DRUG | SUPPLIED | DOSE | COMMENTS |
---|---|---|---|
SODIUM PHENYLBUTYRATE (Paeds) (Pheburane) Mechanism of action: Prodrug of phenylacetate, which conjugates with glutamine to form phenylacetylglutamine, a nitrogen waste product that is excreted by the kidneys. Ref: 61,110, 483 Last update: 2019-07-07 |
Granules: 483 mg sodium phenylbutyrate per gram of granules. Bottles of 174 |
Oral: < 20 kg: 250-600 mg/kg/24 hr PO added to daily formula >20 kg: 5 -13 g/m2/24 hr PO added to daily formula Max 20 g/24 h |
If total daily dose is not added to daily formula, then may be divided BID-TID Oral administration: The total daily dose should be divided into equal amounts and given with each meal or feeding (e.g. 4-6 times per day in small children). The granules can be directly swallowed with a drink (water, fruit juices, protein-free infant formulas) or sprinkled on to a spoonful of solid food (mashed potatoes or apple sauce); in this case, it is important that the Pheburane granules and food is taken immediately in order to preserve the taste-masking. A calibrated dosing spoon is provided which dispenses up to 3 g of sodium phenylbutyrate, in graduations of 250 mg. Only use the dosing spoon provided with the medicine to measure out the dose. Administration by nasogastric or gastrostomy tube: Pheburane granules should not be administered by tube. A solution of Pheburane (50 mg/ml of sodium phenylbutyrate) must be prepared by hospital or pharmacy personnel for administration through a nasogastric or gastrostomy tube Standard Prescription: sodium phenylbutyrate __ mg/kg/24hour PO added to daily formula sodium phenylbutyrate __ g/m2/24 hour PO added to daily formula |