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A-Z of Drugs : A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

A-Z of Drugs

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Search Results : Urea cycle disorders - EMERGENCY TREATMENT (Sodium phenylbutyrate - Urea cycle disorders - EMERGENCY TREATMENT)

Records returned : 5 (on 17 Jun 2026 at 07:30:56). Return to search results for ' Urea cycle disorders - EMERGENCY TREATMENT '.

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Icon Key

NFD1

Non formulary

NFD2

Not assessed for formulary status. Apply to APC / DTC before use.

Controlled drug. Prescribing and/or storage requirements may apply

Restrictions apply

Safety Alert

Unlicensed - no UK marketing license in place

Off Label

Not licensed for this indication.

NHSE

Treatment commissioned by NHS England

CDF

Cancer Drugs Fund

ICB

Treatment commissioned by the ICB

BlueTeq form to be completed

NICE

Link to the NICE website

Low carbon device

SPC

View the treatment summary for {padindicationname} on https://www.medicines.org.uk

Status Key

Red

Specialist ONLY drugs - treatment initiated and continued by specialist clinicians

Amber

Prescribing initiated and stabilised by specialist but has potential to transfer to primary care under a formal shared care agreement

Green

GPs (or non-medical prescribers in primary care) are able to take full responsibility for initiation and continuation of prescribing.

Green (see narrative)

GPs (or non-medical prescribers in primary care) are able to take full responsibility for initiation and continuation of prescribing. Please refer to the narrative on the Drug Profile page where additional information may be conveyed as to the place in therapy or restrictions for use that have been locally agreed.

Non Formulary

Not recommended for use in any health setting across the Surrey Heartlands Integrated Care System

Blue

Prescribing initiated and stabilised by specialist but has potential to transfer to primary care WITHOUT a formal shared care agreement. Please note that in some circumstances a specialist may recommend that prescribing can be started in primary care.

See narrative

See narrative - may be applied in instances where a traffic light status has not yet been agreed but an entry to the formulary has been made to clarify interim arrangements.

Drug

Indication

Status

Restrictions/Comments

Formulary Status

Links

1. Sodium phenylbutyrate

Drug:

Sodium phenylbutyrate

Indication :

Urea cycle disorders - EMERGENCY TREATMENT

Status :

Red

Formulations :

Granules
Oral solution
Tablets

Restrictions / Comments:

Important

For use in the emergency treatment of urea cycle disorders in patients on acute admission.

For routine management of metabolic disorders it is for use by NHSE specialised commissioned services only. Not commissioned from any acute Trust in Surrey Heartlands.

Formulary Status :

Primary Care

ASPH

RSFT

SASH

SABP

Links :

SPC

2. L-arginine

Drug:

L-arginine

Indication :

Urea cycle disorders - EMERGENCY TREATMENT

Status :

Red

Restrictions / Comments:

Important

For use in the emergency treatment of urea cycle disorders in patients on acute admission.

For routine management of metabolic disorders it is for use by NHSE specialised commissioned services only. Not commissioned from any acute Trust in Surrey Heartlands.

Formulary Status :

Primary Care

ASPH

RSFT

SASH

SABP

Links :

SPC

3. Levocarnitine

Drug:

Levocarnitine

Indication :

Urea cycle disorders - EMERGENCY TREATMENT

Status :

Red

Formulations :

Capsules

Restrictions / Comments:

Important

For use in the emergency treatment of urea cycle disorders in patients on acute admission.

For routine management of metabolic disorders it is for use by NHSE specialised commissioned services only. Not commissioned from any acute Trust in Surrey Heartlands.

Formulary Status :

Primary Care

ASPH

RSFT

SASH

SABP

Links :

SPC

4. Levocarnitine

Drug:

Levocarnitine

Indication :

Urea cycle disorders - EMERGENCY TREATMENT

Status :

Red

Formulations :

Oral solution

Restrictions / Comments:

Important

For use in the emergency treatment of urea cycle disorders in patients on acute admission.

For routine management of metabolic disorders it is for use by NHSE specialised commissioned services only. Not commissioned from any acute Trust in Surrey Heartlands.

30% paediatric solution (1.5 gram in 5ml)

Formulary Status :

Primary Care

ASPH

RSFT

SASH

SABP

Links :

SPC

5. Sodium benzoate

Drug:

Sodium benzoate

Indication :

Urea cycle disorders - EMERGENCY TREATMENT

Status :

Red

Restrictions / Comments:

Important

For use in the emergency treatment of urea cycle disorders in patients on acute admission.

For routine management of metabolic disorders it is for use by NHSE specialised commissioned services only. Not commissioned from any acute Trust in Surrey Heartlands.

Formulary Status :

Primary Care

ASPH

RSFT

SASH

SABP

Links :

SPC