摘要
目的通过对肝淀粉样变性患者的临床资料总结，提高对以肝脏为突出表现的淀粉样变性疾病的认识。方法回顾性分析6例肝淀粉样变性患者的临床资料、实验室指标、检查结果、治疗及预后。结果6例均有消瘦、乏力、肝大、尿蛋白，4例脾大，3例心肌肥厚，6例碱性磷酸酶及γ谷氨酰转肽酶升高且在正常值1.5倍以上，3例行免疫球蛋白电泳检查，均有异常，3例行肝脏穿刺，3例行肾脏穿刺，均为受累部位活检，且结果均阳性，无穿刺出血或死亡病例。系统的脏器评估例数较少，观察预后的指标考虑有胆红素、碱性磷酸酶大于500 U/L、B型钠尿肽、骨髓浆细胞、β2微球蛋白以及其他脏器功能。结论肝淀粉样变性患者均有不同程度肝肿大，肝功能损害轻微，以γ谷氨酰转肽酶、碱性磷酸酶升高为主，常伴肾、脾、心脏等多个脏器病变及高脂血症、血（尿）免疫轻链异常。确诊依靠病理。
ObjectiveTo summarize the clinical characteristics of hepatic amyloidosis disease by analyzing the data of this disease.MethodsSix cases of hepatic amyloidosis out of 32 cases of amyloidosis were selected and the patients clinical characteristics, laboratory tests, treatments and prognosis were analyzed retrospectively.ResultsAll the patients presented weight loss, fatigue, hepatomegaly as well as proteinuria. Four patients had splenomegaly and 3 patients had ventricular hypertrophy. The levels of alkaline phosphatase （ALP） and γ-glutamyl transpeptidase （GGT） in all patients elevated 1.5 times more than the upper limit of normal. Three of them performed immunoglobulin electrophoresis, and the results of which were abnormal. All of them did involved organ biopsy, including liver （3 cases） and kidney（3 cases）. All of them demonstrated amyloid deposition. None of them suffered bleeding and death during the biopsy. Organ pathology assessing was not enough. Prognostic markers included hyperbilirubinemia, ALP more than 500 U/L, B type natriuretic peptides, plasma cells clone, β2 microglobulin and other organ function.ConclusionsThe patients with hepatic amyloidosis disease present hepatomegaly with different sizes and mildly abnormal liver test （mainly with elevated GGT and ALP）. Most cases have multiple organs pathology including kidney, spleen and heart. Laboratory test often shows hyperlipidemia and abnormal serum free light chain. It is always confirmed by pathology.
作者
姬蒙
夏兴洲
Ji Meng;Xia Xingzhou.(Department of Gastroenterology, the Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, Chin)
出处
《中国医师进修杂志》
2018年第2期109-112,共4页
Chinese Journal of Postgraduates of Medicine
关键词
淀粉样变性
疾病特征
回顾性研究
Amyloidosis
Disease attributes
Retrospective studies
