Pulmonary function in patients with transfusion-dependent thalassemia and its associations with iron overload

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作者
Kate C. Chan
Chun T. Au
Alex W. K. Leung
Albert M. Li
Chi-kong Li
Matthew M. T. Wong
Carol S. T. Li
Hang L. Cheung
Philip Fan
Siu C. Ling
Rever C. H. Li
S. Y. Ha
机构
[1] The Chinese University of Hong Kong,Department of Paediatrics, Faculty of Medicine
[2] The Chinese University of Hong Kong,Laboratory for Paediatric Respiratory Research, Faculty of Medicine, Li Ka Shing Institute of Health Sciences
[3] The Chinese University of Hong Kong,Hong Kong Hub of Paediatric Excellence
[4] Princess Margaret Hospital,Department of Paediatrics and Adolescent Medicine
[5] Tuen Mun Hospital,Department of Paediatrics and Adolescent Medicine
[6] Queen Mary Hospital,Department of Paediatrics and Adolescent Medicine
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In patients with transfusion-dependent thalassemia (TDT), pulmonary function impairment has been reported but data are conflicting. Moreover, it remains unclear whether pulmonary dysfunction is associated with iron overload. This study aimed to evaluate the pulmonary function in patients with TDT and to investigate the associations between pulmonary dysfunction and iron overload. It was a retrospective observational study. 101 patients with TDT were recruited for lung function tests. The most recent ferritin levels (pmol/L) and the magnetic resonance imaging (MRI) measurements of the myocardial and liver iron status, as measured by heart and liver T2* relaxation time (millisecond, ms) respectively, were retrieved from the computerized medical records. Only data within 12 months from the lung function measurement were included in the analysis. The serum ferritin, and the cardiac and liver T2* relaxation time were the surrogate indexes of body iron content. The threshold of abnormality in lung function was defined as under 80% of the predicted value. 101 subjects were recruited with a mean age of 25.1 years (standard deviation (SD) 7.9 years). Thirty-eight (38%) and five (5%) demonstrated restrictive and obstructive lung function deficits, respectively. A weak correlation of FVC %Predicted and TLC %Predicted with MRI myocardial T2* relaxation time (rho = 0.32, p = 0.03 and rho = 0.33, p = 0.03 respectively) was observed. By logistic regression, MRI cardiac T2* relaxation time was negatively associated with restrictive lung function deficit (B − 0.06; SE 0.03; Odds ratio 0.94; 95% confidence interval (CI) 0.89–0.99; p = 0.023) after adjusting for age, sex and body mass index. Restrictive pulmonary function deficit was commonly observed in patients with TDT, and the severity potentially correlates with myocardial iron content. Monitoring of lung function in this group of patients, particularly for those with iron overload, is important.
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