title: Pulmonary function abnormalities in thalassemia patients on a hypertransfusion program.
abstract: Pulmonary function tests were performed in 12 thalassemia patients on a hypertransfusion program (age 18.4 +/- 2.6 SEM years) to determine the presence of any abnormalities of lung function. These included spirometry, expiratory flow rates, body plethysmography, single-breath nitrogen washout, single breath carbon monoxide diffusing capacity, and arterial blood gases. Only one patient had normal pulmonary function. Arterial hypoxemia was present in ten of 12 patients at rest. The total lung capacity (TLC) was normal. The residual volume was abnormally increased in five of 12 patients. The slope of phase III of single breath nitrogen washout curve was abnormal in five of 12 patients, but the closing volume was normal. The maximal expiratory flow rate at 60% total lung capacity was decreased in four of 12 patients, suggesting the presence of small airway disease. The single breath carbon monoxide diffusing capacity was normal in all patients. These pulmonary function abnormalities did not correlate with age or the cumulative amount of iron via blood transfused. The small airway obstruction, hyperinflation; and hypoxemia observed in thalassemia patients on a hypertransfusion program may result from the basic disease, iron deposition in the lungs, or other factors.
mesh_terms: D000293:Adolescent; D000328:Adult; D000403:Airway Resistance; D001784:Blood Gas Analysis; D001803:Blood Transfusion; D002648:Child; D003011:Closing Volume; D005260:Female; D005540:Forced Expiratory Flow Rates; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008449:Maximal Expiratory Flow-Volume Curves; D010366:Peak Expiratory Flow Rate; D010993:Plethysmography, Whole Body; D011653:Pulmonary Diffusing Capacity; D013789:Thalassemia / Q000503:physiopathology* / Q000628:therapy; D014109:Total Lung Capacity