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9.8 Metabolic disorders

9.8 METABOLIC DISORDERS

9.8.1  Drugs used in metabolic disorders

Carnitine deficiency
RED           L-Carnitine paediatric solution 30% (3gram/10ml) – 20ml bottle
RED           L-Carnitine injection 1gram/5ml
 
Nephropathic cystinosis
RED          Mercaptamine (Cystagon®) capsules 50mg, 150mg
                  Please prescribe by brand to avoid confusion with similar sounding names.
RED          Mercaptamine (Cysteamine) eye drops
 
This is a "special" for use in ophthalmic involvement of nephropathic cystinosis, available
on named patient basis only.
 

Metabolic disorders
RED          Sodium Bicarbonate 420mg/5ml (1mmol/1ml) sugar free oral solution 
                  prescribe as SodiBic® (Arjun) 
RED          Sodium Benzoate 500mg/5ml oral solution
RED          Joulies Solution (oral solution - unlicensed)
 
Other drugs
AMBER     L-Arginine 10% infusion 200ml
                  (may be given orally, see BNF-C for further information on administration)
RED          Sodium phenylbutyrate 500mg


9.8.2 Acute porphyrias

For use as haem replacement in moderate, severe or unremitting acute porphyria crises.

RED           Haem arginate (Normosang®) injection 25mg/ml