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…
ID
Bron
Verkorte titel
Aandoening
UCD; Urea cycle defect; ureumcyclusdefect; PKU; Pheylketonuria; fenylketonurie; microbiome; microbioom; gut bacteria; darmbacterien
Ondersteuning
Onderzoeksproduct en/of interventie
Uitkomstmaten
Primaire uitkomstmaten
differences in the abundance of bacterial strains between healthy volunteers and UCD patients and between PKU and UCD patients
Achtergrond van het onderzoek
Urea 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)
Doel van het onderzoek
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
Onderzoeksopzet
single measurement
Onderzoeksproduct en/of interventie
one day food diary
and
a one time stool (feces) sample
Algemeen / deelnemers
Wetenschappers
Belangrijkste voorwaarden om deel te mogen nemen (Inclusiecriteria)
Patients:
- 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.
Belangrijkste redenen om niet deel te kunnen nemen (Exclusiecriteria)
Patients:
-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
Opzet
Deelname
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In overige registers
| Register | ID |
|---|---|
| NTR-new | NL6106 |
| NTR-old | NTR6447 |
| Ander register | NL61031 : ABR nummer |