Tuğçe KAZGAN, Begül YAĞCI KÜPELİ, Aylin KONT ÖZHAN

Talasemi major hastalarında asemptomatik solunum fonksiyon bozukluğu

Amaç: Düzenli transfüzyonlar, transfüzyona bağımlı talasemide (TDT) kalp, karaciğer ve akciğer gibi kritik organlarda ilerleyici demir birikmesine neden olur. Bu çalışmada TDT'li hastalarda solunum fonksiyon bozukluğunu ve solunum fonksiyon bozukluğunu etkileyen faktörlerin değerlendirilmesi amaçlanmıştır.Gereç ve Yöntem: Sağlık bilimleri üniversitesi, Adana Eğitim Ve Araştırma Merkezi Çocuk Hematolojisi / Onkoloji Ünitesi’nde TDT nedeniyle takip ve tedavi edilmekte olan hastalar içinden solunum fonksiyon testi yapılan hastalar retrospektif olarak değerlendirildi.Bulgular: Solunum fonksiyon testleri 40 TDT tanılı hastada (19 kadın; 21 erkek) yapıldı. Tanı sırasındaki ortalama yaş 6.63 ± 5.74 ay, ortalama vücut kitle indeksi 18.57 ± 2.27 kg / m², ortalama transfüzyon programı süresi 151.67 ± 50.48 ay idi. Splenektomi oranı%48 idi. Bütün hastalar demir şelasyon tedavisi kullanıyordu. Solunum fonksiyon testlerinde hastaların%57,5’inde olan normal bulgular, %27,5’inde küçük hava yolu tıkanıklığı bulguları ile restriktif akciğer disfonksiyonu ve%15’inde izole restriktif disfonksiyon saptandı. Solunum fonksiyonlarında yaş, cinsiyet, transfüzyon programı süresi, ferritin ve hemoglobin düzeyleri, splenektomi varlığı veya şelasyon tedavisi tipi açısından istatistiksel olarak anlamlı bir fark saptanmadı. Ferritin düzeyi 2500 ng / dl'nin üzerinde olan hastalarda zorlu ekspiratuar hacim (FEV1) ve zorlu vital kapasite (FVC) için anlamlı olarak düşük değerler bulundu.Sonuç: Çalışmamızda, solunum semptomu olmayan TDT tanılı hastaların yaklaşık yarısında kısıtlayıcı solunum fonksiyon bozukluğu tespit edildi. Yüksek ferritin düzeyleri solunum disfonksiyonunu işaret edebileceğinden hastaların bu açıdan yakın takip edilmesi uygun olacaktır.

Anahtar Kelimeler:

Talasemi, solunum fonksiyon bozukluğu, solunum fonksiyon testleri

Asymptomatic respiratory dysfunction in patients with thalassemia major

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.

Keywords:

Thalassemia, respiratory dysfunction, pulmonary function tests,

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