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Asymptomatic respiratory dysfunction in patients with thalassemia major

Year 2019, Volume: 44 Issue: 4, 1317 - 1322, 29.12.2019
https://doi.org/10.17826/cumj.525791

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. 


References

  • References1. Cunningham M. The Thalassemias. In: Nathan and Oski's Hematology of Infancy and Childhood (Nathan David G., IV Samuel Lux, Look A. Thomas, Ginsburg David, Orkin Stuart H., Fisher David E.). Philadelphia: WB Saunders. 2009; 1015-106.2. Keser I. Hemoglobinopatilerde moleküler çalışmalar. Türkiye Klinikleri J Pediatr Sci 2007;3: 9.3. Martin A, Thompson A. Thalassemias. Pediatr Clin North Am 2013; 60: 1383-91.4. Whetherall DJ. Pathophsiology of thalassemia. Bailliere’s Clinical Hematology 1998; 11: 127-46.5. Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med 2005; 353: 1135-46.6. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood 2011; 118: 3479-88.7. Ware HM, Kwiatkowski JL. Evaluation and treatment of transfusional iron overload in children. Pediatr Clin North Am. 2013; 60: 1393-406.8. Lands LC, Woods S, Katsardis CH et al. The effects of diuresis and transfusion on pulmonary function in children with thalassemia Major. . Pediatr Pulmonol 1991; 11: 340-4.9. Miller MR, Hankinson J, Brusasco V. Standardisation of spirometry. In: Ats/Ers/Task Force: Standardisation Of Lung Function Testing: Eur Respir J. 2005; 319–38.10. Factor JM, Pottipati SR, Rappoport I et al. Pulmonary function abnormalities in thalassemia major and the role of iron overload. Am J Respir Crit Care Med 1994; 149: 1570-411. Piatti G, Allegra L, Ambrosetti U et al. Beta-thalassemia and pulmonary function. Haematologica 1999; 84: 804-8.12. Borgna-Pignatti C, Galanello R. Thalassemias and related disorders : quantitative disorders of hemoglobin synthesis In: Lippincott Williams & Wilkins (JP Greer, DA Arber, B Glader), 13 edn. Philadelphia. 2013; 862-913.13. Ozyoruk D, Misirlioglu ED. Pulmonary Functions in Children With Thalassemia Major. J Pediatr Hematol Oncol 2015; 37: 605-10.14. Abu-Ekteish FM, Al-Rimawi HS, Al-Ali MK et al. Pulmonary function tests in children with beta-thalassemia major. Chron Respir Dis 2007; 4: 19-22.15. Yang G, Liu R, Peng P et al. How Early Can Myocardial Iron Overload Occur in Beta Thalassemia Major? PLoS ONE 2014; 9.16. Borgna-Pignatti C, Meloni A, Guerrini G et al. Myocardial iron overload in thalassaemia major. How early to check? Br J Haematol 2014; 164: 579-85.17. Evangalia B, Meropi D, Marina E. Restrictive Pulmonary Dysfunction and Its Predictors in Young Patients With B-Thalassemia Major. Pediatr Pulmonol 2012; 47: 801-7.18. Landing BH, Nadorra R, Hyman CB et al. Pulmonary lesions of thalassemia major. Perspect Pediatr Pathol 1987; 11: 82-96.19. Carnelli V, D'Angelo E, Pecchiari M et al. Pulmonary dysfunction in transfusion-dependent patients with thalassemia major. Am J Respir Crit Care Med 2003; 168: 180-4.20. Canatan D, Koc N. The effect of transfusion on pulmonary function tests in patients with thalassemia. Turk J Haematol 2004; 21: 137-921. Azarkeivan A, Mehrvar A, SohrabPour H et al. Pulmonary function test in transfusion-dependent-thalassemia patients. Pediatr Hematol Oncol 2009; 598: 598-606.22. Sağlam N, Yaroğlu S, Bülbül L. Bakırköy Tıp Dergisi 2015; 11: 43-8.23. Li AM, Chan D, Li CK et al. Respiratory function in patients with thalassaemia major: relation with iron overload. Arch Dis Child 2002; 87: 328-30.24. Khong PL, Chan GC, Lee SL et al. Beta-thalassemia major: thin-section CT features and correlation with pulmonary function and iron overload. Radiology 2003; 229: 507-1225. King GG, Brown NJ, Diba C et al. The effects of body weight on airway calibre. Eur Respir J 2005; 25: 896-901.26. Parakh A, Dubey AP, Chowdhury V et al. Study of pulmonary function tests in thalassemic children. J Pediatr Hematol Oncol 2007; 29: 151-5.27. Priftis KN, Anthracopoulos MB, Tsakanika C et al. Quantification of siderophages in bronchoalveolar fluid in transfusional and primary pulmonary hemosiderosis. Pediatr Pulmonol 2006; 41: 972-7.

Talasemi major hastalarında asemptomatik solunum fonksiyon bozukluğu

Year 2019, Volume: 44 Issue: 4, 1317 - 1322, 29.12.2019
https://doi.org/10.17826/cumj.525791

Abstract

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.


References

  • References1. Cunningham M. The Thalassemias. In: Nathan and Oski's Hematology of Infancy and Childhood (Nathan David G., IV Samuel Lux, Look A. Thomas, Ginsburg David, Orkin Stuart H., Fisher David E.). Philadelphia: WB Saunders. 2009; 1015-106.2. Keser I. Hemoglobinopatilerde moleküler çalışmalar. Türkiye Klinikleri J Pediatr Sci 2007;3: 9.3. Martin A, Thompson A. Thalassemias. Pediatr Clin North Am 2013; 60: 1383-91.4. Whetherall DJ. Pathophsiology of thalassemia. Bailliere’s Clinical Hematology 1998; 11: 127-46.5. Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med 2005; 353: 1135-46.6. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood 2011; 118: 3479-88.7. Ware HM, Kwiatkowski JL. Evaluation and treatment of transfusional iron overload in children. Pediatr Clin North Am. 2013; 60: 1393-406.8. Lands LC, Woods S, Katsardis CH et al. The effects of diuresis and transfusion on pulmonary function in children with thalassemia Major. . Pediatr Pulmonol 1991; 11: 340-4.9. Miller MR, Hankinson J, Brusasco V. Standardisation of spirometry. In: Ats/Ers/Task Force: Standardisation Of Lung Function Testing: Eur Respir J. 2005; 319–38.10. Factor JM, Pottipati SR, Rappoport I et al. Pulmonary function abnormalities in thalassemia major and the role of iron overload. Am J Respir Crit Care Med 1994; 149: 1570-411. Piatti G, Allegra L, Ambrosetti U et al. Beta-thalassemia and pulmonary function. Haematologica 1999; 84: 804-8.12. Borgna-Pignatti C, Galanello R. Thalassemias and related disorders : quantitative disorders of hemoglobin synthesis In: Lippincott Williams & Wilkins (JP Greer, DA Arber, B Glader), 13 edn. Philadelphia. 2013; 862-913.13. Ozyoruk D, Misirlioglu ED. Pulmonary Functions in Children With Thalassemia Major. J Pediatr Hematol Oncol 2015; 37: 605-10.14. Abu-Ekteish FM, Al-Rimawi HS, Al-Ali MK et al. Pulmonary function tests in children with beta-thalassemia major. Chron Respir Dis 2007; 4: 19-22.15. Yang G, Liu R, Peng P et al. How Early Can Myocardial Iron Overload Occur in Beta Thalassemia Major? PLoS ONE 2014; 9.16. Borgna-Pignatti C, Meloni A, Guerrini G et al. Myocardial iron overload in thalassaemia major. How early to check? Br J Haematol 2014; 164: 579-85.17. Evangalia B, Meropi D, Marina E. Restrictive Pulmonary Dysfunction and Its Predictors in Young Patients With B-Thalassemia Major. Pediatr Pulmonol 2012; 47: 801-7.18. Landing BH, Nadorra R, Hyman CB et al. Pulmonary lesions of thalassemia major. Perspect Pediatr Pathol 1987; 11: 82-96.19. Carnelli V, D'Angelo E, Pecchiari M et al. Pulmonary dysfunction in transfusion-dependent patients with thalassemia major. Am J Respir Crit Care Med 2003; 168: 180-4.20. Canatan D, Koc N. The effect of transfusion on pulmonary function tests in patients with thalassemia. Turk J Haematol 2004; 21: 137-921. Azarkeivan A, Mehrvar A, SohrabPour H et al. Pulmonary function test in transfusion-dependent-thalassemia patients. Pediatr Hematol Oncol 2009; 598: 598-606.22. Sağlam N, Yaroğlu S, Bülbül L. Bakırköy Tıp Dergisi 2015; 11: 43-8.23. Li AM, Chan D, Li CK et al. Respiratory function in patients with thalassaemia major: relation with iron overload. Arch Dis Child 2002; 87: 328-30.24. Khong PL, Chan GC, Lee SL et al. Beta-thalassemia major: thin-section CT features and correlation with pulmonary function and iron overload. Radiology 2003; 229: 507-1225. King GG, Brown NJ, Diba C et al. The effects of body weight on airway calibre. Eur Respir J 2005; 25: 896-901.26. Parakh A, Dubey AP, Chowdhury V et al. Study of pulmonary function tests in thalassemic children. J Pediatr Hematol Oncol 2007; 29: 151-5.27. Priftis KN, Anthracopoulos MB, Tsakanika C et al. Quantification of siderophages in bronchoalveolar fluid in transfusional and primary pulmonary hemosiderosis. Pediatr Pulmonol 2006; 41: 972-7.
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Details

Primary Language English
Subjects Paediatrics, Respiratory Diseases, Clinical Sciences
Journal Section Research
Authors

Tuğçe Kazgan This is me

Begül Yağcı Küpeli

Aylin Kont Özhan This is me

Publication Date December 29, 2019
Acceptance Date April 5, 2019
Published in Issue Year 2019 Volume: 44 Issue: 4

Cite

MLA Kazgan, Tuğçe et al. “Asymptomatic Respiratory Dysfunction in Patients With Thalassemia Major”. Cukurova Medical Journal, vol. 44, no. 4, 2019, pp. 1317-22, doi:10.17826/cumj.525791.