Osteopenia and vitamin D deficiency in children with sickle cell disease
Article first published online: 13 AUG 2009
© 2009 John Wiley & Sons A/S
European Journal of Haematology
Volume 83, Issue 6, pages 572–578, December 2009
How to Cite
Chapelon, E., Garabedian, M., Brousse, V., Souberbielle, J. C., Bresson, J. L. and De Montalembert, M. (2009), Osteopenia and vitamin D deficiency in children with sickle cell disease. European Journal of Haematology, 83: 572–578. doi: 10.1111/j.1600-0609.2009.01333.x
- Issue published online: 10 NOV 2009
- Article first published online: 13 AUG 2009
- Accepted for publication 10 August 2009
- sickle cell disease;
- bone mineral density;
- vitamin D
Objectives: To assess the prevalence in children with sickle cell disease of low bone mineral density (BMD), a feature found in up to 82% of adults but not well known in children.
Methods: In 53 children (45 SS, 4 SC, 4 Sβ-thalassemia) with a mean age of 12.8 ± 2.4 years, we assessed height; weight; sexual maturation; number of hospitalizations, painful crises, and transfusions in the last 3 years; calcium intake; steady-state hemoglobin and leukocyte count; calcaemia, phosphataemia, and calciuria/creatinuria; serum 25-(OH)D and PTH concentrations; and osteocalcin, urinary deoxypyridinoline, and the C-terminal component of pro-collagen type I. BMD was assessed using dual X-ray absorptiometry.
Results: Mean lumbar spine Z-score was −1.1 ± 1.3 (−3.9 to +1.8). The Z score was significantly lower in girls than in boys in the prepubertal subgroup (−1.74 ± 0.27 vs. −0.53 ± 0.31) (P = 0.0169), but not in the pubertal group (−1.15 ± 0.41 vs. −1.33 ± 0.70). BMD was not associated with any of the disease-severity markers in girls but was unexpectedly associated with fewer vaso-occlusive crises and hospitalizations in boys. BMD did not correlate with hemoglobin or leukocyte counts. Vitamin D deficiency [25-(OH)D < 12 ng/mL] was found in 76% of patients and secondary hyperparathyroidism (PTH > 46 pg/mL) in 38%. BMD was not related to calcium intake, vitamin D status, osteocalcin, or bone resorption markers.
Conclusion: A slight BMD decrease was found in SCD children, starting before puberty and being more marked in females. The decrease was unrelated to disease severity, vitamin D deficiency, or bone hyperresorption, suggesting abnormal bone formation as the underlying mechanism.