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Restrictive lung disease and serum TGF-beta1 in thalassemia major children
Suchada Sritippayawan1, Piyachat Lekhanont, Chanthana Harnruthakorn
1Division of Pulmonology and Critical Care Medicine, King Chulalongkorn Memorial Hospital, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. suchadacu@hotmail.com
Insights
Restrictive lung disease in thalassemia major is linked to longer transfusion duration and higher ferritin. Serum TGF-beta1 levels did not show a significant association with restrictive lung disease in these children.
Area of Science:
- Pediatric Hematology
- Pulmonary Medicine
- Biochemistry
Background:
- Thalassemia major (TM) is a severe inherited blood disorder requiring lifelong transfusions.
- Pulmonary complications, including restrictive lung disease (RLD), can affect TM patients.
- The role of serum transforming growth factor-beta 1 (TGF-beta1) in RLD development in TM is not well understood.
Purpose of the Study:
- To investigate the relationship between restrictive lung disease (RLD) and serum transforming growth factor-beta 1 (TGF-beta1) levels in children with thalassemia major.
- To identify factors associated with RLD in this patient population.
Main Methods:
- A cross-sectional study involving 21 children with thalassemia major.
- Evaluation of pulmonary function, serum ferritin, and serum TGF-beta1 levels.
- Comparison of RLD patients with non-RLD patients.
Main Results:
- 24% of TM children exhibited restrictive lung disease (RLD).
- RLD patients had significantly longer transfusion durations and higher serum ferritin levels compared to non-RLD patients.
- Serum TGF-beta1 levels were significantly lower in TM children than in normal children, but not significantly different between RLD and non-RLD TM patients.
Conclusions:
- Restrictive lung disease in thalassemia major is associated with prolonged transfusion history and elevated serum ferritin.
- Serum TGF-beta1 levels do not appear to be a significant factor in the development of RLD in children with thalassemia major.
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.
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