RT Journal Article
SR Electronic
T1 Ventilatory restrictive impairment in thalassemic patients: Gender differences and correlation with hypogonadism and iron overload
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 3120
VO 40
IS Suppl 56
A1 S. Roggero
A1 L. Zito
A1 A. Piga
A1 C. Ciacco
A1 M. Guglielmo
A1 M.V. de Vita
A1 R. Torchio
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/3120.abstract
AB BACKGROUND and AIMVentilatory restrictive (RES) impairment has been described in β-thalassemia, but no evidence exists on the causal mechanism. We investigated relationships among lung function, iron overload and clinical parameters in a homogeneous series of β-thalassemia major adult patients.METHODSWe studied 79 patients (males M/ females F 44/35; age 34.5±6.8 years) with β-thalassemia major on regular transfusion and iron chelation. Iron overload was assessed by serum ferritin, liver iron concentration (LIC) by SQUID susceptometry, cardiac iron by MRI T2*. Lung volumes, diffusion capacity, chelator drugs, hypogonadism (H), hypothyroidism and osteoporosis were evaluated in stable hematologic conditions.RESULTS30/79 pt (38%) [ 21/44 (47.7%) M and 9/35 (25.7%) F] showed RES, 6 bronchial obstruction with no gender differences (3M 3F). In F LIC was higher 2249±903 vs 1497±553 mgFe/gliver dw; p<0.008 in RES vs normals and correlated with total lung capacity (r=-0.402 p<0.03). No differences were present for hypothyroidism, osteoporosis or 3 iron chelators. Among RES patients 57%M and 88%F were H. H males showed higher cardiac iron MRI T2* 29.6±12.7 vs 39.6±9.2 ms; p<0.03) but similar LIC in respect to non H, without correlation with total lung capacity. In F cardiac and hepatic iron levels were not different between H and non H.CONCLUSIONSRES is present in 1/3 of well compensated adult thalassemic (M/F 1.88). Iron (LIC) was higher in RES F vs normals and H males have higher MRI T2*. Different gender impact of H on lung and chest growth, severity and efficacy of replacement therapy can explain data but the involved mechanisms is still unclear.