Bone mineral density in children and young adults with beta-thalassemia trait.

Abstract

Homozygous beta-thalassemia is a hereditary hematological disease due to defective beta-globin synthesis. Consequently, there is ineffective erythropoiesis and increased peripheral hemolysis. Increased erythropoiesis in bone marrow results in expansion of marrow cavity and reduced bone mass. Patients with heterozygous beta-thalassemia or beta-thalassemia trait may have mild anemia, and consequently mildly increased erythropoiesis. Whether modestly increased erythropoiesis might decrease bone mass is not well established. To evaluate bone mineral density (BMD) in children and young adults with beta-thalassemia trait. Thirty-one healthy young adults aged 20-45 yr and 26 healthy children aged 8-15 yr with beta-thalassemia trait were enrolled in the study. BMD was determined by dual X-ray absorptiometry (DEXA). Determinations of intact parathormone (PTH), 25-hydroxyvitamin D (25-OHD), and bone markers were performed. In adults, all had z-scores of BMD more than -2 above the mean. The mean z-scores of BMD of lumbar spine, radius and femoral neck were 0.11, -0.10 and 0.41, respectively. In children, only two of 26 had z-scores of lumbar spine BMD more than -2 below the mean. The mean z-scores of BMD of total body, lumbar spine, radius and femoral neck were 0.12, -0.28, 0.30 and -0.14, respectively. All subjects had normal PTH, 25-OHD and bone markers levels. beta-Thalassemia trait is not a contributing factor for osteopenia/osteoporosis in children and young adults.