- Porphyria
- Group of inherited disorders of haem metabolism
- Classification
- Acute
- More important for anaesthetists
- All result in an increase in 5-aminolaevulinic acid (ALA)
- Urinary porphobilinogen levels are used to diagnose an acute crisis
- Acute intermittent porphyria
- Variagate porphyria
- Hereditary coproporphyria
- ALA dehydratase deficiency
- Non-acute
- Do not deteriorate into neurovisceral crises
- Porphyria cutanea tarda
- Congenital erythropoietic porphyria
- Ertythropoietic protoporphyria
- Triggers
- Fasting
- Smoking
- Alcohol
- Drugs
- Dehydration
- Infection
- Stress
- Symptoms
- Abdominal pain
- Cardiovascular
- Weakness
- Psychiatric disturbance
- Seizures
- May be due to hyponatraemia
- Autonomic disturbances
- Drugs
- If in doubt then CHECK
- Safe
Link:www.drugs-porphyria.org
- Propofol
- Isoflurane, desflurane, nitrous oxide
- Bupivicaine
- Suxamethonium
- All NDMRs
- Neostigmine
- Fentanyl, alfentanil, remifentanil, morphine, tramadol, ibuprofen, aspirin, diamorphine
- Penicillins, Vancomycin
- Adrenaline, NA, atropine, glycopyrrolate, β-blockers
- Syntocinon, carboprost, tranexamic acid
- Unsafe
- Sevoflurane
- Thiopentone, ketamine
- Diclofenac
- Rifampicin
- Erythromycin
- Ephedrine
- Unsure
- Levobupivicaine, ropivicaine
- Metaraminol
- Dexamethasone
- Clopidogrel
- Hydrocortisone
- Treatment
- Remove precipitants
- Treat infection
- Carbohydrate (10% dextrose with 0.9% saline to avoid hyponatraemia)
- IV haem arginate (3mg/kg over 30 mins)
- β-blockers for tachycardia
- Haloperidol safe for ψ disturbances
- Seizures treated with benzodiazepines and magnesium
- GTN for hypertension
- CEACCP
Link:ceaccp.oxfordjournals.org/content/12/3/128.full.pdf