• Cheng Jiao, Wei Liu, Jin-Hui Chen, Jun Guo, and Yi-Ming Qiao.
• Department of General Surgery, Bethune International Peace Hospital, Shijiazhuang, Hebei 050000, China.
• Medicine (Baltimore). 2023 Nov 17; 102 (46): e36036e36036.

RationaleAcute intermittent porphyria (AIP) is a rare genetic disorder that affects porphyrin metabolism in the blood. The disease causes defects in specific enzymes in the body, which in turn leads to the accumulation of porphyrin metabolites. Patients may experience abdominal pain, neurological symptoms, muscle pain, and nausea, but it does not directly cause pancreatitis.Patient ConcernsThe patient is a young woman, 23 years old, who was admitted to our hospital with intermittent abdominal pain for 2 days, the pain was not fixed, episodic, with no obvious trigger, and 1 day before admission, the patient started to experience nausea and vomiting, with gastric contents as the vomitus, and similar symptoms had occurred many times in the past. Blood amylase 600 U/L, blood sodium 120.6 mmol/L, blood routine, and coagulation function results were normal; abdominal CT showed pancreatic swelling with unclear surrounding fat interstitial, acute pancreatitis was considered. The patient's urine was dark red, and the results of the qualitative urine porphyrin test were positive.DiagnosesAIP complicated with acute pancreatitis.InterventionRelief of symptoms, control of pain, correction of electrolyte disturbances, and high-carbohydrate therapy.OutcomesThe patient was discharged with complete symptomatic relief after 10 days of high-carbohydrate therapy.LessonsAIP complicated with acute pancreatitis is very rare. Treatment of AIPs aims to control acute attacks and prevent potential triggers.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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