The importance of gut motility in the metabolic control of propionic acidemia.
J Pediatr
; 144(4): 532-5, 2004 Apr.
Article
em En
| MEDLINE
| ID: mdl-15069406
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Propionatos
/
Motilidade Gastrointestinal
/
Erros Inatos do Metabolismo dos Aminoácidos
/
Glicina
Tipo de estudo:
Observational_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Humans
Idioma:
En
Ano de publicação:
2004
Tipo de documento:
Article