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Respiratory System Disorders in Thalasemia

Yıl 2017, Cilt: 26 Sayı: 3, 352 - 377, 30.09.2017
https://doi.org/10.17827/aktd.303592

Öz

Thalassemia major is a worldwide healthcare problem requiring high cost medical treatment. Patients usually die of treatment associated complications in the second decade. However, improvements in treatment and meticulous follow-up strategies for treatment associated complications have led to an increase in expected life span of the patients. Regular transfusions cause progressive iron deposition in critical organs such as heart, liver and lungs. Iron deposition in the lungs results in respiratory dysfunction and impairment in quality of life of patients with thallassemia. Although iron deposition in respiratory system has been a known reality for many years in thallassemia, the effect of this deposition on respiratory functions has been an ongoing debate. 

Kaynakça

  • 1.Cunningham M. The Thalassemias. Nathan and Oski's Hematology of Infancy and Childhood 2009;1015-1106.
  • 2.Keser I. Hemoglobinopatilerde moleküler çalışmalar. Türkiye Klinikleri J Pediatr Sci. 2007;3:9.
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  • 4.Whetherall DJ. Pathophsiology of thalassemia. Bailliere’s Clinical Hematology 1998;11:127-46.
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  • 6.Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood 2011;118:3479-88.
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  • 8.Lands LC, Woods S, Katsardis CH, Desmond K, Coates AL. The effects of diuresis and transfusion on pulmonary function in children with thalassemia Major. Pediatr Pulmonol. 1991;11:340-4.
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  • 10. Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Guidelines for the Clinical Management of Thalassaemia, 2008.
  • 11. Salomon-Andonie J, Miasnikova G, Sergueeva A, Polyakova LA, Niu X, Nekhai S et al. Effect of congenital upregulation of hypoxia inducible factors on percentage of fetal hemoglobin in the blood. Blood 2013;122:3088-9.
  • 12.Hoffman R. Thalassemia syndromes in hematology basic principles an practice. Hematology : basic principles and practice, 2000;485-509.
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  • 16. Çürük MA, Genç A, Huseynova P, Zeren F, Aksoy K. Çukurova’da alfa talasemi genotipleri ve Hb H hastalığı. Türkiye Klinikleri J Pediatr Sci. 2007;3:17-23.
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  • 20. Gümrük F. Hemoglobinopatilerin Tanı ve Tedavisinde Yenilikler. Türk Hematoloji Dernegi 9. mezuniyet sonrası egitim kursu 2006:62-4.
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  • 28.Taher AT, Musallam KM, Karimi M, Cappellini MD. Contemporary approaches to treatment of beta-thalassemia intermedia. Blood Rev. 2012;26 (Suppl 1):S24-7.
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Talasemilerde Solunum Sistemi Bozuklukları

Yıl 2017, Cilt: 26 Sayı: 3, 352 - 377, 30.09.2017
https://doi.org/10.17827/aktd.303592

Öz

Talasemi majör, tüm dünyada önemli bir sağlık sorunu olup, yüksek maliyetli tedavi gerektiren bir hastalıktır. Hastaların çoğu ikinci dekatta tedaviye bağlı komplikasyonlar nedeniyle kaybedilmektedir. Son yıllarda kullanıma giren yeni tedavi rejimleri ve tedaviye bağlı komplikasyonların dikkatli bir şekilde izlenmesiyle hastaların beklenen yaşam süreleri artmaktadır. Düzenli kan transfüzyonları nedeniyle kalp, karaciğer ve akciğer gibi hayati organlarda ilerleyici demir birikimi görülmektedir. Akciğer de demir birikiminin yaşandığı organlardan birisidir. Akciğerlerdeki demir birikimi akciğer dinamiklerinde değişikliklere yol açmakta ve bu durum hastaların yaşam kalitesini olumsuz etkilemektedir. Bu hastalarda akciğerlerde demir birikiminin olduğu uzun süreden beri bilinen bir gerçek ise de, bu birikimin akciğerler üzerine nasıl etkide bulunduğu konusu hala tartışma konusudur. 

Kaynakça

  • 1.Cunningham M. The Thalassemias. Nathan and Oski's Hematology of Infancy and Childhood 2009;1015-1106.
  • 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, Desmond K, Coates AL. The effects of diuresis and transfusion on pulmonary function in children with thalassemia Major. Pediatr Pulmonol. 1991;11:340-4.
  • 9.Zheng G, Schaefer M, Karplus M. Hemoglobin Bohr effects: atomic origin of the histidine residue contributions. Biochemistry 2013;52:8539-55.
  • 10. Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Guidelines for the Clinical Management of Thalassaemia, 2008.
  • 11. Salomon-Andonie J, Miasnikova G, Sergueeva A, Polyakova LA, Niu X, Nekhai S et al. Effect of congenital upregulation of hypoxia inducible factors on percentage of fetal hemoglobin in the blood. Blood 2013;122:3088-9.
  • 12.Hoffman R. Thalassemia syndromes in hematology basic principles an practice. Hematology : basic principles and practice, 2000;485-509.
  • 13.Andrews NC, Ullrich CK, Fleming MD. Disorders of iron metabolism and sideroblastic anemia. Nathan and Oski's Hematology of Infancy and Childhood 2009;1054-74.
  • 14.Başak N. Βeta Talasemide moleküler tanı ve yöntemleri. Talasemi ve hemoglobinopatiler Tanı ve Tedavi -Talasemi Federasyonu. 2007;49-60.
  • 15. Higgs DR, Thein SL, Wood WG. Human haemoglobin. The Thalassaemia Syndromes 2001;65-120.
  • 16. Çürük MA, Genç A, Huseynova P, Zeren F, Aksoy K. Çukurova’da alfa talasemi genotipleri ve Hb H hastalığı. Türkiye Klinikleri J Pediatr Sci. 2007;3:17-23.
  • 17.Vichinsky E. Complexity of alpha thalassemia: growing health problem with new approaches to screening, diagnosis, and therapy. Ann N Y Acad Sci. 2010;1202:180-7.
  • 18.Higgs DR. Alpha thalassemia: An Owerview. Current Views on Thalassemia 1992;31-40.
  • 19.Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98:833-44.
  • 20. Gümrük F. Hemoglobinopatilerin Tanı ve Tedavisinde Yenilikler. Türk Hematoloji Dernegi 9. mezuniyet sonrası egitim kursu 2006:62-4.
  • 21.Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-7.
  • 22.Kocak R, Alparslan ZN, Agridag G, Başlamisli F, Aksungur PD, Koltaş S. The frequency of anaemia, iron deficiency, hemoglobin S and beta thalassemia in the south of Turkey. Eur J Epidemiol. 1995;11:181-4.
  • 23. Canatan D. Türkiye'de Hemoglobinopatilerin Epidemiyolojisi. Hematolog 2014;4-1:11-21.
  • 24. Musallam KM, Taher AT, Rachmilewitz EA. Beta-thalassemia intermedia: a clinical perspective. Cold Spring Harb Perspect Med. 2012;2:a013482.
  • 25.Galanello R, Origa R. Beta-thalassemia: Orphanet J Rare Dis. Journal of Continuing Education Topics & Issues 2010;21:5-11.
  • 26. Musallam KM, Cappellini MD, Taher AT. Iron overload in beta-thalassemia intermedia: an emerging concern. Curr Opin Hematol. 2013;20:187-92.
  • 27.Weatherall DJ. The definition and epidemiology of non-transfusion-dependent thalassemia. Blood Rev. 2012;26 (Suppl) 1:S3-6.
  • 28.Taher AT, Musallam KM, Karimi M, Cappellini MD. Contemporary approaches to treatment of beta-thalassemia intermedia. Blood Rev. 2012;26 (Suppl 1):S24-7.
  • 29.Rivella S. The role of ineffective erythropoiesis in non-transfusion-dependent thalassemia. Blood Rev. 2012;26 (Suppl 1):S12-5.
  • 30.Akar N. Thalassemia Sendromları. Ankara Üniversitesi Tıp Fakültesi Klinik Kitaplar Serisi,Çocuk Sağlığı ve Hastalıkları 1997;325-43.
  • 31. Lanskowsky P. Hemoglobinopathies. Manual of Pediatric Hematology and Oncology 2011;200-247.
  • 32.Izadyar S, Fazeli M, Izadyar M, Salamati P, Gholamrezanezhad A. Bone mineral density in adult patients with major thalassaemia: our experience and a brief review of the literature. Endokrynol Pol. 2012;63:264-9.
  • 33. Chatterjee R, Shah FT, Davis BA, Byers M, Sooranna D, Bajoria R et al. Prospective study of histomorphometry, biochemical bone markers and bone densitometric response to pamidronate in beta-thalassaemia presenting with osteopenia-osteoporosis syndrome. Br J Haematol. 2012;159:462-71.
  • 34. Gibbons R, Higgs DR, Olivieri NF, Wood WG. The β thalassaemias. The Thalassaemia Syndromes 2001;287-356.
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  • 36.Angelucci E, Brittenham GM, McLaren CE, Ripalti M, Baronciani D, Giardini C et al. Hepatic iron concentration and total body iron stores in thalassemia major. N Engl J Med. 2000;343:327-31.
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Toplam 97 adet kaynakça vardır.

Ayrıntılar

Konular Sağlık Kurumları Yönetimi
Bölüm Derleme
Yazarlar

Tuğçe Kazgan Bu kişi benim

Betül Yağcı Küpeli

Yayımlanma Tarihi 30 Eylül 2017
Kabul Tarihi 10 Nisan 2017
Yayımlandığı Sayı Yıl 2017 Cilt: 26 Sayı: 3

Kaynak Göster

AMA Kazgan T, Yağcı Küpeli B. Talasemilerde Solunum Sistemi Bozuklukları. aktd. Eylül 2017;26(3):352-377. doi:10.17827/aktd.303592