การศึกษาสมรรถภาพปอดในผู้ป่วยเด็กโรคเบต้าธาลัสซีเมียชนิดรุนแรง

Abstract

Abnormal PFT in patients with severe form of β -thalassemia has been reported. Objective: To determine the prevalence and factors a ssociated with abnormal PFT in patients with severe form of β -thalassemia. Methods: PFT of patients with severe form of β-thalassemia was performed. Body plethysmography was used to determine PFT and lung volume. Diffusion capacity of carbon monoxide (DLCO) was also measured. Results:Twenty-one patients with β-thalassemia. Seventeen patients (81%) had abnormal PFT. Eight patients (47%) had mild  degree of air trapping (RV/TLC > 30%). even patients (41%) had combined restrictive and air trapping. wo patients   ad restrictive defect. None of the patients has abnormality of DLCO or hypoxemia in room  air. Age duration  of disease and history of splenectomy are associated with those who had abnormal PFT. Conclusion:Majority  of patients with severe form of β-thalassemia have mild degree of abnormal pulmonary function  tests. Age uration of disease and history of splenectomy were associated with abnormal PFT.