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Sport-related concussion — MRCEM SBA MCQ

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HardConcussionSport-related concussionMRCEM SBA

A patient with known acute intermittent porphyria has severe persistent abdominal pain and vomiting despite supportive treatment. Urine porphobilinogen is raised, sodium is 121 mmol/L and new proximal weakness is developing. Which disease-specific escalation is most appropriate?

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Correct answer: A — Contact NAPS urgently and arrange intravenous haem arginate while supporting complications

The best answer is “Contact NAPS urgently and arrange intravenous haem arginate while supporting complications”. Progressive neuropathy and hyponatraemia mark a severe acute porphyria attack and are indications to consider haem arginate promptly with National Acute Porphyria Service advice. Treatment should not await full porphyrin speciation once an acute attack is biochemically supported. Potentially porphyrinogenic medicines are stopped, safe analgesia and antiemesis are provided, fluid balance and sodium are corrected cautiously, and respiratory function is monitored because motor neuropathy can progress to ventilatory failure. Carbohydrate may help mild attacks but is not an adequate substitute for haem arginate in this severe presentation.

Reference: British Porphyria Association: acute porphyria emergency treatment: https://porphyria.org.uk/acute-porphyrias/