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目的观察抗肾小球基底膜(GBM)肾炎患者的临床和病理特征。方法收集2000—2010年在南京军区南京总医院全军肾脏病研究所住院并行肾活检诊断为抗GBM肾炎患者38例,根据入院时的血清肌酐浓度(Scr)分为2组:Scr<530μmol/L为A组(16例);Scr≥530μmol/L为B组(22例)。比较两组患者临床、实验室检查和肾活检病理特征。结果 38例患者中,男21例,女17例;平均年龄(44.3±18.2)岁;从发病到确诊时间平均为7.43周,确诊时已有半数以上(57.9%)的患者Scr≥530μmol/L,表现为急进性肾炎综合征26例患者(68.4%),急进性肾炎合并大量蛋白尿低蛋白血症者12例(31.6%),肉眼血尿高达65.8%。月体发生率为40%~100%,≥85%占34.2%。与A组相比较,B组患者年龄较大,病程较长,临床表现为少尿无尿者较多见,Scr明显升高;肾间质损伤的实验室指标差异具有统计学意义;而A组患者尿蛋白定量较多、血尿程度相对较重,但差异无统计学意义。病理上B组患者包囊壁断裂、小管炎、炎症细胞浸润均明显高于A组,新月体形成百分比差异则无统计学意义。结论本组患者肉眼血尿发生率高,诊断时多数表现为尿毒症,全部患者新月体形成≥40%。确诊时Scr水平、病程中是否出现少尿、无尿及肾间质损伤的实验室指标可预测肾脏损伤程度。
Objective To observe the clinical and pathological features of glomerular basement membrane (GBM) nephritis patients. Methods Thirty-eight patients diagnosed as having anti-GBM nephritis by renal biopsy at Nanjing General Hospital of Nanjing Military Region from 2000 to 2010 were divided into two groups according to serum creatinine concentration (Scr) at admission: Scr <530μmol / L group A (16 cases); Scr≥530μmol / L group B (22 cases). The clinical, laboratory and renal biopsy characteristics of the two groups were compared. Results Among 38 patients, there were 21 males and 17 females with an average age of (44.3 ± 18.2) years. The average time from onset to diagnosis was 7.43 weeks. More than half of the patients (57.9%) had Scr ≥530 μmol / L (26.4%) with acute nephritic syndrome, 12 cases (31.6%) with acute nephritis with massive proteinuria and hypoproteinemia, and gross hematuria (65.8%). The incidence of lunate was 40% ~ 100%, ≥85% 34.2%. Compared with group A, patients in group B were older and had a longer course of disease. Clinical manifestations of oliguric and anuria were more common, and Scr was significantly increased. Laboratory differences in renal interstitial injury were statistically significant; while group A Urine protein in patients with more quantitative, hematuria is relatively heavy, but the difference was not statistically significant. Pathological B group patients with cystic wall rupture, tubulitis, infiltration of inflammatory cells were significantly higher than the A group, no significant difference in the percentage of crescent formation. Conclusion This group of patients with high incidence of gross hematuria, uremia at diagnosis most of the performance, all patients with ≥ 40% of the formation of crescent. The level of Scr at the time of diagnosis, the presence of oliguria in the course of the disease, laboratory tests of anuria and renal interstitial lesions predict the extent of renal damage.