Assessment of Lung Function and Its Correlation With Iron Overload in Children With Thalassemia Major

评估肺功能及其与重型地中海贫血患儿铁过载的相关性

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Abstract

INTRODUCTION:  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. OBJECTIVES: 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. METHODOLOGY: 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. RESULTS: 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). CONCLUSION: 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.

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