Pulmonary Function Test in Transfusion-Dependent β-Thalassemia Major Patients: A Pilot Study

Abstract

Lung involvement is one of known complications of thalassemia. The aim of this study was to determine predominant type of pulmonary dysfunction and its relationship to iron overload in β-thalassemia children. Fifty thalassemia major children with treatment of regular blood transfusion and desferrioxamine participated in the study. Thirty-three boys and 17 girls (median age 12.5 years) with β-thalassemia enrolled in the study. Other information including body mass index, hematocrit, and the number of years of blood transfusion were recorded. Serum ferritin level and hematocrit were 3346 ± 1667 mg/dL and 27.7 ± 2, respectively. Pulmonary function tests were performed in all subjects for detecting pulmonary dysfunction. Thirty-five patients (70%) with thalassemia had abnormal result of spirometry. Obstructive airway disease based on reduced forced expiratory volume in 1 second (FEV₁) and FEV₁/forced vital capacity (FVC) ratio <80% was detected in 4 patients (8%). Six patients (12%) showed restrictive pattern, as defined by a reduction FVC <80% and FEV₁/FVC ratio ≥80%. In this study, small airway involvement based on presence of forced expiratory flow (FEF_25%–75%) <60%, FEV₁/FVC ratio >70%, and FVC >80% was detected in 25 subjects (50%). Decreased values of peak expiratory flow rate (PEF) were detected in 23 (46%) and low FEV₁ in 10 (20%) subjects. There was no significant correlation between abnormal pulmonary function test and serum ferritin level in children with thalassemia. This study showed small airway disease was predominant abnormality in thalassemia patients, although additional larger studies are needed to evaluate underlying mechanisms and validate these findings.

Keywords :

• β-thalassemia major
• pulmonary function test