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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 ,特别AS诊断及其遗传方式分析上具有重要意义
Objective: To analyze the significance of immunofluorescent staining of type Ⅳ collagen α3 and α5 chains in Alport syndrome (AS) in renal tissues. Methods: The indirect immunofluorescence staining was performed on four normal subjects and 43 cases of hereditary glomerulonephritis patients with monoclonal antibodies specific to the α3 and α5 chain NC1 regions of type Ⅳ collagen. RESULTS: Fluorescent staining of anti-α3 and α5 chains was abnormal in renal tissues of 10 patients and showed three types. In these 10 patients, according to the abnormal distribution of α3 and α5 chains in renal tissues, the genetic patterns were analyzed. Among them, 7 were X-linked dominant and 3 were autosomal recessive. CONCLUSIONS: Immunofluorescence staining of α3 and α5 chains of type Ⅳ collagen in renal biopsies is of great importance in the diagnosis of hereditary nephritis, special AS diagnosis and genetic analysis