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Subsequent Transition from Minimal Change Disease to Secondary form of Focal Segmental Glomerulosclerosis: Not a Sampling Error yet Still Amenable to Immunosuppressive Therapy
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作者 Kamel El-Reshaid Shaikha Al-Bader +1 位作者 Hossameldin Tawfik Sallam John Patrick Madda 《Open Journal of Nephrology》 2024年第4期447-453,共7页
Background: Previous studies suggested that the focal and segmental nature of focal segmental glomerulosclerosis (FSGS) may lead to sample error or diagnosis error that results in minimal change disease (MCD) misdiagn... Background: Previous studies suggested that the focal and segmental nature of focal segmental glomerulosclerosis (FSGS) may lead to sample error or diagnosis error that results in minimal change disease (MCD) misdiagnosis, or FSGS being missed especially in patients with early lesions or limited glomeruli in the biopsy specimens. Patients and methods: Over the past 5 years, patients were included in the study if they had (a) relapse of nephrotic syndrome (NS), (b) biopsy-proven FSGS without immune deposits, (c) long-remission (years) after biopsy-proven Corticosteroid-refractory NS due to MCD, (c) negative history, clinical examination, radiological scans as well as laboratory and serological tests for autoimmune diseases, infections, malignancy and drugs-side effect. Results: After 3 months of therapy with Losartan alone;Proteinuria decreased only by 22% improvement with a mild decrease in Creatinine clearance (ClCr) by 1.5%. However, the addition of Mycophenolate mofetil (MMF) resulted in a further significant decrease in Pr to 72% compared to Losartan alone. Moreover, there were no significant changes in CrCl after 1- and 2 years of follow up. Our data indicates that such a transition may not be due to inadequate sampling but a new lesion. Initial hemodynamic therapy with Losartan was not adequate and immunosuppressive therapy with MMF significantly improved proteinuria and stabilized their kidney function. The data is promising with regard to the management of patients with such relentless disease. In conclusion, a true transition from MCD to FSGS is amenable to therapy with MMF. 展开更多
关键词 CORTICOSTEROIDS focal segmental glomerulosclerosis minimal change disease Mycophenolate Mofetil Nephrotic Syndrome TRANSITION
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Transition from minimal change disease to focal segmental glomerulosclerosis related to occupational exposure:A case report
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作者 Long Tang Zhen Cai +1 位作者 Su-Xia Wang Wen-Jing Zhao 《World Journal of Clinical Cases》 SCIE 2022年第17期5861-5868,共8页
BACKGROUND Although minimal change disease(MCD)and focal segmental glomerulosclerosis(FSGS)have been described as two separate forms of nephrotic syndrome(NS),they are not completely independent.We report a case of a ... BACKGROUND Although minimal change disease(MCD)and focal segmental glomerulosclerosis(FSGS)have been described as two separate forms of nephrotic syndrome(NS),they are not completely independent.We report a case of a patient transitioning from MCD to FSGS,review the literature,and explore the relationship between the two diseases.CASE SUMMARY A 42-year-old male welder,presenting with lower extremity edema and elevated serum creatinine,was diagnosed with NS and end-stage kidney disease(ESKD)based on laboratory test results.The patient had undergone a kidney biopsy for NS 20 years previously,which indicated MCD,and a second recent kidney biopsy suggested FSGS.The patient was an electric welder with excessive levels of cadmium and lead in his blood.Consequently,we suspect that his aggravated pathology and occurrence of ESKD were related to metal nephrotoxicity.The patient eventually received kidney replacement therapy and quit his job which involved long-term exposure to metals.During the 1-year follow-up period,the patient was negative for metal elements in the blood and urine and recovered partial kidney function.CONCLUSION MCD and FSGS may be different stages of the same disease.The transition from MCD to FSGS in this case indicates disease progression,which may be related to excessive metal contaminants caused by the patient’s occupation. 展开更多
关键词 minimal change disease focal segmental glomerulosclerosis Occupational exposure CADMIUM LEAD Case report
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Review of plasma exchange and rituximab for prevention of recurrent focal segmental glomerulosclerosis after a prior graft loss
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作者 Sophie Gharaei Hashim Abbas Durga Anil Kanigicherla 《World Journal of Transplantation》 2024年第4期133-139,共7页
BACKGROUND Focal segmental glomerulosclerosis(FSGS)often recurs after transplantation,leading to graft dysfunction and graft loss.Patients who have lost prior grafts due to recurrence are at particularly high risk of ... BACKGROUND Focal segmental glomerulosclerosis(FSGS)often recurs after transplantation,leading to graft dysfunction and graft loss.Patients who have lost prior grafts due to recurrence are at particularly high risk of re-recurrence in subsequent grafts.Rituximab and plasma exchange have been used pre-emptively to prevent post-transplant recurrence.However,the efficacy of such preventative measures remains unclear.AIM To investigate the outcomes of preventative rituximab and plasma exchange for recurrent FSGS in transplant recipients after prior graft loss.METHODS We conducted a systematic review of 11 studies with 32 patients who had experienced prior graft loss due to post-transplant FSGS recurrence and were treated with either pre-emptive plasma exchange alone,rituximab alone,or a combination of both.RESULTS Overall,47%of the 32 patients experienced recurrence despite prophylactic treatment.Re-recurrence was seen in 25%(1/4)with pre-emptive rituximab alone,and 45%recurrence(9/20)with plasma exchange alone.Re-recurrence was noted in 63%with the use of combined plasma exchange and rituximab.CONCLUSION There is a paucity of available evidence in the literature to draw clear conclusions on the benefits of pre-emptive measures to prevent FSGS re-recurrence.The small sample sizes and variations in protocols call for larger and controlled studies to serve this patient population at high risk of recurrence and graft loss. 展开更多
关键词 focal segmental glomerulosclerosis Glomerular disease Plasma exchange RITUXIMAB TRANSPLANTATION
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Collapsing focal segmental glomerulosclerosis: Current concepts 被引量:1
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作者 Muhammed Mubarak 《World Journal of Nephrology》 2012年第2期35-42,共8页
Collapsing focal segmental glomerulosclerosis (cFSGS), also known as collapsing glomerulopathy is currently classified under the rubric of FSGS. However, its de-fining morphological features are in stark contrast to... Collapsing focal segmental glomerulosclerosis (cFSGS), also known as collapsing glomerulopathy is currently classified under the rubric of FSGS. However, its de-fining morphological features are in stark contrast to those observed in most other variants of FSGS. During the early stage of the disease, the lesion is character-ized pathologically by an implosive segmental and/or global collapse of the glomerular capillary tufts, marked hypertrophy and hyperplasia of podocytes, and severe tubulointerstitial disease. With advancement of the disease, segmental and/or global glomerulosclerosis is also observed in association with the collapsing le-sions. The etiology of this enigmatic disorder is still elusive, but a growing list of diseases/conditions is being reported in association with this morphological pattern of renal parenchymal injury. The pathogenesis of cFSGS involves discreet epithelial cell injury leadingto cell cycle dysregulation and a proliferative cellularphenotype. From the clinical perspective, cFSGS is no-torious for its propensity to affect black people, a highincidence and severity of nephrotic syndrome, markedresistance to empirical therapy, and rapid progressionto end-stage renal disease. The lesion has also beenreported in transplanted kidneys either as recurrent orde novo disease, frequently leading to graft loss. Mostcases have been reported in western countries, but the lesion is also being increasingly recognized in the tropi-cal regions. The recent increase in reporting of cFSGS partly refects a true increase in the incidence and part-ly a detection bias. There is no specifc treatment for the disorder at present. Newer insights into the patho-genesis may lead to the development of targeted and specifc therapy in near future. There is an urgent need to increase awareness of the lesion among pathologists and nephrologists, especially those from developing countries, to ensure accurate diagnosis and appropriate managment. With the accumulation of more and more data, it is hoped that the prevailing confusion about the nosological identity of the lesion will also be resolved in a more logical way. 展开更多
关键词 Collapsing focal segmental glomerulosclerosis End-stage renal disease ETIOLOGY PATHOLOGY Renal biopsy PROGNOSIS
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Exploring kidney biopsy findings in congenital heart diseases:Insights beyond cyanotic nephropathy
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作者 Jose Daniel Juarez-Villa Iván Zepeda-Quiroz +7 位作者 Sebastián Toledo-Ramírez Victor Hugo Gomez-Johnson Francisco Pérez-Allende Brian Ricardo Garibay-Vega Francisco E Rodríguez Castellanos Bernardo Moguel-González Edgar Garcia-Cruz Salvador Lopez-Gil 《World Journal of Nephrology》 2024年第1期25-32,共8页
BACKGROUND The association between congenital heart disease and chronic kidney disease is well known.Various mechanisms of kidney damage associated with congenital heart disease have been established.The etiology of k... BACKGROUND The association between congenital heart disease and chronic kidney disease is well known.Various mechanisms of kidney damage associated with congenital heart disease have been established.The etiology of kidneydisease has commonly been considered to be secondary to focal segmental glomerulosclerosis(FSGS),however,this has only been demonstrated in case reports and not in observational or clinical trials.AIM To identify baseline and clinical characteristics,as well as the findings in kidney biopsies of patients with congenital heart disease in our hospital.METHODS This is a retrospective observational study conducted at the Nephrology Depart-ment of the National Institute of Cardiology“Ignacio Chávez”.All patients over 16 years old who underwent percutaneous kidney biopsy from January 2000 to January 2023 with congenital heart disease were included in the study.RESULTS Ten patients with congenital heart disease and kidney biopsy were found.The average age was 29.00 years±15.87 years with pre-biopsy proteinuria of 6193 mg/24 h±6165 mg/24 h.The most common congenital heart disease was Fallot’s tetralogy with 2 cases(20%)and ventricular septal defect with 2(20%)cases.Among the 10 cases,one case of IgA nephropathy and one case of membranoproliferative glomerulonephritis associated with immune complexes were found,receiving specific treatment after histopathological diagnosis,delaying the initiation of kidney replacement therapy.Among remaining 8 cases(80%),one case of FSGS with perihilar variety was found,while the other 7 cases were non-specific FSGS.CONCLUSION Determining the cause of chronic kidney disease can help in delaying the need for kidney replacement therapy.In 2 out of 10 patients in our study,interventions were performed,and initiation of kidney replacement therapy was delayed.Prospective studies are needed to determine the usefulness of kidney biopsy in patients with congenital heart disease. 展开更多
关键词 Renal biopsy Congenital heart disease Chronic kidney disease focal segmental glomerulosclerosis
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Advances in the pathophysiology and treatment of focal segmental glomerulosclerosis:The importance of a timely and tailored approach
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作者 Guido Gembillo Concetto Sessa Domenico Santoro 《World Journal of Nephrology》 2025年第2期1-8,共8页
Focal segmental glomerulosclerosis(FSGS)is a histological pattern of glomerular damage that significantly contributes to chronic kidney disease and end-stage renal disease.Its incidence is rising globally,necessitatin... Focal segmental glomerulosclerosis(FSGS)is a histological pattern of glomerular damage that significantly contributes to chronic kidney disease and end-stage renal disease.Its incidence is rising globally,necessitating timely and personalized management strategies.This paper aims to provide an updated overview of the pathophysiology,diagnosis,and therapeutic strategies for FSGS,emphasizing the importance of early interventions and tailored treatments.This editorial synthesizes key findings from recent literature to highlight advancements in understanding and managing FSGS.Emerging evidence supports the role of targeted therapies and personalized approaches in improving outcomes for FSGS patients.Advances include novel biomarkers,genetic testing,and innovative therapeutics such as transient receptor potential ion channel blockers and antisense oligonucleotides for apolipoprotein 1-related FSGS.Effective mana-gement of FSGS requires a combination of timely diagnosis,evidence-based therapeutic strategies,and ongoing research to optimize patient outcomes and address gaps in the current understanding of the disease. 展开更多
关键词 focal segmental glomerulosclerosis Chronic kidney disease Glomerulonephritis Renal failure Immunosuppressive therapy Calcineurin inhibitors Mycophenolate mofetil Rituximab Sparsentan Plasmapheresis
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基于叶天士络病理论探讨中医药治疗局灶节段性肾小球硬化
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作者 赵京 邢海涛 赵菁莉 《时珍国医国药》 北大核心 2025年第1期109-113,共5页
局灶节段性肾小球硬化是目前最常见的难治性肾病综合征的病理类型之一,目前临床尚无系统治疗方案。文章以叶天士络病理论为基础,通过梳理《临证指南医案》的相关医案,基于“络以通为用”的治疗原则,针对局灶节段性肾小球硬化的病因病机... 局灶节段性肾小球硬化是目前最常见的难治性肾病综合征的病理类型之一,目前临床尚无系统治疗方案。文章以叶天士络病理论为基础,通过梳理《临证指南医案》的相关医案,基于“络以通为用”的治疗原则,针对局灶节段性肾小球硬化的病因病机和疾病特点,从虚、瘀着手,谈及了补虚通络、辛畅通络、虫蚁剃络等方法,为局灶节段性肾小球硬化的治疗提供新的思路。 展开更多
关键词 局灶节段性肾小球硬化 叶天士 络病理论 络脉 中医药
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中药干预相关信号通路治疗局灶节段肾小球硬化型肾病的研究进展
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作者 李维笑 周博 +3 位作者 肖紫煜 张尔昱 李冰 杨小红 《河南中医》 2025年第1期150-156,共7页
局灶节段肾小球硬化(focal segmental glomerulosclerosis,FSGS)型肾病是一种常见的肾小球疾病,是导致终末期肾病的主要原因。中药可通过干预磷脂酰肌醇3激酶(phosphoinositide 3-kinase,PI3K)/蛋白激酶B(protein kinase B,PKB,又称AKT... 局灶节段肾小球硬化(focal segmental glomerulosclerosis,FSGS)型肾病是一种常见的肾小球疾病,是导致终末期肾病的主要原因。中药可通过干预磷脂酰肌醇3激酶(phosphoinositide 3-kinase,PI3K)/蛋白激酶B(protein kinase B,PKB,又称AKT)信号通路、核转录因子-κB(nuclear factor-κB,NF-κB)信号通路、转化生长因子-β(transforming growth factor-β,TGF-β)信号通路、β-catenin信号通路、Notch信号通路、核转录因子红系2相关因子2(nuclear factor-erythroid 2-related factor 2,Nrf2)信号通路、沉默信息调节因子(silence infor-mation regulator,SIRT)信号通路等减少细胞外基质积累和上皮-间质转化,减少活性氧和炎症因子的产生、激活自噬、修复裂孔隔膜相关蛋白减轻FSGS型肾病的足细胞损伤。尽管中药调控相关信号通路治疗FSGS型肾病有很多优势,但目前中药干预FSGS型肾病信号传导的研究还处于前期阶段,且多停留在动物实验阶段,存在研究内容不足、研究方法差异较大、缺乏统一的FSGS型肾病中医指南或共识等问题。今后,应将基础实验与临床研究相结合进行验证,进一步明确中药发挥治疗作用的相关机制,同时,在动物实验中选用中药复方时应根据辨证论治的思维进行选择,更利于提高疗效及作用机制探讨。除此之外,非中药疗法如火龙罐、艾灸、推拿等也对FSGS型肾病治疗有一定的疗效,未来可从非药物疗法的作用机制进行探讨。 展开更多
关键词 局灶节段肾小球硬化型肾病 PI3K/AKT信号通路 NF-κB信号通路 TGF-Β信号通路 Β-CATENIN信号通路 Notch信号通路 中医药疗法
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Efficacy of tacrolimus in the treatment of children with focal segmental glomerulosclerosis 被引量:2
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作者 Mahmoud Kallash Diego Aviles 《World Journal of Pediatrics》 SCIE 2014年第2期151-154,共4页
Background:Focal segmental glomerulosclerosis(FSGS)is the most common glomerular condition leading to end-stage renal disease(ESRD)and the third most common cause of ESRD in pediatric patients.Methods:This is a retros... Background:Focal segmental glomerulosclerosis(FSGS)is the most common glomerular condition leading to end-stage renal disease(ESRD)and the third most common cause of ESRD in pediatric patients.Methods:This is a retrospective study consisting of 22 pediatric patients with FSGS and heavy proteinuria.After demonstrating steroids resistance,the patients were treated with tacrolimus,targeting a trough level 5-8 ng/mL.The primary outcome is the induction of remission with tacrolimus.Results:Thirteen patients(59%)achieved remission(complete in 31.8%and partial in 27.2%)and 12 patients showed stable or improved renal function over an average follow-up of 2.9 years(range:0.5-7 years).There was no significant difference in response rate between African American and Caucasian patients.None of the patients had significant side-effect to tacrolimus and none of the repeat biopsies showed an increase in interstitial fibrosis compared to baseline.The best renal outcome was for patients who achieved complete remission.Partially responsive patients had improved renal function compared with resistant patients.Conclusion:Tacrolimus is a viable option in the treatment of children with idiopathic steroid resistant FSGS. 展开更多
关键词 chronic kidney disease focal segmental glomerulosclerosis(FSGS) PROTEINURIA TACROLIMUS
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TRPC6基因突变患者基因变异特征与临床病理及预后分析
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作者 钟清 张昌明 +3 位作者 朱丽 王钢 张丽华 刘志红 《肾脏病与透析肾移植杂志》 CSCD 2024年第6期501-507,共7页
目的:探讨TRPC6基因突变患者的基因变异特征和临床病理表现。方法:筛选国家肾脏疾病临床医学研究中心经全外显子或肾脏疾病panel基因检测证实存在TRPC6基因突变患者,对患者的基因变异特征、临床病理及预后进行分析。结果:发现10例患者... 目的:探讨TRPC6基因突变患者的基因变异特征和临床病理表现。方法:筛选国家肾脏疾病临床医学研究中心经全外显子或肾脏疾病panel基因检测证实存在TRPC6基因突变患者,对患者的基因变异特征、临床病理及预后进行分析。结果:发现10例患者存在致病性TRPC6基因变异,共检测到TRPC6基因的8个杂合突变,其中6个为错义突变,以c.2683C>T(p.R895C)最为常见。肾脏病家族史阳性者9例。10例患者中,男女各5例,首次就诊年龄16~35岁。患者均以蛋白尿起病,4例起病即表现为肾病综合征(NS)。所有患者均接受过血管紧张素Ⅱ受体拮抗剂(ARB)治疗,5例患者接受足量激素联合钙调神经蛋白抑制剂(CNI)治疗,2例患者接受中等量激素治疗。6例患者于病程1~17年进展至终末期肾病(ESKD)。6例患者行肾活检病理表现均为局灶节段性肾小球硬化(FSGS),肾小球系膜增生不明显,部分患者伴轻至中度的慢性肾小管间质病变,超微结构下足细胞病变突出,足突广泛融合(50%~80%)4例、节段融合(30%~50%)2例,无肾小球基膜病变。结论:TRPC6基因变异以错义突变为主,最常见突变位点为c.2683C>T(p.R895C)。患者均以蛋白尿起病,半数为NS状态,肾脏病理以FSGS为主,足细胞足突融合明显,ARB及CNI治疗效果不佳,多数患者最终进展至ESKD。疾病的早期诊断和及时特异性干预治疗可能是改善预后的关键。 展开更多
关键词 TRPC6基因变异 蛋白尿 局灶节段性肾小球硬化 终末期肾病
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Long-term prognosis of focal segmental glomerulosclerosis treated with therapeutic low-density lipoprotein-apheresis in patients with severe kidney dysfunction and proteinuria
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作者 Shinji Kitajima Megumi Osima +10 位作者 Hisayuki Ogura Shiori Nakagawa Yuta Yamamura Taito Miyake Taro Miyagawa Tadashi Toyama Akinori Hara Norihiko Sakai Miho Shimizu Takashi Wada Yasunori Iwata 《Rheumatology & Autoimmunity》 2023年第1期35-42,共8页
Background:The prognosis of focal segmental glomerulosclerosis patients with nephrotic syndrome is estimated to be 10%-20%in 5 years and 30%-50%in 10 years,leading to end-stage kidney disease.The response rate with st... Background:The prognosis of focal segmental glomerulosclerosis patients with nephrotic syndrome is estimated to be 10%-20%in 5 years and 30%-50%in 10 years,leading to end-stage kidney disease.The response rate with steroid therapy is 40%-60%.Therapeutic low-density lipoprotein-apheresis(LDL-A)may be effective in patients with steroid resistance.Information regarding the long-term prognosis of patients with focal segmental glomerulosclerosis receiving this therapy is scarce.Methods:We investigated the effectiveness of treatment in 50 patients with primary focal segmental glomerulosclerosis diagnosed between 1961 and 2017 at Kanazawa University Hospital and related facilities.The patients were observed at least 12 months after biopsy or until end-stage kidney disease occurrence or death.Results:LDL-A was performed in four patients who presented with steroidresistant nephrotic syndrome(two patients had concurrent acute renal failure for which hemodialysis was performed).In comparison with 17 patients who did not receive LDL-A after 1989,the LDL-A group had higher urinary protein excretion(13.7 vs.5.2 g/day,P=0.053)and serum creatinine(4.11 vs.1.65 mg/dL)levels at onset,and a numerically higher remission rate(75.0%vs.58.7%)compared with the nonlipoprotein-apheresis group.Conclusion:Therapeutic LDL-A can be performed for critical cases and may improve the remission rate. 展开更多
关键词 APHERESIS end-stage kidney disease focal segmental glomerulosclerosis LDL PROGNOSIS
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改良阿霉素肾病大鼠模型的建立 被引量:49
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作者 杨维娜 于琳华 +2 位作者 郭尚温 成少利 柴云 《西安交通大学学报(医学版)》 CAS CSCD 北大核心 2009年第4期445-448,452,共5页
目的探讨阿霉素肾病在不同病理发展阶段动物模型的制作。方法采用间隔2周两次尾静脉注射阿霉素的方法复制大鼠阿霉素肾病模型,观察各组大鼠血、尿生化指标和肾组织病理学的改变。结果该模型表现为严重水肿、大量蛋白尿、低蛋白血症和高... 目的探讨阿霉素肾病在不同病理发展阶段动物模型的制作。方法采用间隔2周两次尾静脉注射阿霉素的方法复制大鼠阿霉素肾病模型,观察各组大鼠血、尿生化指标和肾组织病理学的改变。结果该模型表现为严重水肿、大量蛋白尿、低蛋白血症和高脂血症;病理学检查显示,4周末肾小球足细胞肿胀,系膜细胞轻度增生;8周末有球囊粘连,肾小管上皮细胞萎缩;12周末部分肾小球节段性轻、中度硬化,间质内有明显灶性淋巴细胞浸润,并有轻度纤维化。结论经改良间隔2周两次尾静脉内注射阿霉素4mg/kg,可以成功复制阿霉素肾病模型。急性模型病理学改变类似人的微小病变性肾病,慢性模型病理学改变类似人的局灶节段性肾小球硬化。 展开更多
关键词 阿霉素肾病 足细胞 微小病变性肾病 局灶节段性.肾小球硬化
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肾病综合征患者血浆抗凝血酶Ⅲ检测及其临床意义 被引量:9
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作者 汤曦 王旭方 +5 位作者 张丽华 陈朝红 张炯 张羽 曾彩虹 刘志红 《肾脏病与透析肾移植杂志》 CAS CSCD 北大核心 2010年第5期407-412,共6页
目的:比较分析不同病理类型肾病综合征患者血浆抗凝血酶III(antithrombin Ⅲ,AT-Ⅲ)浓度的差异及相关影响因素。方法:比较微小病变(minimal change disease,MCD,n=26)、局灶节段性肾小球硬化(focalsegmental glomerulosclerosis,FSGS,n=... 目的:比较分析不同病理类型肾病综合征患者血浆抗凝血酶III(antithrombin Ⅲ,AT-Ⅲ)浓度的差异及相关影响因素。方法:比较微小病变(minimal change disease,MCD,n=26)、局灶节段性肾小球硬化(focalsegmental glomerulosclerosis,FSGS,n=26)及膜性肾病(membranous nephropathy,MN,n=20)三组成人肾病综合征患者肾活检术前血尿AT-Ⅲ浓度及临床特点。选取20例健康成人作为正常对照。血尿AT-III浓度采用免疫速率比浊法检测。采用Person、Sperman方法及多元线性回归分析FSGS、MCD患者中影响血浆AT-III浓度的因素。结果:肾活检术时MN组尿蛋白、血清白蛋白、肌酐、总胆固醇、IgG与MCD和FSGS组差异显著(P<0.05);后两组间以上指标的差异无统计学意义(P>0.05)。FSGS组尿C3、α2巨球蛋白、N-乙酰-β-D-葡萄糖苷酶、视黄醇结合蛋白高于MCD和MN组(P<0.05)。26例(100%)MCD、23例(88.46%)FSGS、3例(15%)MN患者血浆AT-Ⅲ降低(P<0.05)。FSGS与MCD患者血浆AT-III浓度分别为(15.64±3.86)mg/dl、(18.10±4.8)mg/dl(P<0.05),均低于MN患者(28.08±4.23)mg/dl(P<0.05)。FSGS、MCD组尿AT-III>5mg/dl者分别为8/20例(40%)、4/22例(18.18%)(P>0.05),而MN组患者尿液AT-III含量均<5mg/dl(P<0.05)。一元回归发现FSGS患者中血浆AT-III浓度与尿蛋白(r=-0.505,P<0.05)负相关、血清白蛋白(r=0.559,P<0.01)正相关;MCD患者中血浆AT-III浓度与尿蛋白(r=-0.429,P<0.05)负相关,与血清白蛋白(r=0.564,P<0.01)、IgG(r=0.529,P<0.01)正相关。多元回归分析提示FSGS、MCD患者中血浆AT-III与血清白蛋白相关(R2=0.312、0.320,P<0.05)。随访中2例A-TⅢ降低的FSGS患者发生静脉血栓。结论:肾病综合征患者血浆AT-III浓度与病理类型有关。FSGS、MCD血浆AT-Ⅲ浓度较MN明显下降,可能源于尿液丢失的差异。 展开更多
关键词 肾病综合征 抗凝血酶Ⅲ 微小病变 局灶节段性肾小球硬化 膜性肾病
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局灶节段性肾小球硬化患者内皮细胞功能异常及临床意义 被引量:7
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作者 张庆燕 曾彩虹 +6 位作者 陈朝红 程震 李世军 贺红光 张炯 伏幼娟 刘志红 《肾脏病与透析肾移植杂志》 CAS CSCD 北大核心 2010年第4期309-316,共8页
目的:前瞻性观察局灶节段性肾小球硬化(FSGS)患者的内皮细胞功能及其与疾病活动程度和并发症的关系。方法:63例经肾活检及临床确诊且蛋白尿≥3.5g/d的特发性FSGS患者进入本研究,选取年龄、性别相匹配的32例健康志愿者作对照。分析内皮... 目的:前瞻性观察局灶节段性肾小球硬化(FSGS)患者的内皮细胞功能及其与疾病活动程度和并发症的关系。方法:63例经肾活检及临床确诊且蛋白尿≥3.5g/d的特发性FSGS患者进入本研究,选取年龄、性别相匹配的32例健康志愿者作对照。分析内皮细胞损伤指标循环内皮细胞计数(CECs)、血管性血友病因子(vWF)、可溶性血栓调节蛋白(sTM)、血管细胞粘附分子(VCAM)和E选择素(ES)的阳性率,观察起点内皮细胞损伤指标与其他临床指标及静脉血栓栓塞(VTE)并发症的关系,观察内皮细胞损伤指标的动态变化。结果:(1)FSGS患者的各项内皮细胞损伤指标均显著高于正常对照组。(2)无VTE的52例患者内皮细胞损伤指标的阳性率分别为CECs48.1%、vWF92.3%,sTM96.2%,VCAM67.3%,ES28.9%,其中VCAM阳性患者的血压水平、尿蛋白定量、血肌酐水平及肾小管损伤指标NAG酶、RBP均显著高于阴性者,CECs及ES阳性与阴性患者各项指标间无明显差异。相关性分析显示,sTM、VCAM与SCr显著正相关,VCAM、vWF与NAG、RBP均有正相关性。随访2月时15例达完全缓解(CR),13例部分缓解(PR),15例无效(NR),8例失随访,1例进入腹膜透析治疗退出本研究。CR组治疗后sTM、VCAM均明显下降,而vWF、ES水平变化不明显,PR组治疗前后相比sTM明显下降,余三项指标变化无统计学意义,NR组治疗前后各项内皮损伤指标均无明显变化。随访1年期间11例患者获得持续缓解,该组患者各观察点ES水平与正常对照组均无明显差异,sTM于随访2月时即降至正常,VCAM、vWF分别于随访2月、6月时开始下降,随访12月时仍高于正常对照。(3)合并VTE者CECs、vWF水平显著高于无VTE者。结论:FSGS患者存在明显内皮细胞功能异常,其中VCAM及CECs水平的异常升高分别与疾病的活动程度及血栓栓塞并发症的发生关系密切,随疾病的缓解内皮细胞功能得以不同程度的改善。内皮细胞损伤标志物的检测有助于对FSGS患者的病情及血栓栓塞并发症进行评估。 展开更多
关键词 局灶节段性肾小球硬化 内皮细胞 疾病活动度 血栓栓塞
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成人微小病变患者尿中CD80水平及临床意义 被引量:4
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作者 王晓荣 李绍梅 +3 位作者 杨林 谭会斌 李素敏 傅淑霞 《肾脏病与透析肾移植杂志》 CAS CSCD 北大核心 2014年第3期216-220,共5页
目的:探讨成人微小病变(MCD)患者尿液CD80在MCD发生与发展中的作用及其对MCD与局灶节段性肾小球硬化(FSGS)鉴别诊断的意义. 方法:MCD患者35例(肾病组初治9例和复发10例,完全缓解组16例),其中9例初治患者经随访后完全缓解,行自... 目的:探讨成人微小病变(MCD)患者尿液CD80在MCD发生与发展中的作用及其对MCD与局灶节段性肾小球硬化(FSGS)鉴别诊断的意义. 方法:MCD患者35例(肾病组初治9例和复发10例,完全缓解组16例),其中9例初治患者经随访后完全缓解,行自身配对研究;同期原发FSGS肾病综合征患者18例.记录所有患者相关资料.ELISA法测尿CD80浓度(ng/ml)及血清白细胞介素13(IL-13) (pg/ml)水平,其中尿CD80值用尿肌酐值(g/ml)校正. 结果:(1)尿CD80/肌酐水平:MCD肾病组明显高于MCD完全缓解组或FSGS肾病组[247.0ng/g(196.0~ 378.0ng/g)vs 42.0 ng/g(24.0~76.3 ng/g) 、73.0 ng/g(35.3~ 172.0 ng/g),P<0.05],MCD完全缓解组与FSGS肾病组无统计学差异;MCD初治组与复发组无统计学差异.(2)血IL-13水平在MCD肾病组、MCD完全缓解组及FSGS肾病组中无统计学差异.(3)自身配对研究示:MCD完全缓解后较治疗前尿CD80/肌酐水平显著下降[41.0 ng/g(17.0~61.5 ng/g) vs 323.0 ng/g(225.5~587.5 ng/g),Z=2.666,P=0.008],而血IL-13水平无统计学差异(Z=0.847,P=0.397).(4) MCD肾病组尿CD80/肌酐与24h尿蛋白定量及血IL-13水平均无相关性(分别r=-0.219,P=0.369;r=0.303,P=0.205).(5)尿CD80鉴别诊断MCD和FSGS的ROC曲线下面积(AUC)为0.883(截点为194.5 ng/ml,其敏感度为78.9%,特异性为88.9%). 结论:CD80可能参与了MCD发生及发展,且可作为成人肾病综合征患者MCD与FSGS鉴别诊断的新指标. 展开更多
关键词 尿CD80 肾病综合征 微小病变 局灶节段性肾小球硬化 白细胞介素13
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局灶节段性肾小球硬化患者足细胞钙神经蛋白的表达 被引量:3
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作者 汤曦 吴青 +5 位作者 郑春霞 张明超 曾彩虹 张炯 朱晓东 刘志红 《肾脏病与透析肾移植杂志》 CAS CSCD 北大核心 2010年第4期301-308,共8页
目的:观察成人局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)患者足细胞钙神经蛋白的表达并探讨其意义。方法:选取临床表现为肾病综合征且经肾活检确诊的成人特发性FSGS63例,微小病变(minimal change disease,MCD)24... 目的:观察成人局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)患者足细胞钙神经蛋白的表达并探讨其意义。方法:选取临床表现为肾病综合征且经肾活检确诊的成人特发性FSGS63例,微小病变(minimal change disease,MCD)24例和肾移植供肾组织10例作为对照。免疫组化法检测肾组织钙神经蛋白A(CnA)异构体α、β、γ的表达。免疫荧光法行肾组织CnAα和synaptopodin双标记,并采用胶体金免疫电镜对肾小球CnAα的表达定位。两位病理医生分别盲法评分染色结果。结果:供肾组织肾小球CnAα、β、γ免疫组化染色均阴性,肾小管CnAα、β微弱表达,CnAγ阴性。26例FSGS患者肾小球足细胞CnAα表达上调,其阳性率为41.27%;而MCD患者肾小球CnAα阴性(P<0.01);两者肾小球CnAα、β、γ染色均阴性。足细胞CnAα阳性患者肾小管损伤指标尿视黄醇结合蛋白[20.91(0.17~54.37)mg/L]、血清肌酐[(128.18±56.58)μmol/L]高于阴性患者(P<0.05);两者间病理类型构成差异存在统计学意义(P<0.01);而年龄、性别、尿蛋白、尿N-乙酰-β-葡萄糖苷酶、血清白蛋白及胆固醇无统计学差异。塌陷型、细胞型、顶部型、门周型FSGS中足细胞CnAα阳性病例分别为5例(83.33%)、10例(66.67%)、8例(61.54%)、3例(20%),14例经典型FSGS患者均阴性。多因素Logistic回归分析示血清肌酐(OR4.855,P<0.01)、塌陷型(OR11.069,P<0.05)为FSGS患者足细胞CnAα过表达的主要相关因素。69.23%足细胞CnAα阳性患者肾小球syanptopodin表达减弱且不连续。结论:本研究首次发现部分FSGS患者足细胞上CnAα表达增强,这可能参与了FSGS的发病。FSGS与MCD患者足细胞CnAα的表达差异,提示二者发病机制不同。对临床怀疑FSGS而未见明确节段病变的患者,足细胞CnAα阳性有助于诊断。 展开更多
关键词 局灶节段性肾小球硬化 微小病变 足细胞 钙神经蛋白
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补肾活血方对阿霉素肾病小鼠肾小球足细胞nephrin表达的影响 被引量:6
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作者 贾胜琴 谭小月 +2 位作者 阎丽娜 左春霞 张勉之 《天津医药》 CAS 北大核心 2013年第5期471-474,I0004,共5页
目的观察补肾活血方治疗对阿霉素(ADR)诱导的阿霉素肾病小鼠肾小球足细胞裂孔膜蛋白nephrin表达的影响,并探讨其作用机制。方法用ADR诱导产生局灶性节段性肾小球硬化(FSGS)肾病模型,1周后用补肾活血方治疗(治疗组),持续6周。并设空白对... 目的观察补肾活血方治疗对阿霉素(ADR)诱导的阿霉素肾病小鼠肾小球足细胞裂孔膜蛋白nephrin表达的影响,并探讨其作用机制。方法用ADR诱导产生局灶性节段性肾小球硬化(FSGS)肾病模型,1周后用补肾活血方治疗(治疗组),持续6周。并设空白对照组、模型组。收集各组血、尿及肾组织,检测其体质量、右肾质量、血清白蛋白(ALB)、尿白蛋白/尿肌酐比值(UACR)。光镜观察肾组织形态改变;免疫荧光测定小鼠肾小球足细胞nephrin的表达;RT-PCR检测各组肾组织nephrin mRNA的表达。结果模型组UACR显著高于对照组与治疗组,体质量和ALB显著低于对照组和治疗组(均P<0.01);对照组肾小球足细胞nephrin免疫荧光呈连续性表达,治疗组呈轻微缺失,模型组呈少量点状或片状表达,较对照组和治疗组表达强度明显减少。模型组nephrin mRNA表达水平均显著低于对照组和治疗组(P<0.01)。结论补肾活血方能减少阿霉素肾病小鼠的蛋白尿,可能与改善肾小球足细胞nephrin的表达减少有关。 展开更多
关键词 补肾 活血 表柔比星 肾小球硬化症 局灶节段性 足细胞 疾病模型 动物 小鼠 近交BALB C NEPHRIN
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循环microRNAs与局灶节段性肾小球硬化患者疾病活动的关系 被引量:4
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作者 张昌明 张婉芬 +2 位作者 刘春蓓 曾科 刘志红 《肾脏病与透析肾移植杂志》 CAS CSCD 北大核心 2013年第4期309-314,323,共7页
目的:分析循环中与局灶节段性肾小球硬化(FSGS)患者疾病活动相关microRNAs(miRNAs),为后续探讨循环miRNAs在FSGS发病中的作用奠定基础。方法:选取年龄、性别匹配的活动性FSGS患者及正常对照各9例,提取FSGS患者及对照组血浆总RNA,采用Taq... 目的:分析循环中与局灶节段性肾小球硬化(FSGS)患者疾病活动相关microRNAs(miRNAs),为后续探讨循环miRNAs在FSGS发病中的作用奠定基础。方法:选取年龄、性别匹配的活动性FSGS患者及正常对照各9例,提取FSGS患者及对照组血浆总RNA,采用TaqMan低密度芯片(TaqMan Low Density Array)进行miRNA表达谱分析,筛选出在活动性FSGS患者循环中表达上调的miRNAs分子;然后再选取32例活动性FSGS患者血浆,采用实时荧光定量逆转录聚合酶链反应(qRT-PCR)方法对筛选出的miRNAs分子进行验证,同时与非活动性FSGS患者进行比较。采用ROC曲线分析循环miRNA区分活动性FSGS、非活动性FSGS和正常对照的效能。结果:与正常对照相比,活动性FSGS患者循环miRNA-125b,miRNA-186和miRNA-193a-3p水平显著上调(P<0.05)。ROC曲线分析显示,循环miRNA-125b、miRNA-186和miRNA-193a-3p区分活动性FSGS与正常对照的ROC曲线下面积(AUC)分别为0.882、0.789和0.910。与非活动性FSGS相比,活动性FSGS患者循环中miRNA-186水平也明显上调。结论:活动性FSGS患者循环miRNA-125b、miRNA-186及miRNA-193a-3p较正常对照显著升高,其中miRNA-186的升高与疾病的活动性关系更密切。 展开更多
关键词 局灶节段性肾小球硬化 MICRORNA 疾病活动
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血清miRNAs在局灶节段性肾小球硬化及微小病变性肾病中的表达 被引量:3
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作者 蔡晓懿 夏正坤 +7 位作者 张春妮 罗阳 高远赋 樊忠民 刘梦原 张莹 李静 张婕 《临床儿科杂志》 CAS CSCD 北大核心 2013年第4期328-330,共3页
目的研究局灶节段性肾小球硬化(FSGS)及微小病变性肾病(MCD)患儿miRNA的表达水平,及其与尿蛋白水平的相关性。方法选取60例经肾活检及临床确诊为FSGS(30例)、MCD(30例)的患儿,以及年龄、性别相匹配的20例正常对照儿童,用实时荧光定量PC... 目的研究局灶节段性肾小球硬化(FSGS)及微小病变性肾病(MCD)患儿miRNA的表达水平,及其与尿蛋白水平的相关性。方法选取60例经肾活检及临床确诊为FSGS(30例)、MCD(30例)的患儿,以及年龄、性别相匹配的20例正常对照儿童,用实时荧光定量PCR检测血清miR-192、miR-205水平,同时收集FSGS、MCD患儿的24 h尿蛋白及其他生化指标。结果 FSGS患儿血清miR-192、miR205表达水平明显高于MCD患儿及正常对照儿童,且与尿蛋白水平呈正相关;MCD患儿miR-205表达水平与正常对照儿童差异无统计学意义。结论 FSGS患儿血清miR-192、miR-205表达显著上调,有望作为FSGS诊断的生物标记物。 展开更多
关键词 MIRNA 局灶节段性肾小球硬化 微小病变性肾病 足细胞病
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儿童原发性肾病综合征α-辅肌动蛋白4mRNA表达的临床意义 被引量:4
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作者 康郁林 吴滢 +1 位作者 朱光华 何威逊 《临床儿科杂志》 CAS CSCD 北大核心 2010年第12期1169-1172,共4页
目的探讨在儿童原发性肾病综合征中α-辅肌动蛋白4(α-actinin-4)mRNA表达及其意义。方法经肾活检获取34例原发性肾病综合征患儿(病例组)的肾组织,其中局灶节段硬化性肾小球肾炎(FSGS)21例、微小病变(MCNS)6例、系膜增生性肾小球肾炎(Ms... 目的探讨在儿童原发性肾病综合征中α-辅肌动蛋白4(α-actinin-4)mRNA表达及其意义。方法经肾活检获取34例原发性肾病综合征患儿(病例组)的肾组织,其中局灶节段硬化性肾小球肾炎(FSGS)21例、微小病变(MCNS)6例、系膜增生性肾小球肾炎(MsPGN)7例;另外,12例对照组标本取自肾肿瘤切除术后肿瘤周边的正常组织。通过实时荧光定量PCR(quantitative real-time PCR)的方法检测每份肾组织中α-actinin-4 mRNA表达,同时分析FSGS患儿中mRNA表达与24 h尿蛋白、内生肌酐清除率(Ccr)的相关性。结果 FSGS组α-actinin-4 mRNA表达明显高于对照组,差异有统计学意义(P<0.05),而MCNS组和MsPGN组α-actinin-4 mRNA表达与正常对照组的差异均无统计学意义(P>0.05)。在FSGS组中,表现为肾炎型肾病(伴发镜下血尿)患儿的α-actinin-4 mRNA表达明显高于表现为单纯性肾病的患儿,差异有统计学意义(P<0.05);FSGS患儿α-actinin-4mRNA表达量与24 h尿蛋白及Ccr无相关性(P>0.05)。结论在儿童原发性肾病综合征中,α-actinin-4 mRNA表达量增高可能是促进肾小球足细胞骨架修复的因素之一;该骨架分子的mRNA表达变化与病程及病理类型相关。 展开更多
关键词 α-辅肌动蛋白4 实时荧光定量PCR 局灶节段硬化性肾小球肾炎 微小病变 系膜增生性肾小球肾炎
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