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Pubmed ID :28916119
Publication Date : 2017/09/16

Safety and efficacy of glycerol phenylbutyrate for management of urea cycle disorders in patients aged 2months to 2years.


Glycerol phenylbutyrate (GPB) is approved in the US for the management of patients 2months of age and older with urea cycle disorders (UCDs) that cannot be managed with protein restriction and/or amino acid supplementation alone. Limited data exist on the use of nitrogen conjugation agents in very young patients.

Authors : Berry Susan A , Longo Nicola , Diaz George A , McCandless Shawn E , Smith Wendy E , Harding Cary O , Zori Roberto , Ficicioglu Can , Lichter-Konecki Uta , Robinson Beth , Vockley Jerry ,

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