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題 名 | Acute Intermittent Porphyria Presenting as Acute Pancreatitis and Posterior Reversible Encephalopathy Syndrome=急性間歇性紫質症以急性胰臟炎及後側腦部可逆性腦病變症候群表現 |
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作 者 | 沈峰志; 謝青華; 黃致任; 呂鎮中; 戴維震; 莊曜聰; | 書刊名 | Acta Neurologica Taiwanica |
卷 期 | 17:3 2008.09[民97.09] |
頁 次 | 頁177-183 |
分類號 | 415.9 |
關鍵詞 | 急性間歇性紫質症; 胰臟炎; 腦病變; Acute intermittent porphyria; Acute pancreatitis; Encephalopathy; |
語 文 | 英文(English) |
英文摘要 | 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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