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clinical management of gastrointestinal amyloidosis胃肠道淀粉样变的临床管理.pdf

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clinical management of gastrointestinal amyloidosis胃肠道淀粉样变的临床管理

Open Journal of Gastroenterology, 2012, 2, 155-162 OJGas /10.4236/ojgas.2012.24030 Published Online November 2012 (http://www.SciRP.org/journal/ojgas/) Clinical management of gastrointestinal amyloidosis * Hajime Isomoto , Yasuhiro Kamo, Chun Chuan Chen, Kazuhiko Nakao Department of Gastroenterology and Hepatology, Nagasaki University Hospital, Nagasaki, Japan * Email: hajimei2002@yahoo.co.jp Received 13 August 2012; revised 15 September 2012; accepted 16 October 2012 ABSTRACT There has been a progress in the understanding of the pathogenesis, clinical features, diagnostic measures and Amyloidosis is characterized by extracellular deposi- therapy of this disorder. Herein, we will discuss clinical tion of abnormal protein, consisting of primary, sec- management of gastrointestinal (GI) amyloidosis. ondary, hemodialysis-related, hereditary, senile and localized type. Primary amyloidosis is associated with 2. PATHOGENESIS monoclonal light chains. Secondary amyloidosis is associated with inflammatory, infectious, and neo- Amyloidosis is defined as an extracellular deposit of plastic diseases. Amyloid deposition in the gastroin- protein fibrils with a β-sheet fibrillar structure and char- testinal tract can manifest the symptoms including acteristic properties typically after staining with Congo diarrhea, steatorrhea, or constipation. For diagnosis,