Oxidant and antioxidant status in beta thalassemia major patients
Amaç: Oksidan ve antioksidan dengedeki bozukluklar talasemi ve orak hücreli anemi gibi hemoglobinopatilerde saptanmıştır. Çalışmada düzenli olarak transfüzyon yapılan ve şelasyon tedavisi alan 11 beta talasemi major hastasında oksidan ve antioksidan özellikler çalışılmıştır. Gereç ve yöntem: Hastaların laboratuar değerleri cinsiyet ve yaşlan çalışma grubuna uygun 10 sağlıklı çocuk ile karşılaştırmıştır. Bulgular: Koruyucu bir antioksidan olan eritrosit superoksid dismutazı (ESOD) ve lipit peroksidasyonunun yıkım ürünü olan plazma malonildialdehid (MDA) düzeyleri çalışma grubunda sağlıklı çocuklara göre yüksek bulunmuştur. Serum vitamin E düzeyleri ise sağlıklı çocuklara gore düşük bulunmuştur. Sonuç: Talasemilerin patogenezinde demir yüklenmesi sonucu oluşan artmış oksidatif yıkım önemlidir. Ayrıca vitamin E gibi yağda eriyen vitaminlerin yetersizliği sonucu olarak antioksidan durumun bozulması oksidatif yıkımda artışa neden olur.
Beta talasemi major hastalarında oksidan ve antioksidan düzeyleri
Purpose: It is well documented that disturbances of oxidantantioxidant balance occur in hemoglobinopathies especially in thalassemia and sickle cell diseases. Materials and Methods: Oxidant and antioxidant status were studied in 11 regularly transfused thalassemia major patients who were under chelation therapy and their status were compared with 10 sex and age-matched healthy subjects. Results: Erythrocyte superoxide dismutase (ESOD), which is a preventive antioxidant value, and plasma malonyldialdehyde (MDA) levels, which is the breakdown product of lipid peroxidation were found to be higher in thalassemia major patients. Serum vitamin E levels were lower in patients with thalassemia major than healthy children. Conclusion: Oxidative damage especially due to iron overload and depletion of antioxidant status play an important role in pathogenesis of thalassemias. Increased oxidative damage in thalassemias may be due to the depletion of lipid soluble antioxidants such as vitamin E
___
- 1. Chiu D, Kuypers F, Lubin B: Lipid peroxidation in human red cells. Semin Hematol 1989; 26:257-276. 2. Clemens MR: Antioxidant therapy in hematological disorders. In Antioxidants in TherapyandPreventiveMedicineI.Emeritetal(ed), Plenum Press, New York 1990:423-433. 3. Kattamis C, Kattamis AC. Oxidative stress disturbances in erythrocytes of β-thalassemia. Pediat Hematol Oncol 2001;18:85-88. 4. McDonagh KT, Nienhuis AW: Thethalassemias.InNathanDG,Oski FA, ed. Hematology of Infancy and Childhood, 4th ed. WB Saunders, Philadelphia 1993: 783-879. 5. Scott MD, Van den Berg JJM, Repka T, et al. Effectofexcessβ-hemoglobin chains on cellular and membrane oxidation in model β-thalassemic erythrocytes. J Clin Invest 1993; 91:1706-1712. 6. Livrea MA, Tesoriere L, Pintaudi AM, et al. Oxidative stress and antioxidant status in beta-thalassemia major: iron overload and depletion of lipid soluble antioxidants. Blood 1996; 88:3608-3614.
- 7. Rachmilewitz EA, Kornberg A, Acker M. Vitamin E deficiencydue to increased consumption in β-thalassemia and in Gaucher’s disease. Ann NY Acad Sci 1982;393:336-347. 8. Miniero R, Canducci E, Ghigo D, et al. Vitamin E in beta-thalassemia. Acta Vitaminol Enzymol 1982; 4:21-25. 9. Kurtel H, Granger DN, Tso P, et al: Vulnerability of intestinal interstitial fluidtooxidantstress.AmJPhysiol1992;263:G573-578. 10. Winterbourn CC, Hawkins RE, Brian M, et al. Theestimationofred cell superoxide dismutase activity. J Lab Clin Med 1975;85: 337-341. 11. Rindi G. A rapid colorimetric method for determination of tocopherol and tocopheryl acetate in plasma. Int Rev Vit Res 1958; 28:225. 12. Meral A, Tuncel P, Surmen-Gur E, et al. Lipid peroxidation and and antioxidant status in beta-thalassemia. Pediatr Hematol Oncol 2000; 17:687-693. 13. Tesoriero L, D’Arpa D, Butera D,et al. Oral supplements of vitamin E improve measures of oxidative stress in plasma and reduce oxidative damage to LDL and erythrocytes in beta-thalassemia intermedia patients. Free Radical Res 2001;34(5): 529-540. 14. Cighetti G, Duca L, Bortone L,et al. Oxidative status and malonyldialdehyde in beta-thalassemia patients. Eur J Clin Invest 2002;32 Suppl 1:55-60. 15. Cappelini MD, Tavazzi D, Duca L, et al. G. Non-transferrin-bound iron, iron related oxidative stress and lipid preoxidation in beta-thalassemia intermedia. Transfusion Science 2000;23:245-246. 16. Yenchitsomanus P, Wasi P: Increased erythrocyte superoxide dismutase activities in ß-thalassaemia/haemoglobin H diseases. J Clin Pathol 1983;36:329-333. 17. Chakraborty D, Bhattacharyya M. Antioxidant defence status of red blood cells of patients with β-thalassemia and Eβ-thalassemia. Clinica Chimica Acta 2001;305:123-129. 18. Laksmitawati DR, Handayani S, Udyaningsih-Freisleben SK, et al. Iron status and oxidative stress in beta-thalassemia patients in Jakarta. Biofactors 2003;19:53-62. 19. Tekin M, Akar N, Egin Y, et al. Serum superoxide dismutase levels of beta thalassemia patients and effectsofhighdosageofintravenous desferrioxamine treatment on superoxide dismutase levels. Pediatr Hematol Oncol 1998;15:371-374.