina A. Ivanova |
| collection | DOAJ |
| description | Introduction: Osteopenia and osteoporosis are well-known hemophilia A comorbidities. The pathogenesis of bone turnover alteration resulting in reduced bone mass includes impaired osteoblastic differentiation and disinhibition of RANKL-induced osteoclastogenesis as a result of a low FVIII level.Aim: To evaluate the bone mineral density (BMD) in adult patients with severe hemophilia A and assess a possible correlation with the bone remodeling biomarkers OPG/RANKL, CTX-1, osteocalcin, and Vit D.Materials and methods: 28 male subjects with severe hemophilia A and 33 age-matched controls were recruited. The biomarkers were tested with the ELISA assay and BMD with DEXA of the lumbar spine (LS) and total hip (TH).Results: The patients had lower LS-BMD (−0.955±0.145 vs. 1.118±0.079, p=0.05) and TH-BMD (−0.840±0.147 vs. 0.951±0.075, p=0.05) than those of the controls. The TH T-scores were −1.41±0.91 vs. 0.4±0.49 (p=0.05) and the LS T-scores −1.16±1.046 vs. 0.14±0.72 (p=0.05). 66.6% of patients under 50 years had osteopenia and 8.3% had osteoporosis. Fifty percent of those over 50 years old had osteopenia and 20% had osteoporosis. We found significantly higher OPG levels (123.69±107.05 vs. 41.98±18.95, p=0.05) than that in controls and lower sRANKL levels (23.49±29.39 vs. 131.32±201.27, p=0.05) and sRANKL/OPG ratio (0.27±0.35 vs. 5.28±10.01, p=0.05) than those in controls. A positive correlation was found between sRANKL and the BMD T-score of lumbar spine (p=0.001) in the patient group.Conclusions: sRANKL level and ratio can be used as predictors of low BMD. |
| first_indexed | 2024-04-10T06:14:46Z |
| format | Article |
| id | doaj.art-268e2b17ef4d4399841589839984a66f |
| institution | Directory Open Access Journal |
| issn | 1314-2143 |
| language | English |
| last_indexed | 2024-04-10T06:14:46Z |
| publishDate | 2023-02-01 |
| publisher | Pensoft Publishers |
| record_format | Article |
| series | Folia Medica |
| spelling | doaj.art-268e2b17ef4d4399841589839984a66f2023-03-02T09:11:09ZengPensoft PublishersFolia Medica1314-21432023-02-01651879210.3897/folmed.65.e7541475414A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolismHristina A. Ivanova0Zhanet Grudeva-Popova1Tanya Deneva2Silvia Tsvetkova3Nonka Mateva4St George University HospitalMedical University of PlovdivSt George University HospitalMedical University of PlovdivMedical University of PlovdivIntroduction: Osteopenia and osteoporosis are well-known hemophilia A comorbidities. The pathogenesis of bone turnover alteration resulting in reduced bone mass includes impaired osteoblastic differentiation and disinhibition of RANKL-induced osteoclastogenesis as a result of a low FVIII level.Aim: To evaluate the bone mineral density (BMD) in adult patients with severe hemophilia A and assess a possible correlation with the bone remodeling biomarkers OPG/RANKL, CTX-1, osteocalcin, and Vit D.Materials and methods: 28 male subjects with severe hemophilia A and 33 age-matched controls were recruited. The biomarkers were tested with the ELISA assay and BMD with DEXA of the lumbar spine (LS) and total hip (TH).Results: The patients had lower LS-BMD (−0.955±0.145 vs. 1.118±0.079, p=0.05) and TH-BMD (−0.840±0.147 vs. 0.951±0.075, p=0.05) than those of the controls. The TH T-scores were −1.41±0.91 vs. 0.4±0.49 (p=0.05) and the LS T-scores −1.16±1.046 vs. 0.14±0.72 (p=0.05). 66.6% of patients under 50 years had osteopenia and 8.3% had osteoporosis. Fifty percent of those over 50 years old had osteopenia and 20% had osteoporosis. We found significantly higher OPG levels (123.69±107.05 vs. 41.98±18.95, p=0.05) than that in controls and lower sRANKL levels (23.49±29.39 vs. 131.32±201.27, p=0.05) and sRANKL/OPG ratio (0.27±0.35 vs. 5.28±10.01, p=0.05) than those in controls. A positive correlation was found between sRANKL and the BMD T-score of lumbar spine (p=0.001) in the patient group.Conclusions: sRANKL level and ratio can be used as predictors of low BMD.https://foliamedica.bg/article/75414/download/pdf/osteoporosisFVIII deficiencyRANKLOPG |
| spellingShingle | Hristina A. Ivanova Zhanet Grudeva-Popova Tanya Deneva Silvia Tsvetkova Nonka Mateva A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism Folia Medica osteoporosis FVIII deficiency RANKL OPG |
| title | A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism |
| title_full | A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism |
| title_fullStr | A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism |
| title_full_unstemmed | A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism |
| title_short | A single-center study of bone mineral density in adult patients with severe hemophilia A in correlation with markers of bone metabolism |
| title_sort | single center study of bone mineral density in adult patients with severe hemophilia a in correlation with markers of bone metabolism |
| topic | osteoporosis FVIII deficiency RANKL OPG |
| url | https://foliamedica.bg/article/75414/download/pdf/ |
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