Adjunct in long-term treatment of urea cycle disorders. It is indicated as adjuvant in all patient with neonatal onset disease and in those with late onset disease who have a history of hyperammonaemic encephalopathy
Treatment of urea cycle disorders
Treatment of hyperammonaemia due to urea cycle disorders and non-ketotic hyperglycinaemia
Section Title (top level)
Section Title (sub level)
First Choice item
Non Formulary section
Display tracking information
Link to adult BNF
Link to children's BNF
Link to SPCs
Scottish Medicines Consortium
High Cost Medicine
High Cost Drug Approval System
Traffic Light Status Information
Clinicians are free to prescribe these drugs. They are regarded as cost effective choices which have evidence to support their use.
These drugs should only be prescribed in the scenarios described in the formulary after preferred drug(s) have been ruled out.
Non-formulary choice - the vast majority of a patientís needs should be met by use of formulary preferred choices and second line options when appropriate. Therefore the Clinician should only use a non-formulary drug if this can be justified and it is recommended that all such instances are recorded in the patient record.
Specialist Advised Ė
Specialists may simply advise a patientís GP to initiate these drugs themselves after they have made an initial assessment.
Note: SA drugs can be initiated by the specialist according to patient need and also depending on local commissioning arrangements which may mandate, through the contract between provider and commissioner, that all SA drugs are treated as SI.
Specialist Initiation -
These drugs must be initiated, i.e. the first dose prescribed, by the specialist and then may be continued when appropriate by the patientís GP following communication from the specialist.
Shared Care -
Responsibility for prescribing may be transferred from secondary to primary care with the agreement of an individual GP and when agreed shared care arrangements have been established. The specialist MUST stabilize the patient before asking for care to be transferred.
Only specialists should initiate these drugs. Prescribing should be transferred to GPs according to an Shared Care Agreement [SCA]
Specialist Only -
These drugs are deemed to be not appropriate for prescribing by GPs.
Specialists should not ask GPs to prescribe these drugs.
Black Listed Medicines - These drugs are considered inappropriate for prescribing in Coventry & Warwickshire as they are either not cost-effective or of proven benefit or both.
Not Recommended - These are drugs that are not recommended for use.
Discontinued medicines - These are drugs that are no longer available to prescribe