Background: Chronic hemolytic anemias may compromise growth through multiple mechanisms. To date, no data exist on growth and body composition (BC; bone, muscle and fat mass) of children and adolescents with chronic hemolysis.
Purpose: To evaluate growth and BC of patients with thalassemia intermedia (not on regular transfusions; thal-intermedia), alpha-thalassemia and congenital spherocytosis.
Methods: Patients and controls underwent clinical examination, dual-energy X-ray absorptiometry (DXA) of the spine and total body (less head) and laboratory bone profile. All growth and BC parameters were converted to Z-scores.
Results: 35 patients (17 female, 19 pubertal), aged 11.9±3.4 years, of which 13 with thal-intermedia, 16 with α-thalassemia and six with spherocytosis were studied. The control group consisted of 57 subjects. As a whole, our patients had lower weight and body mass index (BMI) (Z-scores −0.2 and −0.3, respectively, P<0.01). They had lower lumbar spine bone mineral density (LS BMD), (Z-score=−0.6, P<0.01), whereas muscle and fat mass were unaffected. A positive correlation was found between LS BMD and total body BMD (r=0.582, P<0.01), as well as between total body BMD and height (r=0.368, P=0.03). Of note, patients with adequate calcium intake and regular exercise were taller than the other patients (Ht Z-score=0.3, P=0.02). Laboratory markers for bone formation and resorption were normal in the majority of cases (85%), as well as vitamin D and PTH levels. When each subgroup was analysed separately, patients with thal-intermedia appeared more affected in terms of BMD and BMI, whereas in the other subgroups only LS BMD was lower, all other parameters being comparable to controls.
Conclusion: Chronic hemolysis may adversely affect BMI and LS BMD. Muscle and fat mass are not particularly affected. Counselling on a healthy lifestyle and regular surveillance of bone health is justifiable, especially in Thal-intermedia patients.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology