From ACP Gastroenterology Monthly, selected recommendations regarding Acute Hepatic Porphyria from the American Gastroenterological Association


  • Consider screening for AHP in women ages 15 to 50 years with unexplained, recurrent severe abdominal pain of unknown etiology despite a workup.
  • Diagnosis of AHP should be made by biochemical testing, measuring δ aminolevulinic acid, porphobilinogen, and creatinine on a random urine sample.
  • In patients with positive biochemical testing, genetic testing should be performed to confirm the diagnosis
  • In patients with four or more attacks per year, consider prophylactic heme therapy or givosiran, administered in an outpatient setting.
  • Liver transplantation should be limited to patients with intractable symptoms and who are refractory to pharmacotherapy.
  • Patients should undergo annual monitoring for liver disease.
  • Patients, regardless of the severity of symptoms, should undergo surveillance for hepatocellular carcinoma, beginning at age 50 years, with liver ultrasound every six months.
  • Patients with AHP on treatment should undergo surveillance for chronic kidney disease annually.

Pearl – The triad of seizures, abdominal pain, and hyponatremia in a young woman is highly suggestive of acute porphyria.