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Acute Hepatic Porphyria
Declaration of sponsorship Alnylam Pharmaceuticals

Clinical Manifestations

Declaration of sponsorship Alnylam Pharmaceuticals
Read time: 10 mins
Last updated:3rd Mar 2021
Published:3rd Mar 2021
  • Patients may present with various neurovisceral manifestations, such as autonomic neuropathy: abdominal pain, tachycardia, and hypertension; peripheral neuropathy: muscle weakness and paralysis; CNS manifestations: seizures and mental status changes; metabolic: hyponatremia; and red-brownish urine on exposure to light and air.2-9
  • Abdominal pain is recurrent, severe and unremitting, typically requiring sizeable doses of narcotics for pain relief.2,3,14
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Acute Symptoms

  • A review of more than 100 patients with acute intermittent porphyria (AIP) found that the most commonly reported symptoms during acute attacks included abdominal pain, nausea/vomiting, weakness, and constipation (see figure).1
  •  the most commonly reported symptoms during acute attacks included abdominal pain, nausea/vomiting, weakness, and constipation (see figure).1
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Constellation of Symptoms

Gastroenterologists should consider a diagnosis of acute hepatic porphyria (AHP) in any patient experiencing recurrent and prolonged episodes of severe, diffuse abdominal pain of unknown etiology with concurrent symptoms shown in the figure below. It's important to increase awareness and suspicion of some key symptoms of AHP:

 

  • Neurovisceral pain: severe abdominal pain which is usually lower but can occur anywhere. Many consider it generalised, and the pain doesn’t particularly radiate into the back, so it is not suggestive of an acute pancreatitis, other patients complain of right, upper quadrant pain, which increases the consideration of gallstones or sludge.1
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