The clinical and pathological features of idiopathic membranous nephropathy in 246 Chinese adults

Objective: To investigate the clinical and pathological features of idiopathic membranous nephropathy (IMN) in Chinese adults. Methods: From 1986 to 1997, 264 patients with biopsy proven membranous nephropathy were selected in this study. Clinical and pathological features were compared between patients at different ages by t test. Results: (1) Patients from 21- 40 years old were inclined to membranous nephropathy. (2) One hundred and six of the patients had heavy proteinuria at presentation. Hypertension was found in 35 patients. Renal insuffeiency occurred in 7.7% of the patients in renal biopsy. Microscopic hematuria was found in 40.2% of the patients. Seventy-four patients presented nephrotic syndrome. (3) Eight of the 57 patients had deterioration of renal function during an average 49-month follow-up. (4) Patients of stage Ⅰ, Ⅱ, Ⅲ and Ⅳ accounted for 42.3%, 48.7%, 6.0% and 3.0% respectively. Glomeruli IgG, C3 and C1qdeposition was found in 93.2%, 98.8% and 58.3% of the patients. Conclusion: Younger patients are inclined to membranous nephropathy. The incidence of hypertension, microscopic hematuria and renal insuffcieney is similar to that of other countries, while nephrotic syndrome is uncommon.     

NON-IgA MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS CLINICAL AND PATHOLOGICAL ANALYSIS OF 77 CASES

Primary mesangial proliferative glomerulonephritis without IgA deposition (non-IgA MsPGN)is one of the most common types of glomerular disease in China .In an attempt to investigate its clinical and pathological features ,we reviewed 77 such cases from 380 patients with primary glomerulonephritis taken renal biopsies during 1980-1987. Prodromal upper respiratoty tract infection occurred in 31 cases (40%) .In immunofluorescence Microscopy .prominent IgG granular deposits in mesangium were observed in 45 cases (58%) .These features are quite different from those in western countries ,indicating it might have different pathogenetic ptocesses. According to the severity of mesangial lesions , the 77cases were divided into 3 groups: mild (55 cases ) ,moderate (14) and severe (8) .In the patients with mild mesangial lesion and massive proteinuria , the therapeutic response to prednisone was similar to that in adult minimal change disease . In the moderate and severe groups ,there was a significantly higher incidence of superimposed tubulo – interstitial lesions associated with hypertension, persistant renal insufficiency and a poor response to prednisone . This work showed non-IgA MsPGN covered about 20% of our primary glomerulopathy ,which may be related to a higher incidence of infection. It was suggested that minimal change nephritic syndrome ,inspite of the variety of immunoglobulin mesangium deposits, could be treated as a single disease entity, and light microscopy is most important in offering prognostic information.     

Tumor suppressor cylindromatosis: expressed in IgA nephropathy and negatively associated with renal tubulo-interstitial lesion

Background IgA nephropathy is the major cause of end-stage renal failure in patients with primary glomerular diseases. Tumor suppressor cylindromatosis （CYLD）, the recently identified member of the deubiquitinating enzymes, has been actively involved in regulation of inflammation. This study was undertaken to investigate the CYLD expression profile in IgA nephropathy and identify factors associated with CYLD expression. Methods Forty-one cases of IgA nephropathy were selected. CYLD expression in the kidney biopsy tissue was measured by immunohistochemical staining. Relevant clinical and pathological data were analyzed, and Logistic regression analysis was carried out to identify factors associated with CYLD expression. Results CYLD was specifically expressed in renal tubular epithelial cells in 70% of the studied patients with IgA nephropathy. All patients with positive CYLD staining had proteinuria, while only 72.7% of patients with negative CYLD had proteinuria （P=0.003）. Among studied proteinuric patients, those with positive CYLD had significantly less tubulo-interstitial lesions and higher estimated glomerular filtration rate （eGFR） levels when compared with those patients showed negative CYLD results. Logistic regression analysis indicated that the urinary protein excretion and eGFR were identified as predictors for the CYLD expression. Conclusion CYLD is expressed in renal tubular epithelial cells and appears to be associated negatively with tubulointerstitial lesions, however, its exact functional role remains to be clarified in further experiments.     

Lipoprotein glomerulopathy: clinical features and pathological characteristics in Chinese

Background Lipoprotein glomerulopathy (LPG), once recognized as a rare glomerular disease, has been reported around the world in recent years. In this study, we reported 8 patients of LPG and aimed to explore the clinical features and pathological characteristics of LPG under light microscope, immunofluorescence staining, and electron microscope.Methods Clinical manifestations were recorded on the day of renal biopsy. Biochemical patterns of lipids and lipoproteins were detected by routine examination. Plasma concentrations of apo B and apo E were determined by radial immunodiffusion assays. Biopsy specimens were then processed for lightmicroscopy, immunohistochemical staining for immunoglobulins and complement components, and electron microscopy. Glomerular deposition of apo A, B, and E were detected using monoclonal antibodies on cryostatic sections.Results All of the eight patients presented with edema, microscopic hematuria, severe proteinuria,anemia, and enlarged kidney size. Biochemical profiles revealed high levels of triglycerides, apo B,and apo E. We noted increments of glomerular size and lipoprotein thrombi occupying capillary lumina in the glomeruli of all patients. Immunofluorescence staining showed that the thrombi were strongly positive for apo A, B, and E. Granules and various sizes of vacuoles were observed in the thrombi under electron microscope.Conclusion Compared with previous reports on LPG in other countries, unique clinical and pathological features were found in this group of Chinese LPG patients.     

The clinical and pathological characteristics of Chinese patients with pauci-immune crescent golmerulonephritis

Objective　To investigate the clinical and pathological characteristics of pauci-immune crescent glomerulo～nephritis (PICGN) in Chinese patients. Methods　During 13 years (1985-1998), 6400 patients underwent non-transplanting renal biopsy. Twenty-four patients were diagnosed as PICGN. All clinical and laboratory data of these patients were collected from the patients’ records and used for detailed analysis. The diagnosis is based on clinico-pathologic findings. Results　Of the 24 patients, 16 were females and 8 were males, with median age of 33 years (ranged 10-76 years). Microscopic polyarteritis (MPA) (33.3%) and systemic vasculitis (8.3%) were the secondary diseases. The incidence of PICGN was 0.38% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. At onset, gross hematuria was noted in 58.3% of patients, hypertension in 45.8%, nephrotic syndrome in 41.7%, and oliguria in 25.0%. However, systemic symptoms were rare except for anemia. Pathologically, necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in the interstitium (53.3%) were observed. In addition, glomerulosclerosis was noted in 45.8%, severe tubular atrophy in 83.3% and interstitial fibrosis in 75.0%. Anti-neutrophil cytoplasmic antibodies (ANCAs) were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were available for long-term follow up (median 29.8 months, range 8-92 months). Twelve of them had life-sustaining renal function, four had normal serum creatinine (<124?μmol/L) and only 4 patients were dialysis-dependent. Conclusion　PICGN is not rare in China. Early diagnosis and administration of immunosuppressive therapy, particularly in patients with rapidly progressive glomerulonephritis (RPGN), are important for good prognosis.     

Renal thrombotic microangiopathies induced by severe hypertension

Renal thrombotic microangiopathy(TMA) is an uncommon vascular complication of severe hypertension.Until now,its clinical-pathological characteristics and renal survival have been unclear.Twenty-one patients with biopsy-proven renal TMA and with severe or malignant hypertension were retrospectively studied.All the patients exhibited severe hypertension,with systolic blood pressure at 200-280 mmHg and diastolic pressure at 110-180 mmHg.No patients had hemorrhagic manifestations.Elevated lactate dehydrogenase and thrombocytopenia were found in 6 and 5 patients,respectively.Significant proteinuria(> 3 g/day) was present in 2 patients and microscopic hematuria in 18 patients.All patients presented with renal insufficiency(creatinine 3.1 +/-2.1 mg/dL).The level of von Willebrand factor:antigen(vWF:Ag) in patients was not significantly higher than that in the healthy subjects,while the ADAMTS13(a disintegrin and metalloprotease,with thrombospondin-1-like domains) activity was not significantly lower than that in the healthy subjects.Renal histology showed a TMA involving preglomerular arterioles and/or interlobular arteries character-ized by fibrin deposits and vascular wall sclerosis.Fibrin glomerular microthrombi were not observed in these patients.Four patients required hemodialysis upon admission for severe acute renal failure.On follow-up,3 patients had recovered normal renal function and 14 had mild renal insufficiency(creatinine 1.8 +/-0.3 mg/dL),while 4 patients still required persistent hemodialysis.In conclusion,compared with patients having hemolytic uremic syndrome/thrombocytopenic purpura,our patients showed a low incidence of throm-bocytopenia and better renal outcome.     

Role of multi-slice CT urography over ultrasonography in patients with hematuria

Objective:Understanding the role of Multi-slice CT Urography(MSCTU)over Ultrasonography(US)in patients presenting with hematuria.Materials and Methods:Retrospective study enrolled 131 patients presenting with hematuria[microscopic hematuria(n=60)]and macroscopic hematuria(n=71)]who have undergone both MSCTU and US of urinary tract system simultaneously.Results of tests were compared with respective surgical and histopathological analysis of lesion.The cases obtained were bladder carcinoma,ureter carcinoma,renal carcinoma,urinary tract calculi and bladder inflammation.PASW-18thstatistical tool was used for obtaining statistical analysis and final interpretation of results.Results:The sensitivity and specificity of MSCTU and US for recognition of lesions presenting with macroscopic hematuria were 95.38%,83.33%and 81.54%,66.67%respectively and for those with microscopic hematuria were 96.08%,88.89%and 86.27%,77.8%respectively.The positive and negative likelihood ratios of MSCTU and US in macroscopic category were 5.73,0.055 and 2.46,0.277 respectively while for those in microscopic category were 8.65,0.044 and 3.88,0.176 respectively.In context to the sensitivity of MSCTU and US in patients presenting with macroscopic hematuriathedifferenceswere significant(McNemar’s test,P=0.039)suggesting the tests are not similar whereas for those with microscopic hematuria the differences were not significant(Mc Nemar’s test,P=2.68)indicatingsimilarity between these tests.Conclusion:Diagnostic efficacy of MSCTU is found to be far superior over US for patients presenting with macroscopic hematuria,thus current practice of using it as a first line modality seems to be justified.However,for those presenting with microscopic hematuria MSCTU and ultrasonography shows near to similar resultsin accordance to MSCTU,thus US alone seems sufficient to exclude significant urinary tract lesions.     

Clinical spectrum of diffuse crescentic glomerulonephritis in Chinese patients

Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.Results The distribution of patients among the three classifications was 8.7% type Ⅰ, 68.6% type Ⅱ and 22.7% type Ⅲ. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30.2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61.6% had hypertension, 50.6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type Ⅲ DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.Conclusion DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30.2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.     

Treatment of six cases of left renal nutcracker phenomenon： surgery and endografting

Objective A retrospective study was undertaken to examine therapeutic strategies used in 6 patients with left renal venal nutcracker phenomenon.Methods Three patients underwent surgical transposition of the superior mesenteric artery. Three patients underwent endograft stent implantations with intervention.Results All 6 cases were completely relieved of left renal vein compression after the operations, leading to improved clinical symptoms and normal urine tests.Conclusions Transposition of the superior mesenteric artery is a feasible method, but leads to complications and great trauma. Treating left renal venal nutcracker phenomenon with endografting techniques is minimally invasive in certain cases, and seems to have a prospective future. Chin Med J 2003; 116(11):1782-1784Left renal venal nutcracker phenomenon or aortomesenteric left renal vein entrapment syndrome involves compression of the left renal vein (LRV) in the fork between the abdominal aorta and the proximal superior mesenteric artery (SMA) close to its point of origin. The main symptoms of this phenomenon are hematuria and proteinuria. In this paper, we report our experiences with surgical and interventional management of left renal venal nutcracker phenomenon.     

A clinicopathological study of Alport syndrome and detection of type IV collagen chains in Alport patients

OBJECTIVE To summarize the clinical and pathological findings of Alport syndrome (AS), detect the distribution of type IV collagen within basement membrane of patients with AS and evaluate the diagnostic value of indirect immunofluorescence (iIF) study of type IV collagen in AS.

METHODS Fourteen patients belonging to 12 families were collected from January 1990 to June 1996. The clinical examinations include biochemical examination, audiometry and ocular examination. IIF technique was used to detect the location of chains of type IV collagen in 6 renal and 5 skin specimens from 8 Alport patients.

RESULTS Among fourteen patients, 11 were male and 3 female (mean age 29.4 years). Microscopic hematuria was found in 13 patients, and recurrent gross hematuria in 7. All had proteinuria. Three patients presented nephrotic syndrome. Slowly progressive renal failure occurred in 10 of 11 males (11-39 years) and 1 female (40 years). Sensorineural deafness was observed in 9 patients particularly high frequency sound. Anterior lenticonus were presented in 2. Five families transmitted as X-linked dominant (XD) trait and 3 autosomal dominant, 3 autosomal recessive inheritance. In 7 renal biopsies, the findings by light microscopy mostly revealed focal and segmental sclerosis glomerulonephritis (4/7). The results of IF were negative in 4. Ultrastructural studies showed variable thickening, thinning of glomerular basement membrane (GBM) in 7 specimens with lamellation and basket wearing of GBM in 1. Using the iIF technique, the alpha 3, 4, 5 (IV) chains were observed to be absent within both GBM and EBM of 4 male XD-AS patients. Six patients were treated with hemodialysis, 2/6 with transplantation.

CONCLUSIONS Alport syndrome (AS) is a heterogeneous hereditary disease characterized by progressive hematuric nephritis with or without sensorineural hearing loss and ocular defects. Ultrastructural alterations of GBM are helpful to the diagnosis of AS. IIF study suggests that type IV collagen in basement membrane of AS was abnormal and iIF study of type IV collagen chains distribution is useful for confirming the diagnosis of AS.

     

免疫触须样肾小球病

目的:探讨免疫触须样肾小球病的病因、临床病理特征、诊断、鉴别诊断、治疗及预后。方法:报道2例罕见的免疫触须样肾小球病,并结合文献进行复习。结累:2例均为中青年男性,临床表现蛋白尿、肾病综合征及镜下血尿,1例伴有高血压,1例肾功能不全。光镜下2例病理类型均为膜增殖性肾小球肾炎,肾小球系膜区和基底膜有IgG、IgM、C3和/或IgA免疫复合物沉积,但刚果红染色阴性。电镜下系膜区和基底膜内皮下扩大,电子致密物沉积,其中可见直径30～50nm、无分支的中空微管状结构。结论:ITG具有典型的形态学特征,确诊需依赖电镜特征性徽管状结构。病情发展快,预后差,多数肾功能持续恶化。     

成人无症状性血尿蛋白尿的临床病理分析及预后

目的：探讨成人无症状性肾小球肾炎的临床与病理关系，了解其早期肾功能的变化。方法：54例临床诊断为隐匿性肾炎的患者均行肾组织活检术，肾组织行光镜，免疫组化及电镜检查，同时查肝肾功，血脂，尿蛋白定量，肾功效免，自身抗体，乙肝系列等。结果：单纯性血尿30例占55．5％；余为单纯性蛋白尿11例及血尿，蛋白尿13例，肾脏病理改变以轻度系膜增生性肾炎（MsPGN24例）及IgA肾病(IgAN15例）为主，分别占44．4％，27．7％，余为局灶增生性肾炎（FPGN），局灶段肾小球硬化症（FSGS），IgM肾病，Ⅰ型膜增生性肾炎（MPGN），毛细血管内增生性肾炎（EnPGN),51.8%伴小血管炎（28例）。结合临床可见：发作性肉眼血尿多见于IgAN,单纯性蛋白尿多见于MsPGN,早期肾功损害主要见于IgAN,FSGS,28例随访6月至5年，仅2例血Scr轻度升高。结论；成人隐匿性肾炎临床以单纯血尿和／或蛋白尿为主要表现，其病理类型多样，但以MsPGN及IgAN为主，发作性肉眼血尿多为IgAN,单纯性蛋白尿多见于MsPGN,约51．8％伴明显小血管炎，约40％有早期肾功能损害，对于发作性肉眼血尿或单纯性血尿，蛋白尿，肾活检有重要价值。     

成人原发性肾病综合征的病理类型、临床特征和疗效的关系

在175例经肾穿刺明确诊断的成人原发性肾病综合征中,分析其病理类型及临床表现,实验室检查和疗效的关系。肾病综合征疗效和病理类型关系密切,微小病变性肾病和局灶增生性肾炎的缓解率分别达96.3％和83.3％,而局灶性肾小球硬化症和膜增生性肾炎则分别为21.4％和23.76％,膜性肾病为48.1％。肾病综合征伴血尿,尿纤维蛋白降解产物阳性和非选择性蛋白尿者,疗效显著地差,p<0.001。     

成年人原发性肾病综合征的不同病理类型对泼尼松的治疗反应

目的 明确成年人原发性肾病综合征的常见病理类型及对泼尼松的治疗反应。方法 对220例原发性肾病综合征患者进行了经皮肾穿刺病理检查并给予泼尼松口服治疗10周，通过测定治疗前后24h尿蛋白定量及血浆白蛋白的变化评价治疗效果。结果 成年人原发性肾病综合征的常见病理类型为：系膜增生性肾炎、膜增生性肾炎、轻微肾小球病变、膜性肾病、局灶性节段性硬化。泼尼松治疗10周后总有效率为63％。结论 原发性肾病综合征对泼尼松的治疗反应与病理类型及是否合并肾小管损伤有关。     

Clinicopathological spectrum and treatment outcome of idiopathic steroid-resistant nephrotic syndrome in children at a tertiary care center

BackgroundIdiopathic steroid-resistant nephrotic syndrome (iSRNS) is a common problem in pediatric nephrology. About 10%–20% of children with nephrotic syndrome have iSRNS and almost 50 percent progress to end-stage renal disease. Very few studies have tried to study the histopathological spectrum, correlate clinical features, renal biopsy patterns and treatment outcome in children presenting with iSRNS in India. In this study, we compared the histopathologic distribution of different subtypes of glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy.MethodsIt is a retrospective cross-sectional study involving review of records of all children with iSRNS presenting to our pediatric nephrology OPD who underwent renal needle biopsy and followed-up for a period of atleast six months post biopsy. Histopathological subtypes were correlated with clinical features (viz. age of onset, gender, oliguria, microscopic hematuria, hypertension, serum creatinine at presentation, serum total protein, serum albumin, 24 h urine protein, persistent proteinuria after 12 weeks of calcineurin inhibitor therapy, and progress to end stage renal disease (ERSD), if any, outcome.ResultIt was found that minimal change disease (MCD) was overall the most common cause of iSRNS. These patients have a significantly greater incidence of remission with immunosuppression as compared with focal segmental glomerulosclerosis (FSGS) and IgA nephropathy subgroup.ConclusionKidney biopsy is of prognostic value in children with iSRNS. The prognosis of children with SRNS owing to MCD is much better than with other nephropathy.     

肾病水平蛋白尿的急性链球菌感染后肾小球肾炎患儿临床特点及预后分析

目的　探讨肾病水平蛋白尿的急性链球菌感染后肾小球肾炎患儿的临床特点、病理及预后特点。方法　回顾性分析西安市儿童医院2013年1月—2017年1月确诊为急性链球菌感染后肾小球肾炎、肾病水平蛋白尿并行肾穿刺活组织检查的21例患儿（肾病水平蛋白尿组）的诊治资料,选择同期80例非肾病水平蛋白尿患儿（非肾病水平蛋白尿组）的诊治资料,总结肾病水平蛋白尿组肾脏病理检查特点,对比分析两组患儿的临床资料、治疗情况及预后情况。结果　肾病水平蛋白尿组患儿肾脏病理光镜检查显示均为毛细血管内增生性肾小球肾炎,9例（42.9%）有新月体形成,但比例均<10.0%;电镜检查显示上皮下“驼峰”样电子致密物沉积,其中1例电子致密物沉积较多。免疫荧光检查显示仅9例（42.9%）有C3沉积（≥++）而无IgG沉积,2例伴明显的IgG沉积（≥++）。肾病水平蛋白尿组肉眼血尿发生率、急性肾损伤（AKI）发生率高于非肾病水平蛋白尿组,清蛋白水平、抗链球菌溶血素“O”试验阳性率低于非肾病水平蛋白尿组（P<0.05）。肾病水平蛋白尿组患儿除了给予抗感染及对症治疗外,6例患儿给予糖皮质激素治疗,非肾病水平蛋白尿组均未使用激素治疗。肾病水平蛋白尿组肉眼血尿消失时间、水肿消退时间、尿蛋白转阴时间及补体恢复时间长于非肾病水平蛋白尿组（P<0.05）。结论　 肾病水平蛋白尿的急性链球菌感染后肾小球肾炎患儿AKI发生率更高,近期预后良好,仅少数遗留镜下血尿。     

伴毛细血管内增生的儿童紫癜性肾炎的临床病理及转归分析

目的：探讨伴毛细血管内增生的儿童紫癜性肾炎（EP-HSPN）的临床、病理和疾病转归。方法：回顾性分析2005年1月-2013年6月本院收治的经肾活检确诊为EP-HSPN患儿的临床、病理和随访资料,选取同时段的非EP-HSPN患儿作为对照,将2组进行比较。进一步将EP-HSPN患儿毛细血管内增生的受累肾小球比例按≥50%、＜50%分为DEP-HSPN组（弥漫性毛细血管内增生的HSPN）和非DEP-HSPN组,将2组进行比较。结果：①EP-HSPN组46例,非EP-HSPN组44例,与非EP-HSPN组患儿比较,EP-HSPN组患儿年龄小,起病较急,临床多见关节肿痛、胃肠道受累、肾病水平蛋白尿及低白蛋白血症,更多表现为肾病综合征型;病理上,EP-HSPN组患儿较多呈III级病理表现,新月体比例、毛细血管袢坏死比例高,肾小管间质病变损伤程度轻。②46例EP-HSPN患儿中,DEP-HSPN组13例,非DEP-HSPN组33例,与非DEP-HSPN组患儿相比,DEP-HSPN组患儿有较高的24 h尿蛋白量、血尿素氮,更多表现为肾病水平蛋白尿、低白蛋白血症和肾病综合征型,较少表现为血尿和蛋白尿型。③针对EP-HSPN患儿多采取激素联合环磷酰胺冲击治疗（占50.0%）,非EP-HSPN患儿则较多采用对症治疗（占47.7%）。随访结果中,2组患儿在随访时间和疾病转归上差异无统计学意义。结论：EP-HSPN患儿临床表现和肾小球病理改变较重,早期积极予以激素联合免疫抑制剂干预可获得良好的短期疗效。     

38例儿童特发性IgM肾病临床与病理分析

目的 探讨小儿IgM肾病的临床病理特点及预后。方法 收集整理同济医院38例IgM肾病患儿病例，对其临床表现、病理及疗效进行分析。结果 IgM肾病多表现为肾病综合征(17例)，其次为单纯性血尿(13例)，也可表现为持续性蛋白尿、血尿伴蛋白尿。38例均可见IgM弥漫性沉积于肾小球系膜区，31例(81．58％)可见电子致密物的沉积，20例(52．63％)存在球囊粘连，9例(23．68％)伴有间质炎性细胞浸润。17例肾病综合征者经激素或激素加免疫抑制剂治疗后均可完全缓解。13例单纯性血尿者经中药及对症治疗后，肉眼血尿消失，镜下血尿有不同程度的减轻但仍持续存在。随访25例，21例复发(84％)但无患儿发生肾功能不全，其预后有待进一步观察。结论 儿童IgM肾病有其独特的临床病理特征及预后，应将其作为一种独立的疾病，以便更好地指导临床治疗。     

Proliferative glomerulonephritis and primary antiphospholipid syndrome

Little is known regarding the association of primary antiphospholipid syndrome APLS and proliferative glomerulonephritis GN. We describe a biopsy-documented case with primary APLS and proliferative GN with no evidence of thrombotic microangiopathy TMA, and in the absence of other manifestations of systemic lupus erythematosus SLE. She presented initially with left popliteal deep venous thrombosis and nephrotic syndrome. Her first pregnancy at the age of 26 years resulted in intra-uterine fetal death at term. Two subsequent pregnancies ended up with miscarriages at 3 and 4 months of gestation. Urinalysis revealed glomerular red blood cells of 1.0000.000/ml and granular cast; proteinuria of 13.4 grams/24 hours, which was non-selective; hemoglobin 12 gm/dl, normal white blood cell and platelets; serum albumin 2.6 gm/dl; anti-nuclear antibody ANA and anti DNA were negative and complement levels normal. Lupus anticoagulant was positive leading to a diagnosis of primary APLS. The biopsy findings were consistent with membranoproliferative GN. She continued to have steroid-resistant proteinuria, but stable renal function after a 12-year follow up period. She had 2 pregnancies during this period and was delivered at term using caesarian section. She received heparin during the pregnancies. Later she developed hypertension easily controlled by atenolol. This case provides evidence that primary APLS can be associated with proliferative GN due to immune deposits and not only TMA as previously reported, and in the complete absence of SLE. Performing more renal biopsies in this group of patients may disclose a greater prevalence of proliferative GN and may help in devising a rationale for treatment.     

新疆地区无症状镜下血尿患者158例分析

目的探讨新疆地区无症状镜下血尿患者临床与病理特点及其意义。方法对158例接受经皮肾活检的无症状镜下血尿患者进行临床及病理资料分析。结果本组158例患者中,非IgA系膜增生性肾小球肾炎者最多见79例（50%）,IgA肾病54例（34.2%）,局灶节段性硬化性肾炎15例（9.5%）,其次为毛细血管内增生性肾炎10例（6.3%）。汉族患者原发性肾小球疾病病理类型以IgA肾病为主（26.0%）,其次为非IgA系膜增生性肾小球肾炎（14.6%）;维吾尔族原发性肾小球疾病患者病理类型以非IgA系膜增生性肾小球肾炎为主（35.4%）其次为IgA肾病（8.2%）。结论无症状镜下血尿患者的病理类型为原发性肾小球疾病,以男性青壮年为主,其中汉族患者以IgA肾病为主,维吾尔族患者以非IgA系膜增生性肾小球肾炎为主。