Acute intermittent porphyria presenting as acute pancreatitis and posterior reversible encephalopathy syndrome.
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.
Shen
Feng-Chih
FC
Department of Internal Medicine, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Hsieh
Ching-Hua
CH
Huang
Chi-Ren
CR
Lui
Chun-Chung
CC
Tai
Wei-Chen
WC
Chuang
Yao-Chung
YC
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