Clinical Food Study to Evaluate the Effect of KB195 on Gut Nitrogen Metabolism in Patients With Urea Cycle Disorders
Clinical Food Study to Evaluate the Effect of KB195 on Gut Nitrogen Metabolism in Patients With Urea Cycle Disorders
This clinical food study aims to explore the effect of KB195, a novel mixture of
 oligosaccharides, on the metabolism of nitrogen by the microbiome in patients with urea cycle
 disorders (UCDs). This will be done using a stable isotope to assess nitrogen metabolism in
 the blood, urine, and stool. The study will also assess the safety and tolerability of KB195
 in patients with UCDs.
Inclusion Criteria:

 -  Confirmed UCD patient at any age ≥ 14 years

 -  Parental/legal guardian permission or patient's written informed consent or assent, as
 applicable

 -  Be willing and able to comply with study requirements

 Exclusion Criteria:

 -  Any medical condition unrelated to the sequelae of UCD

 -  Patient has N-acetylglutamate synthase (NAGS) deficiency

 -  Recent hospitalization or risk for metabolic decompensation

 -  Liver transplantation

 -  Systemic antibiotics, probiotics or prebiotics that do not align with study guidances

 -  Change in dose or frequency of any drug or other compound to modulate GI motility

 -  Contraindications or known allergy/sensitivity to the use of the study products

 -  Use of an investigational drug, product or device within 30 days prior to Screening
 Visit, or current enrolment in another investigational drug, product, or device study

 -  Considered to be at risk for noncompliance or unlikely for any reason to be able to
 comply with the study procedures