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Olpruva
sodium phenylbutyrate
Adult Dosing .
Dosage forms: PELLET: 2 g convenience pack, 3 g convenience pack, 4 g convenience pack, 5 g convenience pack, 6 g convenience pack, 6.67 g convenience pack
Dosage Form Details
- [2 g convenience pack components]
- Info: single-dose pack contains 2 g packet x1 and suspending agent
- [3 g convenience pack components]
- Info: single-dose pack contains 3 g packet x1 and suspending agent
- [4 g convenience pack components]
- Info: single-dose pack contains 2 g packet x2 and suspending agent
- [5 g convenience pack components]
- Info: single-dose pack contains 2 g packet x1, 3 g packet x1, and suspending agent
- [6 g convenience pack components]
- Info: single-dose pack contains 3 g packet x2 and suspending agent
- [6.67 g convenience pack components]
- Info: single-dose pack contains 3 g packet x1, 3.67 g packet x1, and suspending agent
chronic hyperammonemia, urea cycle disorder-assoc.
- [9.9-13 g/m^2/day PO divided 3-6x/day]
- Max: 20 g/day; Info: for pts w/ carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase deficiency; adjust dose based on pt clinical response and plasma ammonia levels; give w/ food; separate doses by at least 3h
renal dosing
- [see below]
- renal impairment: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [adjust dose amount]
- hepatic impairment: use lowest usual start dose
Peds Dosing .
- Dosage forms: PELLET: 2 g convenience pack, 3 g convenience pack, 4 g convenience pack, 5 g convenience pack, 6 g convenience pack, 6.67 g convenience pack
Dosage Form Details
- [2 g convenience pack components]
- Info: single-dose pack contains 2 g packet x1 and suspending agent
- [3 g convenience pack components]
- Info: single-dose pack contains 3 g packet x1 and suspending agent
- [4 g convenience pack components]
- Info: single-dose pack contains 2 g packet x2 and suspending agent
- [5 g convenience pack components]
- Info: single-dose pack contains 2 g packet x1, 3 g packet x1, and suspending agent
- [6 g convenience pack components]
- Info: single-dose pack contains 3 g packet x2 and suspending agent
- [6.67 g convenience pack components]
- Info: single-dose pack contains 3 g packet x1, 3.67 g packet x1, and suspending agent
chronic hyperammonemia, urea cycle disorder-assoc.
- [>20 kg and BSA >1.2 m^2]
- Dose: 9.9-13 g/m^2/day PO divided 3-6x/day; Max: 20 g/day; Info: for pts w/ carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase deficiency; adjust dose based on pt clinical response and plasma ammonia levels; give w/ food; separate doses by at least 3h
renal dosing
- [see below]
- renal impairment: not defined, caution advised
- HD/PD: not defined, caution advised
hepatic dosing
- [adjust dose amount]
- hepatic impairment: use lowest usual start dose
Contraindications / Cautions .
- hypersensitivity to drug or ingredient
- caution: inborn error of beta-oxidation
- caution: hepatic impairment
- caution: renal impairment
- caution: nephrosis
- caution: cirrhosis
- caution: CHF
- caution: sodium retention w/ edema
Drug Interactions .
Overview
sodium phenylbutyrate
nitrogen binding agent
- OAT1 substrate
- anti-hyperammonemia agent
- hypokalemia
Avoid/Use Alternative
- arsenic trioxide
Monitor/Modify Tx
- abiraterone acetate
- acetazolamide
- albuterol
- albuterol inhaled
- aloe
- amphotericin
- arformoterol inhaled
- asparaginase
- betamethasone
- budesonide
- bumetanide
- carbamazepine
- carboplatin
- carfilzomib
- cetuximab
- chlorothiazide
- chlorthalidone
- cisplatin
- corticotropin
- cortisone
- crizotinib
- decitabine
- deflazacort
- dexamethasone
- dichlorphenamide
- digoxin
- dinutuximab
- emapalumab
- epinephrine
- ethacrynic acid
- fludrocortisone
- fluorouracil
- formoterol inhaled
- foscarnet
- furosemide
- haloperidol
- hydrochlorothiazide
- hydrocortisone
- indapamide
- insulin
- isoproterenol
- lenalidomide
- levalbuterol inhaled
- licorice
- magnesium citrate
- mannitol
- methazolamide
- methylprednisolone
- metolazone
- mifepristone
- motixafortide
- necitumumab
- nilotinib
- olodaterol inhaled
- osilodrostat
- pamidronate
- panitumumab
- polyethylene glycol
- polyethylene glycol/electrolytes
- posaconazole
- prednisolone
- prednisone
- sacituzumab govitecan
- salmeterol inhaled
- selinexor
- sirolimus albumin-bound
- sodium benzoate
- sodium phenylacetate
- sodium polystyrene sulfonate
- sodium thiosulfate
- sulfate bowel prep
- tafasitamab
- terbutaline
- topiramate
- torsemide
- trastuzumab
- triamcinolone
- valproic acid
- vamorolone
- vilanterol inhaled
- zonisamide
Caution Advised
- probenecid
Adverse Reactions .
Serious Reactions
- neurotoxicity
- anemia
- leukopenia
- thrombocytopenia
- renal tubular acidosis
Common Reactions
- menstrual irregularities
- anorexia
- body odor
- taste disturbance
- metabolic acidosis
- hypoalbuminemia
- anemia
- hyperchloremia
- metabolic alkalosis
- hypophosphatemia
- LFTs incr.
- leukopenia
- leukocytosis
- total protein decr.
- hypokalemia
- thrombocytopenia
- hyperuricemia
- hyperphosphatemia
- hypernatremia
Safety/Monitoring .
Monitoring Parameters
CBC; chemistry profile; plasma phenylacetate; plasma phenylacetylglutamine; urinalysis
Pregnancy/Lactation .
Pregnancy
Clinical Summary
weigh risk/benefit during pregnancy; no human data available; possible risk of neonatal neurotoxicity based on animal data
Lactation
Clinical Summary
weigh risk/benefit while breastfeeding; no human data available to assess risk of infant harm or effects on milk production
Pharmacology .
Metabolism: liver, kidney; CYP450: unknown; Info: prodrug converted to phenylacetate
Excretion: urine 80-100%; Half-life: 1.15-1.29h
Subclass: Inherited Metabolic Disorders
Mechanism of Action
provides alternate urea cycle pathway, reducing excess nitrogen
Formulary .
No Formulary Selected
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