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Hypertensive emergency — USMLE Step 2 CK MCQ

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HardInternal MedicineHypertensive emergencyUSMLE Step 2 CK

A 27-year-old woman taking phenobarbital develops severe diffuse abdominal pain, constipation, tachycardia, hypertension, confusion and sodium 121 mEq/L. CT is normal, and a spot urine porphobilinogen concentration is markedly elevated. Which specific treatment is most appropriate?

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Correct answer: E — Stop the trigger and administer intravenous hemin

This is a severe acute hepatic porphyria attack: neurovisceral abdominal pain, autonomic activation, hyponatremia and neuropsychiatric features with markedly increased urinary porphobilinogen. The porphyrinogenic drug should be stopped and intravenous hemin given; hemin suppresses hepatic ALAS1 and precursor production. Glucose alone is reserved for mild attacks or as a bridge while hemin is obtained. Surgery does not treat the metabolic disorder, and unsafe porphyrinogenic or paralysis-producing drugs can worsen morbidity.

Reference: American Porphyria Foundation: Emergency room guidelines for acute porphyrias. https://porphyriafoundation.org/for-healthcare-professionals/emergency-room-guidelines-for-acute-porphyrias/