ASIAN PACIFIC JOURNAL OF ALLERGY AND IMMUNOLOGY (2005) 23: 121-126 Restrictive Lung Disease and Serum TGF-β1 in Thalassemia Major Children Suchada Sritippayawan 1 , Piyachat Lekhanont 1 , Chanthana Harnruthakorn 1 , Rujipat Samransamruajkit 1 , Jitladda Deerojanawong 1 , Panya Seksarn 2 , Yong Poovorawan 3 and Nuanchan Prapphal 1 SUMMARY 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-β1). 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-β1 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-β1 levels than normal children (7.9 vs 78.8 pg/ml; p < 0.001). The serum TGF-β1 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-β1 levels. From the 1 Division of Pulmonology and Critical Care Medicine, 2 Division of Hemato-oncology, 3 Center of Excellence Viral Hepa- titis Research Unit, King Chulalongkorn Memorial Hospital, De- partment of Pediatrics, Faculty of Medicine, Chulalongkorn Uni- versity, Bangkok, Thailand. Correspondence: Suchada Sritippayawan E-mail: suchadacu@hotmail.com Thalassemia major is an inherited disorder of the hemoglobin synthesis. Clinical presentations in- clude severe anemia and transfusion dependence. Frequent hemolysis and frequent blood transfusions as well as increased iron absorption from the gut result in an iron overload of the tissues of various organs and lead to multiple organ dysfunctions. 1 Pulmonary function abnormalities, especially restrictive lung disease (RLD), have been reported in many thalas- semic patients. The prevalence of RLD varied from 0-80%, depending on the definition of RLD and the method of pulmonary function testing used in each study. However, the nature and pathogenesis of the abnormal lung functions are still controversial. 2-15 Several studies demonstrated the association between increased body iron storage and lung dys- functions in thalassemic patients. 3,4,7,12,15 Radiologic evidences of interstitial lung disease were reported in some thalassemia major patients who had RLD. 12,16 However, whether increased fibrous tissue in the lungs contributes to RLD in these patients has not been well established. Transforming growth factor-β1 (TGF-β1) is an inflammatory cytokine that plays a role in pro- moting fibroblastic proliferation and matrix accumu- lation in tissues. It is secreted by antigen-stimulated T cells, lipopolysaccharide-activated mononuclear phagocytes and many other cell types including platelets, epithelial cells and fibroblasts. The major biologic action is to promote the synthesis of ex-