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Aminothiol — cystine-depleting

Mercaptamine (cysteamine)

Brand names: Cystagon, Cystadrops, Procysbi

Adult dose

Dose: Per BNF/SmPC; titrated to leucocyte cystine level
Route: PO/Topical (eye)
Frequency: QDS or BD MR

Clinical pearls

  • Nephropathic cystinosis; specialist metabolic
  • Eye drops for corneal cystine deposits

Contraindications

  • Hypersensitivity to penicillamine

Side effects

  • GI upset
  • Halitosis (sulphurous body odour)
  • Skin lesions / EDS-like syndrome (rare)
  • Headache

Interactions

  • Bicarbonate-containing antacids (reduced absorption)

Monitoring

  • Leucocyte cystine
  • Renal function
  • Skeletal symptoms

Reference: BNF; SmPC; https://bnf.nice.org.uk/drugs/mercaptamine/. Verify against your local formulary and the latest BNF before prescribing.

Related

Curated clinical cross-links plus same-class fallbacks.