VITAMIN E LEVEL AND BIOMARKERS OF OXIDATIVE STRESS IN PATIENTS WITH HEREDITARY SPHEROCYTOSIS AND THEIR RELATION TO GROWTH AND QUALITY OF LIFE

Abstract

ereditary spherocytosis is a common inherited disorder that is characterised by a triad of anaemia, jaundice, and splenomegaly. Most patients have compensated hemolytic anemia and are asymptomatic, while others have severe hemolytic anemia requiring transfusion. Oxidative stress is not the primary etiology of most hemolytic anemias but may aggravate hemolysis. To protect against oxidative stress, cells maintain an effective antioxidant system consisting of water- or lipid-soluble antioxidants and enzymes that remove reactive oxygen species by metabolic conversion. There is an important role of vitamin E as a lipid-soluble antioxidant in cellular membranes and lipo-proteins. The aim of this study is to find out if vitamin E, as an antioxidant, is protective to erythrocytes in hereditary spherocytosis decreasing the oxidative stress associated with this condition. This was assessed by measuring level of vitamin E in blood in relation to biomarkers of oxidative stress and level of hemoglobin. Quality of life for those patients was additionally assessed. A cross-sectional study including 40 children with hereditary spherocytosis recruited from the hematology clinic- Pediatric Hospital, Ain Shams University during the period from September 2017 to February 2018. They were compared to 40 apparently healthy children attending the outpatien