PULMONARY FUNCTION TESTS IN CHILDREN WITH BETA THALASSEMIA AND ITS CORRELATION WITH IRON OVERLOAD

Abstract

Iron overload is the principal determinant of major complications in conditions requiring repeated blood transfusions, like Beta Thalassemia Major and Sickle Cell Anemia. Although pulmonary dysfunction is not the most signicant clinical manifestation of thalassemias, or indeed does not produce any symptoms, a certain reduction of pulmonary volumes has been reported to occur in most subjects with beta thalassemia receiving regular blood transfusions. This study was done to assess the pulmonary function of such patients by Spirometry and the type of pulmonary dysfunction and to study its co-relation with iron overload. A total of 54 patients with Beta Thalassemia major on regular blood transfusions, of ages 6 years and above, were made to undergo Pulmonary Function Tests, of which 21 (38.89%) were females and 33 (61.11%) were males. Serum Ferritin values were recorded in all patients as a measure of iron overload. Pulmonary dysfunction was observed in 42 (77.78%) patients.`A predominantly restrictive pattern was found in 38 (90.48%) patients. An obstructive pattern was found in 4 (9.52%) patients. Patients with an FEV1/FVC ratio of less than 70% of the predicted value were labeled as having obstructive pulmonary dysfunction. Patients with FVC values of less than 80% of the predicted value and a FEV1/FVC ratio of more than 80 percent of th6e predicted value were labeled as having restrictive pulmonary dysfunction . Among patients with restrictive dysfunction, those having FVC values between 70-79%, 50-69% and 35 to 49% were graded as having mild, moderate and severe restrictive dysfunction respectively. Among subjects with obstructive dysfunction, those having FEV1/FVC ratio values between 70-79%, 50-69% and 35-49% were graded as having mild, moderate and severe obstructive dysfunction respectively. The number of patients with mild, moderate and severe pulmonary dysfunction were 13 (30.95%), 25 (59.52%) and 4 (9.52%) respectively. The mean ferritin value was calculated for each category of severity to see if any kind of correlation exists between pulmonary dysfunction and iron overload. Pulmonary dysfunction was found in a signicant majority of the patients, however no signicant correlation was found between severity of pulmonary dysfunction and iron overload.