Cambridgeshire and Peterborough
Formulary
 
back
9 Nutrition and blood
09-08-01 Urea cycle disorders

Arginine
Formulary

  • unlicensed - All preparations are unlicensed.



  • CUHFT:

    • Indicated for urea cycle disorders, MELAS and mitochondrial disorders (Neuro). 

    • For paediatric metabolic disorders on specialist advice only. 

    • Available as:

      • L-arginine 100mg/ml oral solution.

      • 500mg tablets.

      • 1g tablets. 



    • Restricted Item 5g in 10ml injection only - restricted to emergency treatment for hyperammonaemia.





  • NWAFT - Restricted Item 5g in 10ml injection only - restricted to treating hyperammonaemia in a patient with ornithine transcarbamylase deficiency as per protocol.



  • Non formulary at all other trusts.


 

Link  Protocol for treating Hyperammonaemia in a patient with Ornithine Transcarbamylase Deficiency
 

Red Hospital View adult BNF  View SPC online  View childrens BNF
Betaine Cystadane®
Formulary

 

    • Available as Cystadane® Powder or 500mg tablets (unlicensed - unlicensed).




    • CUHFT only - Commissioned by NHSE for highly specialised services (metabolic liposomal disorders). Powder to be considered on case-by-case basis. Contact formulary/commissioning for further info.




    • Non formulary at all other trusts.



 


Red Hospital View adult BNF  View SPC online  View childrens BNF
Carglumic Acid Carbaglu®
Formulary

  • CUHFT only: Hyperammonaemia due to N-acetylglutamate synthase (NAGS) deficiency - Specialist use only.


Red Hospital View adult BNF  View SPC online  View childrens BNF  HCD
Sodium Benzoate
Formulary

  •  CUHFT:

    • For urea cycle disorders.

    • For paediatric metabolic disorders on specialist advice only.

    • Available as (all unlicensed unlicensed):

      • 500mg/5ml oral solution

      • 125mg/5ml oral solution (paeds only)

      • 500mg tablets

      • 2g/10ml injection 







  • NWAFTRestricted Item injection only restricted to treating hyperammonaemia in a patient with ornithine transcarbamylase deficiency as per protocol.


 

Link  Protocol for treating Hyperammonaemia in a patient with Ornithine Transcarbamylase Deficiency
 

Red Hospital
Sodium Phenylbutyrate
Formulary

  • CUHFT:

    • Available as:

      • Tablets 500mg (Ammonaps)

      • Granules (Ammonaps)

      • Oral liquid (unlicensed unlicensed)

      • Injection 200mg/ml (unlicensed unlicensed)



    • For paediatric metabolic disorders on specialist advice only. 





  • NWAFT: Restricted Item Injection 1g in 5ml only (unlicensed unlicensed) - restricted to treating hyperammonaemia in a patient with ornithine transcarbamylase deficiency as per protocol. 


 

Link  Protocol for treating Hyperammonaemia in a patient with Ornithine Transcarbamylase Deficiency
 

Red Hospital
Sodium Benzoate
Non Formulary

  • CUHFT only: 1.25g/5ml oral solution unlicensed unlicensed  - requires individual approval.


 


Grey View adult BNF  View SPC online  View childrens BNF