The improved survival of patients with Thalassemia Major (TM) has allowed new complications to develop in the adult patients. These previously unknown clinical entities require a new approach to the management of thalassemia. Pulmonary dysfunction is one of the least understood, previously unrecognized, complications of TM. Patients and Methods The aim of our study was to define the spirometric pattern and lung damage in 35 TM patients (18 M and 17 F, age range from 18 to 44 years). All were regularly transfused (pre transfusion hemoglobin level 9+/-0,4 g/dl) and chelated (25 with deferoxamine,5 with deferiprone and 5 with both chelators). Their serum ferritin level was 1071+/-628 ng/ml All patients had normal cardiac ejection fraction. Nine patients were active smokers (10+2 pack/years) while 9 had been smokers in the past (6+2 pack/years). Four had a mild allergic asthma under good clinical control, 1 had a mild pulmonary hypertension and 1 had mediastinal hematopoietic tissue. Pulmonary function tests were performed before transfusion and included pulmonary volumes and flows recorded by spirometry; residual volume (RV) and total lung capacity (TLC)measured by helium closed-circuit rebreathing technique; carbon monoxide diffusion capacity (TLCO)measured by single breath method.
Pulmonary dysfunction in Thalassemia Major patients.
GENNARI, Alessandra;BELLAGAMBA, Maria Paola;DI STASIO, Alessandra;FERRARO, Andrea;COGO, Annaluisa;BORGNA, Caterina;
2005
Abstract
The improved survival of patients with Thalassemia Major (TM) has allowed new complications to develop in the adult patients. These previously unknown clinical entities require a new approach to the management of thalassemia. Pulmonary dysfunction is one of the least understood, previously unrecognized, complications of TM. Patients and Methods The aim of our study was to define the spirometric pattern and lung damage in 35 TM patients (18 M and 17 F, age range from 18 to 44 years). All were regularly transfused (pre transfusion hemoglobin level 9+/-0,4 g/dl) and chelated (25 with deferoxamine,5 with deferiprone and 5 with both chelators). Their serum ferritin level was 1071+/-628 ng/ml All patients had normal cardiac ejection fraction. Nine patients were active smokers (10+2 pack/years) while 9 had been smokers in the past (6+2 pack/years). Four had a mild allergic asthma under good clinical control, 1 had a mild pulmonary hypertension and 1 had mediastinal hematopoietic tissue. Pulmonary function tests were performed before transfusion and included pulmonary volumes and flows recorded by spirometry; residual volume (RV) and total lung capacity (TLC)measured by helium closed-circuit rebreathing technique; carbon monoxide diffusion capacity (TLCO)measured by single breath method.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
