Status:

UNKNOWN

Fasting Tolerance in MCADD-infants

Lead Sponsor:

University Medical Center Groningen

Conditions:

Mcad Deficiency

Eligibility:

All Genders

2-6 years

Phase:

NA

Brief Summary

MCAD deficiency (MCADD; #OMIM 201450) is the most common inherited disorder of mitochondrial fatty acid oxidation. Already before the introduction of population newborn bloodspot screening (NBS), larg...

Detailed Description

Rationale: MCAD deficiency (MCADD; #OMIM 201450) is the most common inborn error of mitochondrial fatty acid oxidation. Already before the introduction of population newborn bloodspot screening (NBS),...

Eligibility Criteria

Inclusion

  • A child must be at least younger than 6 months of life at inclusion. In case of prematurity, the child will be included and treated according to the adjusted age.
  • Established MCADD diagnosis. The diagnosis should be confirmed by a combination of (a) NBS outcome (b) MCAD enzyme activity measured with phenylpropionyl-CoA as a substrate, ideally in lymphocytes (considered to be the golden standard) and (c) ACADM gene mutation-analysis.

Exclusion

  • Any other chronic and/or genetic condition that is deemed an exclusion criterion based on the judgement of the treating metabolic paediatrician.

Key Trial Info

Start Date :

May 15 2019

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

January 1 2024

Estimated Enrollment :

20 Patients enrolled

Trial Details

Trial ID

NCT03761693

Start Date

May 15 2019

End Date

January 1 2024

Last Update

May 14 2019

Active Locations (1)

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University Medical Center Groningen

Groningen, Netherlands, 9700 RB