Propionic acidaemia. Other disorders of branched-chain amino-acid metabolism_Propionic acidaemia
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
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
Exclusion criteria: Parental dissent for participation in the study and daily intake of fruit juice
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Amino acid profile. Timepoint: Before intervention, after intervention. Method of measurement: HPLC method.;Ammonia. Timepoint: Before intervention, after intervention. Method of measurement: Spectrophotometry. | — |
Secondary
| Measure | Time frame |
|---|---|
| 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. | — |
Countries
Iran (Islamic Republic of)
Contacts
Shiraz University of Medical Sciences