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題 名 | Systemic Amyloidosis Manifesting as a Rare Cause of Hepatic Failure=全身性類澱粉沉積症造成肝衰竭 |
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作 者 | 洪宏緒; 黃德豐; 曾成槐; 蘇正熙; 蘇東平; 陳虹潔; 蔡世豪; 林漢傑; 吳肇卿; 李壽東; 蘇建維; | 書刊名 | Journal of the Chinese Medical Association |
卷 期 | 73:3 2010.03[民99.03] |
頁 次 | 頁161-165+CA27 |
分類號 | 415.53 |
關鍵詞 | 類澱粉沉積症; 肝衰竭; 黃膽; 血小板減少; Amyloidosis; Hepatic failure; Jaundice; Thrombocytopenia; |
語 文 | 英文(English) |
英文摘要 | In 1854, the term “amyloid” was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and jaundice. Disseminated intravascular coagulation profile was positive. Abdominal sonography showed ascites, and abdominal computed tomography disclosed heterogeneous enhancement of the liver, with focal low attenuation regions and splenomegaly with poor contrast enhancement. Liver decompensation was highly suspected. Diagnostic laparoscopy with liver biopsy and colonoscopic biopsy from the rectum were subsequently performed. Typical apple-green birefringence was demonstrated on polarized light microscopy by Congo red staining. Systemic amyloidosis was diagnosed and colchicine prescribed. However, liver function deteriorated and intermittent gastrointestinal bleeding was found during the patient’s hospitalization. The patient died due to uncorrectable coagulopathy and massive gastrointestinal bleeding. The final diagnosis was idiopathic amyloidosis with hepatic failure. Although amyloidosis rarely presents with hepatic failure, it should be considered in patients with signs of liver decompensation. Clinicians should be aware of this rare but potentially lethal presentation and arrange appropriate treatment promptly. |
本系統中英文摘要資訊取自各篇刊載內容。