title: Oxidative Stress -a Phenotypic Hallmark of Fanconi Anemia and Down Syndrome: The Effect of Antioxidants.
abstract: BACKGROUND
  Oxidative stress plays a major role in the pathogenesis of leukemia-prone diseases such as Fanconi anemia (FA) and Down syndrome (DS).
  
  
  OBJECTIVE
  To explore the oxidative stress state in children with DS and FA by estimating the levels of antioxidants (e.g., malondialdehyde [MDA], total antioxidant capacity, and superoxide dismutase [SOD] activity) and DNA damage, and to evaluate of the effect of antioxidant treatment on these patients.
  
  
  METHODS
  The study included 32 children clinically diagnosed with (15 patients) and FA (17 patients) in addition to 17 controls matched for age and sex. MDA, total antioxidant capacity, SOD activity, and DNA damage were measured. Antioxidants including Vitamin A, E, and C were given to the patients according to the recommended daily allowance for 6 months. Clinical follow-up and re-evaluation were conducted for all patients. Laboratory tests including complete blood count, karyotyping, DNA damage, and oxidative stress were re-evaluated. Statistical analysis was performed using statistical computer program Statistical Package for the Social Sciences version 14.0.
  
  
  RESULTS
  Children with FA and DS had elevated levels of oxidative stress and more DNA damage than controls. Oxidative stress parameters and DNA damage improved in FA and DS patients after antioxidant administration.
  
  
  CONCLUSIONS
  Early administration of antioxidants to FA and DS patients is recommended for slowing of the disease course with symptoms amelioration and improvement of general health.
authors.affiliation: Department of Clinical Genetics, National Research Centre, Cairo, Egypt.
authors.forename: H T
authors.initials: HT
authors.lastname: El-Bassyouni
authors.affiliation: Department of Clinical Genetics, National Research Centre, Cairo, Egypt.
authors.forename: H H
authors.initials: HH
authors.lastname: Afifi
authors.affiliation: Department of Human Cytogenetics, National Research Centre, Cairo, Egypt.
authors.forename: M M
authors.initials: MM
authors.lastname: Eid
authors.affiliation: Institute of Postgraduate Childhood Studies, Ain Shams University, Cairo, Egypt.
authors.forename: R M
authors.initials: RM
authors.lastname: Kamal
authors.affiliation: Institute of Postgraduate Childhood Studies, Ain Shams University, Cairo, Egypt.
authors.forename: H H
authors.initials: HH
authors.lastname: El-Gebali
authors.affiliation: Department of Medical Biochemistry, National Research Centre, Cairo, Egypt.
authors.forename: Gsm
authors.initials: G
authors.lastname: El-Saeed
authors.affiliation: Department of Clinical Genetics, National Research Centre, Cairo, Egypt.
authors.forename: M M
authors.initials: MM
authors.lastname: Thomas
authors.affiliation: Department of Clinical Pathology, National Research Centre, Cairo, Egypt.
authors.forename: S A
authors.initials: SA
authors.lastname: Abdel-Maksoud
keywords: Down syndrome; Oxidative DNA damage; Oxidative stress