The effects of diuresis and transfusion on pulmonary function in children with thalassemia major

Previous pulmonary function studies in subjects with thalassemia major (TM) who were on regular transfusion programs have demonstrated results ranging from small airway obstruction to a restrictive pattern. Ten subjects with TM were studied pre- and postdiuresis, and again 24 hr after transfusion, in order to evaluate the role of possible fluid overload in altering pulmonary function. Subjects underwent spirometry and had lung volume and flow volume curves (MEFVC) measured in a volume displacement plethysmograph while breathing air and a mixture of 80% helium and 20% oxygen (HeO2). Six patients had pulmonary mechanics measured with esophageal balloons in place. Baseline function was normal and no change occurred following diuresis. Following transfusion, the volume of isoflow (VisoV) decreased, but other parameters did not change. Subsequent analysis revealed 5 subjects with an initial VisoV greater than 20 (% FVC) but, paradoxically, less evidence of flow limitation in the small airways than those with a VisoV less than 20 (% FVC). Posttransfusion, in those subjects with an initially high VisoV, the Vmax25(air) tended to fall without a change of MEFVC in HeO2, resulting in a decreased VisoV. This was interpreted as evidence of subtle abnormalities in the small airways caused by volume expansion, raising doubts about the value of the VisoV as a measure of small airway disease. As a group, our subjects did not demonstrate any abnormalities in baseline function. Some subjects had mild flow limitation in small airway while other developed comparable levels of flow limitation following the volume expansion associated with transfusion.