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    Investigation of thiol/disulfide homeostasis in familial mediterranean fever patients
    (Derman Medical Publ, 2018) Cakirca, Gokhan; Celik, Muhammet Murat; Erdal, Huseyin; Neselioglu, Salim; Erel, Ozcan; Basarali, Mustafa Kemal; Cakirca, Tuba Damar
    Aim: To determine the thiol/disulfide homeostasis in patients with familial Mediterranean fever (FMF) and its correlation with the levels of inflammatory markers consisting of white blood cell count, erythrocyte sedimentation rate, C-reactive protein and fibrinogen. Material and Method: This study was performed in the internal Medicine department of Mustafa Kemal University Hospital in Turkey. A total of 27 FMF patients in the attack period (AP), 33 FMF patients in the attack-free period (AFP), and 36 healthy controls participated in this study. Thiol/disulfide profile parameters were detected using the novel method of Erel and Neselioglu. Results: Total and native thiol levels of the FMF-AP group were markedly lower than those of healthy controls, while the difference in disulfide level was not statistically significant. Thiol/disulfide levels in the FMF-AFP group were similar to the levels in both the FMF-AP group and healthy controls. Correlation analysis showed a negative correlation between fibrinogen levels and total and native thiol levels, while there was a positive correlation between white blood cell count and disulfide levels in the FMF-AP group. Discussion: The findings suggest that decreased concentrations of total and native thiol in patients with FMF-AP are likely to be an outcome of inflammation-induced oxidative stress.
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    Plasma Ischemia-Modified Albumin Levels and Dynamic Thiol/Disulfide Balance in Sickle Cell Disease: A Case-Control Study
    (Galenos Yayincilik, 2018) Ozcan, Oguzhan; Erdal, Huseyin; Ilhan, Gul; Demir, Damla; Gurpinar, Ahmet Burak; Neselioglu, Salim; Erel, Ozcan
    Objective: Sickle cell disease (SCR described as a group of inherited blood disorders, affects millions of people throughout the world and is particularly common in the southern part of Turkey. We aimed to determine the relationship between ischemia-modified albumin (IMA) and the dynamic thiol/disulfide balance in SCD. Materials and Methods: Fifty-four adult SCD patients and 30 healthy controls were included in the study. The 54 adult patients included 30 (56% males and 24 (44%/) females with a mean age of 28.3 +/- 8.4 years (minimum-maximum: 18-46 years). Of the 54 patients, 46 had homozygous sickle cell anemia (HbSS) and 8 had sickleni/beta-thalassemia (HbS/(beta(+)-thalassemia). Fasting blood samples were collected. After centrifugation at 1500xg for 10 min, plasma samples were portioned and stored at -80 degrees C. IMA levels were determined by albumin cobalt binding test, a colorimetric method. Total and native thiols and disulfide were analyzed with a novel spectrophotometric method. Results: We found significantly lower levels of native thiol (-SH) (284.0 +/- 86.3 mu mol/L), disulfide levels (14.6 +/- 7 pmol/L), and total thiols (-SH + -S-S-) (313.0 +/- 89.3 ilmol/L) in SCD patients compared to healthy controls (respectively 417.0+54.2, 22.7+11.3, and 462.0 +/- 58.7 mu twill.). Plasma albumin levels (34.9 +/- 7.9 g/L) were lower and IMA levels (13.6 +/- 3.1 g/L) were higher in SCD patients compared to controls (respectively 43.5 +/- 3.1 and 8.4 +/- 1.6 g/L). Plasma albumin levels were strongly correlated with both plasma native (r=0.853; p=0.0001) and total thiols (r=0.866; p=0.0001). Conclusion: Decreased plasma native and total thiol levels and increased IMA levels are related to increased oxidative stress and provide an indirect and quick reflection of the oxidative damage in SCD patients.