Diet Challenge in G6PD Deficient Egyptian Children: A One- Year Prospective Single Center Study With Genotype - Phenotype Correlation

Glucose-6-Phosphate Dehydrogenase Deficiency Clinical Trial

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT02498340
• Other study ID #: Diet challenge in G6PD
• Status: Not yet recruiting
• Phase: Phase 2/Phase 3
• First received: July 10, 2015
• Last updated: July 14, 2015
• Start date: December 2015

Study information

• Verified date: July 2015
• Source: Ain Shams University
• Contact: Amira Adly, ass. prof
• Phone: 0105245837
• Email: amiradiabetes[@]yahoo.com
• Is FDA regulated: No
• Health authority: Egypt: Institutional Review Board
• Study type: Interventional

Clinical Trial Summary

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is prevalent and add a burden on families in Egypt and Middle East due to lifelong diet restriction, non-fava beans diet is main food for most families in the region and parents and doctors consider it as a prohibited food whatever the genetic or clinical phenotypes. The effective management is avoiding a spectrum of food and drugs causing oxidative stress. No data is available about the hazards of consumption of non-fava beans diet.

Description:

Investigate the effect of challenge of non-fava beans diet on occurrence of hemolysis in both common and rare mutations causing G6PD deficiency in Egyptian children as well as making a genotype-phenotype correlation. Patients and Methods: The study will include all G6PD deficient children who were regularly followed up in Pediatric Hematology Center, Ain Shams University over last decade from 2004-2014 who stopped eating non fava-bean diet since their diagnosis as G6PD deficient and willing to participate in the diet challenge. They will be enrolled in a one year prospective study involving quantitative analyses for enzymatic activity, and molecular typing of G6PD enzyme using a polymerase chain reaction-amplification refractory mutation system (PCR-ARMS) technique. Patient's medical records will be reviewed as history of blood transfusion and G6PD level at diagnosis. Initial phase will be dietetic challenge with ingestion of non-fava beans taken in small amount (10-20 gm/day for 3 successive days ) for children with haemoglobin level ≥ 11 gm/dl with daily clinical and laboratory monitoring by complete blood count, and markers of hemolysis as well as measurement of malondialdehyde (MDA) level both basal and at study end. A drop of Hb of ≥0.5 gm/dl and / or appearance of hemoglobinuria is considered a significant hemolysis. Patients who will not meet the definition of significant hemolysis will be prospectively followed up for one year with follow up during their chronic exposure by complete blood count and hemolysis markers/ 3 months.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 100
• Est. primary completion date: July 2016
• Accepts healthy volunteers: No
• Gender: Both
• Age group: N/A and older

Eligibility

Inclusion Criteria:

• All children with proved enzymatic deficiency of G6PD.

• Patients who met the criteria of hemoglobin level at study time = 11 gm/dl

Exclusion Criteria:

• patients with another enzyme deficiency

Study Design

Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment

Related Conditions & MeSH terms

• Glucose-6-Phosphate Dehydrogenase Deficiency
• Glucosephosphate Dehydrogenase Deficiency

Intervention

Dietary Supplement:
• non- fava beans
Patients will be subjected to eat non- fava beans diet at doses of 10 -30 gm (small to moderate amount) of 3 different types of non- fava beans, each one will be given once daily for 3 days.

Locations

Country	Name	City	State
Egypt	Pediatric Hematology clinic, Ain Shams University	Cairo

Sponsors (1)

Lead Sponsor	Collaborator
Ain Shams University

Country where clinical trial is conducted

Egypt, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Diet challenge		one year	Yes