肾病综合征诊断治疗指南
抗肾病综合征药治疗肾病综合征的西药使用指南
抗肾病综合征药治疗肾病综合征的西药使用指南肾病综合征是一种常见的肾脏疾病，其特征是蛋白尿、水肿和低蛋白血症。
目前，西药在抗肾病综合征的治疗中起到了重要的作用。
本文将介绍一些常用的西药，并提供肾病综合征的西药使用指南。
1. 血管紧张素转化酶抑制剂（ACEI）ACEI是一类常用的降压药物，也被用于肾病综合征的治疗。
它通过抑制血管紧张素转化酶，减少血管收缩和尿蛋白排泄。
常见的ACEI 包括依那普利和雷米普利。
在使用ACEI时，需要密切监测患者的肾功能和血压。
2. 血管紧张素Ⅱ受体拮抗剂（ARB）ARB也是常用的治疗肾病综合征的药物。
它通过阻断血管紧张素Ⅱ受体，减少肾小球滤过膜的通透性和尿蛋白排泄。
洛沙坦和氯沙坦是常见的ARB药物。
与ACEI相比，ARB更适合那些不能耐受ACEI的患者。
3. 肾上腺皮质激素肾上腺皮质激素，如泼尼松，是治疗肾病综合征的常用药物。
它可以抑制免疫系统的过度反应，减少蛋白尿和水肿。
然而，长期使用肾上腺皮质激素可能导致一系列的副作用，如骨质疏松和免疫抑制。
因此，在使用肾上腺皮质激素时需要权衡利弊。
4. 免疫抑制剂免疫抑制剂在肾病综合征的治疗中也发挥着重要作用。
它们通过抑制免疫系统的活性，减少肾脏损伤和尿蛋白排泄。
常用的免疫抑制剂包括环磷酰胺和他克莫司。
然而，免疫抑制剂的使用需要密切监测患者的免疫状态和副作用。
5. 利尿剂利尿剂被广泛应用于肾病综合征的治疗中，它们可以通过增加尿液排出来减轻水肿。
常见的利尿剂包括袢利尿剂和噻嗪类利尿剂。
然而，在使用利尿剂时需要注意监测患者的电解质水平，以避免不良反应。
总之，以上介绍的西药在抗肾病综合征的治疗中起到了重要的作用。
然而，每个患者的情况各异，治疗方案需要根据具体情况来调整。
因此，在使用西药治疗肾病综合征时，建议在医生的指导下进行，以确保疗效和安全性的最大化。
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中国肾病诊疗指南(2024版)
中国肾病诊疗指南(2024版)前言本指南根据我国肾脏病防治的实际情况，结合国际肾脏病研究的最新进展，为肾脏病专科医生提供了一套完整的诊疗规范，旨在提高我国肾脏疾病的诊疗水平，促进医疗资源的合理配置。
目录1. 概述2. 诊断方法3. 慢性肾病4. 急性肾损伤5. 慢性肾衰竭6. 常见肾小球疾病7. 常见肾实质疾病8. 儿童肾病9. 老年肾病10. 肾病综合征11. 治疗原则12. 药物治疗13. 中医治疗14. 护理与康复15. 预防与调养16. 研究进展与展望1. 概述肾病是指影响肾脏正常功能的疾病，包括肾小球、肾小管、肾血管等部位的病变。
我国肾病发病率逐年上升，已成为威胁人民健康的重大疾病之一。
2. 诊断方法肾病诊断主要包括病史询问、体格检查、实验室检查、影像学检查和肾活检等。
其中，肾活检是确诊肾脏疾病类型的重要手段。
3. 慢性肾病慢性肾病（CKD）是指持续损害肾脏功能的一种疾病。
根据我国CKD流行病学调查结果，我国CKD患病率为10.8%。
4. 急性肾损伤急性肾损伤（AKI）是指肾功能在短时间内急剧下降，表现为氮质血症、水电解质失衡和全身炎症反应。
5. 慢性肾衰竭慢性肾衰竭（CRF）是慢性肾病发展的终末阶段，表现为肾功能进行性减退、代谢产物潴留和全身多系统受累。
6. 常见肾小球疾病肾小球疾病包括IgA肾病、膜性肾病、局灶节段性肾小球硬化症等。
7. 常见肾实质疾病肾实质疾病包括肾炎、肾病、肾肿瘤等。
8. 儿童肾病儿童肾病包括急性肾小球肾炎、肾病综合征等。
9. 老年肾病老年肾病是指老年人发生的肾脏疾病，常见病因为糖尿病肾病、高血压肾损害等。
10. 肾病综合征肾病综合征是指由多种原因引起的肾小球滤过膜通透性增加，表现为蛋白尿、低蛋白血症、水肿和高脂血症。
11. 治疗原则肾病治疗原则为：控制原发病，缓解症状，改善生活质量，延缓疾病进展，防止并发症。
12. 药物治疗药物治疗主要包括降压、降脂、抗凝、免疫抑制等。
IGA肾病治疗指南
I G A肾病治疗指南Document serial number【NL89WT-NY98YT-NC8CB-NNUUT-NUT108】KDIGO指南解读：IgA肾病的治疗IgA肾病是青壮年最为常见的肾脏疾病之一，在亚裔人群中，IgA肾病占原发性肾病的30%～45%，其临床和病理表现、进展速度及预后迥异，20%的IgA肾病患者在10年内进展至终末期肾脏病，30%的患者最终进展至终末期肾脏病。
IgA肾病的诊断、治疗和预后评估问题，新加坡、日本和澳大利亚分别发布了相关临床指南，但由于缺乏大样本RCT研究，故大部分指南的证据仅为B、C级。
2011年3月肾脏病预后组织（KidneyDisease:ImprovingGlobalOutcomes,KDIGO）发布了IgA肾病的临床实践指南（公众审阅稿），近期将发布正式版本。
KDIGO将指南推荐等级分为1级、2级或未分级，循征依据等级分为A、B、C、D（见表一、表二）。
KDIGOIgA 肾病指南共有19条。
推荐等级，1级1个（占5.3%），2级12个（占63.2%），未分级6个（占31.6%）。
证据等级，A级0个，B级3个（占23.1%），C级6个（占46.2%），D级4个（占30.8%）。
表一：推荐等级推荐等级含义患者临床医生政府部门1级“推荐”recommend 绝大多数患者将按照推荐的要求治疗绝大多数患者给予推荐的治疗方案推荐意见可用作制定政策参考2级“建议”suggest 多数患者可按照建议来做，但还有很多人不需要有多种不同的方案可供不同患者选择。
强调根据患者的要求和意愿制定治疗方案需要与相关利益方在进行进一步的协商表二：证据等级标准分级证据标准A级（高质量）试验结果和真实情况非常接近B级（中等质量）试验结果与真实情况可能较接近，不排除存在偏差之可能C级（低质量）试验结果与真实情况之间可能存在较大偏差D级（极低质量）试验结果本身多属推测，与实际情况相差甚远.评估肾脏病进展的风险IgA肾病临床表现多种多样，从孤立性血尿到快速进展的肾小球肾炎；进展至肾衰竭的速率也各不相同，且相似的临床表现其预后可能截然不同。
肾病诊疗指南2022版最新
肾病诊疗指南2022版最新下载温馨提示：该文档是我店铺精心编制而成，希望大家下载以后，能够帮助大家解决实际的问题。
文档下载后可定制随意修改，请根据实际需要进行相应的调整和使用，谢谢!并且，本店铺为大家提供各种各样类型的实用资料，如教育随笔、日记赏析、句子摘抄、古诗大全、经典美文、话题作文、工作总结、词语解析、文案摘录、其他资料等等，如想了解不同资料格式和写法，敬请关注!Download tips: This document is carefully compiled by the editor. I hope that after you download them, they can help yousolve practical problems. The document can be customized and modified after downloading, please adjust and use it according to actual needs, thank you!In addition, our shop provides you with various types of practical materials, such as educational essays, diary appreciation, sentence excerpts, ancient poems, classic articles, topic composition, work summary, word parsing, copy excerpts,other materials and so on, want to know different data formats and writing methods, please pay attention!慢性肾病是一种持续进展的疾病，严重影响患者的生活质量。
膜性肾病治疗指南
膜性肾病治疗指南膜性肾病（Membranous nephropathy）是一种以肾小球基底膜肾小球损伤为特征的肾脏疾病，其临床特点是肾病综合征，具有高蛋白尿、低蛋白血症和水肿等症状。
膜性肾病的治疗主要包括药物治疗和支持性治疗。
以下是膜性肾病治疗的指南：1.药物治疗：-常用的一线药物是ACEI/ARB（血管紧张素转换酶抑制剂/血管紧张素受体拮抗剂），用于控制高血压和减少肾脏蛋白丢失。
同时，它们还具有减少蛋白尿的作用。
-有些患者可能需要使用免疫抑制剂，如糖皮质激素和细胞毒药物。
糖皮质激素，如泼尼松，可减少肾小球蛋白尿和炎症反应。
细胞毒药物，如环磷酰胺和环孢素，可抑制免疫反应并降低蛋白尿。
- 对于无法耐受或无反应的患者，可考虑使用雷米刚（Rituximab）或人工免疫球蛋白（Intravenous Immunoglobulin），以减少肾小球蛋白尿。
2.支持性治疗：-对于肾功能不全的患者，特别是伴有水肿和高血压的患者，应进行液体平衡和电解质调节，以减轻症状和维持肾功能。
-高脂血症是膜性肾病患者的常见合并症，因此需要密切监测血脂并进行必要的调节，如限制饱和脂肪酸和胆固醇的摄入，并使用降血脂药物。
-心血管疾病是膜性肾病患者的主要死亡原因之一，因此应进行心血管风险评估，并给予适当的预防，如使用抗血小板药物和控制血压。
3.近年来的新治疗：-近年来，人们对膜性肾病的治疗进行了深入的研究，并取得了一些新的进展。
其中包括对免疫抑制治疗的优化，如联合应用糖皮质激素和环孢素，并在需要时加入其他免疫抑制剂。
- 另外，一些新型药物如B淋巴细胞抗原（CD20）抗体奥利司他韦（Olxacarbazepine）和复显海马霉素（Remidoxyl）等，也被证实对膜性肾病具有一定的疗效。
总的来说，膜性肾病的治疗主要以控制症状、保护肾功能和降低并发症为目标。
早期诊断和治疗对于预防或延缓疾病进展非常重要。
同时，临床医生也应根据患者的具体情况制定个体化的治疗方案，并定期随访评估疗效和调整治疗方案。
肾病综合征诊断治疗指南
肾病综合征诊断治疗指南肾病综合征是肾小球疾病的一种表现，其病因多种多样，对治疗的反应和预后也有很大的差异。
因此，在临床上不能仅仅满足于肾病综合征的诊断，还必须对其作出病因、病理、并发症乃至完整的诊断，以提高治疗的缓解率，改善患者的预后。
原发性肾病综合征的病理类型有多种，而微小病变肾病、肾小球局灶节段硬化、系膜增生性肾炎、膜性肾病、系膜毛细血管性肾炎等几种类型最为常见。
肾病综合征的分类根据病因分为原发性和继发性，前者之诊断主要依靠排除继发性NS。
继发性NS的病因常见于糖尿病肾病、狼疮性肾炎、肾淀粉样变性、药物、肿瘤等。
肾病综合征的症状和体征不一定出现在任何特定年龄段，而可能发生在任何年龄。
在发病前可能有职业病史、有毒有害物接触史、服用药物或食物过敏史等情况。
此外，肾病综合征也可能继发于呼吸道、皮肤的感染、病毒性肝炎、肿瘤、糖尿病、系统性疾病等。
肾病综合征的起病可能急骤也可能隐匿，患者可能有乏力、恶心、腰酸、食欲下降等，部分患者甚至可能没有明显的临床症状。
除了水肿和蛋白尿外，肾病综合征还可能表现为血尿、高血压及不同程度的肾功能减退。
肾病综合征的典型实验室检查表现为大量蛋白尿（尿蛋白定量>3.5g/d）、低白蛋白血症（血浆白蛋白<30g/L）和高脂血症。
此外，尿沉渣镜检红细胞可能增多，可见管型，肾功能可能正常或受损（GFR下降），还可能伴有免疫指标（抗核抗体、抗双链DNA、ANCA、免疫球蛋白等）、肿瘤指标（CEA、AFP、PSA等）、病毒指标（HBV、HCV、HIV等）以及骨髓穿刺活检异常。
肾穿刺活检可以明确病理分型。
肾病综合征的主要并发症包括水肿、蛋白质丢失、感染、血栓栓塞、肾衰竭等。
在治疗肾病综合征时，必须综合考虑患者的临床表现、实验室检查结果以及病因、病理等方面的信息，以制定出个性化的治疗方案。
1.感染：肾病综合征患者由于营养不良、免疫状态异常、激素及免疫抑制剂的使用，感染的风险增加。
激素敏感性肾病综合征的治疗--新KDIGO指南
EDUCATIONAL REVIEWTreatment of steroid-sensitive nephrotic syndrome:new guidelines from KDIGORebecca M.Lombel &Debbie S.Gipson &Elisabeth M.HodsonReceived:31May 2012/Revised:30July 2012/Accepted:16August 2012/Published online:3October 2012#IPNA 2012Abstract The 2012Kidney Disease:Improving Global Outcomes (KDIGO)clinical practice guideline on glomeru-lonephritis (GN)is intended to assist the practitioner caring for patients with GN.Two chapters of this guideline focus specifically on nephrotic syndrome in children.Guideline development followed a thorough evidence review,and management recommendations and suggestions were based on the best available evidence.Critical appraisal of the quality of evidence and strength of recommendations fol-lowed the Grades of Recommendation Assessment,Devel-opment and Evaluation (GRADE)approach.Chapters 3and 4of the guideline focus on the management of nephrotic syndrome in children aged 1–18years.Guideline recom-mendations for children who have steroid-sensitivenephrotic syndrome (SNSS),defined by their response to corticosteroid therapy with complete remission,are addressed here.Recommendations for those with steroid-resistant nephrotic syndrome (SRNS)(i.e.,do not achieve complete remission)are discussed in the companion article.Limitations of the evidence,including the paucity of large-scale randomized controlled trials,are discussed.This arti-cle provides a short description of the KDIGO process,the guideline recommendations for treatment of SSNS in chil-dren and a brief review of relevant treatment trials related to each recommendation.Keywords Clinical practice guidelines .Steroid-responsive nephrotic syndrome .Management .ChildrenIntroductionIdiopathic nephrotic syndrome is a major contributor to the workload of pediatric nephrologists.Most children have steroid-sensitive nephrotic syndrome (SSNS),with about 20%of children having steroid-resistant nephrotic syn-drome (SRNS),depending on the geographic area [1].Most children with SSNS have minimal change disease (MCD)[2],while children with SRNS have MCD,mesangial pro-liferative glomerulonephritis (MesPGN)or focal and seg-mental glomerulosclerosis (FSGS)[2].Studies by the International Study of Kidney Disease in Children (ISKDC)and the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN)during the 1960s,1970s,and 1980s,and subsequent studies by individual research teams form the basis of the clinical management of SSNS and SRNS in children.Transformation of local interpretation of the liter-ature into a regional or national clinical practice guideline typically incorporates a systematic review and scoring of the literature.Published guidelines from France,NorthR.M.Lombel D.S.Gipson Division of Nephrology,Department of Pediatrics and Communicable Diseases,University of Michigan,Ann Arbor,MI,USAD.S.GipsonMichigan Institute for Clinical and Health Research,University of Michigan,Ann Arbor,MI,USAE.M.HodsonCentre for Kidney Research,The Children ’s Hospital at Westmead,Westmead,Sydney,AustraliaE.M.HodsonSydney School of Public Health,University of Sydney,Sydney,AustraliaR.M.Lombel (*)Pediatric Nephrology,C.S.Mott Children ’s Hospital,1540E.Hospital Drive,SPC 4297,Ann Arbor,MI 48109-4297,USA e-mail:rlombel@Pediatr Nephrol (2013)28:415–426DOI 10.1007/s00467-012-2310-xAmerica,and India have been generated using this approach [3–5].While reflecting available evidence,these guidelines were largely based on consensus among experts in the field. Now KDIGO(Kidney Disease:Improving Global Out-comes)has published clinical practice guidelines on various glomerulonephritides including SSNS and SRNS in children using evidence-based principles[6].In this article,we dis-cuss the KDIGO process,present the guidelines for SSNS, and outline the evidence used to develop these guidelines. For reference,definitions typically used to discuss nephrotic syndrome are found in Table1.The guideline for SRNS is presented in a separate article[7].What is KDIGO?KDIGO(),which was established in2003,is an international non-profit organization aiming to improve the outcomes of kidney disease patients worldwide.It is governed by an international board and is managed by the National Kidney Foundation in the United States.The board comprises approximately50members of whom most are practicing nephrologists.The board elects an11-person executive committee,which plans and oversees KDIGO activities and is chaired by two board members(currently Dr.Bertram Kasiske and Dr.Kai-Uwe Eckardt).Topics for guidelines are chosen based on the worldwide burden of disease,the likelihood of the condition respond-ing to prevention and/or treatment,the expected impact of management,the existence of sufficient evidence from which to develop evidence-based guidelines,and the poten-tial that such guidelines can improve outcomes.The KDIGO guideline processGuidelines are developed by independent,international,mul-tidisciplinary workgroups comprising12–20members.The two co-chairs choose the workgroup members.The Glomer-ulonephritis Workgroup was chaired by Drs.Daniel Cattran (Canada)and John Feehally(the United Kingdom)and com-prised16members from the United States of America,the United Kingdom,Australia,Canada,Chile,China,Germany, India,the Netherlands,and Spain.Each guideline workgroup is supported by an independent review team to provide meth-odological expertise using evidence-based principles to work-group members.They perform literature searches for systematic reviews,randomized controlled trials(RCTs)and non-randomized comparator studies,undertake data extrac-tion and data quality assessment,and ensure that the grading of guidelines is consistent with the available evidence.The Glomerulonephritis Workgroup was supported by a team led by Dr.Ethan Balk from the Tufts Center for Kidney Disease Guideline Development and Implementation.After comple-tion,the guidelines were opened for public comment before further final revisions so that the published guideline[8]is based on the best available evidence to June2011.KDIGO has an implementation team that works with national and regional societies to encourage dissemination.KDIGO has procedures in place to ensure that guidelines are updated everyTable1Definitions of nephrotic syndrome in childrenClassification DefinitionNephrotic syndrome Edema,uPCR≥2,000mg/g(≥200mg/mmol),or≥300mg/dl or3+protein on urine dipstick,hypoalbuminemia≤2.5mg/l(≤25g/l)Complete remission uPCR<200mg/g(<20mg/mmol)or<1+of protein on urine dipstick for3consecutive days Partial remission Proteinuria reduction of50%or greater from the presenting value andabsolute uPCR between200and2,000mg/g(20–200mg/mmol)No remission Failure to reduce urine protein excretion by50%from baseline or persistent excretionuPCR>2,000mg/g(>200mg/mmol)Initial responder Attainment of complete remission within initial4weeks of corticosteroid therapyInitial nonresponder/steroid resistance Failure to achieve complete remission after8weeks of corticosteroid therapyRelapse uPCR≥2,000mg/g(≥200mg/mmol),or≥300mg/dl or3+protein on urine dipstick Infrequent relapse One relapse within6months of initial response,or one to three relapses in any12-month period Frequent relapse Two or more relapses within6months of initial response,or four or more relapses in any12-month periodSteroid dependence Two consecutive relapses during corticosteroid therapy,or within14days of ceasing therapy Late nonresponder Persistent proteinuria during4or more weeks of corticosteroids following one or more remissionuPCR urine protein:creatinine ratioReproduced with permission from Kidney Disease:Improving Global Outcomes(KDIGO)Glomerulonephritis Work Group.KDIGO Clinical Practice Guideline for Glomerulonephritis[8]416Pediatr Nephrol(2013)28:415–4265years and that earlier updates occur to one or more recom-mendations based on important new evidence.KDIGO guidelines are intended to provide guidance rather than rules.Although the purpose of the recommendations is to assist in decision-making,a guideline recommendation does not take into account individual patient characteristics,pro-vider variation,and system factors.Thus,each provider retains the privilege and responsibility to assess the appropri-ateness of a particular recommendation in a specific context. Strength of recommendations and quality of supporting evidenceKDIGO workgroups use the GRADE system[6,9]to assign separate grades for the quality of the evidence(Table2)and the strength of the recommendations(Table3).As a starting point in the GRADE classification,systematic reviews of RCTs and individual RCTs are considered high-quality ev-idence(level A),observational studies are considered low-quality evidence(level C)and other studies including small case series are considered very low quality evidence(level D).However,the level in RCTs can be downgraded or the level in observational studies upgraded according to a num-ber of criteria[6].The strength of the recommendations is graded as level1(“we recommend”)if there is high-quality evidence that the intervention’s effects are clearly greater than its adverse effects(or the opposite),or as level2(“we suggest”)if the evidence is of lower quality and there remains some uncertainties about the trade-offs between benefits and harms(Table3).Recommendations that pro-vide general guidance about evaluation or general manage-ment are marked as“not graded”.The scope of the KDIGO Glomerulonephritis Clinical Practice GuidelinesThe scope for each topic in the Glomerulonephritis Guide-line was determined by the Glomerulonephritis Workgroup. The Glomerulonephritis Guideline aims to provide treat-ment guidelines for patients already diagnosed with a form of glomerulonephritis[8].The guideline covers SSNS in children,SRNS in children,minimal change disease (adults),focal and segmental glomerulosclerosis(adults), idiopathic membranous nephropathy,membranoprolifera-tive glomerulonephritis,infection-related glomerulonephri-tis,IgA nephropathy,Henoch–Schönlein purpura nephritis, lupus nephritis,pauci-immune focal and necrotizing glo-merulonephritis,and anti-glomerular basement membrane antibody glomerulonephritis.Corticosteroids for the initial episode of steroid-sensitive nephrotic syndromeKDIGO Glomerulonephritis Workgroup,2012:“3.1:Treatment of the initial episode of SSNS3.1.1:We recommend that corticosteroid therapy(prednisone or prednisolone)1be given for atleast12weeks.(1B)3.1.1.1:We recommend that oral prednisone beadministered as a single daily dose(1B)starting at60mg/m2/day or2mg/kg/day to amaximum60mg/day.(1D)3.1.1.2:We recommend that daily oral prednisonebe given for4–6weeks(1C)followed byalternate-day medication as a single dailydose starting at40mg/m2or1.5mg/kg(maximum40mg on alternate days)(1D)and continued for2–5months with taperingof the dose.(1B)”(163).The risk of relapse was reduced by30%at12–24months by12weeks or more of prednisone compared with8weeks (six RCTs;422children;risk ratio[RR]of relapse0.70; 95%confidence intervals[CI]0.58-0.84)[10].The quality of the evidence was downgraded from A(high)to BTable2Grading of the quality of the evidenceReproduced with permission from Kidney Disease:Improving Global Outcomes(KDIGO) Glomerulonephritis Work Group.KDIGO Clinical Practice Guideline for Glomerulonephritis[8]Grade Quality of the evidence MeaningA High We are confident that the true effect lies close to that ofthe estimate of the effectB Moderate The true effect is likely to be close to the estimate of the effect,but there is a possibility that it is substantially differentC Low The true effect may be substantially different from theestimate of the effectD Very low The estimate of the effect is very uncertain,and often willbe far from the truth1Prednisone and prednisolone are equivalent,used in the same dosage,and have both been used in RCTs.All later references to prednisone ororal corticosteroids refer to prednisone or prednisolone.Pediatr Nephrol(2013)28:415–426417(moderate)because of the poor methodological quality of some of the RCTs included in the systematic review.The daily dose of 60mg/m 2/day and the starting dose of 40mg/m 2for alternate day therapy were used empiri-cally by the ISKDC [11]and the APN [12]and have not been examined in RCTs so while these doses are recom-mended,the quality of evidence supporting these data is very low.Theoretical studies [13]have suggested that under-dosage with prednisone can occur if a per-kilogram dose is used particularly in children weighing below 30kg.However,there are few data [14]to suggest that this is of clinical relevance,so the workgroup concluded that either method for calculating prednisone dose may be used.The mean time to remission did not differ significantly when prednisone was given as a single daily dose compared to divided doses (two RCTs;66children;weighted mean difference 0.04days;95%CI −0.98to 1.06)[10].The duration of daily and alternate day prednisone is harder to define.Daily prednisone should be given for at least 4weeks based on one RCT [12],which showed that 8weeks of prednisone (4weeks of daily,4weeks of alternate day)was significantly more effective in maintaining remission at 6and 12months com-pared with prednisone given daily until remission and on alternate days until the albumin level exceeded 35g/l (total duration about 4weeks).An increase in benefit was seen when alternate-day prednisone was given for up to 6months (risk ratio 01.26–0.112duration;r 200.56,p 00.03)[10].Corticosteroids for relapsing steroid-sensitive nephrotic syndromeKDIGO Glomerulonephritis Workgroup,2012:“3.2:Treatment of relapsing SSNS with corticosteroids 3.2.1:Corticosteroid therapy for children with infre-quent relapses of SSNS:3.2.1.1:We suggest that infrequent relapses of SSNSin children be treated with a single daily dose of prednisone 60mg/m 2or 2mg/kg (maxi-mum of 60mg/day)until the child has been in complete remission for at least 3days.(2D )3.2.1.2:We suggest that,after achieving completeremission,children be given prednisone as a single dose on alternate days (40mg/m 2per dose or 1.5mg/kg per dose:maximum 40mg on alternate days)for at least 4weeks.(2C )3.2.2:Corticosteroid therapy for frequently relapsing(FR)and steroid-dependent (SD)SSNS:3.2.2.1:We suggest that relapses in children with FRor SD SSNS be treated with daily prednisoneT a b l e 3D e s c r i p t i o n o f c r i t e r i a f o r s t r e n g t h o f r e c o m m e n d a t i o n s u s e d b y K D I G OG r a d eI m p l i c a t i o n sP a t i e n t sC l i n i c i a n sP o l i c y L e v e l 1,“W e r e c o m m e n d ”M o s t p e o p l e i n y o u r s i t u a t i o n w o u l d w a n t t h e r e c o m m e n d e d c o u r s e o f a c t i o n a n d o n l y a s m a l l p r o p o r t i o n w o u l d n o t M o s t p a t i e n t s s h o u l d r e c e i v e t h e r e c o m m e n d e d c o u r s e o f a c t i o nT h e r e c o m m e n d a t i o n c a n b e e v a l u a t e d a s a c a n d i d a t e f o r d e v e l o p i n g a p o l i c y o r p e r f o r m a n c e m e a s u r e L e v e l 2,“W e s u g g e s t ”T h e m a j o r i t y o f p e o p l e i n y o u r s i t u a t i o n w o u l d w a n t t h e r e c o m m e n d e d c o u r s e o f a c t i o n b u t m a n y w o u l d n o tD i f f e r e n t c h o i c e s w i l l b e a p p r o p r i a t e f o r d i f f e r e n t p a t i e n t s .E a c h p a t i e n t n e e d s h e l p t o a r r i v e a t a m a n a g e m e n t d e c i s i o n c o n s i s t e n t w i t h h e r o r h i s v a l u e s a n d p r e f e r e n c e sT h e r e c o m m e n d a t i o n i s l i k e l y t o r e q u i r e s u b s t a n t i a l d e b a t e a n d i n v o l v e m e n t o f s t a k e h o l d e r s b e f o r e p o l i c y c a n b e d e t e r m i n e dR e p r o d u c e d w i t h p e r m i s s i o n f r o m K i d n e y D i s e a s e :I m p r o v i n g G l o b a l O u t c o m e s (K D I G O )G l o m e r u l o n e p h r i t i s W o r k G r o u p .K D I G O C l i n i c a l P r a c t i c e G u i d e l i n e f o r G l o m e r u l o n e p h r i t i s [8]418Pediatr Nephrol (2013)28:415–426until the child has been in remission for at least3days,followed by alternate-day prednisonefor at least3months.(2C)3.2.2.2:We suggest that prednisone be given on alter-nate days in the lowest dose to maintain re-mission without major adverse effects inchildren with FR and SD SSNS.(2D)3.2.2.3:We suggest that daily prednisone at the lowestdose be given to maintain remission withoutmajor adverse effects in children with SDSSNS where alternate-day prednisone therapyis not effective.(2D)3.2.2.4:We suggest that daily prednisone be givenduring episodes of upper respiratory tractand other infections to reduce the risk forrelapse in children with FR and SD SSNSalready on alternate-day prednisone.(2C)”(164–165).Children with SSNS have an80–90%chance of having one or more relapses[15,16].Of those who relapse,about half will relapse infrequently while the remainder have frequently relapsing or steroid-dependent SSNS.There are few RCT data on prednisone treatment of relapsing SSNS.The Guideline for infrequently relapsing SSNS is based on data from an RCT, which found no significant difference in the number of chil-dren who relapsed between8weeks of daily prednisone compared with daily prednisone until remission followed by 4weeks of intermittent prednisone(further relapse by 9months RR1.07;95%CI0.77–1.50)[10].The guideline for frequently relapsing and steroid-dependent SSNS is based on an RCT[10],which showed that the risk for relapse at12 and24months was significantly reduced in children treated for7months of prednisone compared with2months.Data from three RCTs[17–19](158children)show that prednisone therapy for5–7days during an infection reduces the risk of relapse in children with steroid-dependent SSNS.There are no RCT data on the use of low-dose alternate-day or daily prednisone to maintain remission in frequently relapsing or steroid-dependent SSNS.Observational studies have shown that low-dose alternate-day prednisone(mean dose0.48mg/kg on alternate days)or daily prednisone (0.25mg/kg/day)reduced the risk of relapse compared with historical controls[20,21].Corticosteroid-sparing agents for frequently relapsing and steroid-dependent SSNSKDIGO Glomerulonephritis Workgroup,2012:“3.3:Treatment of FR and SD SSNS with corticosteroid-sparing agents 3.3.1:We recommend that corticosteroid-sparing agents beprescribed for children with FR SSNS and SDSSNS,who develop steroid-related adverse effects.(1B)”(165).The corticosteroid-sparing agents considered by the workgroup were alkylating agents(cyclophosphamide, chlorambucil),levamisole,calcineurin inhibitors(cyclo-sporine,tacrolimus),mycophenolate mofetil,rituximab, mizoribine,and azathioprine.Alkylating agentsKDIGO Glomerulonephritis Workgroup,2012:“3.3.2:We recommend that alkylating agents,cyclo-phosphamide or chlorambucil,be given ascorticosteroid-sparing agents for FR SSNS.(1B)We suggest that alkylating agents,cy-clophosphamide or chlorambucil,be given ascorticosteroid-sparing agents for SD SSNS.(2C)3.3.2.1:We suggest that cyclophosphamide(2mg/kg/day)be given for8–12weeks(maximum cu-mulative dose168mg/kg).(2C)3.3.2.2:We suggest that cyclophosphamide not bestarted until the child has achieved remissionwith corticosteroids.(2D)3.3.2.3:We suggest that chlorambucil(0.1–0.2mg/kg/day)may be given for8weeks(maximumcumulative dose11.2mg/kg)as an alternativeto cyclophosphamide.(2C)3.3.2.4:We suggest that second courses of alkylatingagents not be given.(2D)”(165–166).Five RCTs(134children)comparing alkylating agents and prednisone with prednisone alone have demonstrated that alkylating agents significantly reduce the risk of relapse (Table4).These trials did not differentiate between fre-quently relapsing and steroid-dependent SSNS.There were no significant differences at12months in efficacy between cyclophosphamide and chlorambucil(RR1.15,95%CI 0.69to1.94)[22,23].Since a post hoc analysis of this trial [22]and a review[24]of observational studies and RCTs showed that alkylating agents resulted in longer durations of remission in frequently relapsing compared with steroid-dependent SSNS(72vs.40%after2years and36vs. 24%after5years),the workgroup recommended alkylating agents for frequently relapsing SSNS(level1B)but only suggested their use for steroid-dependent SSNS(level2C).Because of potential gonadal,hematological,and other toxicities of both agents[24–27]and some data suggesting that the margin between therapeutic effect and toxicity isPediatr Nephrol(2013)28:415–426419less for chlorambucil[25]than for cyclophosphamide,the guideline suggests maximum durations and cumulative doses for these agents and suggests that second courses of an alky-lating agent not be given.Because cyclophosphamide is rarely associated with hemorrhagic cystitis,it is suggested that cy-clophosphamide should be administered when the child is in remission and can receive a high fluid intake.Though not included as a guideline,cyclophosphamide may be given intravenously when non-adherence to therapy is a risk.Two RCTs[23,28,29]have demonstrated no significant difference(RR0.99;95%CI0.76to1.29)in the risk of relapse at12–24months follow-up between oral cyclophosphamide(8–12weeks)and IV cyclophosphamide (monthly pulses for6months).LevamisoleKDIGO Glomerulonephritis Workgroup,2012:“3.3.3:We recommend that levamisole be given as a corticosteroid-sparing agent.(1B)3.3.3.1:We suggest that levamisole be given at a doseof2.5mg/kg on alternate days(2B)for at least12months(2C)as most children will relapsewhen levamisole is stopped”(166).Five RCTs,comparing levamisole with/without predni-sone with prednisone or no specific therapy,have shown that the risk for relapse was reduced by57%(Table4).A sixth RCT[30]using a low dose(2.5mg/kg given on two consecutive days each week)showed no significant benefit. Observational studies suggest that levamisole can be used for12–24months[31–33].Levamisole is generally well tolerated,with minor leucopenia and/or gastrointestinal upsets described in some patients.Rarely,cutaneous vasculitis has been reported[34].Levamisole is not avail-able in many countries and currently there is limited avail-ability in other countries.The results of a large multicenter, double-blind,placebo-controlled RCT to assess the efficacy of levamisole(2.5mg/kg on alternate days)in SSNS are expected in2013[35].Calcineurin inhibitorsKDIGO Glomerulonephritis Workgroup,2012:“3.3.4:We recommend that the calcineurin inhibitors (CNI),cyclosporine or tacrolimus,be givenas corticosteroid-sparing agents.(1C)3.3.4.1:We suggest that cyclosporine be administeredat a dose of4–5mg/kg/day(starting dose)intwo divided doses.(2C)3.3.4.2:We suggest that tacrolimus0.1mg/kg/day(starting dose)in two divided doses be usedinstead of cyclosporine when the cosmeticside-effects of cyclosporine are unacceptable.(2D)3.3.4.3:Monitor CNI levels during therapy to limittoxicity.(Not Graded)3.3.4.4:We suggest that CNIs be given for at least12months as most children will relapse whenCNIs are stopped.(2C)”(166).Two RCTs comparing cyclosporine with alkylating agents have demonstrated that there was no significant difference in the risk of relapse(95children;RR0.91;95%CI0.55to 1.48)during cyclosporine therapy[23,36,37].However, since most children relapse when cyclosporine is ceased,the remission rate is lower after cyclosporine compared with alkylating agents when assessed at12–24months afterTable4Meta-analyses of randomized controlled trials of corticosteroid-sparing agents compared with prednisone and/or placebo in children with frequently relapsing or steroid-dependent steroid-sensitive nephrotic syndromeAgent RCTs a N Patients N Risk ratio of relapse(95%CI b)Time of outcome(months)Relative risk reductionCyclophosphamide31020.44(0.26,0.73)6–1256% Chlorambucil2320.13(0.03,0.57)1287% Levamisole52690.43(0.27,0.68)4–1257% Mizoribine1197Relapse rate ratio c0.81(0.61,1.05)18Not significant Azathioprine2600.90(0.59,1.38)6Not significanta Randomized controlled trialsb Confidence intervalsc Relapse risk ratio0[total number of relapses÷observation period in treatment group]÷[total number of relapses÷observation period in control group]Reproduced with permission from Kidney Disease:Improving Global Outcomes(KDIGO)Glomerulonephritis Workgroup.KDIGO Clinical Practice Guideline for Glomerulonephritis[8]420Pediatr Nephrol(2013)28:415–426completing treatment.In observational studies,cyclosporine maintains remission in60–90%of children with SD SSNS, who had relapsed after alkylating-agent therapy[38–40].Tacrolimus has not been studied in RCTs and there are limited observational data[41,42]to support its use in SSNS. It is used increasingly because of the cosmetic side-effects of cyclosporine(hypertrichosis,gum hyperplasia).However, since there are no RCTs that compare the relative benefits and harms of tacrolimus and cyclosporine in SSNS,there are currently limited data to show that tacrolimus can replace cyclosporine in SSNS despite the grade1C recommendation for its use by the KDIGO workgroup.This designation is tempered by the grade2D recommendation to prescribe tacro-limus when cyclosporine’s cosmetic side-effects limit its use.Both medications may cause hypertension,kidney dys-function[43],renal interstitial fibrosis[38,44]and,in trans-plant recipients,diabetes mellitus[45].Adding ketoconazole to cyclosporine resulted in no loss of efficacy but a48% reduction in the mean dose of cyclosporine with net cost savings of38%in a non-randomized comparator study[46].The length of treatment with CNIs is controversial,with some authors suggesting that CNI therapy should be restricted to2years because of the adverse effects on kidney function and histology[47].In children receiving cyclosporine for 12months or more,tubulointerstitial lesions on kidney biopsy are reported in30–40%of cases.This increases to80%after 4or more years of treatment[47].However,cyclosporine-associated arteriopathy is uncommon.In an ungraded state-ment(not shown),the guideline suggests that kidney biopsy should be performed in children with decreasing glomerular filtration rates which persist after CNI doses are reduced.The guideline does not support the suggestion[47]that children should undergo annual biopsies if CNI therapy is continued beyond2years,because it is unclear whether the benefits of regular kidney biopsies exceed the harms.In an RCT,the sustained remission rate was significantly higher in children established on cyclosporine and maintaining a12-h cyclosporine trough level of60–80ng/ml(mean dose 4.7mg/kg/day)compared to children treated with a fixed dose of2.5mg/kg/day[48].Limited data suggest peak(C2)levels rather than trough(C0)levels can be used for monitoring[49].Mycophenolate mofetil(MMF)KDIGO Glomerulonephritis Workgroup,2012:“3.3.5:We suggest that MMF be given as acorticosteroid-sparing agent.(2C)3.3.5.1:We suggest that MMF(starting dose1,200mg/m2/day)be given in two divideddoses for at least12months,as most chil-dren will relapse when MMF is stopped.(2C)”(p166).To date,most studies on mycophenolic acid prodrugs in SSNS have used MMF.Though a small RCT(24children) found no significant difference in the risk of relapse between MMF and cyclosporine(RR5;95%CI0.68,36.66),there was considerable imprecision in the results indicated by the very wide confidence interval[23,50].As it underpowered with small patient numbers,a difference in the efficacy be-tween these medications has not been excluded by this study. Preliminary data from a crossover study[51]suggest that MMF is less effective than cyclosporine in maintaining remis-sion,with one or more relapses occurring in21children when receiving MMF and in nine children during cyclosporine treatment among the60children included in the study.Also, this study suggested that relapses were related to lower myco-phenolic acid levels.However,glomerular filtration rate is maintained with MMF while it falls during CNI therapy[50, 51].In observational studies,MMF has been used for up to 45months and has been well tolerated[52],with few children developing abdominal symptoms or leucopenia. RituximabKDIGO Glomerulonephritis Workgroup,2012:“3.3.6:We suggest that rituximab be considered only in children with SD SSNS,who have continu-ing frequent relapses despite optimal combina-tions of prednisone and corticosteroid-sparingagents,and/or who have serious adverseeffects of therapy.(2C)”(166).The place of rituximab,an anti-CD20monoclonal anti-body,in the treatment of steroid and CNI-dependent SSNS remains to be established.A single open-labeled RCT en-rolling54children with SD SSNS dependent on prednisone and CNIs found that rituximab significantly reduced the rate of relapse at3months(18.5%and48.1%in experimental and control arms,respectively)and increased the probability of a child not requiring prednisone and CNI treatment[53]. Case series report prolonged remissions in80%of children following rituximab[54,55].Rituximab caused acute reac-tions(fever,vomiting,diarrhea,skin rash,bronchospasm)in about one-third of patients[55].Other reported serious side-effects include Pneumocystis jiroveci pneumonia and pul-monary fibrosis[54,56].Other medicationsKDIGO Glomerulonephritis Workgroup,2012:“3.3.7:We suggest that mizoribine not be used as a corticosteroid-sparing agent in FR and SDSSNS.(2C)Pediatr Nephrol(2013)28:415–426421。
慢性肾病的诊断与治疗(2024版)
慢性肾病的诊断与治疗（2024版）1. 引言慢性肾病（Chronic Kidney Disease, CKD）是指持续的肾脏功能损害，已成为全球范围内严重的公共卫生问题。
根据2024年全球肾脏病流行病学调查，我国慢性肾病发病率呈逐年上升趋势，给社会及家庭带来了沉重的经济负担。
为此，提高对慢性肾病的认识，加强早期诊断、治疗及管理显得尤为重要。
本文档旨在提供关于慢性肾病的诊断与治疗的最新指南，以助于临床医生、药师及相关专业人士更好地了解和应对这一挑战。
2. 慢性肾病的诊断2.1 病史询问详细询问病史对于诊断慢性肾病至关重要，应包括：- 肾脏病史：如蛋白尿、血尿、水肿、高血压等；- 全身性疾病史：如糖尿病、高血压、 autoimmune disease 等；- 药物及毒物暴露史：如氨基糖苷类抗生素、非甾体抗炎药等；- 家族史：如家族性肾病综合征等。
2.2 体格检查体格检查时应注意：- 血压：高血压是慢性肾病的重要危险因素；- 腹部：有无肿块、触痛等；- 下肢：水肿、静脉曲张等。
2.3 实验室检查慢性肾病的实验室检查主要包括：- 尿液检查：蛋白尿、血尿、白细胞、红细胞等；- 血液检查：血肌酐、血尿素氮、电解质、贫血指标等；- 肾脏生物标志物：如肾小球滤过率(eGFR)、肾小球损伤标志物等。
2.4 影像学检查影像学检查有助于了解肾脏大小、形态及尿路情况，主要包括：- 超声检查：常规首选，可评估肾脏大小、结构及血流情况；- 计算机断层扫描（CT）：对肾结石、肾肿瘤等具有较高诊断价值；- 核磁共振成像（MRI）：对肾脏结构及功能具有较高分辨率。
3. 慢性肾病的治疗3.1 药物治疗慢性肾病的药物治疗主要包括：- 降压药物：ACEI、ARB、钙通道阻滞剂等；- 降脂药物：他汀类、贝特类等；- 贫血治疗药物：重组人促红细胞生成素、铁剂等；- 骨代谢调节药物：钙剂、维生素D及其衍生物等；- 肾脏保护药物：ACEI、ARB、碳酸氢钠等。
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肾病综合征诊断治疗指南【概述】肾病综合征（nephrotic syndrome, NS）是肾小球疾病的常见表现，由多种病因引起，其对治疗的反应和预后差异甚大。
临床上不能仅满足肾病综合征的诊断，必须对其作出病因、病理、并发症乃至完整诊断，以提高肾病综合征治疗的缓解率，改善患者的预后。
引起原发性肾病综合征的病理类型有多种，以微小病变肾病、肾小球局灶节段硬化、系膜增生性肾炎、膜性肾病、系膜毛细血管性肾炎等几种类型最为常见。
肾病综合征的分类根据病因分为原发性和继发性，前者之诊断主要依靠排除继发性NS。
继发性NS 的病因常见于糖尿病肾病、狼疮性肾炎、肾淀粉样变性、药物、肿瘤等。
【临床表现】1．症状和体征可发生于任何年龄，发病前可有职业病史、有毒有害物接触史、服用药物或食物过敏史等情况；可继发于呼吸道、皮肤的感染、病毒性肝炎、肿瘤、糖尿病、系统性疾病等，起病可急骤也可隐匿，患者可有乏力、恶心、腰酸、食欲下降等，部分患者可无明显临床症状。
除水肿、蛋白尿外，临床还可表现为血尿、高血压及不同程度肾功能减退。
其主要症状为水肿，特点是水肿首先出现于皮下组织较疏松部位，如眼睑、颜面等处，然后出现于下肢（常从踝部开始），多为指压凹陷性水肿，严重的可发展至全身，引起胸水、腹水、心包积液。
水肿与体位有明显的关系，如出现一侧下肢与体位无关的固定性水肿时应怀疑下肢深静脉血栓形成。
但也有部分患者可水肿不明显。
2．实验室检查典型的肾病综合征实验室检查表现为：⑴大量蛋白尿（尿蛋白定量>3.5g/d）；⑵低白蛋白血症（血浆白蛋白<30g/L）；⑶高脂血症。
此外，尿沉渣镜检红细胞可增多，可见管型，肾功能正常或受损（GFR 下降），可伴免疫指标（抗核抗体、抗双链DNA、ANCA、免疫球蛋白等）、肿瘤指标（CEA、AFP、PSA 等）、病毒指标（HBV、HCV、HIV 等）、骨髓穿刺活检异常。
肾穿刺活检可明确病理分型。
3．肾病综合征的主要并发症：（1）感染:肾病综合征患者由于存在营养不良、免疫状态异常、激素及免疫抑制剂的应用，感染的机会增加。
感染部位多发生在呼吸道、泌尿系统和消化道。
常见的致病菌有肺炎球菌、溶血链球菌和大肠埃希菌等，引起呼吸道感染：肺炎、支气管炎；胸膜炎。
其他如结核杆菌、病毒（疱疹病毒等）、真菌的感染机会也明显增加。
在严重肾病综合征伴大量腹水时，易在腹水的基础上发生自发性细菌性腹膜炎（spontaneous bacterial peritonitis,SBP）。
（2）血栓栓塞:血栓栓塞是肾病综合征常见的甚至严重致死性的并发症之一。
临床上以肾静脉和深静脉血栓最为常见，部分可呈典型肺梗死表现。
大多数肾静脉血栓的患者表现为亚临床型。
膜性肾病中肾静脉血栓的发生率最高，可达50%以上，其次为膜增生性肾炎。
（3）急性肾衰竭:急性肾衰竭是肾病综合征的主要并发症。
可发生在肾病综合征的不同阶段，但以疾病初期和肾病未获缓解时的发生率为最高。
合并急性肾衰的原因主要有①严重血容量不足所致的肾前性氮质血症；②缺血、感染或药物引起的急性肾小管坏死；③感染、药物及过敏所致的急性间质性肾炎；④高凝所致的急性肾静脉血栓形成。
⑤肾间质水肿。
对肾病综合征合并急性肾衰者应积极寻找原因，及早给予对因治疗，肾功能大多可恢复正常。
（4）代谢紊乱:肾病综合征患者存在明显的低白蛋白血症，蛋白代谢呈负平衡。
长期低白蛋白血症可造成患者营养不良、贫血、机体抵抗力下降、生长发育迟缓、甲状腺素水平低下、钙磷代谢紊乱、维生素D 缺乏等。
【诊断要点】1. 大量蛋白尿（尿蛋白定量>3.5g/d）2. 低白蛋白血症（血浆白蛋白<30g/L）3. 高度水肿4. 高脂血症（血浆胆固醇、甘油三酯均明显增高）前两项是诊断肾病综合征的必要条件，后两项为次要条件。
临床上只要满足上述 2 项必要条件，肾病综合征的诊断即成立。
对肾病综合征患者应肾活检明确病理类型，指导临床治疗。
肾病综合征可为原发性和继发性。
如考虑为继发性应积极寻找病因，在排除继发性NS,如糖尿病肾病、紫癜性肾炎、狼疮性肾炎、乙肝相关性肾炎、肾淀粉样变等之后才能诊断为原发性NS。
肾病综合征并非独立疾病，在肾活检基础上完善病理类型的诊断尤为重要。
原发性肾小球肾炎所致的肾病综合征常见的病理类型分为：（1）微小病变型（MCD）：光镜下肾小球基本正常，近端肾小管上皮细胞可见脂肪变性，故又被称为“类脂性肾病”。
免疫荧光阴性，电镜下特征性表现为弥漫性足突融合，肾小球内一般无电子致密物沉积。
（2）系膜增生性肾小球肾炎（MsPGN）：光镜可见肾小球弥漫性系膜细胞增生伴系膜基质增多，而肾小球毛细血管壁和基底膜正常。
按免疫荧光结果可分为IgA 肾病（单纯IgA或以IgA 沉积为主）和非IgA 系膜增生性肾小球肾炎（以IgG 或IgM 沉积为主）。
（3）局灶节段性硬化（FSGS）：其病理特征为局灶损害。
病变以系膜基质增多、血浆蛋白沉积、球囊粘连、玻璃样变性为特征，伴或不伴球性硬化。
电镜可见弥漫性足细胞足突消失，免疫荧光呈现IgM 和C3 沉积。
（4）膜性肾病（MN）：以局限于肾小球基膜的免疫复合物沿肾小球基底膜外侧（上皮下）沉积，刺激基底膜增殖，致使“钉突”形成、基底膜弥漫增厚为特征的一种疾病。
（5）膜增生性肾小球肾炎（MPGN）：其共同特点为肾小球基底膜增厚、系膜细胞增生及系膜基质扩张，毛细血管袢呈“双轨征”为其典型特征性病理改变。
难治性肾病综合征是指部分患者表现为对激素依赖或激素抵抗。
激素依赖是指激素治疗有效，激素减量或停药后 2 周内复发。
激素抵抗是指使用足量泼尼松（龙）1mg/(kg·d)或甲泼尼龙0.8mg/(kg·d)，8～12 周无效，局灶节段肾小球硬化的判断时间应延长为16 周。
【治疗方案及原则】1. 病因治疗有继发性原因者应积极治疗原发病。
对基础疾病采取积极有效的治疗：包括手术或化疗治疗肿瘤；停用相关药物；进行积极有效的抗肝炎病毒治疗；治疗感染性疾病；有效控制自身免疫性疾病等。
2. 对症支持治疗（1）一般治疗①休息:肾病综合征患者应适当注意休息，有严重浮肿及低白蛋白血症者应以卧床休息为主。
病情稳定者应适当活动，以防止血栓形成。
②饮食:在肾病综合征严重低白蛋白血症时蛋白质的摄入量为1.2-1.5g/(kg·d)。
在严重水肿或高血压时，应限制钠盐及水的摄入量，一般摄入钠为2~3g/d。
少油、低胆固醇饮食。
（2）利尿消肿：对于浮肿明显，限钠限水后仍不能消肿者可适当选用利尿剂。
①噻嗪类利尿剂:主要作用于远曲小管，通过抑制氯和钠在髓袢升支粗段及远端小管前段的重吸收而发挥利尿作用。
常用的有氢氯噻嗪，剂量一般为50~100mg/d，分次口服。
使用时需注意低钠和低钾的发生。
②襻利尿剂:主要作用于髓袢升支粗段，抑制钠、钾和氯的重吸收。
利尿作用快速而强大。
常用的有呋塞米，20~100mg/d，分次口服。
其他袢利尿剂如托拉塞米，利尿作用较强而持久，尿钾、钙的排出作用较呋塞米弱。
使用时注意低钠、低钾和低氯的发生。
③潴钾利尿剂：主要作用于远端小管后段，抑制钠和氯的重吸收，但有潴钾作用，潴钾利尿剂单独使用利尿效果欠佳，与噻嗪类利尿剂合用能增强利尿效果，并减少电解质紊乱的发生。
常用的有螺内酯，20~40mg，每日2~3 次口服。
使用时注意高血钾的发生，肾功能不全者慎用。
④补充白蛋白：可提高血浆胶体渗透压，促进组织间隙中的水分回吸收到血管内而发挥利尿作用。
补充白蛋白的适应症为肾病综合征严重水肿、明显低白蛋白血症，使用利尿剂不能达到利尿消肿效果时。
补充白蛋白可以减轻水肿等症状，但对病程没有明显的影响。
NS治疗不应过度补充白蛋白而应强调针对原发病的治疗。
（3）降压治疗肾病综合征患者应严格控制血压，降压的靶目标应低于130/80mmHg，虽然血管紧张素转换酶抑制剂（ACEI）和血管紧张素受体拮抗剂（ARB）能有效控制血压、降低蛋白尿、延缓肾衰进展、降低心血管并发症的发生率和死亡率等，但在肾病综合征严重水肿时，存在肾血流量相对不足时，应避免使用，以免引起肾前性急性肾衰。
在肾病综合征部分缓解或稳定后开始应用，并可根据病情剂量翻倍，降低蛋白尿。
（4）糖皮质激素原发性肾病综合征治疗的最基本药物仍为糖皮质激素。
激素使用的原则为①起始剂量要足。
成人泼尼松1mg/(kg·d)，最大剂量不超过60－80mg/d；儿童可用至2mg/(kg·d)，最大剂量不超过80mg/d。
足量治疗维持4~12 周，视病理类型而定。
目前一般不主张膜性肾病采用足量激素治疗，而采用半量糖皮质激素联合免疫抑制剂治疗。
②肾病综合征缓解后逐渐递减药物。
③激素治疗的总疗程一般在6~12 个月，对于常复发的肾病综合征患者，在激素减至0.5mg/(kg·d)或接近肾病综合征复发的剂量时，维持足够长的时间，然后再逐渐减量。
激素剂量在10mg 左右时，副作用较大剂量时明显减少。
目前常用的激素是泼尼松，在有肝功能损害的患者选用泼尼松龙或甲泼尼龙口服。
糖皮质激素治疗NS 时要注意个体化，应尽可能采用每天一次顿服。
长程糖皮质激素治疗时应注意药物副作用（如高血糖、高血压、股骨头无菌性坏死、消化道溃疡、感染等），定期进行相关检查。
（5）免疫抑制治疗对激素依赖或激素抵抗，或激素有反指征患者可考虑在激素基础上加用或单用免疫抑制剂治疗。
但要密切注意药物的毒副反应。
①烷化剂：环磷酰胺（cyclophosphamide，CTX）是临床应用最多的烷化剂。
CTX 的一般剂量为2mg/(kg·d)，口服2~3 个月；或每次0.5-0.75g/m2，静脉滴注，每月一次。
病情稳定后减量，累积剂量一般不超过10－12g。
CTX 的主要副作用为骨髓抑制、肝功能损害、性腺抑制、脱发、出血性膀胱炎、感染加重及消化道反应。
使用过程中应定期检查血常规和肝功能。
②环孢素A（cyclosporin A，CsA）：是神经钙调酶抑制剂，可通过选择性抑制T 辅助细胞及细胞毒效应而起作用。
起始剂量为3~5mg/(kg·d)，大部分患者在治疗的一个月内起效。
起效后逐渐减量，维持剂量≥6 个月。
血药浓度应维持在谷浓度100~200ng/ml，峰浓度800ng/ml 左右。
环孢素A 的副作用主要为齿龈增生、多毛、肝、肾毒性等。
肾功能不全及小管间质病变严重的患者慎用。
③其他：吗替麦考酚酯（mycophenolate mofetil，MMF）、他克莫司（Tacrolimus，FK506）等用于治疗激素抵抗和激素依赖的原发性肾病综合征有一定疗效。
主要抑制T、B淋巴细胞增殖。
能增加肾病综合征的缓解率、降低复发率、减少激素等的副反应。
具体剂量、疗程视个体而异。