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Osteopenia and vitamin D deficiency in children with sickle cell disease
E Chapelon1, M Garabedian, V Brousse
1Pediatrie Generale, Hopital Jean Verdier, Paris, France.
Insights
Children with sickle cell disease (SCD) experience decreased bone mineral density (BMD) starting before puberty, particularly in females. This bone loss in pediatric SCD patients appears linked to impaired bone formation, not disease severity or vitamin D deficiency.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Bone Metabolism
Background:
- Sickle cell disease (SCD) is associated with low bone mineral density (BMD) in adults.
- The prevalence and characteristics of low BMD in children with SCD are not well-established.
Purpose of the Study:
- To determine the prevalence of low BMD in children diagnosed with SCD.
- To explore potential associations between BMD and disease severity, nutritional status, and biochemical markers.
Main Methods:
- Dual-energy X-ray absorptiometry (DXA) was used to assess BMD in 53 children with SCD.
- Evaluated clinical data including hospitalizations, painful crises, transfusions, and nutritional intake.
- Assessed biochemical markers such as vitamin D, parathyroid hormone (PTH), and bone turnover markers.
Main Results:
- A mean lumbar spine Z-score of -1.1 ± 1.3 was observed, indicating reduced BMD in pediatric SCD patients.
- Lower BMD Z-scores were noted in prepubertal girls compared to prepubertal boys.
- Vitamin D deficiency (76%) and secondary hyperparathyroidism (38%) were prevalent, but BMD did not correlate with these or other disease severity markers.
Conclusions:
- Children with SCD exhibit decreased BMD, beginning before puberty and more pronounced in females.
- The observed low BMD is not directly linked to disease severity, vitamin D status, or bone resorption.
- Abnormal bone formation is suggested as the primary mechanism underlying reduced BMD in pediatric SCD.
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 Sbeta-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.
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