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Published on: September 18, 2013
ED presentation of acute porphyria
Yueh-Ping Liu1, Wan-Ching Lien, Cheng-Chung Fang
1Department of Emergency Medicine, National Taiwan University Hospital, Taipei, Taiwan.
Acute porphyria is often overlooked in emergency departments, leading to delayed diagnosis and unnecessary surgeries for patients with severe abdominal pain. Consider acute porphyria in patients with recurrent, unexplained abdominal pain requiring narcotic analgesia.
Area of Science:
- Internal Medicine
- Medical Diagnostics
- Emergency Medicine
Background:
- Abdominal pain is a frequent emergency department (ED) complaint.
- Acute porphyria presents with nonspecific symptoms, often confusing ED physicians.
- Delayed diagnosis of acute porphyria can lead to unnecessary surgical interventions.
Purpose of the Study:
- To evaluate the diagnostic challenges of acute porphyria in the ED.
- To identify key indicators for considering acute porphyria in patients with acute abdomen.
Main Methods:
- Retrospective review of 32 patients with acute porphyric attacks at a tertiary hospital ED over 13 years.
- Analysis of clinical presentation, diagnostic process, and treatment of patients newly diagnosed with acute porphyria in the ED.
Main Results:
- Ten patients were diagnosed with acute porphyria for the first time in the ED.
- Patients presented with abdominal pain but lacked typical fever, dermatologic, or neurologic symptoms.
- Most patients required multiple medical visits and narcotic analgesia before diagnosis; epigastric tenderness was common.
- Standard laboratory and imaging studies were unhelpful, except for the Watson-Schwartz test.
Conclusions:
- Acute porphyria is an underrecognized cause of acute abdomen in the ED.
- Consider acute porphyria in post-pubertal patients with recurrent, severe abdominal pain without clear cause, especially those requiring narcotic pain relief.
- Early consideration of acute porphyria can prevent diagnostic delays and inappropriate surgeries.
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