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Impairment of bone health in pediatric patients with hemolytic anemia
Michael M Schündeln1, Sarah C Goretzki1, Pia K Hauffa1
1Department of Pediatric Hematology and Oncology, Kinderklinik III, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
Insights
Pediatric patients with chronic hemolytic anemia often have poor bone health, characterized by vitamin D deficiency and altered bone modeling markers. Sickle cell anemia patients showed more severe bone impairment than those with hereditary spherocytosis.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Bone Metabolism
Background:
- Chronic hemolytic anemias, including sickle cell anemia and thalassemia, are linked to compromised bone health in children and adults.
- Pediatric patients with various chronic hemolytic anemias may exhibit impaired bone development and health.
Purpose of the Study:
- To evaluate bone health status in pediatric patients diagnosed with chronic hemolytic anemia.
- To identify specific bone health parameters affected in this patient population.
Main Methods:
- A cross-sectional study involving 45 pediatric patients with diverse hemolytic anemias (17 sickle cell disease, 14 hereditary spherocytosis).
- Assessment included biochemical markers (e.g., 25 OH-vitamin D, RANKL, osteoprotegerin, osteocalcin), radiographic data, and patient history.
- Statistical analysis, including multiple stepwise regression, was used to determine influencing factors on bone health markers.
Main Results:
- A high prevalence of vitamin D deficiency (80.5%) was observed, with serum 25 OH-vitamin D levels below 20 ng/ml.
- Elevated RANKL/osteoprotegerin (OPG) ratios (0.26 vs. 0.08 in controls) and lower osteocalcin levels (68.5 vs. 118.0 ng/ml in controls) indicate altered bone modeling.
- Bone pain was reported in 31% of patients. Lactate dehydrogenase (LDH), diagnosis, and age significantly influenced osteocalcin levels.
- Patients with sickle cell anemia experienced more severe bone health impairment compared to those with hereditary spherocytosis.
Conclusions:
- Pediatric patients with chronic hemolytic anemia exhibit significantly impaired bone health.
- Vitamin D deficiency, an imbalanced RANKL/OPG system, and low osteocalcin levels are key contributors to bone impairment in these children.
- These findings highlight the need for monitoring and intervention strategies for bone health in pediatric hemolytic anemia patients.
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.
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