solution ( 30 %) not licensed in children over 12 years. Not
licensed for use in organic acidaemias.
lCAUTIONSDiabetes mellitus
lSIDE-EFFECTS
▶Rare or very rareAbdominal cramps.diarrhoea.nausea.
skin odour abnormal.vomiting
SIDE-EFFECTS, FURTHER INFORMATIONSide-effects may be
dose-related—monitor tolerance duringfirst week and
after any dose increase.
lPREGNANCYAppropriate to use; no evidence of
teratogenicity inanimalstudies.
lRENAL IMPAIRMENTAccumulation of metabolites may
occur with chronic oral administration in severe
impairment.
lMONITORING REQUIREMENTS
▶Monitoring of free and acyl carnitine in blood and urine
recommended.
lDIRECTIONS FOR ADMINISTRATIONForintravenous
infusion, dilute injection with Sodium Chloride 0. 9 %or
Glucose 5 %or 10 %.
lPRESCRIBING AND DISPENSING INFORMATION
▶When used for Organic acidaemiasLevocarnitine is used in the
treatment of some organic acidaemias; however, use in
fatty acid oxidation is controversial.
lPATIENT AND CARER ADVICE
Medicines for Children leaflet: Carnitine for metabolic
disorderswww.medicinesforchildren.org.uk/carnitine-
metabolic-disorders- 0
lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug. Forms available from special-order
manufacturers include: capsule
Solution for injection
▶Carnitor(Logixx Pharma Solutions Ltd)
L-Carnitine 200 mg per 1 mlCarnitor 1 g/ 5 ml solution for injection
ampoules| 5 ampouleP£ 59. 50
Oral solution
▶Levocarnitine (Non-proprietary)
L-Carnitine 300 mg per 1 mlLevocarnitine 1. 5 g/ 5 ml ( 30 %) oral
solution paediatric| 20 mlP£ 71. 40 DT = £ 71. 40 | 40 mlP
£ 69. 80
▶Carnitor(Logixx Pharma Solutions Ltd)
L-Carnitine 100 mg per 1 mlCarnitor oral single dose 1 g solution
sugar-free| 10 unit doseP£ 35. 00
Chewable tablet
▶Carnitor(Logixx Pharma Solutions Ltd)
L-Carnitine 1 gramCarnitor 1 g chewable tablets| 10 tabletP
£ 35. 00
3.3 Cystinosis
3.3aNephropathic cystinosis
AMINO ACIDS AND DERIVATIVES
Mercaptamine 21-Nov-2017
(Cysteamine)
lINDICATIONS AND DOSE
Nephropathic cystinosis (specialist use only)
▶BY MOUTH
▶Neonate:Initially one-sixth to one-quarter of the
expected maintenance dose, increased gradually over
4 – 6 weeks to avoid intolerance, maintenance 1. 3 g/m^2
daily in 4 divided doses.▶Child 1 month–11 years (body-weight up to 50 kg):Initially
one-sixth to one-quarter of the expected maintenancedose, increased gradually over 4 – 6 weeks to avoid
intolerance, maintenance 1. 3 g/m2
daily in 4 divided
doses
▶Child 12–17 years (body-weight up to 50 kg):Initially one-
sixth to one-quarter of the expected maintenance dose,
increased gradually over 4 – 6 weeks to avoid
intolerance, maintenance 1. 3 g/m^2 daily in 4 divided
doses
▶Child 12–17 years (body-weight 50 kg and above):Initially
one-sixth to one-quarter of the expected maintenance
dose, increased gradually over 4 – 6 weeks to avoid
intolerance, maintenance 2 g daily in 4 divided doses
Corneal cystine crystal deposits in patients with cystinosis
(specialist use only)
▶TO THE EYE
▶Child 2–17 years:Apply 1 drop 4 times a day, to be
applied to both eyes (minimum 4 hours between
doses); dose may be reduced according to response
(minimum daily dose 1 drop in each eye)
DOSE EQUIVALENCE AND CONVERSION
▶With oral use
▶ 1. 3 g/m^2 is approximately equivalent to 50 mg/kg.IMPORTANT SAFETY INFORMATION
SAFE PRACTICE
Mercaptamine has been confused with mercaptopurine;
care must be taken to ensure the correct drug is
prescribed and dispensed.lCAUTIONS
▶When used by eyeContact lens wearers
▶With oral useDose of phosphate supplement may need to
be adjusted if transferring from phosphocysteamine to
mercaptamine
lSIDE-EFFECTS
▶Common or very common
▶When used by eyeDry eye.eye discomfort.eye erythema.
excessive tearing.vision blurred
▶With oral useAppetite decreased.asthenia.breath odour.
diarrhoea.drowsiness.encephalopathy.fever.
gastroenteritis.gastrointestinal discomfort.headache.
nausea.skin reactions.vomiting
▶Uncommon
▶With oral useCompression fracture.gastrointestinal ulcer.
hair colour changes.hallucination.joint hyperextension.
leg pain.leucopenia.musculoskeletal disorders.
nephrotic syndrome.nervousness.osteopenia.seizure
▶Frequency not known
▶With oral useDepression.intracranial pressure increased.
papilloedema
lALLERGY AND CROSS-SENSITIVITYContra-indicated if
history of hypersensitivity to penicillamine.
lPREGNANCY
▶With oral useManufacturer advises avoid—teratogenic and
toxic inanimalstudies.
lBREAST FEEDING
▶With oral useManufacturer advises avoid—no information
available.
lMONITORING REQUIREMENTS
▶With oral useLeucocyte-cystine concentration and
haematological monitoring required—consult product
literature.
▶All patients receiving mercaptamine should be registered
(contact local specialist centre for details).
lDIRECTIONS FOR ADMINISTRATION
▶With oral useFor children under 6 years at risk of
aspiration, capsules can be opened and contents sprinkled
on food (at a temperature suitable for eating); avoid
adding to acidic drinks (e.g. orange juice).BNFC 2018 – 2019 Nephropathic cystinosis 605
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