作者
Francesca Guidotti, Gioia Piatti, Alessia Marcon, Elena Cassinerio, Marianna Giuditta, Alberto Roghi, Valter Fasano, Dario Consonni, Maria Domenica Cappellini
发表日期
2017/1
期刊
British journal of haematology
卷号
176
期号
2
页码范围
309-314
简介
Although pulmonary function abnormalities in thalassaemia major (TM) were described in 1980, the pathogenetic mechanism is not clear and data are contradictory, probably because of study heterogeneity and the multifactorial nature of the pathogenesis. We retrospectively analysed 73 adult TM patients to evaluate the prevalence of pulmonary dysfunction in adult TM and investigate relationships with iron load. All patients underwent body plethysmography and carbon monoxide diffusion (DLCO) was assessed in 63, in addition to blood tests, echocardiogram and T2* myocardial and liver magnetic resonance imaging. Restrictive lung disease was present in 26 (35·6%) patients. Serum ferritin levels were higher in patients with restrictive pattern (1526 μg/l vs. 975 μg/l, P = 0·05). Restrictive lung disease did not correlate with cardiac or liver iron overload. However, considering only patients with serum ferritin >2500 …
引用总数
2016201720182019202020212022202313232342
学术搜索中的文章
F Guidotti, G Piatti, A Marcon, E Cassinerio, M Giuditta… - British journal of haematology, 2017