Clinical Trial: The NIH UNI Study: Urea Cycle Disorders, Nutrition and Immunity

Study Status: Terminated
Recruit Status: Terminated
Study Type: Observational




Official Title: The NIH UNI Study: Urea Cycle Disorders, Nutrition and Immunity

Brief Summary:

Objectives:

  • To study nutrition and immune system problems in people with urea cycle disorders.
  • To study how people with urea cycle disorders and healthy volunteers respond to standard flu and/or hepatitis A vaccines.
  • To compare differences in nutrition and immune systems of people with urea cycle disorders with that of healthy volunteers.

Eligibility:

  • Healthy males and females at least 2 years of age who are able to travel to the National Institutes of Health hospital in Bethesda, MD
  • Males and females at least 2 years of age who have a urea cycle disorder and are able to travel to the National Institutes of Health hospital in Bethesda, MD.

Design:

For Patients with urea cycle disorder:

  • Participants will spend 2 to 3 days in the National Institutes of Health hospital for the following tests:
  • A physical exam and review of medical history
  • Food log for 3 days before the start of the study
  • Blood tests
  • 24-hour urine collection
  • Resting metabolism test
  • DEXA scan imaging study of bones and body fat
  • Participants who are old enough to do certain tasks by themselves (like dressing and eating) can choose to have the following extra tests:
  • 24-hour metabol