Ammonia control in children with urea cycle disorders (UCDs); Phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate

Molecular Genetics and Metabolism

Volumn 103, Issue 4, 2011, Pages 323-329

  Lichter Konecki, Uta ^a   Diaz, G A ^b   Merritt, J L ^c   Feigenbaum, A ^d   Jomphe, C ^e   Marier, J F ^e   Beliveau, M ^e   Mauney, J ^f   Dickinson, K ^g   Martinez, A ^g   Mokhtarani, M ^g   Scharschmidt, B ^g   Rhead, W ^h  

^a CHILDREN'S NATIONAL MEDICAL CENTER   (United States)

^b MOUNT SINAI SCHOOL OF MEDICINE   (United States)

^c SEATTLE CHILDREN S HOSPITAL   (United States)

^d HOSPITAL FOR SICK CHILDREN   (Canada)

^e Pharsight   (Canada)

^f Chiltern   (United States)

^g Hyperion Therapeutics Inc   (United States)

^h MEDICAL COLLEGE OF WISCONSIN   (United States)

Author keywords

Ammonia scavengers; Glycerol phenylbutyrate; Hyperammonemia; Phenylacetate; Sodium phenylbutyrate; Urea cycle disorders

Indexed keywords

4 PHENYLBUTYRIC ACID; AMMONIA; ARYLBUTYRIC ACID DERIVATIVE; DRUG METABOLITE; GLYCEROL PHENYLBUTYRATE; PHENYLACETIC ACID DERIVATIVE; PHENYLACETYLGLUTAMINE; UNCLASSIFIED DRUG;

ADOLESCENT; AMINO ACID BLOOD LEVEL; AMMONIA BLOOD LEVEL; ARGININOSUCCINIC ACIDURIA; ARTICLE; BLOOD LEVEL; BLOOD SAMPLING; CHILD; CITRULLINEMIA; CLINICAL ARTICLE; COMPARATIVE STUDY; CONTACT DERMATITIS; CROSSOVER PROCEDURE; DECREASED APPETITE; DRUG BLOOD LEVEL; DRUG EFFECT; DRUG EFFICACY; DRUG SAFETY; DRUG TOLERABILITY; EAR INFECTION; FEMALE; HEART MURMUR; HUMAN; HYPERAMMONEMIA; LABORATORY TEST; LYMPHADENOPATHY; MALE; MONITORING; ORNITHINE TRANSCARBAMYLASE DEFICIENCY; PATIENT COMPLIANCE; PHASE 2 CLINICAL TRIAL; POSTPRANDIAL STATE; POWDER; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN RESTRICTION; SCHOOL CHILD; TABLET; TREATMENT DURATION; UPPER ABDOMINAL PAIN; UPPER RESPIRATORY TRACT INFECTION; UREA CYCLE DISORDER; URINE LEVEL; VOMITING;

ADOLESCENT; AMMONIA; CHILD; DOSE-RESPONSE RELATIONSHIP, DRUG; GLYCEROL; HUMANS; MALE; PHENYLBUTYRATES; UREA; UREA CYCLE DISORDERS, INBORN;

EID: 79960848652     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.04.013     Document Type: Article

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