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

NCT01795170 · Status: WITHDRAWN · Type: OBSERVATIONAL

Last updated 2014-09-30

No results posted yet for this study

Summary

Restricting dietary lysine intake in infants from age 3 months or less with confirmed diagnosis of pyridoxine-dependent epilepsy due to Antiquitin (ATQ) deficiency will: reduce the accumulation of neurotoxic substratesα-aminoadipicsemialdehydeandits cyclic equivalent 1-piperideine-6-carboxylate;and will improve overall neurodevelopmental outcome at 3 years of age by acting as an effective intervention into the complex pathophysiology of the condition.

Conditions

  • Pyridoxine Dependant Epilepsy

Interventions

DIETARY_SUPPLEMENT

Lysine Restricted Diet

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.

DRUG

Pyridoxine

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

Sponsors & Collaborators

  • March of Dimes

    collaborator OTHER

  • British Columbia Childrens Hospital Foundation

    collaborator OTHER

  • University of British Columbia

    lead OTHER

Principal Investigators

  • Clara van Karnebeek · University of British Columbia

  • Sylvia Stockler · University of British Columbia

Eligibility

Min Age
3 Months
Max Age
3 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2013-04-30
Primary Completion
2016-11-30
Completion
2016-11-30

Countries

  • United States
  • Canada
  • Germany
  • Netherlands
  • Switzerland
  • United Kingdom

Study Locations

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Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT01795170 on ClinicalTrials.gov