表现为肾病综合征的毛细血管内增生性肾炎临床分析

病理为毛细血管内增生性肾炎（endocapillary proliferative glomerulonephritis,EPGN）通常表现为急性感染后肾小球肾炎即急性肾炎,临床上主要表现为。肾炎综合征,但表现为肾病综合征的也并非少见。现对我科住院的经皮肾脏活体组织检查（简称肾活检）证实为EPGN的合并肾病综合征10例患者进行回顾性临床分析,报道如下。     

卡托普利不能抑制激素对大鼠阿霉素肾病肾小球硬化的恶化作用

目的 :探讨血管紧张素转换酶抑制剂 (ACEI)卡托普利能否抑制肾上腺糖皮质激素 (激素 )对大鼠阿霉素肾病肾脏病理损害的加重作用。方法 :40只SD雄性大鼠分成A(正常对照 )组、B(阿霉素 )组、C(阿霉素 +泼尼松 )组和D(阿霉素 +泼尼松 +卡托普利 )组。注射阿霉素后 4周开始经饮水给予入泼尼松及卡托普利 ,其浓度分别为 5 0mg/dl和 2 5mg/dl。在注射阿霉素之后的第 16周及 2 8周末每组分别用 5只大鼠取血及肾脏组织 ,分别作生化及病理检查。结果 :注射阿霉素 1周以后尿蛋白 (Upro)开始升高 ,4周以后达到 (16 8.45± 76 .2 8)mg/d。B组、C组及D组注射阿霉素后第 16周末及 2 8周末Upro、血清肌酐 (Scr)、甘油三酯 (Trg)及血清总胆固醇 (ChL)都显著高于A组 ,血清白蛋白 (Salb)则显著低于A组。B组、C组及D组组内第 2 8周与第 16周比较 ,Upro、Scr升高有显著性差异 ,Salb降低有显著差异。C组与B组比较 ,Upro、Scr、Trg及ChL显著升高。而D组上述生化指标的变化与C组比较都无显著性差异。B组肾脏病理积分显著高于A组 ,且第 2 8周末显著高于第 16周末 ;C组及D组第 16周末肾脏病理积分显著高于B组 ,但D组与C组比较无显著差异。结论 :卡托普利不能阻抑激素对大鼠阿霉素肾病病理损害的加重作用     

厄贝沙坦联合甲基泼尼松龙冲击治疗原发性系膜增生性肾小球肾炎疗效的观察

目的观察厄贝沙坦联合大剂量甲基泼尼松龙冲击治疗原发性系膜增生性肾小球。肾炎（MsPGN）的早期疗效。方法将43例通过。肾脏活体组织检查确诊为MsPGN患者,分为厄贝沙坦联合大剂量甲基泼尼松龙冲击治疗组（A组）15例;大剂量甲基泼尼松龙冲击治疗组（B组）14例;甲基泼尼松龙片常规剂量治疗组（C组）14例,疗程共4周。检测治疗前及治疗后第1、2、3、4周血清白蛋白（Alb）、24h尿蛋白定量、内生肌酐清除率（Ccr）、尿素氮（BUN）、丙氨酸氨基转氨酶（ALT）、天门冬氨酸氨基转氨酶（AST）及空腹血糖（FBS）。结果A、B组在治疗后第3、4周24h尿蛋白定量减少、Alb升高,与C组比较有统计学差异（P〈0．01）。A组在治疗后第3、4周24h尿蛋白定量减少,与B组比较有统计学差异（P〈0．05）;各组治疗前、后Ccr、BUN、ALT、AST及FBS均无统计学差异（P〉0．05）。结论大剂量甲基泼尼松龙冲击治疗原发性MsPGN能迅速缓解临床症状,起效时间短,降低尿蛋白快。     

肾康灵冲剂治疗小儿肾炎性肾病综合征临床探析

目的:分析探讨中药治疗小儿肾炎性肾病综合征机制的作用.方法:动态观察血浆皮质醇(F),外周血白细胞糖皮质激素受体(GCR)及24 h尿蛋白定量变化,60例肾炎性肾病综合征患者随机分为治疗组和对照组各30例,均予常规西药治疗,治疗组加用肾康灵系列冲剂,观察二组在治疗后8周、12周、24周F,外周血GCR,24 h尿蛋白定量等变化.结果:治疗组在治疗后24周GCR较对照组明显增加,24 h尿蛋白定量明显下降;F下降幅度明显减少.结论:肾康灵系列冲剂治疗肾炎性肾病综合征患者可增加其外周血GCR数目,提高激素治疗敏感性,从而增强疗效;同时还能从一定程度上保护受损的肾上腺皮质功能,减轻激素副作用,调节机体免疫稳态,恢复机体阴阳平衡.     

小儿激素依赖性肾病综合征长期激素治疗对生长发育的影响

目的探讨难治性肾病综合征及糖皮质激素治疗对小儿生长发育的影响，增强对小儿肾病综合征生长发育迟缓的防治意识。方法针对1例病情长期未得到良好控制的激素依赖性肾病综合征并发垂体性侏儒症的患儿成功诊治经验，回顾分析激素依赖性肾病综合征对患儿生长发育影响的相关文献。结果肾病综合征疾病本身及糖皮质激素长期应用都会对患儿生长发育造成影响，具体机制还不清楚。结论在治疗小儿肾病综合征时，应尽可能对初发病例采用较短疗程的治疗方案。对此患儿应避免长期使用激素，可换用其它免疫抑制剂，并注意监测身高和生长速度，必要时可加用毕长激索治疗．     

中西医结合治疗成人原发性 系膜增生性肾炎肾病综合征的临床观察

目的探讨中西医结合治疗成人原发性肾病综合征(NS)病理类型为系膜增生性肾炎(MesPGN)临床疗效。方法对48例成人原发性系膜增生性肾炎肾病综合征(MesPGN－INS)分为治疗组和对照组。治疗组24例用强的松和环磷酰胺(CTX),并辨证加用中药;对照组24例单纯用西药治疗,观察临床缓解率和副反应。结果治疗组完全缓解率为54.1％,总缓解率为91.6％;对照组完全缓解率为33.3％,总缓解率为62.4％。治疗组完全缓解率和总缓解率显著优于对照组(P＝0.016;P＝0.008)。治疗组副反应率为37.5％,对照组为87.5％;治疗组副反应极显著低于对照组(P＜0.001)。结论中西医结合是治疗MsPGN－INS的有效方法。     

成人毛细血管内增生性肾炎91例临床与疗效

毛细血管内增生性肾炎（endocapillary proliferativc glomeru-lonephritis,EnPGN）是临床常见的急性肾小球肾炎的病理类型,以儿童最为常见,成人较少见。现将我科1983年3月～2009年8月经肾穿刺确诊的成人EnPGN91例进行临床与疗效分析。     

Three elderly cases of endocapillary glomerulonephritis and nephrotic syndrome

Three patients aged over 60 with endocapillary proliferative glomerulonephritis and nephrotic syndrome were reported. Immunofluorescence and electron microscopical findings were similar in all of them: granular deposits of IgG and C 3 along the capillary loops, electron dense deposits in the subendothelial area, and partial mesangial interposition. The levels of CH 50 were slightly suppressed in two of them, but neither preceding infection nor elevation in ASLO were noticed. None of then responded to steroid therapy. One patient fell in renal failure in spite of intensive steroid therapy, and died of bronchopneumonia. In another patient, proteinuria was remitted with systemic treatment against high blood pressure. The remaining patient took a favorable course during the admission without any special treatment, but proteinuria recurred after the discharge. These clinical manifestations and clinical courses were not compatible with the diagnoses of acute glomerulonephritis, mesangiocapillary glomerulonephritis, or vasculitis. We concluded that the endocapillary proliferative glomerulonephritis in adults over 60 years might be different form of glomerulonephritis from that of AGM, MPGN, and vasculitis, in which diffuse endocapillary proliferative changes in the glomeruli are seen in younger people.     

乙肝相关性肾炎病理为毛细血管内增生性肾炎一例

患者,男,43岁,于2008年1月因感冒后出现双下肢水肿,未做尿液检查,口服私人中药(具体药物成分不详)后水肿缓解.3月再次出现下肢水肿,后渐及全身,血压高达160/110 mm Hg c,查尿蛋白4+,RBC 20～30个/HP.仍用中药及不规律用卡托普利治疗,症状和尿蛋白无明显好转,于7月24日就诊我院.既往有先天心病史.查体:BP 160/100 mm Hg,心率80次/min,律齐,叩诊心界向左右两侧增大,胸骨左缘第3、4肋间可闻及全收缩期杂音伴震颤,双下肢明显水肿.     

IgA deficiency and membranous glomerulonephritis presenting as nephrotic syndrome

Selective IgA deficiency associated with glomerulonephritis is rare and no previous reports in childhood have been made of the association of IgA deficiency and membranous glomerulonephritis (MGN). We report a 5-year-old boy with selective IgA deficiency and MGN. He presented with nephrotic syndrome. Percutaneous renal needle biopsy showed diffuse global thickening on light microscopy and heavy IgG and moderate C3 deposits were found on immunofluorescence. Electron microscopy detected extensive global subepithelial deposition of electron-dense material with frequent intramembranous extension and spike formation. The pathological diagnosis was diffuse MGN stage 1. Oral prednisolone (1 mg kg^–1 day^–1), angiotensin-converting enzyme inhibitors (ACEI), and angiotensin II receptor blocker (ARB) were given resulting in reduction of proteinuria. The prednisolone dose was gradually tapered and discontinued after 2 months. At present the patient has been in complete remission for 10 months despite the discontinuance of prednisolone. In conclusion, our treatment with corticosteroid, ACEI and ARB reduced proteinuria and was effective for our case with selective IgA deficiency and MGN.     

A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosis

We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone. He was admitted to our hospital on November 22 following the appearance of bilateral leg edema in October 2000. Laboratory tests at presentation revealed nephrotic syndrome with renal impairment. Renal biopsy specimens revealed necrotizing glomerulonephritis with crescent, but immunofluorescence study showed lack of staining for immunoglobulins or complement components. Chest X-ray and CT showed abnormal shadows in the right upper lung field, and Cryptococcus neoformans was isolated in a transbronchial lung biopsy. After the diagnosis of pulmonary cryptococcosis was made, the patient was treated with 200mg/day fluconazole. The pulmonary abnormal shadows immediately improved and urinary protein excretion dramatically decreased. A second renal biopsy, performed about 2 months after the first biopsy, showed disappearance of crescent. Electron microscopic examination of the second renal biopsy showed partial effacement of foot processes without electron-dense deposits. Our findings suggest that necrotizing glomerulonephritis with nephrotic syndrome in this patient represented pauci-immune T-cell-mediated injury related to pulmonary cryptococcosis.     

Acute endocapillary proliferative glomerulonephritis associated with human parvovirus B19 infection

A 36-year-old female developed acute nephritic syndrome associated with human parvovirus B19 (HPVB19) infection. Laboratory data showed proteinuria, hypocomplementemia, mild pancytopenia, the presence of immunoglobulin (Ig) M and IgG antibodies to HPVB 19 and positive reaction of serum HPVB19 DNA using a polymerase chain reaction (PCR). A renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental apparent mesangiolytic change; fine granular IgM, IgG and C3 deposits were noted by immunofluorescence microscopy; relatively small electron-dense deposits were observed in the widened subendothelial spaces and the mesangium, and loosening of the mesangial matrix varied from place to place electron microscopically. PCR of HPVB19 DNA in the renal biopsy tissue was positive as well as in the peripheral blood. The histological findings suggested that immune-complex-mediated endocapillary proliferative glomerulonephritis is caused by acute HPVB 19 infection. We discuss the differences from poststreptococcal glomerulonephritis and the possible pathogenesis of acute endocapillary proliferative glomerulonephritis associated with HPVB19 infection.     

Peculiar renal endarteritis in a patient with acute endocapillary proliferative glomerulonephritis

Acute glomerulonephritis (AGN) is one of the most common renal diseases. They are often associated with infections and can result in diffuse proliferative glomerulonephritis (GN). This case report reviews an interesting case in which renal endarteritis coexisted in AGN with diffuse endocapillary proliferation. The discussion highlights important pathological findings and clinical aspects in acute endocapillary proliferative GN with renal endarteritis. Coexisting endarteritis should be in the differential diagnosis of AGN in patients with persistent clinical courses.     

Effect of corticosteroids on coagulation factors in children with nephrotic syndrome

Prothrombin time (PT), activated partial thromboplastin time (APTT) and plasma procoagulant activities were studied in 38 children with nephrotic syndrome in the presence or absence of prednisolone therapy. PT was normal but APTT was prolonged during relapse in untreated patients. Increased factors V, VII, VIII, XI and XIII in both treated and untreated and factor IX in treated patients, as well as decreased factors X and XII in untreated patients, were observed during relapse. These coagulation factor changes were unrelated either to the dose of prednisolone or underlying renal histology and normalized with clinical remission. However, plasma levels of factors II, V, VIII, IX, X and XI were still increased in treated patients. The data suggest that corticosteroids shorten APTT, raise both intrinsic and extrinsic factors, and therefore have favorable and unfavorable effects on the coagulation system in children with nephrotic syndrome.Presented in part at the 7th International Congress on Pediatric Nephrology, 7–12 September 1986, Tokyo, Japan.