Vitamin E Level and Biomarkers of Oxidative Stress in Patients with Hereditary Spherocytosis and Their Relation to Growth and Quality of Life
Authors: Mohsen S. El Alfy, Mohamed T. Hamza, Fatma Abdel Hamid and Nada H. Mohamed
Background: Hereditary spherocytosis (HS), the most common of inherited hemolytic anemia due to membrane dysfunction and has been documented to occur in all racial groups. The clinical spectrum ranges from asymptomatic patients to severe forms requiring transfusions in early childhood. The characteristic features in hereditary spherocytosis are anaemia, jaundice, splenomegaly and family history. The diagnosis can be based on the physical examination, complete red blood cell count, reticulocytes count, medical history and specific tests, preferentially the EMA test (eosin-5-maleimide binding) test and AGLT (Acidified Glycerol Lysis Time).
Aim of the Work: In this study we search for a correlation between vitamin E level and markers of oxidative stress in patients with hereditary spherocytosis, and their correlation to the growth and quality of life of the patients which as far as we searched has not been done in Egypt. Also, we find about the percentage of parents with positive family history and measure the morbidity and mortality of complications of HS.
Patients and Methods: This is a cross sectional study, analyzed as a case-control study conducted on 80 children from Pediatrics Hospital, Ain Shams University Hospitals during the period from September 2017 to February 2018. An informed consent was taken from the parents or caregivers of each participant before enrollment. This study was approved by the ethical committee of Ain Shams University. They were divided into 2 groups: Group 1 (Patients): included 40 patients with hereditary spherocytosis following up in the hematology clinic, Group 2 (controls): included 40 normal children attending the outpatient clinic for a check up. All included neonates were subjected to the following: Detailed medical history with special emphasis on: neonatal history, family history, age at diagnosis, PRBCs transfusion, surgery, crises, mental and motor development, symptoms of fatigue, pallor or jaundice. Measurement of markers of oxidative stress like GSH and Vitamin E.
Results: The results of the present study are shown in Tables 7- 18 and Figures 5-17. This study was carried out on 81 children from Pediatrics Hospital, Ain Shams University Hospitals divided into 2 groups 41 patients with hereditary spherocytosis with male to female ratio 1.05:1 and 40 normal children with male to female ratio 1.1:1. The median age in patient group is 7 ranging from 1-18 and 7.5 in control ranging from 1-18. Both patient and control groups were matched for age and sex.
Conclusion: The role of oxidative stress in the pathophysiology of chronic haemolytic anaemias is controversial. The patients with HS have very low levels of several antioxidants as compared with normal subjects and higher levels of ROS. The decrease in levels of vitamin E may be an indication for the need for supplementation.