Pulmonary function changes in patient beta thalassaemia major

ABSTRACT

Thalassaemia major is the most common cause of chronic hemolytic anemia in Mediterranean population Aggressive blood transfusion therapy for thalassaemia permit better growth and preventing progressive marrow expansion and cosmetic problems. Iron overload from blood transfusion is the main cause of organ dysfunction. Lung function abnormalities in thalassaemia major are various and complex. The aim of the present work is to assess pulmonary function changes in beta thalassaemia major patients. Twenty-four children [14 males and 10 females with their ages ranging from 5 to 15 years] with beta thalassaemia major treated with desferoxamine injection were subjected to history taking, clinical evaluation, Echocardiography and pulmonary function tests [FVC and FEV1/FVC]. Patients with beta thalassaemia major showed significant decrease in FVC [83.37% +/- 6.79%of the predicted] when compared with the control group [92% +/- 1.76%of the predicted] [P=0.001]. This decreased FVC is positively correlated with hemoglobin concentration [P=0.001] and pulmonary acceleration time [P=0001] and negatively with left ventricular end diastolic diameter [P=0.001] and left ventricular end systolic diameter [P=0.001]. There was no significant difference between control group and patients with beta thalassaemia major as regards FEV1/FVC. This decrease in FVC was observed in all groups of the study with either regular or irregular intake of iron chelating agent when compared with control group. This restrictive impaired lung function can be explained by pulmonary hemosiderosis due to repeated blood transfusion and increased gastrointestinal iron absorption and also maybe related to cardiac changes that occurred in these patients