原发性肝淀粉样变性1例报告

李晨; 陈婧; 刘鸿凌; 朱冰; 臧红; 游绍莉; 辛绍杰

doi:10.3969/j.issn.1001-5256.2013.10.017

原发性肝淀粉样变性1例报告

DOI: 10.3969/j.issn.1001-5256.2013.10.017

解放军第302医院肝衰竭诊疗与研究中心

基金项目:

“十二五”国家传染病科技重大专项(2012ZX10002004-005)；

详细信息

中图分类号: R575.5
计量
- 文章访问数: 1798
- HTML全文浏览量: 30
- PDF下载量: 601
- 被引次数: 0
出版历程
- 出版日期: 2013-10-20

One case of primary hepatic amyloidosis

Liver Failure Treatment and Research Centre, 302 Hospital of PLA

Research funding:

摘要

摘要: <正>淀粉样变性,又称淀粉样物质沉积症,是以淀粉样物质在各种组织和脏器细胞外间隙沉积为特征的一种进行性疾病,主要累及心、肝、肾、脾、胃肠、肌肉、皮肤、神经系统等部位,当发生于肝脏时,则称为肝淀粉样变性[1]。根据淀粉样物质中纤维蛋白前体的化学结构种类,淀粉样变性分为6种临床类型,即原发性淀粉样变性、继发性淀粉样变性、透析相关性淀粉样变性、家族性淀粉样变性、老年性淀粉样变性、局限性淀粉样变性[2]。该病整体发病率低,其中原发性淀粉样变
- 肝疾病 /
- 淀粉样变性
- liver diseases /
- amyloidosis

HTML全文

参考文献(14)

[1]LAO MX, WANG JH, CHEN J, et al.Liver amyloidosis with severe cholestasis and corneal Kayser-Fleischer rings:report of one case and literature review[J].Chin J Pract Intern Med, 2012, 32 (9) :721-723. (in Chinese) 劳敏曦, 王锦辉, 陈洁, 等.肝淀粉样变性合并重度淤胆及角膜K-F环1例报告并文献复习[J].中国实用内科杂志, 2012, 32 (9) :721-723.

[2]EBERT EC, NAGAR M.Gastrointestinal manifestations of amyloidosis[J].Am J Gastroenterol, 2008, 103 (3) :776-787.

[3]COHEN AD, COMENZO RL.Systemic light-chain amyloidosis:advances in diagnosis, prognosis, and therapy[J].Hematology Am Soc Hematol Educ Program, 2010, 2010:287-294.

[4]KUMAR S, DISPENZIERI A, KATZMANN JA, et al.Serum immunoglobulin free light-chain measurement in primary amyloidosis:prognostic value and correlations with clinical features[J].Blood, 2010, 116 (24) :5126-5129.

[5]RAJKUMAR SV, GERTZ MA, KYLE RA.Primary systemic amyloidosis with delayed progression to multiple myeloma[J].Cancer, 1998, 82 (8) :1501-1505.

[6]PARK MA, MUELLER PS, KYLE RA, et al.Primary (AL) hepatic amyloidosis:clinical features and natural history in 98 patients[J].Medicine (Baltimore) , 2003, 82 (5) :291-298.

[7]HU YL, PANG Y, XIN GJ, et al.Clinical diagnosis of patients with hepatic amyloid degeneration[J].J Clin Hepatol, 2010, 26 (1) :65-66. (in Chinese) 胡玉琳, 潘煜, 辛桂杰, 等.4例肝淀粉样变性的临床特点和分析[J].临床肝胆病杂志, 2010, 26 (1) :65-66.

[8]HUNG HH, HUANG DF, TZENG CH, et al.Systemic amyloidosis manifesting as a rare cause of hepatic failure[J].J Chin Med Assoc, 2010, 73 (3) :161-165.

[9]NAITO KS, ICHIYAMA T, KAWAKAMI S, et al.AL amyloidosis with spontaneous hepatic rupture:successful treatment by transcatheter hepatic artery embolization[J].Amyloid, 2008, 15 (2) :137-139.

[10]KIM SH, HAN JK, LEE KH, et al.Abdominal amyloidosis:spectrum of radiological findings[J].Clin Radiol, 2003, 58 (8) :610-620.

[11]GIRNIUS S, SELDIN DC, SKINNER M, et al.Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease[J].Haematologica, 2009, 94 (7) :1029-1032.

[12]FAUST D, AKOGLU B, RISTIC G, et al.Ursodeoxycholic acid for treatment of cholestasis in patients with hepatic amyloidosis[J].Vojnosanit Pregl, 2009, 66 (6) :482-486.

[13]SHI XM, WANG YT, YANG TS.The analysis of 11cases with cardiac amyloidosis misdignosised as idiopathic hypertrophic cardiomyopathy[J].J Clin Intern Med, 2012, 29 (2) :112-114. (in Chinese) 时向民, 王玉堂, 杨庭树.心脏淀粉样变误诊为原发性肥厚型心肌病11例临床分析[J].临床内科杂志, 2012, 29 (2) :112-114.

[14]LOUSTAUD-RATTI VR, CYPIERRE A, ROUSSEAU A, et al.Non-invasive detection of hepatic amyloidosis:FibroScan, a new tool[J].Amyloid, 2011, 18 (1) :19-24.

施引文献

资源附件(0)

访问统计