七、遗传性和先天性肾病

　　包括Alport综合征、良性复发性血尿（薄基底膜综合征）、先天性肾病综合征（Finish type）、婴儿肾病综合征（French type）、甲一髌综合征、Fabry’s病等，荧光显微镜观察皆为阳性。

　　八、杂病

　　1．妊娠毒血症性肾病　临床表现为蛋白尿、高血压、或有镜下血尿。光镜可见肾小球毛细血管内皮细胞高度肿胀，使毛细血管腔变狭窄。电镜可见内皮细胞及系膜细胞肿胀，内皮下及系膜区沉积物。荧光可见纤维素沿肾小球毛细血管壁呈颗粒状沉积，可伴有IgG、IgM及C_３。

　　2．放射性肾炎 临床表现为蛋白尿、高血压及肾衰。光镜可见肾小球毛细血管壁增厚及皱曲，节　段性坏死及硬化。电镜可见内皮细胞剥离，内皮下沉积物。荧光为阴性或有IgM呈节　段性沉积。

　　九、肾移植

　　1．超急性排斥反应　发生于移植后几分钟至几小时，临床表现为高热、腰痛、无尿。光镜可见肾小球和间质的毛细血管内大量中性白细胞集聚，继而形成血栓并有血管壁纤维素样坏死。电镜可见肾小球毛细血管内有纤维素，内皮细胞剥落，基底膜疏松肿胀。荧光可见lgG、lgM、和C₃沿肾小球毛细血管壁呈线形沉积，间质小动脉壁有纤维素沉积。

　　2．加速性排斥反应（严重急性排斥反应）　发生于移植后一个月内，临床表现为高热、腰痛、血尿、少尿并发展为无尿。光镜可见肾小球毛细血管及肾小动脉壁有纤维素样坏死及广泛微血栓形成，常继发出血及梗死。电镜所见与前大致相同。荧光可见血管壁有纤维素沉积。

　　3．急性排斥反应　发生于移植后二个月，临床表现为发热、腰痛、少尿及血压升高。光镜可见肾间质水肿及小圆形细胞湿润，人球小动脉及小叶间动脉管壁水肿、内皮细胞剥脱，甚至管壁纤维素性坏死。电镜所见与前大致相同。荧光可见IgG、IgM、C3及纤维素在小动脉壁沉积。

　　4．慢性排斥反应　发生于移植后数月至数年，临床表现为蛋白尿、高血压及肾功能减退。光镜可见细动脉及小动脉的闭塞性血管内膜炎，肾小球毛细血管袢皱缩，基底膜增厚，系膜基质增多，并有局灶性和弥漫性肾间质纤维化。电镜可见内皮下电子密度疏松区。荧光可见IgG、IgM及C_３于肾小球系膜区和动脉壁有颗粒状沉积。

　　十、肾小管间质性疾病

　　主要介绍过敏性间质性肾炎，多由磺胺、青霉素、庆大霉素、卡那霉素等药物引起。临床表现为蛋白尿、少尿甚至无尿。光镜可见肾间质水肿，有淋巴细胞、单核细胞及嗜性白细胞浸润。肾小管上皮细胞有灶状坏死，管腔内有蛋白管型。发展至慢性时，肾间质有淋巴细胞浸润及纤维化，肾小管萎缩。荧光可见IgG及C_３于肾小管基底膜呈线形沉积。

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(魏民)