Restrictive lung disease and serum TGF-beta1 in thalassemia major children.

ABSTRACT

A cross sectional study was performed in 21 thalassemia major (TM) children at King Chulalongkorn Memorial Hospital during March to August, 2003 to determine whether restrictive lung disease (RLD) was related to serum transforming growth factor-beta 1 (TGF-beta1). All studied patients (57% female, age 11.2 +/- 2.6 yrs, duration of transfusion 7.7 +/- 4.1 yrs) never had desferoxamine treatment and their pulmonary function, serum ferritin and serum TGF-beta1 were evaluated. Five (24%) had RLD. RLD patients had significantly longer durations of transfusion and higher serum ferritin levels than non-RLD patients (9.1 +/- 1.9 vs 5.5 +/- 3.2 yrs; p = 0.03 and 3,816.6 +/- 1,715.9 vs 2,084.5 +/- 1,504.8 ng/ml; p = 0.04, respectively). TM children had lower serum TGF-beta1 levels than normal children (7.9 vs 78.8 pg/ml; p < 0.001). The serum TGF-beta1 level was not different between RLD and non-RLD patients (13.3 vs 4.2 pg/ml; ns), concluding that RLD was related to longer duration of transfusion and higher serum ferritin but not related to serum TGF-beta1 levels.