Talasemi major hastalarında asemptomatik solunum fonksiyon bozukluğu

Abstract

Purpose: Regular transfusions cause progressive iron deposition in critical organs such as heart, liver and lungs in transfusion-dependent thalassemia (TDT). We aimed to evaluate respiratory dysfunction and factors affecting respiratory dysfunction in patients with TDT.Materials and Methods: Patients with TDT treated at department of Pediatric Hematology/Oncology of Sağlık Bilimleri University, Adana Education and Research Center were evaluated retrospectively. Results: Respiratory function tests were performed in 40 patients (19 female; 21 male) with TDT. The mean age at diagnosis was 6.63±5.74 months, mean body mass index was 18.57±2.27 kg/m², mean transfusion program duration was 151.67±50.48 months. Splenectomy rate was 48%. All of the patients were using iron chelation therapy. Respiratory function tests reveled normal findings in 57.5%, restrictive lung dysfunction with small airway obstruction findings in 27.5% and isolated restrictive dysfunction in 15% of patients. There was no statistically significant difference in respiratory dysfunction according to age, gender, transfusion program duration, ferritin and hemoglobin levels, presence of splenectomy, or type of chelation therapy. Patients with ferritin levels above 2500 ng/dl had significantly lower values for forced expiratory volüme (FEV1) and forced vital capacity (FVC) Conclusion: In our study, restrictive respiratory dysfunction was detected in nearly half of the patients with TDT who had no respiratory symptoms. Higher ferritin levels may indicate the need for close follow-up for respiratory symptoms.