Acute Intermittent Porphyria: A Rare Cause of Postoperative Abdominal Pain and Hyponatremia

急性间歇性卟啉症:术后腹痛和低钠血症的罕见病因

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Abstract

Acute hepatic porphyrias (AHP) are a group of rare metabolic disorders that develop from heme biosynthesis enzyme deficiencies. They often result in the accumulation of neurotoxic intermediates, such as aminolevulinic acid (ALA) and porphobilinogen (PBG). The disorders can be triggered by specific medications, prolonged fasting, or physiological stress. We present a case of a 34-year-old female patient with no significant medical conditions or comorbidities, who started experiencing severe abdominal pain, tachycardia, and hyponatremia after undergoing laparoscopic cholecystectomy under general anesthesia. The diagnosis of acute intermittent porphyria (AIP), a subtype of AHP, became evident with elevated urinary ALA and PBG upon laboratory testing. The patient received intravenous hemin treatment along with supportive care, which resulted in rapid resolution of symptoms. The case demonstrates why healthcare providers should include porphyria in their diagnostic considerations when patients present with unexplained postoperative abdominal pain and hyponatremia, especially when anesthetic triggers are involved. Early diagnosis through targeted therapy halted neurological deterioration and reduced the duration of hospital stay. Heightened awareness among anesthesiologists and perioperative clinicians would lead to earlier detection and improved outcomes for patients with similar conditions.

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