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9.8 Metabolic disorders

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)
 
          Sapropterin                                                                                                                          NHS England Commissioned
          NICE TA729  Recommended as an option for treating hyperphenylalaninaemia that responds to sapropterin (response as 
                               defined in the summary of product characteristics) in people with phenylketonuria (PKU), only if they are:
                                    >  under 18 and a dose of 10 mg/kg is used, only using a higher dose if target blood phenylalanine levels 
                                        cannot be achieved at 10 mg/kg
                                    >  aged 18 to 21 inclusive, continuing the dose they were having before turning 18 or at a maximum dose of 
                                        10 mg/kg
                                    >  pregnant(from a positive pregnancy test until birth).
                               Sapropterin is recommended only if the company provides it according to the commercial arrangement.
          RED    Sapropterin 100mg soluble tablets [Kuvan®]                                                       
 
     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