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Bone mineral density in children with beta-thalassemia major in Diyarbakir
Ayfer Gözü Pirinççioğlu1, Veysi Akpolat, Orhan Köksal
1Department of Pediatrics, Faculty of Medicine, University of Dicle, 21280, Diyarbakir, Turkey. ayfergozu@hotmail.com
Insights
Beta-thalassemic children show lower bone mineral density (BMD) compared to healthy peers. This complex issue may stem from iron overload and chelation therapy impacting growth and endocrine function.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Metabolic Bone Disease
Background:
- Bone mineral status is well-studied in adult thalassemics, but less so in pediatric populations.
- Beta-thalassemia is a genetic blood disorder requiring lifelong management.
Purpose of the Study:
- To investigate and compare bone mineral density (BMD) and related biochemical markers in children with beta-thalassemia versus healthy controls.
- To explore potential contributing factors to bone health disparities in pediatric thalassemic patients.
Main Methods:
- Cross-sectional study involving 47 children with beta-thalassemia and 50 age-, sex-, and socioeconomically matched healthy controls.
- Measurements included bone mineral density (BMD) at lumbar and femur sites, and various demographic and biochemical parameters.
Main Results:
- Thalassemic children exhibited significantly higher levels of AST, ALT, phosphorus, osteocalcin, CTX-I, iPTH, and ferritin.
- Conversely, patients had significantly lower 25-hydroxy vitamin D (25OH-D), alkaline phosphatase, and Z-scores at both lumbar and femur sites.
- A subset of patients with high intact parathyroid hormone (iPTH) showed lower Z-scores and 25OH-D, with higher osteocalcin.
Conclusions:
- Children with beta-thalassemia have significantly lower bone mineral density (BMD) compared to healthy children.
- This reduced BMD is a complex multifactorial issue, potentially linked to impaired physical development due to iron overload and chelation therapy affecting liver and endocrine functions.
Abstract:
Bone mineral status has extensively been investigated in adult thalassemics but less in thalassemic children. This study involves measurements of the bone mineral density (BMD), various demographic and biochemical parameters in 47 thalassemic children and 50 healthy controls with comparable age, sex, socioeconomic and regional distribution. Patients have significantly higher aspartate aminotransferase, alanine aminotransferase, phosphorous, osteocalcin, serum carboxy terminal teleopeptide fragment of type I collagen, intact parathyroid hormone (iPTH) and ferritin levels while they have significantly lower 25-hydroxy vitamin D (25OH-D), alkaline phosphatase and z-scores both at lumbar and femur compared to controls. Patients with high iPTH (30%) had significantly lower z-scores and 25OH-D while larger osteocalcin. We conclude that a significantly lower BMD in beta-thalassemic children compared with their healthy counterparts is a complex process and may partially attributed to their slower physical development, caused by iron overload and chelation therapy which may influence the liver as well as the endocrine tissues.
