Are pulmonary and extra-pulmonary features affected in patients with Beta-thalassemia?

Abstract

<b>Background:</b> Pulmonary and musculoskeletal system effects can be seen in patients with Beta-thalassemia depending on the disease or the side effects of the treatment methods. <b>Aims and objectives:</b> The aim of this study was to investigate and compare pulmonary functions, respiratory muscle function, exercise capacity in patients with Beta-thalassemia intermedia (β-TI) and Beta-thalassemia major (β-TM). <b>Methods:</b> Forty patients (mean age 43.18±7.75) with diagnosis of β-TI and β-TM (18F/22M) were included in this study. Demographic characteristics were recorded. Pulmonary functions (spirometry), respiratory muscle strength (maximal inspiratory pressure, maximal expiratory pressure), respiratory muscle endurance (incremental threshold loading test) and exercise capacity (6-minute walking test) perception of fatigue (Fatigue Severity Scale) and quality of life (Short Form-36) were evaluated. <b>Results:</b> The age and the body mass index values were significantly lower in the β-TM group (p&lt;0.05) when compared with β-TI group. Pulmonary functions, respiratory muscle strength and endurance, exercise capacity and quality of life were found to be decreased and fatigue perception was increased in patients with beta-thalassemia while there was no statistically significant difference in these values between groups (p&gt;0.05). <b>Conclusions:</b> Pulmonary and extra-pulmonary features can be affected in patients with beta-thalassemia. Although these effects are similar in β-TI and β-TM groups, respiratory muscle function, exercise capacity and quality of life may be negatively affected by presence of disease. Patients with Beta-thalassemia should be included in pulmonary rehabilitation programs.