mild phenylketonuria. Other hyperphenylalaninaemias
Conditions
Interventions
Kuvan,100 mg oral tablet, daily, 20 mg/kg.
Phenylalanine, orally powder, daily, 50 mg (additive phenylalanine of dietary regime), If level of Phe is normal, another 50 mg of Phe is added for next wee
Treatment - Drugs
Kuvan,100 mg oral tablet, daily, 20 mg/kg.
Phenylalanine, orally powder, daily, 50 mg (additive phenylalanine of dietary regime), If level of Phe is normal, another 50 mg of Phe is added for next we
Sponsors
Mashhad University of Medical Sciences, Vice Chancellor for Research
Eligibility
Sex/Gender
All
Age
1 Years to 10 Years
Inclusion criteria
Inclusion criteria: Inclusion criteria: patients with controlled PKU; age range of 1-10 years; lack of underlying disorders (e.g. malnutrition, hepatic and renal diseases). Exclusion criteria: BH4 deficiency; Phe levels less than 600 micromoles/liter or more than 1200 micromoles/liter; presence of fever or acute illness; intolerance to BH4 therapy.
Exclusion criteria
Exclusion criteria:
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Serum levels of phenylalanine. Timepoint: In first phases, after three days of free diet, then 24 and 48 houres after prescription of BH4. In second phase, measured weekly. Method of measurement: HPLC. | — |
Countries
Iran (Islamic Republic of)
Contacts
Public ContactDr Vajihe Chalak
Mashhad University of Medical Sciences
Outcome results
None listed