European Journal of Cardiovascular Medicine

Introduction: In Thalassemia major there is decrease or total suppression of hemoglobin polypeptide chain synthesis occurs. Patient require regular blood transfusion to maintain normal Hb level greater than 10 gm% [17]. An inevitable, important and potentially lethal complication of administering repeated blood transfusion to a child with thalassemia is gradual overloading of body with iron. Iron deposition on various organ affects their function including lungs. Pulmonary deposition of iron ultimately leads to decease in function of lungs which can be assessed by spirometry and their correlation can be derived. From the above facts one can apprehend the problem of associated complication in thalassemia major children receiving regular blood transfusion. Aim &Objectives: To determine the pulmonary function status in beta-thalassemia major patients receiving regular blood transfusion. To study the pattern of respiratory impairment using spirometry. Also to estimate iron overload status by estimating serum ferritin level and with these values correlation of respiratory impairment with iron overload in thalassemia patients. Methodology: Hospital based observational cross-sectional prospective study, of sample size 81 having diagnosed as Thalassemia major of patients between age group of 6year to 14 years. Just before transfusion, venous sample collected from all participants and serum ferritin levels were assessed. Serum ferritin levels were recorded in the chart of patients every 6 months. Serum ferritin was derived by calculating the average measurement over a 2-year period for each patient. Further patients were categorized into population group A and population group B whom having serum ferritin level greater than or equal to 2500ng/ml and less than 2500ng/ml respectively. PFT was performed on the day scheduled for blood transfusion. Results were expressed as a percentage of normal. To compare the clinical parameter and biochemical parameters, chi-square test of association has been used. For comparison of study variable, independent student t test was used. For correlation of number of blood transfusion with serum ferritin, pearson correlation co-efficient has been used. The results were compared by using SPSS software version 17. Results: Patients with higher number of transfusion i.e. greater or equal to 140 showed increased chance of pulmonary abnormality as evidenced by mean FEV1(91.82±3.556) which was significantly higher compared to patients with less number of transfusion (i.e. less than 140) was (86.23±2.224). Pulmonary function test parameter FEV1 when compared to serum ferritin level. It was found that mean FEV1(91.06±3.564) level of population with serum ferritin level less than 2500 is significantly higher than mean FEV1(81.18±4.177) of population with serum ferritin level higher or equal to 2500, which is statistically significant as evidenced by p-value of 0.001 Conclusion: Patients with higher number of transfusion showed  increased chance of pulmonary abnormality. The severity of the restrictive disease increases in older age and with more transfusion iron burden, which is indicative of a central role of iron in the pathogenesis of pulmonary function abnormality, which is associated with thalassemia major. This study will emphasise, patients with Thalassemia major on regular blood transfusion need monitoring throughout treatment to avoid future Pulmonary complications.