The Effect of Consumption of High calorie-Fruit Juice Fortified with Vitamin C in the Management of Propionic Acidemia

Not Applicable

Recruiting

• Conditions: Propionic acidaemia.; Other disorders of branched-chain amino-acid metabolism_Propionic acidaemia

• Registration Number: IRCT20121019011168N3
• Lead Sponsor: Shiraz University of Medical Sciences

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Last Update Posted: 11 September 2018

Recruitment & Eligibility

• Status: Recruiting
• Sex: All
• Target Recruitment: 20

Inclusion Criteria

Children 2-6 years of age
Suffering from propionic acidemia (PA) according to metabolic panel test
Confirmation of the disease by genetic test (if possible)
Parental consent for participation in the study and daily intake of fruit juice
Monthly cycles of one-week metronidazole therapy (10-20 mg/kg).

Exclusion Criteria

Parental dissent for participation in the study and daily intake of fruit juice

Study & Design

• Study Type: interventional
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Amino acid profile. Timepoint: Before intervention, after intervention. Method of measurement: HPLC method.;Ammonia. Timepoint: Before intervention, after intervention. Method of measurement: Spectrophotometry.

Secondary Outcome Measures

Name	Time	Method
Serum vitamin C. Timepoint: Before intervention, after intervention. Method of measurement: HPLC method.;Serum glutathione peroxidase. Timepoint: Before intervention, after intervention. Method of measurement: Enzymatic, colorimetric method.;Serum superoxide dismutase. Timepoint: Before intervention, after intervention. Method of measurement: Enzymatic, colorimetric method.;Total antioxidant capacity. Timepoint: Before intervention, after intervention. Method of measurement: Enzymatic, colorimetric method.;Serum total protein. Timepoint: Before intervention, after intervention. Method of measurement: Autoanalyzer, colorimetric method.;Serum Albumin. Timepoint: Before intervention, after intervention. Method of measurement: Autoanalyzer, colorimetric method.;Serum Prealbumin. Timepoint: Before intervention, after intervention. Method of measurement: Immunoturbidimetric method.