Ⅳ型胶原α3、α5链免疫组化检测在遗传性肾炎诊断中的意义

DIAGNOSTIC VALUE OF IMMUNOHISTOCHEMICAL STUDY OF TYPE Ⅳ COLLAGEN α3 AND α5 CHAINS ON PATIENTS WITH HEREDITARY NEPHRITIS

目的 :分析肾组织Ⅳ型胶原α3、α5链的免疫荧光染色在Alport综合征 (AS)诊断中的意义。 方法 :采用特异性抗Ⅳ型胶原α3和α5链NC1区域的单克隆抗体 ,对 4例正常人及 4 3例临床疑诊为遗传性肾炎患者的肾脏组织进行了间接免疫荧光染色。 结果 :抗α3和α5链的抗体荧光染色在 10例患者的肾组织中分布异常 ,并呈现三种类型。在这 10例患者中 ,根据α3和α5链在肾组织中异常分布的特点 ,对其遗传方式进行分析发现其中 7例为X连锁显性遗传 ,3例为常染色体隐性遗传。 结论 :肾活检组织Ⅳ型胶原α3和α5链的免疫荧光染色 ,在遗传性肾炎 。

OBJECTIVE To evaluate the diagnostic value of immunoflurescent staining of type Ⅳ collagen α3 and α5 chains in renal biopsy specimen in patients with Alport syndrome. METHODOLOGY This study included 49 patients who satisfied the clinical criteria for the diagnosis of hereditary nephritis. Type Ⅳ collagen α3 and α5 chains in renal biopsy sections were detected with indirect immunofluorescence assay. RESULTS Three types of abnormal distribution of renal type Ⅳ collagen α3 and α5 chains were observed in 10 of the 49 patients with hereditary nephritis. Based on the immunohistochemistry features of type Ⅳ collagen chains in glomerular base membrane, 7 of the 10 patients were diagnosed as X linked dominant inherited Alport syndrome,and 3 as autosomal recessive inherited Alport syndrome. CONCLUSION Immunohistochemical staining of type Ⅳ collagen α3 and α5 chains in renal biopsy sections is of diagnostic importance in hereditary nephritis,especially in the diagnosis of Alprot syndrome and the inheritance patten analysis.