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Impact of Prophylaxis on Bone Mineral Metabolism in Children With Hemophilia
Vildan Culha1, Sibel Akpinar Tekgündüz, Hüsniye N Yarali
1Department of Hematology, Ankara Child Health and Disease, Hematology-Oncology Training and Research Hospital, University of Health Sciences, Ankara, Turkey.
Insights
Hemophilia patients often have low bone mineral density and vitamin D deficiency. While prophylaxis didn't improve these, monitoring bone turnover markers and bone density is recommended for early detection of bone loss.
Area of Science:
- Endocrinology
- Hematology
- Bone Metabolism
Background:
- Calcium (Ca) metabolism is crucial for bone health.
- Hemophilia patients (PWH) may face unique challenges affecting bone metabolism.
- Early diagnosis and management of metabolic factors are vital.
Purpose of the Study:
- To investigate calcium metabolism changes in PWH.
- To assess the impact of diagnosis and treatment on calcium metabolism.
- To evaluate the significance of early diagnosis and prophylaxis.
Main Methods:
- Serum levels of calcium, phosphorus, alkaline phosphatase, 25-hydroxy vitamin D (25-OHD), parathormone (PTH), and calcitonin were measured.
- Bone mineral density (BMD) was assessed using dual-energy x-ray absorptiometry.
- Patients were analyzed based on prophylaxis status.
Main Results:
- Low BMD scores (74.4%) and 25-OHD deficiency (87.2%) were prevalent in PWH.
- Prophylaxis did not significantly alter 25-OHD levels or BMD scores.
- Higher PTH levels were observed in the prophylaxis group, with a negative correlation to Z-score.
Conclusions:
- Biochemical markers of bone turnover can aid in detecting bone loss in PWH.
- Annual BMD measurements and exercise programs are recommended for monitoring and management.
- Further research with larger cohorts is warranted to confirm findings.
Abstract:
In this study, we aimed to investigate changes in calcium (Ca) metabolism in hemophilia patients (PWH). We also aimed to investigate the importance of diagnosis and treatment of factors impairing calcium metabolism and the significance of early diagnosis and prophylaxis with respect to these subjects. For all patients, serum calcium, phosphorus, alkaline phosphatase, 25 hydroxy vitamin D (25-OHD), parathormone (PTH), and calcitonin levels were evaluated. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry. Low BMD scores and 25-OHD deficiency were observed in 29 (74.4%) and 34 (87.2%) patients, respectively. Prophylaxis of PWH did not differ significantly in terms of 25-OHD levels and BMD scores. Patients in the prophylaxis group had significantly higher PTH levels (P=0.042). A negative correlation was found between PTH measurements and Z-score (P=0.008). In summary, our findings, with a small number of PWH in our study group, suggest that biochemical markers of bone turnover may be used to detect bone loss. Follow-up through annual BMD measurements coupled with appropriate exercise programs could be recommended.
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