Physiologically Healthy Adult Male Participants

Graded arginine excess intake will start at 50mg/kg/d on study day 1 followed by 100 mg/kg/d on study day 2 till the level reaches 300mg/kg/d on study day 6.
Consisting of oral consumption of eight hourly experimental meals per study day - Includes 4 tracer free experimental meals containing a mixture of free amino acids, calories from a flavored liquid and protein free cookies and 4- labeled amino acid experimental meals.

Arginine

BC Children;s Hospital Research Institute, University of British Columbia

Lysine Oxidation in Response to Arginine Supplementation

Determination of Lysine Oxidation in Response to Arginine Supplementation in Adult Men Using in Vivo Stable Isotope Techniques

Urine, plasma and breath samples will be collected during the study to measure the rate of oxidation of tracer with graded intake of arginine

Rajavel Elango, PhD

Assistant Professor

University of British Columbia

Patients receiving a lysine restricted diet adjunct to pyridoxine therapy will be considered as participants in the 'exposure'/test group

Patients on pyridoxine mono-therapy will be participants in the 'control' group

Pyridoxine Dependant Epilepsy

Daily lysine intake will be managed to maintain a plasma lysine level of 50-80 µmol/L (normal range: 52-196 µmol/L). Diet prescriptions will be based on international guidelines for glutaricaciduria type I, another inborn error of lysine catabolism. In order to meet the recommended daily protein intake (DRI) [23,24], the diet may include commercially available lysine-free amino acid formulas approved for use in conditions affecting lysine metabolism, as well as commercially available low-protein products based on the participant's taste.

Lysine Restricted Diet

All participants will be on 15-30 mg/kg/day of pyridoxine therapy up to a maximum of 500mg/day divided in 2-3 doses enterally

Pyridoxine

Children's Hospital Colorado

Seattle Children's Hospital

BC Children's Hospital

Hannover Medical School

Maxima Medical Center

Kinderspital Zürich

University College London

Neurodevelopmental Outcome of Early Dietary Lysine Restriction in Pyridoxine Dependent Epilepsy Patients

Neurodevelopmental Outcome of Early Dietary Lysine Restriction in Pyridoxine

defined by total developmental index measured using the Bayley Scales for Infant and Toddler Development, 3rd Edition (Bayley-III)

March of Dimes

British Columbia Childrens Hospital Foundation

Oral pyridoxine, 30- 50 mg/kg/day in one daily dose (powder form)for a period of four weeks followed by cross-over to Phenytoin arm for another four weeks

Phenytoin Oral, 5 mg/kg/day in two equally divided doses(powder form)for a period of four weeks and then cross-over to Pyridoxine arm for another four weeks

Sodium valproate oral, 10 - 15 mg/kg/day once daily powder form)for a period of four weeks and then cross-over to placebo arm for another four weeks

Placebo will consist of an inert substance (e.g., gelatin) with an appearance similar to medication in similar dosage as the study arms for a period of 4 weeks and subsequent cross-over to Sodium Valproate arm

Nodding Syndrome

Sodium Valproate

Phenytoin

Placebo

Kitgum Nodding Syndrome Treatment Center, Kitgum General Hospital

Treatment of Nodding Syndrome - A Randomized Blinded Placebo-Controlled Crossover Trial of Oral Pyridoxine and Conventional Anti-Epileptic Therapy, in Northern Uganda - 2012

Change (Reduction) in frequency of observed head nodding or other seizure activity episodes among children with NS

pyridoxine/seizures

Lysine Oxidation in Response to Arginine Supplementation

Neurodevelopmental Outcome of Early Dietary Lysine Restriction in Pyridoxine Dependent Epilepsy Patients

Treatment of Nodding Syndrome - A Randomized Blinded Placebo-Controlled Crossover Trial of Oral Pyridoxine and Conventional Anti-Epileptic Therapy, in Northern Uganda - 2012

Phase II Randomized Study of Early Surgery Vs Multiple Sequential Antiepileptic Drug Therapy for Infantile Spasms Refractory to Standard Treatment

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