Journal of Korean Endocrine Society 2000;15(1):128-132.
Published online January 1, 2001.
A Case of Porphyria with Acute Pancreatitis.
Seok Won Chung, Jeong Hee Han, Young Min Ju, Kwang Hee Yoon, Won Seok Yang, Sung Koo Lee, Sung Kkwan Hong, Eun MEE hAN, Byung Sik Kim, Ki Up Lee
1Department of Internal Medicine, Diagnostic Pathology and General Surgery, Korea.
2Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Abstract
The porphyrias are a group of disorders caused by deficiency in the enzymes of the heme biosynthetic pathway. Patients with porphyria may develop neurovisceral attacks which include severe abdominal pain, neuropsychiatric manifestations and potentially fatal respiratory paralysis. However, these patients may also have abdominal pain not due to porphyria itself, and in such case, careful evaluation is important. We report a case of acute pancreatitis with masquerading acute attack of porphyria, which made us difficult to make a correct diagnosis. A 29-year-old female, previously diagnosed to have hepatic porphyria, presented with acute abdominal pain, back pain and leg pain for 3 days. Serum amylase was normal and 24-hour urine -ALA and PBG showed increased levels. After intravenous infusion of glucose, symptoms were improved. From the 10th day of admission, she complained severe abdominal pain, and was found to have severe metabolic acidosis, shock, and signs of peritonitis on the 12th day of admission. Emergency exploration revealed edematous pancreas. Amylase and lipase levels in serum and ascites were found to be markedly elevated. After conservative management, her general condition gradually improved and serum amylase and lipase levels were normalized.
Key Words: Porphyria, Acute pancreatitis, Acute abdomen


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