Introduction Sickle cell anemia and thalassemia result in impaired bone health
in both adults and youths. Children with other types of chronic hemolytic
anemia may also display impaired bone health. Study Design To assess bone
health in pediatric patients with chronic hemolytic anemia, a cross-sectional
study was conducted involving 45 patients with different forms of hemolytic
anemia (i.e., 17 homozygous sickle cell disease and 14 hereditary
spherocytosis patients). Biochemical, radiographic and anamnestic parameters
of bone health were assessed. Results Vitamin D deficiency with 25 OH-vitamin
D serum levels below 20 ng/ml was a common finding (80.5%) in this cohort.
Bone pain was present in 31% of patients. Analysis of RANKL, osteoprotegerin
(OPG) and osteocalcin levels indicated an alteration in bone modeling with
significantly elevated RANKL/OPG ratios (control: 0.08+0.07; patients:
0.26+0.2, P = 0.0007). Osteocalcin levels were found to be lower in patients
compared with healthy controls (68.5+39.0 ng/ml vs. 118.0+36.6 ng/ml, P =
0.0001). Multiple stepwise regression analysis revealed a significant
(P<0.025) influence of LDH (partial r2 = 0.29), diagnosis of hemolytic anemia
(partial r2 = 0.05) and age (partial r2 = 0.03) on osteocalcin levels.
Patients with homozygous sickle cell anemia were more frequently and more
severely affected by impaired bone health than patients with hereditary
spherocytosis. Conclusion Bone health is impaired in pediatric patients with
hemolytic anemia. In addition to endocrine alterations, an imbalance in the
RANKL/OPG system and low levels of osteocalcin may contribute to this
impairment
