Study of the Levels of Advanced Glycation End Products in Sickle Cell Patients

Abstract

Abstract Background Sickle cell disease (SCD) is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and vaso-occlusion leading to reduced quality of life and life expectancy. SCD is caused by a point mutation in a single gene, which results in a mutant β-globin protein (HbS), in which the sixth amino acid is changed from glutamic acid to valine. In the homozygous and some compound heterozygous states, deoxygenated HbS molecules form polymers, which damage the red cell membrane and increase its rigidity. Aim of the Work to study the AGE levels in sickle cell patients and determine its relationship to the presence of SCD-related organ complications, in addition to exploring the association of AGE levels to other clinical and laboratory data. Patients and Methods This study was conducted on 40 Sickle cell patients, including sickle cell anemia (HbSS) and compound heterozygous states. In addition to 20 race, sex, and age matched healthy control subjects will also be included. They were recruited from patients attending Pediatric Hematology Clinic, Pediatric Hospital, Ain Shams University from September 2017 to September 2018. A verbal informed consent was taken from all patients. Results A statistically significant difference was found between SC patients and control group as regards their CBC data; including TLC, which showed a significant statistical difference being higher in patients group (P < 0.001). Conclusion Plasma levels of pentosidine and CML are increased and associated with haemolysis and haemolysis-related organ complications in sickle cell patients, suggesting that AGEs might be implicated in vascular damage and chronic organ complications in SCD. Measurement of these AGEs might be useful in assessing disease severity and predicting organ complications in SCD.