N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia

Pediatrics

Volumn 126, Issue 1, 2010, Pages

  Mew, Nicholas Ah ^a   McCarter, Robert ^a   Daikhin, Yevgeny ^b   Nissim, Itzhak ^b   Yudkoff, Marc ^b   Tuchman, Mendel ^a  

^a GEORGE WASHINGTON UNIVERSITY   (United States)

^b UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

Acetylglutamate; Carbamylglutamate; Clinical trial; Hyperammonemia; N acetyl l glutamate; Organic acidemia; Stable isotopes; Urea cycle

Indexed keywords

AMINO ACID DERIVATIVE; AMMONIA; CARGLUMIC ACID; GLUTAMIC ACID DERIVATIVE; GLUTAMINE; N CARBAMYLGLUTAMATE; UNCLASSIFIED DRUG; UREA;

ADOLESCENT; ARTICLE; BLOOD SAMPLING; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; FEMALE; HUMAN; HYPERAMMONEMIA; MALE; PRESCHOOL CHILD; PRIORITY JOURNAL; PROPIONIC ACIDEMIA; SCHOOL CHILD; TREATMENT RESPONSE;

ADMINISTRATION, ORAL; ADOLESCENT; AMMONIA; BLOOD CHEMICAL ANALYSIS; CHILD; CHILD, PRESCHOOL; CONFIDENCE INTERVALS; DOSE-RESPONSE RELATIONSHIP, DRUG; DRUG ADMINISTRATION SCHEDULE; FEMALE; FOLLOW-UP STUDIES; GLUTAMATES; GLUTAMINE; HUMANS; INFANT; MALE; PROBABILITY; PROPIONIC ACIDEMIA; PROSPECTIVE STUDIES; RISK ASSESSMENT; TREATMENT OUTCOME; UREA;

EID: 77954377501     PISSN: 00314005     EISSN: 10984275     Source Type: Journal    
DOI: 10.1542/peds.2010-0008     Document Type: Article

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