SODIUM PHENYLBUTYRATE
(Pheburane)Standard Prescription
sodium phenylbutyrate __ mg/kg/24hour PO added to daily formula
sodium phenylbutyrate __ g/m2/24 hour PO added to daily formula
Dosages
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
< 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
Mechanism of Action
Prodrug of phenylacetate, which conjugates with glutamine to form phenylacetylglutamine, a nitrogen waste product that is excreted by the kidneys.
Forms Supplied
Granules: 483 mg sodium phenylbutyrate per gram of granules.
Bottles of 174
Comments
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
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
References
61,110, 483
Last Edited
2019-07-07 19:13:59