论文部分内容阅读
目的:分析8例致密物沉积病(DDD)的临床病理特征和治疗反应,旨在提高对DDD的诊断和治疗水平。方法:对经临床、肾脏病理(光镜、免疫病理和电镜)确诊的8例DDD患者的临床病理资料及疗效进行分析。结果:(1)一般情况:8例患者男性5例,女性3例,起病时平均年龄(18.4±9.20)岁(8~32岁),至肾活检时平均病程(2.49±2.04)年(0.17~7年)。(2)临床特征:患者均有大量蛋白尿(尿蛋白定量>3.5 g/24h)和镜下血尿,伴肉眼血尿、高血压、血清肌酐升高、低补体血症和贫血者分别为3、5、2、6和5例。均未见部分脂肪营养不良与眼脉络膜疣。(3)病理特点:光镜下7例表现为膜增生性肾小球肾炎,1例表现为肾小球系膜增生性病变。肾小球细胞数并无明显增加,肾小球基膜(GBM)病变明显,GBM嗜伊红性明显增强,PAS强阳性。免疫病理以C3为主,伴(或)不伴免疫球蛋白在肾小球毛细血管袢呈线状或绸带状沉积,部分可沉积于肾小管基膜和包曼囊壁。电镜下均表现为GBM内伴(或)不伴肾小管基膜内、包曼囊壁有高电子致密物沉积。(4)治疗及疗效:有5例患者入院前接受泼尼松、环磷酰胺和(或)环孢素治疗,无明显效果,有2例出现股骨头坏死。1例曾接受雷公藤多甙治疗,尿蛋白减少。诊断明确后有6例患者回访,均先后接受雷公藤多甙治疗者,尿蛋白有不同程度的减少,有1例先接受泼尼松联合霉酚酸酯治疗2年无效,切换为雷公藤多甙治疗3月后尿蛋白有所减少。结论:(1)对青少年,临床表现为大量蛋白尿、镜下血尿、高血压,尤其合并低补体C3血症与贫血,对一般免疫抑制剂治疗无效的患者要警惕DDD。(2)肾脏病理光镜下突出病理改变在GBM,表现为嗜伊红性明显增强,PAS强阳性。免疫病理以C3为主在肾小球毛细血管袢呈线状或绸带状沉积,电镜检查可以明确诊断。(3)DDD对一般的免疫抑制治疗无效,有7例患者接受雷公藤多甙治疗,尿蛋白均有不同程度的减少,雷公藤多甙对DDD的确切疗效有待积累更多的临床资料。
OBJECTIVE: To analyze the clinicopathological characteristics and treatment response of 8 cases of dense sedimentosis (DDD) aimed at improving the diagnosis and treatment of DDD. Methods: The clinicopathological data and therapeutic effects of 8 patients with DDD confirmed by clinical pathology, renal pathology (light microscopy, immunopathology and electron microscopy) were analyzed. Results: (1) The general situation: There were 5 males and 3 females in 8 cases, the mean age at onset was (18.4 ± 9.20) years old (ranged from 8 to 32 years), and the mean duration to renal biopsy was 2.49 ± 2.04 years 0. 17 ~ 7 years). (2) Clinical features: All patients had a large amount of proteinuria (urinary protein quantification> 3.5 g / 24h) and microscopic hematuria with gross hematuria, hypertension, elevated serum creatinine, hypocomplementemia and anemia, 5, 2, 6 and 5 cases. No partial fat dystrophy and ocular drusen were seen. (3) pathological features: 7 cases under light microscopy showed membranoproliferative glomerulonephritis, 1 case showed mesangial proliferative lesions. Glomerular cell number did not increase significantly, glomerular basement membrane (GBM) lesions significantly increased GBM eosinophil significantly enhanced PAS strong positive. Immunopathology mainly C3, with or without immunoglobulin in the glomerular capillary loop was linear or ribbon deposition, some can be deposited in the tubular basement membrane and the Buman wall. Electron microscopy showed GBM with or without renal tubular basement membrane, Baoman capsule wall with high electron density deposition. (4) treatment and efficacy: 5 patients admitted to hospital before receiving prednisone, cyclophosphamide and (or) cyclosporine treatment, no significant effect, 2 cases of avascular necrosis. One patient had received treatment with tripterygium glycosides, urinary protein decreased. After the diagnosis was clear, there were 6 patients who were interviewed. All of them received the treatment of tripterygium polyglycoside, urinary protein decreased to varying degrees. One case received prednisone combined with mycophenolate mofetil for 2 years and switched to Tripterygium wilfordii Glycosides decreased after 3 months of treatment. Conclusion: (1) For adolescents, the clinical manifestations of massive proteinuria, microscopic hematuria, hypertension, especially in patients with low complement C3 hyperlipidemia and anemia, general immunosuppressive therapy should be on guard against DDD. (2) pathological changes under the pathological changes of renal pathology in GBM, manifested as significantly increased eosinophilus, PAS strong positive. Immunopathology C3-based glomerular capillary loop was linear or ribbon deposition, electron microscopy can confirm the diagnosis. (3) DDD is ineffective for the general immunosuppressive therapy. Seven patients were treated with tripterygium glycosides, urinary protein were reduced to varying degrees. The clinical efficacy of tripterygium glycosides in DDD still needs to be accumulated.