Drug Action
Glycerol phenylbutyrate is a nitrogen- binding agent that provides an alternative vehicle for waste nitrogen excretion.
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Drug reference
Glycerol phenylbutyrate is a nitrogen- binding agent that provides an alternative vehicle for waste nitrogen excretion.
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Urea cycle disorders (specialist use only) ▶BY MOUTH, OR BY GASTROSTOMY TUBE, OR BY NASOGASTRIC TUBE ▶Adult (body surface area up to 1.3 m2): Initially 9.4 g/m2 daily in divided doses, usual maintenance 5.3–12.4 g/m2 daily in divided doses, each dose should be rounded up to the nearest 0.5 mL and given with each meal. For dose adjustments based on individual requirements—consult product literature ▶Adult (body surface area 1.3 m2 and above): Initially 8 g/m2 daily in divided doses, usual maintenance 5.3–12.4 g/m2 daily in divided doses, each dose should be rounded up to the nearest 0.5 mL and given with each meal. For dose adjustments based on individual requirements—consult product literature DOSE EQUIVALENCE AND CONVERSION ▶1 mL of liquid contains 1.1 g of glycerol phenylbutyrate. ▶For patients switching from sodium phenylbutyrate or sodium benzoate—consult product literature.
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Treatment of acute hyperammonaemia
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Elderly—limited information available. intestinal malabsorption. pancreatic insufficiency
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Scottish Medicines Consortium (SMC) decisions SMC No. 1342/18 The Scottish Medicines Consortium has advised (August 2018) that glycerol phenylbutyrate (Ravicti ®) is accepted for use within NHS Scotland as an adjunctive therapy for chronic management of adult and paediatric patients aged 2 months and older with urea cycle disorders who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. Glycerol phenylbutyrate must be used with dietary protein restriction and, in some cases, dietary supplements. This advice is contingent upon the continuing availability of the patient access scheme in NHS Scotland or a list price that is equivalent or lower. All Wales Medicines Strategy Group (AWMSG) decisions AWMSG No. 2127 The All Wales Medicines Strategy Group has advised (December 2019) that glycerol phenylbutyrate (Ravicti ®) is recommended as an option for use within NHS Wales for use as an adjunctive therapy for chronic management of patients with urea cycle disorders who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. Ravicti ® must be used with dietary protein restriction and, in some cases, dietary supplements. This recommendation applies only in circumstances where the approved Wales Patient Access Scheme (WPAS) is utilised or where the list/contract price is equivalent or lower than the PAS/WPAS price.
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EMERGENCY MANAGEMENT OF UREA CYCLE DISORDERS For further information on the emergency management of urea cycle disorders consult the British Inherited Metabolic Disease Group (BIMDG) website at www.bimdg.org.uk.
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→Appendix 1: glycerol phenylbutyrate
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▶Common or very common Appetite abnormal. constipation . diarrhoea. dizziness. fatigue. food aversion. gastrointestinal discomfort. gastrointestinal disorders. headache. menstrual cycle irregularities. nausea. oral disorders. peripheral oedema. skin reactions. tremor. vomiting ▶Uncommon Akathisia. alopecia. biliary colic. bladder pain . burping. confusion. depressed mood. drowsiness. dry mouth. dysphonia. epistaxis. fever. gastrointestinal infection viral. hot flush. hyperhidrosis. hypoalbuminaemia. hypokalaemia. hypothyroidism. joint swelling. muscle spasms. nasal congestion. oropharyngeal pain. pain. paraesthesia. plantar fasciitis. speech disorder. taste altered. throat irritation. ventricular arrhythmia. weight changes
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Manufacturer advises avoid unless essential—toxicity in animal studies.
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Manufacturer advises avoid—no information available.
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Manufacturer advises caution (risk of increased exposure). Dose adjustments Manufacturer advises use lowest possible dose—consult product literature.
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Manufacturer advises use with caution in severe impairment—no information available.
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Manufacturer advises may be added to a small amount of apple sauce, ketchup, or squash puree and used within 2 hours. For administration advice via nasogastric or gastrostomy tube—consult product literature. l HANDLING AND STORAGE Manufacturer advises discard contents of bottle 14 days after opening.
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There can be variation in the licensing of different medicines containing the same drug. Liquid ▶Ravicti (Immedica Pharma AB) A Glycerol phenylbutyrate 1.1 gram per 1 ml Ravicti 1.1g/ml oral liquid | 25 ml P £161.00
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Driving and skilled tasks Manufacturer advises patients and carers should be counselled on the effects on driving and performance of skilled tasks—increased risk of dizziness.
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Initially 9.4 g/m2
First-line fragment. Read the complete Indications and Dose section above.
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Initially
First-line fragment. Read the complete Indications and Dose section above.
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