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Urea cycle disorders Clinical criteria: Treatment Phase: Initial treatment Patient must have elevated ammonia levels that are not controlled with diet alone and other adjunct care alone. An increase in the maximum quantity will be authorised to provide for up to one month's supply at a dose of up to 600 mg/kg/day in patients weighing less than 20 kg and up to 13 g/m2/day in patients weighing more than 20 kg.
“Sodium phenylbutyrate is an ammonia scavenger. Sodium phenylbutyrate is a pro-drug and is rapidly metabolised to phenylacetate. Phenylacetate is a metabolically active compound that conjugates with glutamine via acetylation to form phenylacetylglutamine which is then excreted by the kidneys.”
“The elimination half-life was estimated to be 0.8 hours.”
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Curated subset. The full adverse-effect list is in the TGA Product Information; click any citation above to open it.
“Hepatotoxicity after rifampicin ceased possibly due to reduction in CYP3A4 leading to toxic accumulation of phenylacetate.”