Patients with β-thalassemia often present with a restrictive pattern at pulmonary function tests (PFTs) due to several pathogenetic factors. However, the long-term evolution is unknown. We performed a longitudinal study of pulmonary function in asymptomatic, non-smoking patients with β-thalassemia major and intermedia. We looked fortemporal changes in lung function and characteristics that would predict the development of PFT abnormalities. In 1996, 18 patients with major β-thalassemia (9 males and 9 females; age range: 18-35 years) and 11 patients with intermediate β-thalassemia (5 males and 6 females; age range: 25-51 years) underwent clinical assessment and PFT, including body plethysmography and gas transfer study (carbon monoxide diffusion capacity, DLCO). Patients were reassessed in 2003. An echocardiographic evaluation was also obtained to exclude pulmonary hypertension. In 55.5% of major and 45.4% of intermediate β-thalassemia patients, a restrictive pattern was found in 1996; in 2003 only 38.8 and 27.2% of patients, respectively, exhibited total lung capacities below the predicted values. DLCO was unchanged in both groups of patients, being reduced in 5 thalassemia major patients and within the normal range in intermediate patients. We conclude that asymptomatic patients with β-thalassemia have a high prevalence of PFT abnormalities, but without significant increases over time. An improvement may be observed when good control of the iron balance is reached with optimal chelation therapy. Copyright

Lung function in beta-thalassemia patients : a longitudinal study / G. Piatti, L. Allegra, V. Fasano, C. Gambardella, M. Bisaccia, M.D. Cappellini. - In: ACTA HAEMATOLOGICA. - ISSN 0001-5792. - 116:1(2006), pp. 25-29. [10.1159/000092344]

Lung function in beta-thalassemia patients : a longitudinal study

G. Piatti
Primo
;
L. Allegra
Secondo
;
V. Fasano;C. Gambardella;M.D. Cappellini
Ultimo
2006

Abstract

Patients with β-thalassemia often present with a restrictive pattern at pulmonary function tests (PFTs) due to several pathogenetic factors. However, the long-term evolution is unknown. We performed a longitudinal study of pulmonary function in asymptomatic, non-smoking patients with β-thalassemia major and intermedia. We looked fortemporal changes in lung function and characteristics that would predict the development of PFT abnormalities. In 1996, 18 patients with major β-thalassemia (9 males and 9 females; age range: 18-35 years) and 11 patients with intermediate β-thalassemia (5 males and 6 females; age range: 25-51 years) underwent clinical assessment and PFT, including body plethysmography and gas transfer study (carbon monoxide diffusion capacity, DLCO). Patients were reassessed in 2003. An echocardiographic evaluation was also obtained to exclude pulmonary hypertension. In 55.5% of major and 45.4% of intermediate β-thalassemia patients, a restrictive pattern was found in 1996; in 2003 only 38.8 and 27.2% of patients, respectively, exhibited total lung capacities below the predicted values. DLCO was unchanged in both groups of patients, being reduced in 5 thalassemia major patients and within the normal range in intermediate patients. We conclude that asymptomatic patients with β-thalassemia have a high prevalence of PFT abnormalities, but without significant increases over time. An improvement may be observed when good control of the iron balance is reached with optimal chelation therapy. Copyright
β-Thalassemia; Hematological disorders; Lung function; Restrictive impairment
Settore MED/10 - Malattie dell'Apparato Respiratorio
Settore MED/09 - Medicina Interna
2006
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/29293
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