Urea cycle disorders Urea cycle disorders
Conditions
Interventions
Protein loading with 2 grams of natural protein per kilogram body weight. If normal plasma and urine values are observed, the protein loading test will be repeated with up to 4 grams per kilogram body
The protein-rich drink consists of a protein shake (Melkunie) with Resource whey protein powder (Nestlé). 
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
18 Years to 64 Years
Inclusion criteria
Inclusion criteria: In order to be eligible to participate in this study, a participant must meet all of the following criteria:Age 18-64HealthyBMI between 18 and 25 kg/m2
Exclusion criteria
Exclusion criteria: A potential participant who meets any of the following criteria will be excluded from participation in this study:Alcohol or substance abuseLaboratory results outside of the normal ranges at screening and judged as clinically relevant by the Investigator for ammonia, liver function (alanine aminotransferase, aspartate aminotransferase, albumin, bilirubin, gamma-glutamyl transferase, alkaline phosphatase, international normalized ratio, and activated partial thromboplastin time), amino acids, carnitine, urea, and creatinineKnown with a condition that is known to cause an impairment of the urea cycle, or with a family history of such condition.Any other acute or chronic condition with a clear negative impact on healthIntolerance for one of the substances of the protein-rich drinkBariatric surgeryNot able to read and comprehend Dutch or English (necessary to read the patient information form)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The concentrations of ammonia and glutamine in plasma of healthy individuals without an impairment of the urea cycle at various time points before and after consuming a large protein bolus (containing 2 or up to 4 grams protein per kilogram body weight) | — |
Secondary
| Measure | Time frame |
|---|---|
| The secondary parameters of this study are (i) concentrations of orotic acid in urine of healthy individuals at baseline and after 360 minutes after consuming a large protein bolus and (ii) the difference in time required to complete the TMT 10 minutes before and 120 minutes after consuming a large protein bolus. | — |
Countries
Netherlands
Contacts
Public ContactM.A.E.M. Wagenmakers
Erasmus MC, Universitair Medisch Centrum Rotterdam
Outcome results
None listed