title: Acute intermittent porphyria presenting as acute pancreatitis and posterior reversible encephalopathy syndrome.
abstract: Acute intermittent porphyria (AIP) is an inherited metabolic disease that can affect the autonomic, peripheral and central nervous systems. Pancreatic diseases assocated with AIP is rarely reported. We report here a 60-year-old non-alcoholic male who had typical manifestations of AIP, including abdominal pain, constipation, tachycardia, hypertension, mental disturbances, psychiatric manifestations, seizures, peripheral neuropathy, and excessive excretion of porphyrin precursors in urine. Increases of serum amylase and lipase, as well as mild pancreatic edema on ultrasonography, were noted during the acute attack of AIP, suggesting concomitant acute pancreatitis. In this patient, brain magnetic resonance imaging revealed reversible multifocal cerebral lesions resembling a posterior reversible encephalopathy syndrome (PRES) during the acute attack of AIP. Because the clinical manifestations of acute pancreatitis could be present with an acute attack of AIP, early confirmation of diagnosis is mandatory to effectively manage the attack and avoid inappropriate treatment.
authors.affiliation: Department of Internal Medicine, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.
authors.forename: Feng-Chih
authors.initials: FC
authors.lastname: Shen
authors.forename: Ching-Hua
authors.initials: CH
authors.lastname: Hsieh
authors.forename: Chi-Ren
authors.initials: CR
authors.lastname: Huang
authors.forename: Chun-Chung
authors.initials: CC
authors.lastname: Lui
authors.forename: Wei-Chen
authors.initials: WC
authors.lastname: Tai
authors.forename: Yao-Chung
authors.initials: YC
authors.lastname: Chuang
mesh_terms: D000208:Acute Disease; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000175:diagnosis*; D017118:Porphyria, Acute Intermittent / Q000175:diagnosis*; D013577:Syndrome