J Rufeena, Sahu Upendra P, Datta Shambhavi, Rajak Prity K, Singh Praveen Kumar
Paediatrics, Rajendra Institute of Medical Sciences, Ranchi, Ranchi, IND.
Paediatric Medicine, Rajendra Institute of Medical Sciences, Ranchi, Ranchi, IND.
Cureus. 2025 Jul 4;17(7):e87278. doi: 10.7759/cureus.87278. eCollection 2025 Jul.
Thalassemia major is a genetic disorder characterised by defective red cell production. Due to the requirement of regular blood transfusions, these patients are at risk of iron overload, leading to iron deposition in all organs, including the lungs, causing hemosiderosis of the lung, thereby causing impairment of lung function.
The primary objective was to assess lung dysfunction in children with thalassemia major and to correlate it with serum iron status. The secondary objectives were to find the prevalence, type and severity of lung dysfunction in these children.
A cross-sectional study was conducted in the paediatrics department of a tertiary care hospital in Jharkhand, Eastern India, from December 2022 to April 2024. Eighty children with a diagnosis of thalassemia major, aged 6 to 18 years, with a history of more than 10 blood transfusions, were enrolled. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC ratio estimation by spirometer, serum ferritin and CRP were assessed.
Of the 80 children included in the study, 52 were males (65%) and 28 were females (35%). A total of 87.5% (n = 70) of them showed a restrictive pattern of lung involvement. Around 52.5% (n = 42) had mild, 18.75% (n = 15) had moderate, 10% (n = 8) had moderately severe, and 6.25% (n = 5) had severe restriction of lung function. The mean serum ferritin values were 3344.55 ng/mL, 5657.07 ng/mL, 5165.5 ng/mL and 8024.2 ng/mL in children with mild, moderate, moderately severe and very severe restriction of pulmonary function, respectively. The serum ferritin levels had a significant correlation with lung function (r = -0.78, p = 0.001).
In this study, we observed varying severity of restrictive type of lung dysfunction in children with thalassemia major despite regular iron chelation. There was a statistically significant association between varying severity of lung impairment and body iron status as measured by serum ferritin levels.
重型地中海贫血是一种以红细胞生成缺陷为特征的遗传性疾病。由于需要定期输血，这些患者存在铁过载的风险，导致铁沉积在包括肺在内的所有器官中，引起肺含铁血黄素沉着症，从而导致肺功能受损。
主要目的是评估重型地中海贫血患儿的肺功能障碍，并将其与血清铁状态相关联。次要目的是确定这些患儿肺功能障碍的患病率、类型和严重程度。
于2022年12月至2024年4月在印度东部贾坎德邦一家三级护理医院的儿科进行了一项横断面研究。纳入80名诊断为重型地中海贫血的儿童，年龄在6至18岁之间，有超过10次输血史。通过肺活量计评估一秒用力呼气容积（FEV1）、用力肺活量（FVC）和FEV1/FVC比值，评估血清铁蛋白和CRP。
在纳入研究的80名儿童中，52名是男性（65%），28名是女性（35%）。其中共有87.5%（n = 70）表现出限制性肺受累模式。约52.5%（n = 42）为轻度，18.75%（n = 15）为中度，10%（n = 8）为中度重度，6.25%（n = 5）为重度肺功能受限。肺功能轻度、中度、中度重度和极重度受限的儿童血清铁蛋白平均值分别为3344.55 ng/mL、5657.07 ng/mL、5165.5 ng/mL和8024.2 ng/mL。血清铁蛋白水平与肺功能有显著相关性（r = -0.78，p = 0.001）。
在本研究中，我们观察到尽管进行了常规铁螯合治疗，但重型地中海贫血患儿仍存在不同严重程度的限制性肺功能障碍。通过血清铁蛋白水平测量，肺损伤的不同严重程度与体内铁状态之间存在统计学上的显著关联。
