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IRCT2016100230082N1 IRCT 2017-06-01 Mashhad University of Medical Sciences, Vice Chancellor for Research Effect of kuvan on relaxation of diet in phenylketonuria Effectiveness of Tetrahydrobiopterin for improvement of dietary regimen of children with Phenylketonuria 2015-01-01 anticipated 20 Complete https://irct.ir/trial/24016 interventional Randomization: N/A, Blinding: Not blinded, Placebo: Not used, Assignment: Single, Purpose: Supportive. N/A mild phenylketonuria. 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 week and this process goes on until Phe dose is twice as the initial dose and if tolerated, they return to normal diet.
public Dr Vajihe Chalak
Department Of Pediatric Endocrinology, Imam Reza Hospital, Imam Reza Square
Mashhad Iran (Islamic Republic of) +98 51 3859 3045 drvch1380@yahoo.com Mashhad University of Medical Sciences scientific Dr Peyman Eshraghi
Department Of Pediatric Endocrinology, Imam Reza Hospital, Imam Reza Square
Mashhad Iran (Islamic Republic of) +98 51 3859 3045 eshraghip2@mums.ac.ir Mashhad University of Medical Sciences Iran (Islamic Republic of) 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. 1 year 10 years Both E70.1 Other hyperphenylalaninaemias 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 week and this process goes on until Phe dose is twice as the initial dose and if tolerated, they return to normal diet 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. Mashhad University of Medical Sciences, Vice Chancellor for Research Approved 2013-11-20 Ethics Committee of Mashhad University of Medical Sciences Central Building of Mashhad University of Medical Sciences (Ghorshi),Daneshgah 16, Daneshgah Street Mashhad Iran (Islamic Republic of)