Acute Porphyria

Porphyria is an autosomal dominant disorder, symptoms beginning after puberty, may be triggered by drugs (barbiturates, sulphonamides, oestrogen, chloroquine), alcohol, menstruation, infection or fasting. Abdominal pain is hallmark of porphyria. Other symptoms include red colour of urine, limb pain, psychiatric symptoms, tachycardia, hypertension, and hyponatraemia, peripheral neuropathy. Markedly increased excretion of porphobilinogen is diagnostic of acute porphyria. Treatment consists of hemiarginate 3 mg/kg for 4 days, and plenty of carbohydrates (glucose intravenously 400 g daily or a carbohydrate-rich diet).


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