| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10833462 | Molecular Genetics and Metabolism | 2012 | 7 Pages |
Abstract
⺠Relation of phenylbutyrate dose and plasma and urinary metabolites is described. ⺠First pass mechanism and degrees of absorption of phenylbutyrate is discussed. ⺠The variability of plasma metabolites levels is discussed. ⺠Urinary PAGN is a biomarker for dose adjustment and assessing compliance.
Keywords
ASLPagNULNPBAgPbDSMBUCDArgininosuccinate synthetase deficiencyArgininosuccinate lyase deficiencyAUC0–24ASsOTCPAAUrea cycle disorderUrea cycle disordersPhenylacetic acidPhenylbutyric acidData safety and monitoring boardITTUpper limit of normalCV%sodium phenylbutyrateCoefficient of VariationpharmacokineticPhenylacetylglutamineintention to treatOrnithine transcarbamylase deficiencyGlycerol phenylbutyrateNaPBA
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Authors
M. Mokhtarani, G.A. Diaz, W. Rhead, U. Lichter-Konecki, J. Bartley, A. Feigenbaum, N. Longo, W. Berquist, S.A. Berry, R. Gallagher, D. Bartholomew, C.O. Harding, M.S. Korson, S.E. McCandless, W. Smith, J. Vockley, S. Bart, D. Kronn, B.F. Scharschmidt,