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08:15

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Published on: December 1, 2010

The importance of gut motility in the metabolic control of propionic acidemia

Chitra Prasad1, Samuel Nurko, Jacob Borovoy

  • 1Division of Genetics, Children's Hospital, Boston, Massachusetts, USA. Chitra.Prasad@lhsc.on.ca

The Journal of Pediatrics
|April 8, 2004
PubMed

Insights

Enhancing gut motility in propionic acidemia (PA) patients improved metabolic stability. The study showed that using an intestinal motility agent significantly reduced blood ammonia and improved carnitine levels in PA patients.

Area of Science:

  • Biochemistry
  • Gastroenterology
  • Metabolic Disorders

Background:

  • Propionic acidemia (PA) is a metabolic disorder.
  • Gut motility's role in PA metabolic stability is not well understood.

Purpose of the Study:

  • To investigate the effect of enhanced gut motility on metabolic stability in PA patients.
  • To assess the impact of an intestinal motility agent on key metabolic markers in PA.

Main Methods:

  • Prospective study of 4 PA patients over 7 days.
  • Measurements included blood ammonia, amino acids, urine organic acids, and lactulose breath test.
  • Gastrointestinal transit time assessed using radio-opaque markers with and without a motility agent (Senekot).

Main Results:

  • Intestinal motility agent significantly decreased blood ammonia levels.
  • Urinary propionylglycine excretion was reduced.
  • The ratio of free to total carnitine increased.

Conclusions:

  • Enhancing gut motility can improve metabolic stability in patients with propionic acidemia.
  • Intestinal motility agents may be a beneficial adjunct therapy for PA.

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