Pulmonary Dysfunction in Thalassemia Major Patients
| Autor: | Alessandra Gennari, Annaluisa Cogo, Andrea Ferraro, Caterina Borgna-Pignatti, Lucetta Capra, Maria Paola Bellagamba, Alessandra di Stasio |
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| Rok vydání: | 2005 |
| Předmět: |
Spirometry
medicine.medical_specialty Lung medicine.diagnostic_test business.industry Thalassemia Immunology Management of thalassemia Cell Biology Hematology medicine.disease Biochemistry Gastroenterology Pulmonary hypertension Pulmonary dysfunction Thalassemia Major Pulmonary function testing Surgery FEV1/FVC ratio medicine.anatomical_structure Internal medicine medicine Lung volumes business |
| Zdroj: | Blood. 106:3814-3814 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v106.11.3814.3814 |
| Popis: | 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. Results: Only the patient with mediastinal masses showed an obstructive pattern with the FEV1/FVC ratio of 53% of predicted value. Thirteen patients (37%)had a restrictive pattern (TLC |
| Databáze: | OpenAIRE |
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