Role of Vitamin E in Gaucher Disease Patients

Ain Shams University40 enrolled

Overview

gaucher disease (GD) can be classified into three clinical types .type 1,the most common type ,is the chronic non neuronpathic form of the disease,which shows gighly variable signs and symptoms and variable course,with visceral,skeletal and hematologic involvement among others.the neurologocal involvement can be observed in types 2 and 3

enzymatic defeciency in gaucher disease patients may induce a cascade of events that result in side effects, such as the production of reactive oxygen species(ROS) and reactive nitrogen species(RNS) that can then generate the oxidative stress whereas in body of healthy individuals,the production and degradation of ROS and RNS are generally balanced. neurochemical abnormalities in patients with gaucher disease might be related to oxidative stress and inflammation in the brain. oxidative stress induces peroxiredoxin-2 as cytoprotective response against RNS .it is able to detoxify a vast range of organic peroxides. vitamin E well accepted nature most effective lipid soluble chain breaking antioxidant .

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Gaucher Disease

Interventions

Vitamin EDRUG

Vitamin E will be taken for 6 months for gaucher disease patients who have oxidative stress and vitamin E defecient

Eligibility

Sex: ALLMin age: 2 YearsMax age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: children and adolescents aged fron 2 to 18 years old with gaucher disease both type 1 and type 3 diagnosed clinically and confirmed by laboratory analysis patients who are on stable enzyme replacement therapy for at least 6 months - Exclusion Criteria: patients with infection or another inflammatory condition patients on multivitamin therapy known chronic liver disease (viral hepatitis,HIV) patients with renal impairement defined as creatinine level greater 1.2 for female and greater than 1.4 for male. \-

Locations (1)

Ain Shams University

Cairo, Abbasia, Egypt

Outcomes

Primary Outcomes

effect of vitamin E on oxidative stress and antioxidant markers in gaucher disease patients

Measuring the oxidative stress markers in gaucher disease MDA and anti oxidant markers which are peroxiredoxin 2 ,Glutathione peroxidase,superoxide dismutase and reduced glutathione

Time frame: 6 months

Data from ClinicalTrials.gov

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