Metabolic Disorders

Question 1

Acute porphyria’s

Answer 1

Acute porphyria’s are a group of disorders affecting the synthesis of HAEM, it is hereditary (inherited).
• Certain drugs can induce an acute crisis, haem arginate can be given for haem replacement in moderate, severe or unremitting acute porphyria crises.

Question 2

• Unsafe drug groups:

Answer 2

o Anabolic steroids
o Antidepressants, MAOIs
o Antidepressants, Tricyclic and related
o Barbiturates (includes primidone and thiopental)
o Contraceptives, hormonal (for detailed advice contact UKPMIS or a porphyria specialist)
o Hormone replacement therapy
o Imidazole antifungals (applies to oral + IV. Topical are safe due to low systemic exposure)
o Non-nucleoside reverse transcriptase inhibitors
o Progestogens
o Protease inhibitors
o Sulfonamides (includes co-trimoxazole and sulfasalazine)
o Sulfonylureas (glipizide and glimepiride are thought to be safe)
o Taxanes
o Triazole antifungals (applies to oral + IV. Topical are safe due to low systemic exposure)

Question 3

• Patients should also avoid

Answer 3

• alcohol, amiodarone, clindamycin, cocaine, erythromycin, isoniazid, gliclazide, nitrofurantoin, phenytoin, rifampicin, spironolactone, trimethoprim + valproate.