以肝肾损害为主要表现的原发性淀粉样变性一例  

A case of primary amyloidosis with main manifestations of liver and kidney damage

作　　者：邹雪 汤绍辉 Zou Xue;Tang Shaohui(The First Affiliated Hospital of Ji'nan University,Guangzhou 510630,China)

机构地区：[1]暨南大学附属第一医院广州华侨医院,广州510630

出　　处：《新医学》2018年第12期924-927,共4页Journal of New Medicine

摘　　要：1例以纳差、腹胀,血碱性磷酸酶(ALP)及γ-谷氨酰转移酶(GGT)升高,肝脏明显肿大,合并存在大量蛋白尿、低白蛋白血症、高脂血症为表现的患者,肠道病变组织刚果红染色提示为淀粉样变性,排除其他继发性因素最后诊断为原发性淀粉样变性。确诊后予沙利度胺为主方案化学治疗,患者纳差较前改善。该例提示对于不明原因的GGT及ALP明显增高而其他肝功受损较轻的肝肿大患者,在排除自身免疫性疾病、酒精性肝病、肝脏肿瘤等其他因素后,应考虑淀粉样变性的可能。淀粉样变性临床表现缺乏特异性,一旦高度怀疑应尽快行病变活组织检查以明确诊断。In this paper,we reported one case of primary amyloidosis presenting with poor appetite,abdominal distension,elevated levels of serum alkaline phosphatase(ALP)and gamma?glutamyl transpeptide(GGT),hepatomegaly,massive proteinuria,hypoalbuminemia and hyperlipidemia.Congo red staining prompted the diagnosis of amyloidosis.The diagnosis of primary amyloidosis was confirmed after alternative secondary factors were excluded.The thalidomide was administered as the main chemotherapy regime.The symptoms of poor appetite were mitigated.This case hints that for hepatomegaly patients with significant elevation of GGT and ALP levels and slight liver function injury,the possibility of primary amyloidosis should be considered after the autoimmune diseases,alcoholic liver diseases and liver tumors are excluded.Primary amyloidosis lacks of specific clinical manifestations.Pathological biopsy is recommended to validate the diagnosis in suspected cases.

关 键 词：腹胀 淀粉样变性 刚果红染色 

分 类 号：R597.2[医药卫生—内科学]