The importance of gut motility in the metabolic control of propionic acidemia.
We hypothesized that gut motility likely plays a critical role in the metabolic stability in propionic acidemia (PA). Therefore, 4 known patients with PA (aged 47 months to 185 months) were prospectively studied over 7 days in the Clinical Research Center at Children's Hospital, Boston. Determinations of ammonia, bicarbonate, and amino acids in blood; organic acids and propionylglycine in urine; and a lactulose breath test were conducted under two study conditions: on regular therapy (for 4 days) and on regular therapy plus Senekot (Purdue Frederick Company, Norwalk, Conn), an intestinal motility agent (for 3 days). The total gastrointestinal transit time was calculated using 20 nonabsorbable, inert, radio-opaque markers. The addition of an intestinal motility agent resulted in a significant decrease in blood ammonia, urinary excretion of propionylglycine, and a rise in the ratio of free to total carnitine over baseline. We concluded that enhancement of gut motility can improve metabolic stability in patients with PA.
Prasad
Chitra
C
Division of Genetics, Children's Hospital, Boston, Massachusetts, USA. Chitra.Prasad@lhsc.on.ca
Nurko
Samuel
S
Borovoy
Jacob
J
Korson
Mark S
MS
D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000201:enzymology* / Q000503:physiopathology / Q000628:therapy*; D000641:Ammonia / Q000097:blood; D002331:Carnitine / Q000032:analysis; D002400:Cathartics / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D005769:Gastrointestinal Motility / Q000502:physiology*; D005998:Glycine / Q000031:analogs & derivatives* / Q000652:urine; D006801:Humans; D011422:Propionates / Q000097:blood*; D011446:Prospective Studies; D012676:Senna Extract / Q000008:administration & dosage
D002400:Cathartics; D011422:Propionates; C050479:N-propionylglycine; D000641:Ammonia; D012676:Senna Extract; D002331:Carnitine; D005998:Glycine