尿激酶治疗肾炎和肾病综合征的疗效

全国16所科研、医疗机构于1980年4月～1981年8月共16个月时间,用尿激酶(Urokinase)治疗123例原发性及继发性肾疾患者,其中原发性慢性肾炎46例。原发性肾病综合征64例、狼疮性肾炎5例、紫癜性肾炎4例、急进性肾炎3例、结节性多动脉炎肾损害1例,肾脏病理形态学的改变是轻微肾小球肾炎7例、微小病变形肾病综合征8例、增殖性肾炎27例、伴有局部半月体形成(<50%)的增殖性肾炎21例,半月体>50%者3例、腺性肾病35例、膜性增殖性肾炎13例、硬化中的肾小球肾炎4例、局灶性肾小球肾炎3例、局灶性肾小球硬化2例。     

耐甲氧西林金黄色葡萄球菌败血症相关肾损害——附5例报道

目的 提高对耐甲氧西林金黄色葡萄球菌(MRSA)败血症相关性肾损害的认识。方法 对我院收治的5例MRSA败血症肾脏损害患者进行临床、病理及免疫学特点分析。结果：MRSA败血症肾脏损害的主要临床表现为急性肾炎综合征、急进性肾炎综合征，均并发急性肾功能衰竭。2例患者接受肾活检，显示增生性肾小球肾炎伴反应性小管间质肾炎。免疫学改变为血清IgG升高，C3、C4下降。结论 积极有效控制感染是治疗MRSA败血症肾脏损害的关键。     

药物性肾损害53例临床分析

目的总结药物性。肾损害患者53例,旨在提高对该病的认识。方法对本科1995至2005年药物性。肾损害患者的临床资料进行回顾分析。结果53例中40岁以下者17例,40岁以上者36例,其中60岁以上者23例。致病药物中抗菌素32例,中药6例,解热镇痛药、避孕药、造影剂、抗肿瘤药、奥美拉唑、阿昔洛韦、卡托普利共15例。临床表现为急性间质性肾炎综合征21例,急性肾小管坏死11例,急进性。肾炎综合征4例,肾病综合征7例,慢性间质性。肾炎综合征9例,过敏性紫癜性。肾炎1例。死亡3例,转为慢性肾损害10例,其余患者均临床痊愈。结论中老年人为药物性。肾损害的高危人群;抗生素。肾损害特别是头孢类抗生素。肾损害发生率上升到所有药物之首。停用致病药物和支持治疗是公认的治疗方法,而对于少数表现为急进性。肾炎综合征、肾病综合征、过敏性紫癜性。肾炎的患者,甲基泼尼松龙加环磷酰冲击治疗后继以肾上腺皮质激素口服也不失为抢救。肾脏的重要方法。     

原发性肾病综合征并发急性肾损伤48例临床病理分析

目的探讨原发性肾病综合征合并急性肾损伤的临床及病理特点,提高此类并发症的防治水平。方法对我院原发性肾病综合征合并急性肾损伤患者的临床和病理改变进行回顾性分析。结果原发性肾病综合征合并急性肾损伤的临床特征表现为大量蛋白尿、高度水肿,常合并胸腹腔积液。肾脏病理类型：系膜增生性肾小球肾炎、肾小球微小病变及IgA肾病多见。其中系膜增生性肾小球肾炎22例,占46%;微小病变型10例,IgA肾病9例。所有患者均依据病理分型给予激素和（或）细胞毒药物,同时行利尿、控制感染、抗凝等综合治疗,其中5例进行血液透析治疗,肾损伤大多好转,但增生硬化型肾炎等预后较差。结论原发性肾病综合征并发急性肾损伤临床并不少见,多发生于系膜增生性肾小球肾炎、肾小球微小病变及IgA肾病,尽早明确病理诊断和去除诱因,并予相应治疗,大多患者预后良好,肾功能可恢复正常。     

单中心肾活检临床和病理特点分析

目的:总结和分析单中心肾脏疾病的病理类型和临床特征。方法:回顾性分析2013年6月1日～2018年5月31日在南充市中心医院行肾活检患者肾脏病理类型及临床分型的特点。结果:283例肾活检患者中,男女比例为1∶1. 18,发病高峰年龄为41岁～65岁。原发性肾脏病211例(74. 56%),其中肾小球疾病208例(73. 50%),常见的病理类型依次为膜性肾病73例(35. 10%)、Ig A肾病61例(29. 33%)、微小病变性肾病54例(25. 96%);继发性肾脏病64例(22. 61%),其中肾小球疾病62例(21. 91%),以狼疮肾炎最常见(35例,占56. 45%)。原发性肾小球疾病的临床类型分别为肾病综合征135例(64. 90%),慢性肾炎综合征52例(25. 00%),无症状血尿和/或蛋白尿19例(9. 13%),急性肾炎综合征1例(0. 48%),急进性肾炎综合征1例(0. 48%)。结论:南充地区肾脏疾病多见于中青年,原发性肾脏病是本地区最常见的肾脏疾病,原发和继发性肾脏病均以肾小球疾病最常见。膜性肾病是原发性肾小球疾病最常见的病理类型。狼疮肾炎是最常见的继发性肾脏病,且病理类型以Ⅳ-G(±V)型最常见。     

利福平相关性移植肾急进性肾炎一例

不少文献报道,利福平可诱发自体肾(native　kidneys)的急性肾功能衰竭、急性间质性肾炎和急进性肾小球肾炎(RPGN)等急性肾损害。我院发现1例应用利福平抗结核诱发移植肾RPGN,停用利福平后完全恢复正常的病例。报告如下。     

149例急进性肾小球肾炎临床病理特点分析

正急进性肾小球肾炎是一组临床上表现为急性肾炎综合征伴肾功能迅速恶化,病理以新月体形成为特点的肾小球疾病。该病起病急、进展快、预后差,如果诊断治疗不及时,大部分病人在数周或数月内发展为肾功能衰竭~([1]),而如果能早期诊断、早期治疗可明显改善预后~([2~4])。本研究分析总结了我院149例急进性肾小球肾炎患者的临床及病理特点,旨在提高我们对急进性肾小球肾炎早期诊断和治疗的水平,从而改善患者     

IgA肾病合并急性肾衰竭的临床与病理分析

目的：探讨IgA肾病合并急性肾衰竭（ARF）的临床与病理特点。方法：1992年～2006年经肾活检确诊IgA肾病合并ARF 20例患者的临床与病理资料进行回顾性分析。根据不同病理选择治疗方案并进行随访。结果：本组20例IgA肾病合并ARF,占活检IgA肾病的3.8%（20/527）。其中急性肾炎综合征4例,急性肾炎综合征合并肾病综合征5例,以浮肿、少尿为主8例,以恶性高血压为主3例。病理改变上系膜增生性肾炎5例,新月体肾炎8例,增生硬化性肾炎伴新月体肾炎3例、轻度系膜增生性肾炎合并急性肾小管间质肾炎4例。14例肾功能恢复正常,4例部分缓解,2例无效性透析治疗后行肾移植。结论：IgA肾病合并急性肾衰竭发生率达3.8%,高于目前文献报道。临床表现多样化,病理表现为多种病理类型。病理轻则预后好,新月体肾炎诊断治疗及时预后好,多数患者肾功能可以恢复正常。因此早期及时肾活检对IgA肾病指导治疗、判断预后有非常重要的价值。     

类风湿性关节炎肾损害的组织病理改变——附4例报告及文献复习

目的 探讨类风湿性关节炎(RA)肾损害的组织病理学改变特点。方法 总结4例RA肾损害的临床病理资料，并结合文献进行分析。结果 4例均呈蛋白尿伴血尿，其中3例肾功能不全，组织病理为IgA肾病2例、节段系膜增生性肾炎及局灶节段性肾小球硬化各1例，文献报道临床表现以蛋白尿、血尿为主，可呈肾病综合征或伴肾功能不全，病理类型以系膜增生性肾炎(包括IgA肾病)、膜性肾病和肾淀粉样变性多见。结论 类风湿性关节炎的组织病理学改变以系镇增生性肾炎最常见，临床表现多样化。     

从干预IgA1异常糖基化角度探讨中医“三焦”论治IgA肾病

正IgA肾病(IgA nephropathy,IgAN)是全球最常见的原发性肾小球疾病,高居我国原发性肾小球疾病的首位(43.5%)[1]。该病临床及病理表现多样,预后相差甚远,较轻者仅表现为无症状性的镜下血尿、蛋白尿,重者可表现为肾病综合征、急进性肾炎,其中约有36%患者在20年内发展为终末期肾脏病(end-stage renal disease,ESRD)[2]。     

Crescentic glomerulonephritis in a child with infective endocarditis

Renal manifestations associated with infective endocarditis (IE) may present with different clinical patterns, and the most common renal histopathological finding is diffuse proliferative and exudative type of glomerulonephritis, leading to hematuria and/or proteinuria. Renal failure due to crescentic glomerulonephritis (CGN) in children with IE is a very rare condition. We report here a 6-year-old boy, who had a history of cardiac surgery for pulmonary atresia and ventricular septal defect, presenting with the clinical findings of IE and hematuria associated with renal failure due to CGN. He was treated with a combination of intravenous (IV) methylprednisolone pulses and appropriate antibiotics, but also received one dose of IV cyclophosphamide. Complete serological, biochemical, and clinical improvement was achieved after 2 months of follow-up. Antibiotic therapy is the essential part of the treatment of IE-associated glomerulonephritis; however, this case also highlights the importance of aggressive immunosuppressive therapy to suppress the immunological process related with infection in this life-threatening condition leading to renal failure.     

Clinicopathological study of patients presenting hematuria and proteinuria by renal biopsy

We performed 308 series renal biopsies during 4 years (1985-1989) and 289 cases were examined by light microscopic, electron microscopic, or immunofluorescent study. Clinically, chronic nephritic syndrome was most frequent (55.4%), followed by nephrotic syndrome (15.1%), and recurrent or persistent hematuria (12.8%). Pathologically, IgA nephropathy was most popular (39.3%), followed by normal glomerulus (9.1%), and thin basement membrane disease (8.7%). Glomerulonephritis clinically recognized with recurrent or persistent hematuria, hardly showing proteinuria, in 81.6% of the cases, consisted of normal glomerulus, or thin basement membrane disease by electron microscopic and immunofluorescent examinations. The remainder (18.4%) was with IgA nephropathy, which was histologically mild. On the other hand, cases of chronic nephritic syndrome (latent type) with persistent proteinuria and hematuria were with glomerulonephritis of various types including IgA nephropathy in 78.8% of the total cases. Therefore, proteinuria is an important sign of glomerulonephritis. In investigation in different age groups, IgA nephropathy was seen in about 40% of both pediatric and adult cases, whereas minor glomerular abnormalities and thin basement membrane disease were more frequent in pediatric cases. Tubulo-interstitial lesions and glomerular lesions in vascular or metabolic diseases were recognized more in adults than in children. Membranous glomerulonephritis (17 cases including 4 pediatric cases), complicated with malignant tumors such as bladder or rectal cancers and hepatoma was found in 3 aged patients. Examination for malignant tumor would be necessary for aged patients with membranous glomerulonephritis. As for the prognosis of IgA nephropathy, because histological changes of IgA nephropathy varied widely from very mild state to severe state, the prognosis is not always good.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pathology, clinical presentations, and outcomes of C1q nephropathy

C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change-like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change-like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex-mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.     

Membranoproliferative glomerulonephritis in congenital portosystemic shunt without liver cirrhosis

We report two cases with congenital portosystemic shunt who developed renal lesions without liver cirrhosis. Clinically, both cases showed proteinuria and mild hematuria at 9 and 6 years of age, respectively. In one case, the renal lesion was associated with normal renal function, but nephrotic syndrome followed by chronic renal failure were noted in the other. Renal biopsies showed characteristic histological features of membranoproliferative glomerulonephritis (MPGN) with IgA deposits along the glomerular capillary wall. Our cases strongly suggest the association between congenital portosystemic shunt and renal region. The shunt ratio may be an important predisposing factor for this type of nephropathy since a high shunt ratio (> 90%) was noted in both cases.     

Prognostic factors in children with membranoproliferative glomerulonephritis type I

The clinical outcome of patients with membranoproliferative glomerulonephritis (MPGN) varies, with some patients progressing to end-stage renal disease. The aim of this retrospective study was to analyze the initial clinical signs and laboratory test results associated with an MPGN prognosis. The study cohort consisted of 47 patients with idiopathic MPGN Type I treated at the National Institute of Pediatrics, Mexico City, between 1971 and 2001. The median follow-up was 3 years. The three different outcomes of interest were death, renal failure, and nephrotic syndrome. The patients’ ages ranged between 4 and 16 years. All patients had different degrees of proteinuria, hyperlipidemia, and microscopic/macroscopic hematuria, and 85.1% of them showed hypocomplementemia. Clinical outcomes varied, however, the most common was nephrotic syndrome, either alone or combined with other syndromes, which accounted for 74.5% of all cases. Fifteen patients died. Treatment with methylprednisolone improved the patient’s condition, while the use of chloroquine or cyclophosphamide worsened it. Twenty-two patients had some degree of renal failure; glomerular filtration rate (GFR) levels and albumin values were negatively associated to renal failure, while treatment with methylprednisolone decreased the probability of renal failure. Nephrotic syndrome persisted in 18 patients; hemolytic complement and hemoglobin values were negatively associated with nephrotic syndrome, while macroscopic hematuria was positively associated with it. Signs that suggested a poor prognosis during diagnosis were low GFR, low albumin, low hemolytic complement, and macroscopic hematuria. Treatment with methylprednisolone seemed to improve prognosis, however, this needs to be confirmed with randomized studies.     

Spontaneous thrombosis of the renal vessels. Rare entities to be considered in differential diagnosis of patients presenting with lumbar flank pain and hematuria.

We report 2 cases of spontaneous thrombotic occlusion of the main renal vessels presenting with acute lumbar flank pain and hematuria suspect of nephrolithiasis. Clinical and laboratory signs of blood hypercoagulability, generalized arterial embolism, nephrotic syndrome or glomerulonephritis were absent. Excretory urography, nephrosonography and retrograde ureteropyelography showed no evidence of upper urinary tract calculi or other causes of obstruction. Renal angiography and cavography demonstrated an acute renal vein thrombosis in 1 patient and a thrombotic occlusion of all but one of the segmental renal arteries in the other patient. These 2 cases demonstrate that thrombotic occlusion of the renal artery or renal vein has to be considered in patients who are presenting with lumbar flank pain and hematuria, in whom the excretory urogram shows severe malfunction of one of the kidneys, and stone disease can be excluded. Renal angiography and cavography as well as CT scan should be carried out in these patients. When the disease is diagnosed at an early stage, an intra-arterial thrombolysis can be attempted.     

Hemolytic uremic syndrome associated with glomerular disease

Secondary hemolytic uremic syndrome (HUS) is uncommon. When it occurs, it is usually in association with pregnancy, malignancy, severe hypertension, drugs, or collagen vascular diseases. It has rarely been reported in patients with glomerular disease. Two such patients with secondary HUS are described. A 17-month-old girl with hematuria and the nephrotic syndrome, negative antistreptolycin O (ASO) titer, and low serum levels of C3 and C4 developed oliguria, progressive azotemia, thrombocytopenia, and microangiopathic hemolytic anemia. A kidney biopsy showed fibrin in glomerular capillaries and cresentic membranoproliferative glomerulonephritis. A 22-year-old man with a 16-year history of relapsing minimal change nephrotic syndrome had been in remission for 5 years when he experienced nephrotic syndrome relapse and developed thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. A kidney biopsy revealed foot process fusion and obstruction of glomerular capillaries with fibrin and platelets. These cases illustrate that HUS can occur in association with other glomerular diseases and should be considered when thrombocytopenia and hemolytic anemia occur in a nephritic or nephrotic patient.     

滥用海洛因合并感染性心内膜炎及急性肾损伤

目的探讨海洛因依赖者感染性心内膜炎合并急性肾损伤的临床特点及预后。方法海洛因依赖者6例,年龄32-46岁,均为男性,均有静注海洛因史,吸毒年限2～10年。行尿吗啡定性、尿蛋白定量、肝功能、肾功能、免疫功能、丙型肝炎病毒抗体、乙型肝炎病毒表面抗原、血培养、心脏彩超及肾活检检查,予抗感染治疗,比较治疗前、后各项指标的变化。结果6例患者均表现为血象升高、贫血、血尿、蛋白尿、低蛋白血症、血肌酐升高、低补体血症、C-反应蛋白升高。丙型肝炎病毒抗体阳性5例,乙型肝炎病毒表面抗原阳性2例,血培养示金黄色葡萄球菌3例。6例患者心脏彩超均示三尖瓣前瓣赘生物形成。肾脏病理表现为中度系膜增生性肾小球肾炎、新月体性肾炎各2例,IgA肾病、毛细血管内增生性肾炎各1例。经治疗后病情均好转,血象、尿蛋白、尿素氮、肌酐明显下降,血清白蛋白明显上升,贫血、肝功能无明显变化。结论滥用海洛因可导致感染性心内膜炎并发肾小球肾炎,常合并肾病综合征、急性肾损伤,病理类型多样。经抗感染治疗后尿蛋白、肾功能明显好转。     

Membranoproliferative glomerulonephritis type I in children: correlation of clinical features with pathologic subtypes

Renal biopsies from 33 patients with membranoproliferative glomerulonephritis (MPGN) type I were reviewed to identify pathologic subtypes of this disease and assess their correlation to clinical features. The patients were divided into two groups: group A included 16 patients in chronic or end-stage renal failure and group B 17 patients with no evidence of renal insufficiency. At presentation, a nephrotic or nephritic syndrome and azotemia were equally common in both groups. The incidence of hypertension was significantly increased in group A (P less than 0.05), while recurrent gross hematuria was more common in group B. Nephrotic syndrome was more common during the course of illness in group A. Three subtypes of MPGN type I were recognized, based on whether duplication of glomerular capillary basement membranes was focal segmental (FS; 9 cases), diffuse global (DG; 18 cases), or mixed segmental and global (6 cases). Eight of nine patients showing FS MPGN type I were in group B (p less than 0.05). In contrast, 11 of 18 patients with DG MPGN type I and 4 of 6 with a segmental and global pattern were in group A (P = not significant). Therefore, FS MPGN is a good predictor of a favorable clinical outcome, whereas the other two subtypes are not. This was confirmed by a 100% actuarial kidney survival for the nine patients with FS MPGN and a 50% kidney survival of 7.5 years for patients with the other two subtypes.     

ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis"

The scleroderma renal crisis is characterized by acute onset of severe hypertension and by rapidly progressive hyperreninemic renal failure. There is, however, a very limited subset of patients with rapidly progressive renal failure who remain normotensive and develop ANCA-positive crescentic glomerulonephritis. We report a case of normotensive acute renal failure secondary to anti-MPO antibody-associated crescentic glomerulonephritis in a patient with diffuse systemic sclerosis. She was referred to our department with normal blood pressure and no extrarenal clinical manifestation ofvasculitis. She presented with rapidly progressive renal failure, microscopic hematuria and minimal proteinuria. P-ANCA were positive by immunofluorescence, with ELISA-confirmed specificity for myeloperoxidase. Renal biopsy revealed typical features of pauciimmune glomerulonephritis with crescent formation and fibrinoid necrosis. The patient was initially treated with i.v. cyclophosphamide only. Because of ongoing deteriorating renal function, additional treatment with intravenous pulses of methylprednisolone followed by oral prednisone was started and allowed renal function improvement. After 9 months, serum creatinine had almost returned to normal level with minimal proteinuria, no hematuria and negative ANCA testing. Control kidney biopsy only revealed scar lesions. The association of ANCA-positive crescentic glomerulonephritis and systemic sclerosis is a very rare event. Treatment with intravenous cyclophosphamide and corticosteroids allows rapid and long-term improvement of renal function. The onset of typical scleroderma renal crisis triggered by high-dose corticosteroids is unlikely but requires a close follow-up of patients with overlapping systemic sclerosis. Diagnosis and treatment are discussed and previously published cases are reviewed.