Biochemical evaluation of vitamin A and zinc and its relation with oxidative stress in patients with ataxia-telangiectasia

• Grant number: 08/08705-5
• Support Opportunities: Regular Research Grants
• Start date: December 01, 2008
• End date: November 30, 2010
• Field of knowledge: Health Sciences - Nutrition - Nutritional Analysis of Population
• Principal Investigator: Roseli Oselka Saccardo Sarni
• Grantee: Roseli Oselka Saccardo Sarni
• Host Institution: Escola Paulista de Medicina (EPM). Universidade Federal de São Paulo (UNIFESP). Campus São Paulo. São Paulo , SP, Brazil

Abstract

Ataxia telangiectasia (A-T) is a rare disease inherited as an autosomal recessive disease with a prevalence as one in 70000 live births, caused by a mutation in the gene responsible for ATM protein encoding, located in chromosome 11q22-23.There is evidence of the exacerbation of oxidative stress in patients with A-T increases neurodegeneration and risk of cancer. The main mechanism is ATM protein alterations, however, there aren't any studies evaluating the micronutrient deficiencies (zinc and vitamin A) participation in lipid peroxidation (oxidative stress).Aim: To evaluate zinc and vitamin A levels in A-T patients and relate them to malondialdehyde levels (lipid peroxidation marker) and CD4/CD8 ratio.Methods: In a prospective and cross-sectional study we will evaluate A-T patients from both genders and all ages, with diagnosis based on Pan-American Group of Immunodeficiency (PAGID) and the European Society of Immunodeficiency (ESID) criteria. The enrollment will consider all A-T patients in the Pediatric Department of Federal University of São Paulo. We will also invite other A-T patients through Grupo Brasileiro de Imunodeficiência (BRAGID: www.bragid.org.br). At the moment there are 30 A-T patients in this group. We will consider biochemical and anthropometrics parameters as: weight, height, tricipital and subscapular skinfolds, arm circumference, serum and erythrocyte zinc, retinol, beta-carotene and malondialdehyde plasma levels.Statistical analysis: Qui-square, Mann Whitney and t-Student tests, ± <0.05. (AU)