Lung function in beta-thalassemia patients: a longitudinal study.
Patients with beta-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 beta-thalassemia major and intermedia. We looked for temporal changes in lung function and characteristics that would predict the development of PFT abnormalities. In 1996, 18 patients with major beta-thalassemia (9 males and 9 females; age range: 18-35 years) and 11 patients with intermediate beta-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, DL(CO)). 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 beta-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. DL(CO) 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 beta-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.
Piatti
G
G
Istituto di Tisiologia e delle Malattie dell'Apparato Respiratorio, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Fondazione IRCCS, Milano, Italia. gioia.piatti@unimi.it
Allegra
L
L
Fasano
V
V
Gambardella
C
C
Bisaccia
M
M
Cappellini
M D
MD
D000293:Adolescent; D000328:Adult; D015913:Chelation Therapy; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism; D008137:Longitudinal Studies; D008168:Lung / Q000002:abnormalities / Q000378:metabolism / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010993:Plethysmography, Whole Body; D015995:Prevalence; D011659:Pulmonary Gas Exchange; D017086:beta-Thalassemia / Q000378:metabolism / Q000503:physiopathology* / Q000628:therapy
D007501:Iron