Pulmoner functi̇ons in chi̇ldren with transfusi̇on dependent B thalassemi̇a major

Background and Aim:Long-term blood transfusion, extravascular hemolysis and increased intestinal iron absorption in patients with β Thalassemia Major (β-TM) lead to excessive iron accumulation in the body.Clinical manifestations of iron overload may occur in different organs and tissues in the body.In this study, we aimed to evaluate the clinical features, findings of the chest X-ray and the results of pulmonary function tests (PFT) of thetransfusion-dependent β TM patients who were followed up in our pediatric hematology clinic. Material and Methods:In this study, lung function was investigated by case control method in children aged between 12 and 18 years who were followed-up in Dicle University Medical Faculty Pediatric Hematology and Oncology Department.The study was approved by Dicle University Medical Faculty Ethics Committee.Children older than 12 years of age were planned to be included in the study in order to adapt to pulmonary function tests. The study included patients with genetically and clinically confirmed β-TM who underwent regular blood transfusions for at least two years, who had regular iron chelators, and had complete PFT compliance.Patients with known lung disease, known asthma and emphysema, who had any pathology on echocardiography, who did not follow their follow-up regularly, and who used cigarette and other tobacco products, were not included in the study. Demographic findings (age, gender, the first diagnosis age, duration of follow-up, name and dose of chelator used), laboratory results (complete blood count, renal function test, liver function test and ferritin value), results of PFT and findings detected by experienced specialist in chest x-ray were recorded. Findings:A total of 26 patients (16 male, 10 female) with a mean age of 14.77±2.97 years were included in our study. The mean hemoglobin level of the patients was 8.56 ± 0.73 g/dL and the ferritin level was 2837±3199 ng/dL.All patients were using deferasirox (DFX) and the mean DFX dose was 1096.15±316.85 mg. In the Chest x-ray findings of the patients; 13 (50%) had normal, 4 (15.4%) had right prominent bronchovascular markings, 2 (7.7%) had bilateral prominent bronchovascular markings, and 2 (7.7%) had right hilar fullness. 13 (50%) of the patients had normal, 7 (26.9%) had restrictive, 4 (15.4%) had mixed and 2 (7.7%) had obstructive type of PFT. All 5 patients who underwent splenectomy were found to be older than 14 years (p = 0.004).There was no statistically significant difference between the two groups with ferritin levels <1500 ng / ml and> 1500 ng / ml in terms of PFT results (p> 0.05). FVC values of 11 patients (42.3%) were below 80%. FVC values were found to be lower (<80%) in older children and longer follow-up children (p = 0.018 and p = 0.025, respectively).There was no significant difference between the genders in terms of PFT results and splenectomy (p> 0.05). It was determined that male patients were diagnosed with TM at a later stage (18 months versus 7.5 months) compared to female patients and this difference was statistically significant (p = 0.024). IgE levels in children with BMI <18 kg/m2 were statistically significantly higher than in children with BMI> 18 kg/m2 (36.8 versus 18.8 IU/mL) (p = 0.003). Conclusion:In asymptomatic periods of patients diagnosed with transfusion-dependent β-TM, we found high pathologic findings on chest radiographs, large restrictive and mixed respiratory distress symptoms, and a lower rate of obstructive respiratory distress.We think that the life expectancy and quality of life can be increased by the early detection of respiratory problems of patients with β-TM. In the clinical follow-up of these patients, we think that more detailed studies should be done on HRCT with different PFT methods and lung findings. Keywords: Thalassemia major, LFT, chest x-ray, iron, chelator