Nitisinone 10 mgOrfadin 10 mg capsules| 60 capsuleP
£ 2 , 062. 00
Nitisinone 20 mgOrfadin 20 mg capsules| 60 capsuleP
£ 4 , 512. 003.13Urea cycle disorders
AMINO ACIDS AND DERIVATIVES
Arginine
lINDICATIONS AND DOSE
Acute hyperammonaemia in carbamylphosphate
synthetase deficiency (specialist use only)|Acute
hyperammonaemia in ornithine transcarbamylase
deficiency (specialist use only)
▶BY INTRAVENOUS INFUSION
▶Neonate: 6 mg/kg/hour.▶Child (body-weight up to 40 kg): 6 mg/kg/hour
▶Child (body-weight 40 kg and above): 4 mg/kg/hour
Maintenance treatment of hyperammonaemia in
carbamylphosphate synthetase deficiency (specialist use
only)|Maintenance treatment of hyperammonaemia in
ornithine transcarbamylase deficiency (specialist use
only)
▶BY MOUTH
▶Neonate: 100 – 200 mg/kg daily in 3 – 4 divided doses,
dose to be taken with feeds.▶Child (body-weight up to 20 kg): 100 – 200 mg/kg daily in
3 – 4 divided doses, dose to be given with feeds or meals
▶Child (body-weight 20 kg and above): 2. 5 – 6 g/m^2 daily in
3 – 4 divided doses, dose to be taken with meals;
maximum 6 g per day
Acute hyperammonaemia in citrullinaemia (specialist use
only)|Acute hyperammonaemia in arginosuccinic
aciduria (specialist use only)
▶BY INTRAVENOUS INFUSION
▶Neonate:Initially 300 mg/kg, to be administered over
90 minutes, followed by 12. 5 mg/kg/hour, to be
administered over 24 hours (maximum 25 mg/kg/hour
thereafter).▶Child (body-weight up to 40 kg):Initially 300 mg/kg, to
be administered over 90 minutes, followed by
12. 5 mg/kg/hour, to be administered over 24 hours
(maximum 25 mg/kg/hour thereafter)
▶Child (body-weight 40 kg and above): 21 mg/kg/hour
Maintenance treatment of hyperammonaemia in
citrullinaemia (specialist use only)|Maintenance
treatment of hyperammonaemia in arginosuccinic
aciduria (specialist use only)
▶BY MOUTH
▶Neonate: 100 – 300 mg/kg daily in 3 – 4 divided doses,
dose to be taken with feeds.▶Child (body-weight up to 20 kg): 100 – 300 mg/kg daily in
3 – 4 divided doses, dose to be taken with feed or meals
▶Child (body-weight 20 kg and above): 2. 5 – 6 g/m^2 daily in
3 – 4 divided doses, doses to be taken with meals;
maximum 6 g per daylUNLICENSED USEInjection not licensed in children.
Tablets not licensed in children. Powder licensed for urea
cycle disorders in children.
lCONTRA-INDICATIONSNot to be used in the treatment of
arginase deficiency
lSIDE-EFFECTSAcidosis hyperchloraemic.flushing.
headache.hypotension.nausea.numbness.vomiting
lPREGNANCYNo information available.
lBREAST FEEDINGNo information available.
lMONITORING REQUIREMENTSMonitor plasma pH and
chloride.
lDIRECTIONS FOR ADMINISTRATIONForintravenous
infusion, dilute to a max. concentration of 50 mg/mL with
glucose 10 %.
lPRESCRIBING AND DISPENSING INFORMATIONPowder to
be prescribed as a borderline substance (ACBS). For use as
a supplement in urea cycle disorders other than arginase
deficiency, such as hyperammonaemia types I and II,
citrullinaemia, arginosuccinic aciduria, and deficiency of
N-acetyl glutamate synthetase.
lPATIENT AND CARER ADVICE
Medicines for Children leaflet: Arginine for urea cycle disorders
http://www.medicinesforchildren.org.uk/arginine-urea-cycle-
disorders- 0lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug. Forms available from special-order
manufacturers include: tablet, capsule, oral solution, solution
for infusion
Tablet
▶Arginine (Non-proprietary)
L-Arginine 500 mgL-Arginine 500 mg tablets| 60 tablet £ 5. 86
L-Arginine 1 gramArginine 1 g tablets| 90 tablet £ 11. 14
Solution for infusion
▶Arginine (Imported (Germany))
L-Arginine monohydrochloride 210.7 mg per 1 mlL-Arginin-
Hydrochlorid 21 % concentrate for solution for infusion 20 ml ampoules
| 5 ampoulePs
Capsule
▶Arginine (Non-proprietary)
L-Arginine 500 mgL-Arginine 500 mg capsules| 30 capsule £ 3. 35 |
120 capsule £ 13. 05
Powder
▶Arginine (Non-proprietary)
L-Arginine 1 gram per 1 gramL-Arginine powder| 100 gram £ 44. 31Carglumic acid
lINDICATIONS AND DOSE
Hyperammonaemia due to N-acetylglutamate synthase
deficiency (under expert supervision)
▶BY MOUTH
▶Neonate:Initially 50 – 125 mg/kg twice daily, to be taken
immediately before feeds, dose adjusted according to
plasma–ammonia concentration; maintenance
5 – 50 mg/kg twice daily, the total daily dose may
alternatively be given in 3 – 4 divided doses.▶Child:Initially 50 – 125 mg/kg twice daily, to be taken
immediately before food, dose adjusted according to
plasma–ammonia concentration; maintenance
5 – 50 mg/kg twice daily, the total daily dose may
alternatively be given in 3 – 4 divided doses
Hyperammonaemia due to organic acidaemia (under
expert supervision)
▶BY MOUTH
▶Neonate:Initially 50 – 125 mg/kg twice daily, to be taken
immediately before feeds, dose adjusted according to
plasma-ammonia concentration, the total daily dose
may alternatively be given in 3 – 4 divided doses.▶Child:Initially 50 – 125 mg/kg twice daily, to be taken
immediately before food, dose adjusted continued→BNFC 2018 – 2019 Urea cycle disorders 613
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