Phenylbutyrate for Monogenetic Developmental and Epileptic Encephalopathy

"Glycerol phenylbutyrate (trade name Ravicti) is an FDA-approved medication used for urea cycle disorders in children and adults. We will titrate to a goal dose of 1.2 mL/m2 (12.4 g/m2) in three equally divided doses given enterally (i.e., by mouth or by g-tube).~The dosing is consistent with the dosing guidelines in the FDA approved Medication Guide (https://www.accessdata.fda.gov/drugsatfda\_docs/label/2017/203284s005lbl.pdf)."