CAJ | 학술논문

目的通过应用多种方法对肾脏淀粉样变性病进行分型诊断,以明确我国肾脏淀粉样变性病的分型特点.方法选择1990年1月至2011年12月间在北京大学第一医院肾内科经肾活检诊断的肾淀粉样变性病患者共205例,应用常见淀粉样变前体蛋白(免疫球蛋白轻链κ、λ,血清淀粉样蛋白A,纤维蛋白原,转甲状腺素蛋白,溶菌酶和载脂蛋白A1)的抗体进行免疫组化染色.对免疫组化染色结果阴性或不确定的患者进行免疫球蛋白轻链的免疫电镜标记和遗传性淀粉样变性病的基因突变筛查.结果 205例患者中,190例(92.7％)诊断为免疫球蛋白轻链淀粉样变,其中165例(80.5％)来源于轻链λ,25例(12.2％)来源于轻链κ;1例(0.5％)诊断为淀粉样蛋白A淀粉样变；1例(0.5％)诊断为遗传性纤维蛋白原淀粉样变；其余13例(6.3％)分型不明确.结论在我国,肾脏淀粉样变性病的主要类型是免疫球蛋白轻链型,占绝大多数,其中致病轻链类型以λ为主.
목적 통과응용다충방법대신장정분양변성병진행분형진단,이명학아국신장정분양변성병적분형특점.방법 선택1990년1월지2011년12월간재북경대학제일의원신내과경신활검진단적신정분양변성병환자공205례,응용상견정분양변전체단백(면역구단백경련κ、λ,혈청정분양단백A,섬유단백원,전갑상선소단백,용균매화재지단백A1)적항체진행면역조화염색.대면역조화염색결과음성혹불학정적환자진행면역구단백경련적면역전경표기화유전성정분양변성병적기인돌변사사.결과 205례환자중,190례(92.7％)진단위면역구단백경련정분양변,기중165례(80.5％)래원우경련λ,25례(12.2％)래원우경련κ;1례(0.5％)진단위정분양단백A정분양변；1례(0.5％)진단위유전성섬유단백원정분양변；기여13례(6.3％)분형불명학.결론 재아국,신장정분양변성병적주요류형시면역구단백경련형,점절대다수,기중치병경련류형이λ위주.
Objective To identify the prevalence of different amyloid types in renal biopsies.Methods The renal biopsies of 205 patients diagnosed as renal amyloidosis from January 1990 to December 2011 were reassessed.Immunohistochemistry was performed with a penal of antibodies directed against λ-light chain,κ-light chain,amyloid A,fibrinogen,transthyretin,apolipoprotein A1,and lysozyme.Immune electron microscopy and gene analysis were performed when the results of immunohistochemistry were indeterminate.Results Among 205 patients,190 cases (92.7％) were classified as immunoglobulin light chain amyloidosis (AL),1 (0.5％) case as amyloid A amyloidosis and 1 (0.5％) case as fibrinogen A α-chain amyloidosis.The amyloid types of remaining 13 (6.3％) cases were undetermined.In the AL patients,the distribution of λ and κ was 6.6∶1.Conclusion AL is the most common form of renal amyloidosis in China,with a predominant light chain type of λ.