Cross-sectional study of pulmonary function and MRI-derived liver and myocardial iron content in young patients with beta-thalassemia major.

摘要

Pulmonary dysfunction stands for one of the most undervalued and less recognized complications in patients with beta-thalassemia, although it has initially been described almost three decades ago (1). Conflict reports exist regarding the type of the observed pneumonopathy (2, 3), and the exact pathogenetic mechanism is not clearly elucidated; however, the chronic effect of iron overload may reasonably intervene in it. In this report, we present findings from a study that we have conducted to cross-sectionally investigate the type of pulmonary dysfunction as assessed by means of spirometry and in correlation with the level of iron accumulation within the liver and the myocardium as determined by MRI in a cohort of children and young adults with beta-thalassemia major.