ABSTRACT
Background: ?-Thalassemia major is a serious hematological problem requiring regular blood transfusions. In regularly transfused thalassemic patients, metabolic bone disease is an important cause of morbidity. Problems include bone pain, deformities, short stature, osteopenia/osteoporosis, rickets, osteomalacia, spinal deformities, nerve compression and fractures. This study was undertaken to evaluate the status of bone health in children with ?-thalassemia major by selected biochemical indices and radiological examination.Methods: Fifty children with ?- thalassemia major were enrolled. Detailed history, examination and anthropometry were done. Serum calcium, phosphorus, magnesium, alkaline phosphatase, and vitamin D levels were measured. X-ray of skull, wrist, and knee were studied for radiological changes. Biochemical indices were analysed with appropriate statistical tests.Results: Stunting and wasting was observed in 58% and 44% children respectively. Hypocalcaemia was seen in 22%, hyperphosphatemia in 56% and 24% children had raised alkaline phosphatase levels. Vitamin D levels were severely deficient in 12%, deficient in 50%, and insufficient in 38% of children. There was no significant difference between vitamin D levels of cases and controls. Radiologically almost all children had a range of osteopathy, like thinned out cortex (96%), medullary expansion (54%), loss of density (40%), diffuse osteoporosis (18%) and growth arrest lines were seen in 8% children.Conclusions: Vitamin D abnormalities and radiological features suggestive of decreased bone mineralization are seen in all the children with ?-thalassemia major treated with periodic blood transfusion at this institute. No biochemical predictors of bone disease in these children were identified.