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The role of microbiome in urea cycle defect disorders


- candidate number26863
- NTR NumberNTR6447
- ISRCTNISRCTN no longer applicable
- Date ISRCTN created
- date ISRCTN requested
- Date Registered NTR10-mrt-2017
- Secondary IDsABR nummer NL61031
- Public TitleThe role of microbiome in urea cycle defect disorders
- Scientific TitleThe role of microbiome in urea cycle defect disorders
- ACRONYMUCD
- hypothesisIn urea cycle defect patients the protein-restricted diet, in combination with essential amino acid supplementation, results in a different gut microbial composition compared to healthy individuals on a normal diet. Selection of ammonia producing bacteria can negatively influence metabolic regulation in UCD patients
- Healt Condition(s) or Problem(s) studiedGut microbiota, Microbiome, Phenylketonuria (PKU), Urea cycle defect
- Inclusion criteriaPatients:
- age 18 years and over.
- confirmed diagnosis of a urea cycle defect or phenylketonuria (enzymatic or mutation analysis and amino acid spectrum)
-treatment with a low protein diet (natural protein intake 0.8 gram/kg/day or lower) and the use of amino acid supplements

Healthy subjects:
-age 18 and over.
- Exclusion criteriaPatients:
-known non-adherence to the diet
-known incompliance with taking the amino acid supplements
-use of antibiotics three months prior to sample collection
-other severe conditions that may influence gut microbiome composition (e.g. liver cirrhosis, renal failure, inflammatory bowel disease)

Healthy subjects:
-illnesses known to influence microbiome composition (e.g. liver disease, bowel disease, diarrhea)
-medication use known to influence microbiome
- mec approval receivedno
- multicenter trialyes
- randomisedno
- groupParallel
- TypeSingle arm
- Studytypeobservational
- planned startdate 1-mrt-2017
- planned closingdate1-mrt-2018
- Target number of participants45
- Interventionsone day food diary and a one time stool (feces) sample
- Primary outcomedifferences in the abundance of bacterial strains between healthy volunteers and UCD patients and between PKU and UCD patients
- Secondary outcomedifferences in the abundance of ammonia producing bacterial strains (e.g. Clostridium species) between healthy volunteers and UCD patients and between PKU and UCD patients
- Timepointssingle measurement
- Trial web site
- statusplanned
- CONTACT FOR PUBLIC QUERIES C. Timmer
- CONTACT for SCIENTIFIC QUERIES M. Langeveld
- Sponsor/Initiator Academic Medical Center (AMC), Amsterdam
- Funding
(Source(s) of Monetary or Material Support)
ESN (erfelijke stofwisselingsziekten Nederlands taalgebied).
- Publications
- Brief summaryUrea cycle disorders (UCDs) are a group of rare inherited metabolic diseases causing hyperammonemic encephalopathy. Despite intensive dietary and pharmacological therapy, outcome is poor in subset of UCD patients. Because of very low protein tolerance, UCD patients follow a strict low protein diet. Both the composition of the diet, as well as the amino acid supplementation these patients use could change gut microbial composition, with a potential negative effect on metabolic control.
Hypothesis:
In urea cycle defect patients the protein-restricted diet, in combination with essential amino acid supplementation, results in a different gut microbial composition compared to healthy individuals on a normal diet. Selection of ammonia producing bacteria can negatively influence metabolic regulation in UCD patients.
Objective:
To detect differences between microbiome composition of UCD patients, healthy controls and phenylketonuria (PKU) patients. PKU patients are included to differentiate between the effect of a low protein diet and the UCD itself on microbial composition)
- Main changes (audit trail)
- RECORD10-mrt-2017 - 30-jun-2017


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