慢性肾功能衰竭患者肾活检的临床意义

目的：对慢性肾功能衰竭（ＣＲＦ）患者肾活检的临床意义进行评价。方法：对２２２例临床诊断为ＣＲＦ,ＳＣｒ〉１７８μｍｏｌ／Ｌ（２ｍｇ／ｄｌ）的口才行肾活检术,分析其病理类型、标本合格率、穿刺成功率、并发症发生率及并发症的危险因素。根据肾穿后有无并发症,将患者分为并发症且和无并发症组两组,对并发症影响因素进行统计学分析。结果：肾活检后能明确诊断者占８９．１％,其中ＩｇＡ肾病、血管炎、慢性间质性肾炎、狼     

Clinicopathological study of nephropathy in patients with rheumatoid arthritis]

We carried out a retrospective study to investigate the clinical and pathological findings in 31 patients with rheumatoid arthritis (RA). In clinical findings, 17 patients showed nephrotic syndrome, five had isolated proteinuria, two had proteinuria and hematuria and seven had renal failure. In pathological findings, there were 16 patients with membranous nephropathy (MN), two with proliferative glomerulonephritis (DPGN), two with minor glomerular abnormality (MGA), six with amyloidosis, 2 with tubulointerstitial nephritis, and three patients had accompanying lupus nephritis. Eleven of 16 with MGN had been treated with gold, bucillamine or D-penicillamine, so they were diagnosed as drug induced MGN. In the other five patients, we could not decide which drugs induced the nephropathy. The 2 cases of MGA were associated with nephrotic syndrome and acute renal failure, which were caused by non-steroidal antiinflammatory drugs. There were two cases of non-Ig A DPGN, which was regarded as the native nephropathy in RA. The three cases with lupus nephritis were diagnosed as systemic lupus erythematosus by the criteria of the American Rheumatism Association (ARA). In conclusion, the nephropathy in patients with RA was varied and renal biopsy was a useful examination.     

Drug-induced interstitial nephritis. Coexistence with glomerular disease

Drug-induced interstitial nephritis is being recognized with increasing frequency. Pharmacologic agents responsible for inducing this entity include antibiotics, diuretics, and nonsteroidal anti-inflammatory drugs. We recently examined five patients with glomerular disease and drug-induced interstitial nephritis. In three patients prior biopsy specimens documented their glomerular disease (membranous nephropathy, crescentic glomerulonephritis, and presumptive lipoid nephrosis). A second biopsy specimen showed acute interstitial nephritis and the glomerular lesion. Two additional patients had single biopsy specimens demonstrating acute interstitial nephritis and either membranous nephropathy or crescentic glomerulonephritis. Our cases emphasize the need for recognizing this complex pattern of renal disease and the difficulties encountered in rendering a proper diagnosis.     

Renal biopsy and diagnosis of angiitis. 13 cases

The renal biopsies of 13 patients presenting with a predominantly renal form of angiitis were reviewed. The principal lesions were glomerular with a segmental and focal extracapillary glomerular nephritis in all cases. Arteriolar lesions with necrosis or granuloma were inconstant (6/13) but necrosis of the tuft reflecting capillary involvement were common (11/13). Interstitial tubular disease and immunofluorescence were not specific. Renal biopsy is a good indication of diagnostic when performed early before the development of sclerosis: arteriolar necrosis and suggestive changes: extracapillary segmental and focal glomerulonephritis. In the latter case, a careful search for extrarenal involvement with guided biopsy studies usually allow a diagnosis to be confirmed. The renal and extrarenal clinical signs, the biochemical changes, treatment and outcome were analysed. Two of the 13 patients had Wegener's disease, one patient had angiitis and linear fixation of IgG along the glomerular and tubular basal membranes.     

Membranoproliferative glomerulonephritis associated with autoimmune thyroiditis

Several cases of glomerular disease have been associated to thyroid diseases. The most frequent lesion described is membranous glomerulopathy, presented as a nephrotic syndrome. Here we report a 67-year-old man who developed a nephrotic syndrome accompanied by rapid derangement of renal function shortly after the onset of a primary hypothyroidism due to autoimmune thyroiditis. High titers of circulating anti-thyroglobulin and anti-microsomal thyroid antigen antibodies were detected. Serum levels of C3 and C4 fractions of complement were markedly decreased. Renal biopsy showed a membranoproliferative glomerulonephritis with severe mesangial proliferation, a type of glomerular involvement non-described previously in the literature, in relation with thyroid diseases. Four boluses of intravenous steroids were administered, followed by oral prednisone for three months. A dramatic recovery of renal function, together with normalization of urinary sediment, proteinuria decrease and normalization of serum complement were observed. Three years later, the patient suffered from a similar event, with a positive response to steroids again. One year later, the patient had a new recurrence and was treated with mycophenolate mofetil , improving his clinical situation.     

Nephrotic Syndrome Associated with Buerger's Disease

We herein report a 43-year-old woman with Buerger''s disease who presented with nephrotic syndrome, renal dysfunction, and mild hypertension. A kidney biopsy revealed focal segmental glomerulosclerosis (FSGS), but there were no findings associated with frequent secondary FSGS or a history of long-term hypertension. A small focal renal infarction was seen on ^99mTc-dimercaptosuccinic acid renal scintigraphy, suggesting that FSGS was due to renal microinfarction associated with Buerger''s disease. After the commencement of angiotensin-converting enzyme inhibitor therapy, the hypertension immediately improved, along with significant attenuation of proteinuria. Renal ischemia by vasoconstriction of the glomerular efferent arterioles in association with Buerger''s disease may result in glomerular hyperfiltration followed by FSGS.     

Prognostic value of renal biopsy and clinical variables in patients with lupus nephritis and normal serum creatinine

OBJECTIVE: To evaluate factors with possible influence on the renal outcome in patients with lupus nephritis but without chronic renal insufficiency (CRI). METHODS: Renal biopsies from 94 patients were re-assessed with regard to WHO class, activity, chronicity and tubulointerstitial indices without knowledge of clinical features. The outcome parameters were CRI defined as irreversibly increased serum creatinine and renal end stage disease. RESULTS: The risk ratios (RR) of developing CRI were 2.6 for active urinary sediment, 3.1 for hyaline thrombi and 7.3 for glomerular leukocyte exudation. The RR of renal end stage disease was 5.0 when the duration of renal disease exceeded one year at the time of biopsy and 4.3 when biopsy disclosed a class IV lesion. Glomerular sclerosis was also associated to renal end stage disease. CONCLUSION: Early renal biopsy and the abovementioned signs of active renal disease carry prognostic information that may have significant therapeutic implications.     

170例糖尿病肾病肾活检分析

目的探讨肾活检对糖尿病肾病(DN)的诊断价值及治疗的指导作用。方法对170例伴有微量白蛋白尿(MAU)或临床蛋白尿(CAU)的2型糖尿病患者(T2DM)进行肾穿刺活检，观察肾活检组织病理与各临床参数之间的关系。结果在170例伴MAU或CAU的DM患者中，DN119例(70．0％)，其中早期系膜增生者22例，典型DN表现者97例。在典型DN表现者中，结节性肾小球硬化47例(39．5％)，弥漫性肾小球硬化50例(42．0％)。DN或DM合并其他肾脏病变51例(30．0％)，其中DN合并IgA肾病13例(25.5％)、间质性肾炎8例(15．7％)、膜增生性肾炎2例(3．9％)，DM合并IgA肾病10例(19．6％)、系膜增生性肾小球肾炎4例(7．8%)、微小病变肾炎4例(7．8％)、间质性肾炎6例(11．8％)、膜性肾病2例(3．9％)、新月体性肾炎2例(3．9％)。结论对临床不能确诊的T2DM蛋白尿患者应提倡做肾活检。     

Collapsing focal segmental glomerulosclerosis in a patient with oral cavity cancer: A case report

Rationale:Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerular diseases, leading to end-stage renal disease. Among the 5 variants of FSGS, the collapsing variant is rare and has the worst prognosis. Solid and hematologic malignancies are associated with glomerular diseases, such as membranous nephropathy, minimal change disease, and FSGS. However, squamous cell carcinoma of the oral cavity is rarely associated with nephrotic syndrome, especially FSGS.Patient concerns:A 55-year-old woman diagnosed with oral cavity cancer presented with generalized edema with heavy proteinuria and renal dysfunction after neoadjuvant chemotherapy and wide surgical excision.Diagnosis:Renal biopsy shows segmental or global collapse of glomerular capillaries with marked hyperplasia and swelling of overlying epithelial cells, suggesting a collapsing variant of FSGS.Interventions:After the renal biopsy, we prescribed oral prednisolone at a dose of 1 mg/kg/day. Despite immunosuppressive treatment, renal function deteriorated, and hemodialysis was started.Outcomes:After 23 sessions of hemodialysis and high-dose oral glucocorticoid treatment, renal function gradually improved, and oral glucocorticoid therapy was discontinued after 8 months. Currently, this patient is in a cancer-free state and has normal renal function without proteinuria.Lessons:Unusual collapsing FSGS might be associated with neoadjuvant chemotherapy and wide surgical excision in patients with oral cavity cancer. Proper diagnostic workup, such as renal biopsy and high-dose glucocorticoid therapy, might have helped recover from nephrotic syndrome and acute renal injury in cancer patients.     

1217例肾活检患者临床与病理资料分析

目的 了解行肾穿刺活检患者的流行病学特点及病理类型与临床表现间的关系.方法 回顾性分析1217例行肾活检者的性别、年龄、病理类型及临床表现等相关资料.结果 1217例患者行肾活检时的平均年龄为（30.86±13.41）岁,男性612例（50.3％）,女性605例（49.7％）.男性占本组原发性肾小球疾病（PGN）的52.2％,继发性肾小球疾病（SGN）的39.0％.肾脏病患者的高发年龄段为20 ～39岁（50.9％）.本组患者最常见的临床表现为肾病综合征（NS）610例（50.1％）,其余分别为尿检异常型（Uab）453例（37.2％）,反复发作性肉眼血尿型（rGH）89例（7.3％）,慢性肾衰竭（CRF）31例（2.5％）,急性肾损伤（AKI） 12例（1.0％）,高血压型（HT）11例（0.9％）,急性肾炎综合征（ANS）8例（0.7％）,孤立性肉眼血尿型（iGH）3例（0.2％）.原发性肾小球疾病以系膜增生性肾炎（MsPGN）,继发性肾小球疾以狼疮性肾炎（LN）为主要病理类型.本组患者中各临床分型均以系膜增生性肾炎为主要病理类型,除系膜增生性肾炎外,肾病综合征以MN,Uab以IgAN为主要病理类型.结论 原发性肾小球疾病是徐州地区最常见的肾脏疾病,男性、青壮年是高发人群,系膜增生性肾炎是最常见病理类型,肾病综合征是最常见的临床表现.     

Nondiabetic renal disease in patients with diabetes mellitus

Renal diseases other than diabetic nephropathy were found in 10 of 122 diabetic patients who underwent renal biopsy between 1960 and 1982. These diseases included lupus glomerulonephritis, acute post-streptococcal glomerulonephritis, membranoproliferative glomerulonephritis (type I), focal glomerulosclerosis, idiopathic membranous nephropathy, and nonspecific immune complex glomerulonephritides. Because some of these disorders can alter the management and prognosis of renal disease in diabetic patients, the appearance of urinary abnormalities or deterioration in renal function inconsistent with the natural history of diabetic nephropathy raises the possibility of a nondiabetic renal disease and should lead to a more detailed evaluation.     

Membraneous glomerulonephritis and non-Hodgkin's lymphoma in a patient with primary Sjögren's syndrome

The most common renal manifestation of Sj?gren's syndrome is tubulointerstitial nephritis, and glomerular disease is rare (3). A 62-year-old woman with primary Sj?gren's syndrome developed nephrotic syndrome. Kidney biopsy was consistent with membraneous glomerulonephritis. Steroid pulse therapy was not effective. Three months later she was diagnosed with non-Hodgkin's lymphoma of the tongue, and she was given CHOP therapy and radiation. Both the lymphoma and membraneous glomerulonephritis were resolved.     

Effects of steroids in focal segmental glomerulosclerosis in a predominantly African-American population

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a common primary glomerulopathy in African Americans. Prolonged treatment with steroids is recommended for FSGS in those with nephrotic-range proteinuria, but strong evidence for this recommendation, especially in African-American adults, is lacking. We reviewed our experience with steroids in FSGS in a predominantly African-American cohort. METHODS: Patients with primary FSGS were identified and their charts were retrospectively reviewed for demographic data, characteristics of renal biopsy, blood pressure, and use of steroids. End-stage renal disease and doubling of creatinine were end-points. RESULTS: Seventy-two patients (65 African Americans) were identified with 48.3 months of follow-up. Patients receiving steroids (n=43) had higher urine protein excretion than those who did not. Seventeen patients reached end-stage renal disease and 26 doubled their creatinine concentration. Factors significant for renal survival on Cox proportional hazards model were initial creatinine level, severity of renal lesion, and blood pressure over the follow-up period. Treatment with steroids did not affect renal survival. About one third of patients receiving steroids developed complications consisting of diabetes (n=4) and greater than 5 kg weight gain (n=10). CONCLUSION: Renal function, severity of the renal lesion, and blood pressure determine renal survival in FSGS. A beneficial effect of steroids was not observed in this predominantly African-American adult cohort.     

The effect of normalization of serum complement and anti-DNA antibody on the course of lupus nephritis: a two year prospective study

A prospective study was carried out in 25 patients with systemic lupus erythematosis (SLE) on the effect of normalizing serum complement (CH50) and anti-DNA antibodies on the course of lupus nephritis. In 16 of the 25 patients, CH50 was maintained within the normal range for two years. Urinary protein excretion increased or remained low in all 16. Repeat renal biopsies were performed in 10 of these 16, and disclosed either stabilization of glomerular disease or diminution. In the nine patients in whom CH50 could not be normalized with tolerated doses of drugs, urinary protein excretion increased or remained increased. Repeat renal biopsies in six of these nine patients were carried out and showed worsening of glomerular disease in five. No clear-cut correlation was found between urinary protein excretion or renal disease and the serum levels of anti-DNA antibody. We conclude from these observations that continuous normalization of CH50 by drug therapy in patients with SLE is associated with stabilization or diminution of lupus nephritis.     

肾小球疾病间质泡沫细胞的分布及其与临床指标的关系

目的观测各种病理类型肾小球疾病间质泡沫细胞的分布特点及其与临床参数间的关系。方法选取2862名肾活检患者为研究对象,观察间质泡沫细胞浸润常见的病理类型及泡沫细胞的分布特点。对诊断明确的Aploa综合征（AS）5例,膜增生性肾小球肾炎（MPGN）28例,局灶节段硬化性肾小球肾炎（FSGS）144例,特发性膜性肾病（IMN）132例,IgA肾病（IgAN）893例按间质是否存在泡沫细胞进行临床参数的比较。结果（1）非继发性肾小球疾病泡沫细胞浸润高发的病理类型依次为AS（100％）、MPGN（46．43％）、FSGS（21．32％）、IMN（13．64％）、IgAN（6．69％）;（2）泡沫细胞浸润组24小时尿蛋白定量、血胆固醇水平以及反映肾小管功能损伤的指标均高于无泡沫细胞组（P〈0．05）。结论肾间质泡沫细胞浸润常见于AS,但在MPGN、FSGS、IMN和IgAN患者中均可出现,大量蛋白尿与高脂血症是导致泡沫细胞浸润的两大因素。间质泡沫细胞的浸润与间质损害有一定的关联。     

木通中毒的肾脏损害

目的;了解中药木通中毒肾脏损害患者的临床病理特点及近期预后。方法：回顾性分析３例木通中毒肾脏损害患者的临床表现,肾活检病理资料及转归情况。结果：３例患者均是在服用１剂木通汤剂后发病,其中２例急性中毒,１例在服药６月才明显诊断,早期主要临床表现为：（１）消化道症状,非少尿型肾功能减退及低钾血症。（２）肾脏损伤除肾功能减退外,３例都有尿ＮＡＧ酶及溶菌酶升高,尿渗量降低及尿酸化功能异常,２／３例有肾性糖     

Renal manifestations of sarcoidosis. A report of nine cases

PURPOSE: Clinical renal outbreaks occurring in the course of sarcoidosis are polymorphous. METHODS: Nine patients presenting with sarcoidosis were followed up for 18 years. RESULTS: Five patients presented with chronic interstitial nephritis. Renal failure accompanying granuloma was also present in three of them. Corticotherapy allowed rapid improvement in renal function in three patients. In two other cases, late treatment prevented recovery and led to end-stage renal failure in one case. In another case, persistent hypercalciuria was responsible for bilateral nephrolithiasis further treated via extracorporeal lithotrity. One case of mesangial glomerulonephritis and two morbid associations (retroperitoneal fibrosis and Henoch-Sch?nlein purpura) were observed. CONCLUSION: Interstitial nephritis is still a severe clinical renal outbreak. Corticotherapy must be prescribed early to avoid renal failure. Calcium metabolism disorders are frequent and often combined with interstitial nephritis. Hypercalcemia can often and rapidly be improved via corticotherapy, while monitoring of hypercalciuria proves to be more difficult. Membranous glomerulonephritis is still the most frequently reported glomerular lesion.     

Renal dysfunction in HIV-1-infected patients

Improved therapy directed against opportunistic infection and HIV-1 itself has resulted in greatly enhanced patient survival in the past decade among patients infected with HIV-1. Since patients are living longer, HIV-1 infection is associated with a rising burden of kidney disease. Approximately 14% of black patients and 6% of white patients dying with HIV-1 infection in 1999 in the United States had renal disease. Overall, 10% of patients dying with HIV-1 infection had renal failure. The most common glomerular diseases are focal segmental glomerulosclerosis and immune complex glomerulonephritis. Appropriate therapy for focal segmental glomerulosclerosis includes effective antiretroviral therapy and angiotensin antagonist medication. Drug toxicity is also common, often manifesting as electrolyte abnormalities, acute renal failure, interstitial nephritis, or nephrolithiasis. In particular, indinavir is associated with crystalluria, nephrolithiasis, interstitial nephritis, and lower urinary tract inflammation. Appropriate screening for renal disease and appropriate intervention will likely reduce the morbidity and mortality associated with progressive renal disease.     

Membranous Glomerulonephritis with ANCA-Associated Necrotizing and Crescentic Glomerulonephritis

Background and objectives: Only rare cases of concurrent membranous glomerulonephritis (MGN) and antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis (NCGN) have been reported.Design, setting, participants, & measurements: The authors report the clinical and pathologic findings in 14 patients with MGN and ANCA-associated NCGN.Results: The cohort consisted of eight men and six women with a mean age of 58.7 yr. ANCA positivity was documented by indirect immunofluorescence or ELISA in all patients. Indirect immunofluorescence was positive in 13 patients (seven P-ANCA, five C-ANCA, one atypical ANCA). ELISA was positive in nine of 10 patients (five MPO-ANCA, three PR3-ANCA, one MPO- and PR3-ANCA). Clinical presentation included heavy proteinuria (mean 24-hr urine protein 6.5 g/d), hematuria, and acute renal failure (mean creatinine 4.4 mg/dl). Pathologic evaluation revealed MGN and NCGN, with crescents involving a mean of 32% of glomeruli. On ultrastructural evaluation, the majority of cases showed stage I or II membranous changes. Follow-up was available for 13 patients, 12 of whom were treated with steroids and cyclophosphamide. At a mean follow-up of 24.3 mo, five patients progressed to ESRD, seven had stabilization or improvement in renal function, and one had worsening renal function. Five patients, including three with ESRD, died during the follow-up period. The only independent predictor of progression to ESRD was serum creatinine at biopsy.Conclusions: MGN with ANCA-associated NCGN is a rare dual glomerulopathy seen in patients with heavy proteinuria, acute renal failure, and active urine sediment. Prognosis is variable, with 50% of patients reaching endpoints of ESRD or death.Membranous glomerulonephritis (MGN) is the most common cause of nephrotic syndrome in white adults, accounting for more than one third of cases (1). At the time of presentation, the majority of patients with MGN have preserved renal function (2). Microscopic evaluation of the urine sediment reveals microscopic hematuria in approximately 50% of cases; however, red blood cell casts are not a feature of this disease. Pathologically, MGN is characterized by the formation of subepithelial immune complex deposits with resultant changes to the glomerular basement membrane (GBM), most notably GBM spike formation. Approximately 75% of cases of MGN are thought to represent primary disease, whereas the remaining 25% of cases represent secondary forms of MGN, most commonly related to systemic lupus erythematosus (SLE), infection (i.e., hepatitis B or C virus), malignancy, or drugs. The natural history of MGN is variable, with approximately one third of patients progressing to ESRD within 10 yr (2).Pauci-immune necrotizing and crescentic glomerulonephritis (PNCGN) is characterized by glomerular necrosis and crescent formation in the absence of significant intracapillary proliferation and in the presence of no more than a “paucity” of glomerular immune complex deposits. The majority of patients with PNCGN, with or without associated systemic vasculitis, have circulating antineutrophil cytoplasmic antibodies (ANCAs), which have been directly implicated in the pathogenesis of this form of glomerular injury. In contrast to patients with MGN, those with PNCGN typically present with rapidly progressive glomerulonephritis (RPGN) and an active urine sediment with red blood cell casts (3,4). PNCGN is an aggressive disease with a 1-yr mortality rate of up to 80% in the absence of immunosuppressive therapy. The prognosis of PNCGN is dramatically improved by immunosuppressive regimens that include corticosteroids and cyclophosphamide (CY).The occurrence of ANCA-associated NCGN and primary MGN in the same patient is rare, with only a handful of reports in the literature (5–10). Herein, we detail the clinical, pathologic, and outcome data of 14 patients with this rare dual glomerulopathy and review the previously reported cases.