Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study
Background: The higher life expectancy in patients with hemophilia (PWH) due to better care has increased the incidence of age-related complications such as osteoporosis. This study was conducted to assess the bone mineral density (BMD) and its correlation with determinants of hemophilic arthropathy...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-09-01
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| Series: | Pediatric Hematology Oncology Journal |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468124522002157 |
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| author | Govind R. Patel Renu Saxena Sadhna Aggarwal Manoranjan Mahapatra |
| author_facet | Govind R. Patel Renu Saxena Sadhna Aggarwal Manoranjan Mahapatra |
| author_sort | Govind R. Patel |
| collection | DOAJ |
| description | Background: The higher life expectancy in patients with hemophilia (PWH) due to better care has increased the incidence of age-related complications such as osteoporosis. This study was conducted to assess the bone mineral density (BMD) and its correlation with determinants of hemophilic arthropathy and to evaluate the effect of type of factor replacement therapy on BMD in Indian hemophilic children. Methods: This study was conducted on 39 hemophilic children and 40 healthy controls. Total range-of-motion (ROM) score was calculated for major affected joints and BMD was measured by dual-energy x-ray absorptiometry at baseline and repeated at follow-up after 6 months period during which all patients received either prophylaxis or on-demand therapy. Results: BMD and BMD Z-scores were significantly reduced in hemophiliacs as compared to controls (p < 0.05). The prevalence of low BMD in hemophiliacs ranged from 30.8 to 41% at various sites. A significant correlation was found between BMD Z-score and episodes of joint bleed, number of target joints, and total joint ROM score (p < 0.05). BMD Z-score showed a significant difference between the prophylaxis group and on-demand therapy group at baseline, and a significant increase in BMD Z-score was observed among patients in prophylaxis group at follow-up after 6 months compared to baseline (p < 0.05) but not among patients in on-demand therapy group. Conclusions: Hemophilic children showed reduced BMD that correlated with hemophilic arthropathy. Prophylaxis, as compared to on-demand therapy, markedly decreased hemarthrosis and consequent immobilization, and thereby retarded the progression of bone mineral loss and improved BMD in these patients. |
| first_indexed | 2024-04-12T23:14:14Z |
| format | Article |
| id | doaj.art-b4e99722c189487f9b63baaf8dc60f67 |
| institution | Directory Open Access Journal |
| issn | 2468-1245 |
| language | English |
| last_indexed | 2024-04-12T23:14:14Z |
| publishDate | 2022-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatric Hematology Oncology Journal |
| spelling | doaj.art-b4e99722c189487f9b63baaf8dc60f672022-12-22T03:12:44ZengElsevierPediatric Hematology Oncology Journal2468-12452022-09-01736773Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational studyGovind R. Patel0Renu Saxena1Sadhna Aggarwal2Manoranjan Mahapatra3Department of Hematology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India; Corresponding author. Department of Hematology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.Department of Hematology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, IndiaDepartment of Biotechnology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, IndiaDepartment of Hematology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, IndiaBackground: The higher life expectancy in patients with hemophilia (PWH) due to better care has increased the incidence of age-related complications such as osteoporosis. This study was conducted to assess the bone mineral density (BMD) and its correlation with determinants of hemophilic arthropathy and to evaluate the effect of type of factor replacement therapy on BMD in Indian hemophilic children. Methods: This study was conducted on 39 hemophilic children and 40 healthy controls. Total range-of-motion (ROM) score was calculated for major affected joints and BMD was measured by dual-energy x-ray absorptiometry at baseline and repeated at follow-up after 6 months period during which all patients received either prophylaxis or on-demand therapy. Results: BMD and BMD Z-scores were significantly reduced in hemophiliacs as compared to controls (p < 0.05). The prevalence of low BMD in hemophiliacs ranged from 30.8 to 41% at various sites. A significant correlation was found between BMD Z-score and episodes of joint bleed, number of target joints, and total joint ROM score (p < 0.05). BMD Z-score showed a significant difference between the prophylaxis group and on-demand therapy group at baseline, and a significant increase in BMD Z-score was observed among patients in prophylaxis group at follow-up after 6 months compared to baseline (p < 0.05) but not among patients in on-demand therapy group. Conclusions: Hemophilic children showed reduced BMD that correlated with hemophilic arthropathy. Prophylaxis, as compared to on-demand therapy, markedly decreased hemarthrosis and consequent immobilization, and thereby retarded the progression of bone mineral loss and improved BMD in these patients.http://www.sciencedirect.com/science/article/pii/S2468124522002157HemophiliaBone mineral densityOsteoporosisHemophilic arthropathyFactor replacement therapy |
| spellingShingle | Govind R. Patel Renu Saxena Sadhna Aggarwal Manoranjan Mahapatra Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study Pediatric Hematology Oncology Journal Hemophilia Bone mineral density Osteoporosis Hemophilic arthropathy Factor replacement therapy |
| title | Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study |
| title_full | Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study |
| title_fullStr | Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study |
| title_full_unstemmed | Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study |
| title_short | Evaluation of bone mineral density (BMD) and the effect of type of factor replacement therapy on BMD in children with hemophilia: A single-center observational study |
| title_sort | evaluation of bone mineral density bmd and the effect of type of factor replacement therapy on bmd in children with hemophilia a single center observational study |
| topic | Hemophilia Bone mineral density Osteoporosis Hemophilic arthropathy Factor replacement therapy |
| url | http://www.sciencedirect.com/science/article/pii/S2468124522002157 |
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