Thalassemia majörlü çocuklarda selenyum, glutatyon peroksidaz ve glutatyon redüktaz

Abstract

7.ÖZET Bu çalışmada C.ü. Tıp Fak. Pediadrik Hematoloji Bilim Dalı'nca thalassemia majör tanısı ile izlenen 6 kız, 7 erkek 6-12 yas arası 13 hasta ile aynı yas grubunda hemoglobin örneği HbAA olan sağlıklı 7 kız 7 erkek 14 kontrol araştırma kapsamına alındı. Hastalar ve kontrollerde rutin hematolojik tetkikler, hemoglobin elektoroforezleri yapıldı. HbA2 ve HbF yüzdelerine bakıldı. Tüm olgularda plazma ve eritrosit selenyumu, eritrosit GSH-Px ve GSH-Rx incelendi. Plazma ve eritrosit selenyumu fluorometrik yöntemle; GSH-Rx Beutler'in yöntemi ile kalitatif olarak, GSH-Px kantitatif olarak çalışıldı. Hastalar ve kontroller arası parametrelerin değerlendirilmesin¬ de Mann-Whitney U testi ve korelasyon gibi istatistiksel metodlar kullanıldı. Thalassemia majörlü hasta grubunda ortalama eritrosit selenyumu 0.056±0.015 wg/ml, plazma selenyumu 0.039±0.010 ug/ml bulundu. Sağlıklı kontrollerde ise ortalama plazma selenyumu 0.054±0.101 ug/ml, eritrosit selenyumu 0.151±0.072 jug/ml olarak bulundu. Hastalar ve kontroller arası eritrosit ve plazma selenyumu ortalama değerleri arası istatistiksel yönden önemli farklılıklar verdi (P<0.05). Thalassemia majörlü hastalarda eritrosit glutatyon peroksidaz 3.198±1.332 ü/g Hb, kontrollerde 9.017±4.730 U/g Hb olarak bulundu. Hastalar ve kontrollerin GSH-Px ortalamaları arasındaki fark istatistiksel yönden önemli bulundu (P<0.05). GSH-Rx aktivitesi üç hastada normal bulunurken., on hastada normalin altında bulundu. Kontrollerin hepsinde ise GSH~Rx aktivitesi normaldi. Thalassemia majörlü hastalarda plazma ve eritrosit selenyum arası pozitif korelasyon vardı (r=0.213) ve bu ilişki istatistiksel yönden önemliliği çok belirgindi (0.05<P<0.1). 41Hastalarda eritrosit selenyum ile eritrosit GSH-Px değerleri arasında pozitif korelasyon bulundu (r=0.844). bu korelasyon önemliydi. (P<0.01). Hastalarda plazma selenyumu ile eritrosit GSH-Px arası pozitif ve güçlü bir korelasyon vardı (rHD.55). bu korelasyon önemliydi (0.01<p<0.02î. 42

8. SUMMARY This study has been done at Pediatric Hematology Clinic of the Medical Faculty of Çukurova University where following groups of chidren have been examined: Group I: 13 patiens with thalassemia Major: ages between 6-12 years. 6 of the patients were female and the rest of them were male. Group II: 14 healthy children (HbAA) in the same age group of which half of them were female and the rest were male. For all children in the above mentioned groups, routine hematologic tests and hemoglobin electrophoresis were done HbA2 and HbF were measured. From each patient plasma and erythrocyte selenium levels, erythrocyte glutathione peroxidase and glutathione reductase were measured plasma and serum selenium levels were measured with fluorometric technique and glutathione reductase was investigated qualitatively with Beutler technique while glutathione peroxidase was studied quantitatively. Mann-VJhithey and correlation tests were used for the statistical evaluation of the groups. For the patients with thalassemia major. the erythrocyte selenium levels were measured as 0.056±0.015 jug/ml and plasma selenium levels were 0.039±0.010 /jg/ml. For the healthy group, the plasma selenium levels were measured as 0.054±0.101 tig/ml and erythrocyte selenium levels were 0.151±0.072 jug/ml. From statistical point of view, important differences were being observed between erythrocyte and plasma selenium average values corresponding to the control and patient groups (FK0.05). The erythrocyte GSH-Px levels were measured as 3.198±1.332 U/gHb and 9.017±4.730 ü/g Hb for the patients with thalassemia major and the healthy group, respectively. Statistically, important differences were being detected between GSH-Px average values corresponding for the control and patient groups (FK0.05). 43<3SH-Rx activity was less then normal values in ten of the patients, while only three of them were normal. On the other hand. GSH-Rx activity was normal in the control group. Positive correlation was present between plasma and erythrocyte selenium levels for the patients with thalassemia major (r=0.213. 0.05<0.1). Also, positive correlation was present between erythrocyte selenium and erythrocyte GSH-Px for the patients (r=0.844. P<0.01). Between plasma selenium and erythrocyte GSH-Px values of the patients, a strongly positive correlation was also seen (r=0.55. 0.01<P<0.02). 44