RESUMO
INTRODUCTION: ß-thalassemia's are hereditary chronic hemolytic diseases, the mainstay of treatment of thalassemia major is regular blood transfusion and iron chelation. They cause many complications, one of the recognized complications related to respiratory system is pulmonary hypertension. Respiratory functions in those patients are not well studied in most of the world. The studies done to assess respiratory function are inconsistent, some found a predominantly restrictive pattern, others found obstructive pattern, and few found normal spirometry. The aim of this study was to assess the spirometric patterns in asymptomatic Omani patients with transfusion-dependent thalassemia using spirometry studies. METHODS: Transfusion-dependent thalassemia patients who are registered at Sultan Qaboos University Hospital who are > 15 years old and able to perform spirometry test were selected for the study after they signed the informed consent. All the patients were free of any respiratory disease. Spirometry was performed in all patients in the sitting position and FVC, FEV1, FEV1/FVC were obtained. RESULTS: Total number of thalassemia patients enrolled in the study was 37. 32 patients are suffering from thalassemia major and 5 are suffering from thalassemia intermedia. The mean age of our patients was 29.95 years. We found that 37.8 % of the patients showed normal spirometry. Most patients had abnormal spirometry (62.1 %). Of these, 35.1 % showed a restrictive pattern while 27 % showed obstructive pattern. CONCLUSION: Spirometry assessment of the lung function in thalassemia patients who are receiving regular transfusion showed that majority had abnormal spirometry results despite being asymptomatic from a respiratory point of view.
