• Oğuzhan Özcan, Hüseyin Erdal, Gül İlhan, Damla Demir, Ahmet Burak Gürpınar, Salim Neşelioğlu, and Özcan Erel.
• Mustafa Kemal University Faculty of Medicine, Department of Biochemistry, Hatay, Turkey
• Turk J Haematol. 2018 Nov 13; 35 (4): 265-270.

ObjectiveSickle cell disease (SCD), 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 MethodsFifty-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 sickle/β-thalassemia (HbS/β+-thalassemia). Fasting blood samples were collected. After centrifugation at 1500×g for 10 min, plasma samples were portioned and stored at -80 °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.ResultsWe found significantly lower levels of native thiol (-SH) (284.0±86.3 µmol/L), disulfide levels (14.6±7 µmol/L), and total thiols (-SH + -S-S-) (313.0±89.3 µmol/L) in SCD patients compared to healthy controls (respectively 417.0±54.2, 22.7±11.3, and 462.0±58.7 µmol/L). 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).ConclusionDecreased 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.

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