Drug reference
Long-term treatment of urea cycle disorders (as adjunctive therapy in all patients with neonatal-onset disease and in those with late-onset disease who have a history of hyperammonaemic encephalopathy) (under expert supervision) ▶BY MOUTH ▶Adult: 9.9–13 g/m2 daily in divided doses, with meals; maximum 20 g per day
PDF page 1153
Conditions involving sodium retention with oedema (preparations contain significant amounts of sodium). congestive heart failure (preparations contain significant amounts of sodium)
PDF page 1153
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.
PDF page 1153
→Appendix 1: sodium phenylbutyrate
PDF page 1153
▶Common or very common Abdominal pain. anaemia. appetite decreased. constipation. depression. headache. irritability. leucocytosis. leucopenia. menstrual cycle irregularities. metabolic acidosis. metabolic alkalosis. nausea. oedema. renal tubular acidosis. skin reactions. syncope. taste altered. thrombocytopenia. thrombocytosis. vomiting. weight increased ▶Uncommon Anorectal haemorrhage. aplastic anaemia. arrhythmia. gastrointestinal disorders. pancreatitis
PDF page 1153
Manufacturer advises adequate contraception during administration in women of child-bearing potential.
PDF page 1153
Avoid—toxicity in animal studies. Blood and nutrition
PDF page 1153
Manufacturer advises avoid—no information available.
PDF page 1153
Manufacturer advises caution.
PDF page 1153
Manufacturer advises use with caution (preparations contain significant amounts of sodium).
PDF page 1153
Granules should be mixed with food before taking. Pheburane ® granules must not be administered by nasogastric or gastrostromy tubes.
PDF page 1153
There can be variation in the licensing of different medicines containing the same drug. Forms available from special-order manufacturers include: capsule, oral suspension, oral solution Granules ▶Ammonaps (Immedica Pharma AB) Sodium phenylbutyrate 940 mg per 1 gram Ammonaps 940mg/g granules sugar-free | 266 gram P £860.00 DT = £860.00 ▶Pheburane (Eurocept International bv) Sodium phenylbutyrate 483 mg per 1 gram Pheburane 483mg/g granules | 174 gram P £331.00 Tablet ▶Ammonaps (Immedica Pharma AB) Sodium phenylbutyrate 500 mg Ammonaps 500mg tablets | 250 tablet P £493.00 DT = £493.00 2.13 Wilson’s disease Other drugs used for Wilson’s disease Penicillamine, p. 1158 ANTIDOTES AND CHELATORS › COPPER ABSORPTION INHIBITORS
PDF page 1153
9.9–13 g/m2 daily in divided doses, with meals;
First-line fragment. Read the complete Indications and Dose section above.
PDF page 1153
