Department of Paediatrics, Faculty of Medicine, The Chinese University of Hong Kong, 6/F, Lui Che Woo Clinical Sciences Building, Prince of Wales Hospital, Shatin, N.T., Hong Kong SAR, China.
Laboratory for Paediatric Respiratory Research, Faculty of Medicine, Li Ka Shing Institute of Health Sciences, The Chinese University of Hong Kong, Shatin, Hong Kong SAR, China.
Sci Rep. 2023 Mar 4;13(1):3674. doi: 10.1038/s41598-023-30784-9.
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.
在依赖输血的地中海贫血症（TDT）患者中，已有报道称存在肺功能损害，但数据相互矛盾。此外，肺功能障碍是否与铁过载有关仍不清楚。本研究旨在评估 TDT 患者的肺功能，并探讨肺功能障碍与铁过载之间的关系。这是一项回顾性观察性研究。共招募了 101 名 TDT 患者进行肺功能检查。从计算机病历中检索了最近的铁蛋白水平（pmol/L）和心脏和肝脏铁状态的磁共振成像（MRI）测量值，分别由心脏和肝脏 T2弛豫时间（毫秒，ms）测量。仅纳入了距肺功能测量 12 个月内的数据进行分析。血清铁蛋白和心脏及肝脏 T2弛豫时间是体内铁含量的替代指标。肺功能异常的阈值定义为低于预测值的 80%。共纳入 101 名受试者，平均年龄为 25.1 岁（标准差 7.9 岁）。分别有 38%（38%）和 5%（5%）的患者表现为限制性和阻塞性肺功能障碍。FVC%预测值和 TLC%预测值与 MRI 心肌 T2弛豫时间呈弱相关（rho=0.32，p=0.03 和 rho=0.33，p=0.03）。通过逻辑回归分析，在校正年龄、性别和体重指数后，MRI 心脏 T2弛豫时间与限制性肺功能障碍缺陷呈负相关（B-0.06；SE 0.03；比值比 0.94；95%置信区间 0.89-0.99；p=0.023）。TDT 患者常出现限制性肺功能障碍，其严重程度可能与心肌铁含量相关。对于这组患者，特别是对于铁过载的患者，监测肺功能非常重要。
