| Acute intermittent porphyria presenting as acute pancreatitis and posterior reversible encephalopathy syndrome. | |
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MedLine Citation:
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PMID: 18975524 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Feng-Chih Shen; Ching-Hua Hsieh; Chi-Ren Huang; Chun-Chung Lui; Wei-Chen Tai; Yao-Chung Chuang |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta neurologica Taiwanica Volume: 17 ISSN: 1028-768X ISO Abbreviation: Acta Neurol Taiwan Publication Date: 2008 Sep |
Date Detail:
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Created Date: 2008-11-03 Completed Date: 2008-12-09 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9815355 Medline TA: Acta Neurol Taiwan Country: China (Republic : 1949- ) |
Other Details:
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Languages: eng Pagination: 177-83 Citation Subset: IM |
Affiliation:
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Department of Internal Medicine, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acute Disease Brain / pathology Brain Diseases / diagnosis*, pathology Humans Magnetic Resonance Imaging Male Middle Aged Pancreatitis / diagnosis* Porphyria, Acute Intermittent / diagnosis* Syndrome |
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