Bone mass and metabolism in thalassemic children and adolescents treated with different iron-chelating drugs

• Published in: Journal of bone and mineral metabolism 2004, Vol.22 (1), p.53-57
• Main Authors: DI STEFANO, Marco, CHIABOTTO, Patrizia, ROGGIA, Cristiana, GAROFALO, Franco, LALA, Roberto, PIGA, Antonio, ISAIA, Giovanni Carlo
• Format: Article
• Language: English
• Subjects: Adolescent; Alkaline Phosphatase - drug effects; Alkaline Phosphatase - metabolism; Amino Acids - drug effects; Amino Acids - metabolism; Amino Acids - urine; Anemias. Hemoglobinopathies; Biological and medical sciences; Body Weights and Measures; Bone and Bones - drug effects; Bone and Bones - metabolism; Bone density; Bone Density - drug effects; Bone Density - physiology; Bones; Child; Deferoxamine - pharmacology; Diseases of red blood cells; Diseases of the osteoarticular system; Female; Femur - chemistry; Femur - drug effects; Ferritins - blood; Hematologic and hematopoietic diseases; Humans; Iron Chelating Agents - pharmacology; Iron Chelating Agents - therapeutic use; Lumbar Vertebrae - chemistry; Lumbar Vertebrae - drug effects; Male; Medical sciences; Older people; Osteoporosis; Prospective Studies; Pyridones - pharmacology; Thalassemia - drug therapy; Time Factors
• ISSN: 0914-8779; 1435-5604
• DOI: 10.1007/s00774-003-0449-z

We evaluated bone mineral density (BMD) and bone turnover in 22 homozygous prepubertal beta-thalassemic patients treated with desferrioxamine. Ten patients underwent treatment with desferrioxamine for the whole study period, while 12 patients stopped desferrioxamine and were then treated with deferiprone (L1). Lumbar and femoral BMD and bone metabolism markers were examined at baseline and after 1 and 3 years of follow up. All patients were prepubertal at baseline and they all became pubertal over the 3 years of follow up. At baseline, the mean lumbar Z score value was -2.048 SD +/- 0.75; the Z score was less than -2 SD in 13 children, within -1 and -2 SD in 6, and within 0 and -1 SD in only 3 subjects. A significant BMD increase (P < 0.0001) was observed at both the lumbar (+8.466%/year) and the femoral level (average of +3.46%/year at neck and +5.83%/year at the intertrochanteric region) after 3 years, without any significant difference being shown between patients treated with desferrioxamine and those treated with L1. The mean Z score SD values increased to -1.957 +/- 0.975 at 1 year (not significantly different from baseline) and to -1.864 +/- 1.221 at 3 year follow up (P < 0.05 vs baseline); an increase in bone turnover was also observed. These findings show that low BMD, a hallmark of beta-thalassemia, improves significantly when puberty begins; this increase involves different skeletal sites, regardless of pharmacological treatment with different iron-chelating drugs.