Background: We reported a case with acute intermittent porphyria who demonstrated acute pancreatitis and liver dysfunction.
Case Report: A 44-year-old white male with type II diabetes mellitus presented with colicky right upper quadrant and epigastric pain, nausea and vomiting during last two days. Two weeks prior to these complaints, he had
had an upper respiratory tract infection and used some drugs including terfenadine and co-trimoxazole. Physical examination revealed that he was mentally confused and he had jaundice. Abdominal examination revealed
diffuse tenderness. Clinical, laboratory findings, liver histology, pancreas cytology and CT scan showed the presence of acute pancreatitis, mild liver failure and pleural effusion. His urine color was pink-purple and got
darker in urine collection bag. Porphyrin studies revealed markedly elevated urine porphobilinogen, aminolevulinic acid, coproporphyrin, and uroporphyrin which suggested the diagnosis of acute intermittent porphyria. All
these studies established the diagnosis of acute intermittent porphyria causing acute pancreatitis, mild hepatic failure, pleural effusion and neurological signs. The patient was kept at rest and treated with total parenteral nutrition without allowing oral food intake. Large amount of glucose was given by central venous route. A broad spectrum antibiotic was administered. Liver function, as well as clinical and laboratory findings of pancreatitis improved after two weeks of hospitalization. He has now been on follow-up for 6 months and he has not had any complaints.
Conclusion: When a clinician cannot find the etiology of acute pancreatitis and/or liver failure in patients, especially with neurological disorders, acute porphyria must be included in differential diagnosis.

Keywords: acute pancreatitis, Liver failure, porphyria, Pleural Effusion