慢性肾脏病（chronic kidney disease，CKD）已成为全球性的公共卫生问题。“肾脏病预后质量倡议（Kidney Disease Outcome Quality Initiative，KDOQI）”和“改善全球肾脏病预后组织（Kidney Disease: Improving Global Outcomes，KDIGO）”工作组先后制定了CKD 临床实践指南。为提高我国CKD防治水平，在参考国外指南基础上，结合中国特点，我们组织上海市肾脏病专家制定了《CKD筛查 诊断及防治指南》，并于2017年发表。该指南对各级全科医师和肾脏病专科医师均有参考价值，有力推动了对CKD的认识和提高了对CKD的管理水平。近年来CKD 研究又取得了很多重大进展，基于临床证据的积累及各种新药的问世，上海市肾内科临床质量控制中心专家组对2017年版《CKD筛查 诊断及防治指南》进行了更新和修订，内容主要包括CKD筛查的对象和方式、进展的评估及防治等方面。

糖尿病肾脏疾病（diabetic kidney disease，DKD）是我国常见病与多发病，同时也是引起终末期肾脏病的主要原因。因此，加强DKD防治具有重要意义。目前，国内外已颁布了多种有关糖尿病诊断、治疗、管理的临床指南或专家共识，其中也涉及了DKD诊疗的部分内容，但并不能满足肾科医生的需求。对DKD患者这一特殊人群，合理用药、规范诊疗、细化管理等问题仍有待解决。另外，随着医疗新技术的发展，有关DKD的知识不断更新，特别是新型药物的临床应用，拓展了DKD诊疗策略，因此有必要编写一部适用于中国人群的DKD诊疗指南。鉴于此，中华医学会肾脏病学分会组织了专家组编写了这部《DKD临床诊疗中国指南》。该指南参考了国内外相关指南与专家共识，整合了中国肾脏病专家的临床经验，系统地介绍了DKD诊断、肾脏病理活检、治疗与管理、合并症处理及常用药物的合理应用等问题。另外，专家组在编写过程中本着严谨、简明、权威的原则，参阅了国际指南格式，提出诊疗要点、逐条列证说明。该指南反映了当今DKD诊疗的新趋势、新观点，对进一步加强DKD认识、规范DKD诊疗体系、制定合理治疗原则、指导精准用药、延缓肾脏病进展、提高患者生活质量具有重要价值，可供广大医师在临床工作中参考。

肾脏在调节人体钾代谢中起着重要作用。肾衰竭患者易发生急性或慢性高钾血症；肾小管酸中毒、失盐性肾病等患者易发生低钾血症。无论高钾血症还是低钾血症均可引起细胞膜电位异常，导致四肢麻痹、心律失常甚至猝死等严重并发症。中华医学会肾脏病学分会专家组参考国内外最新文献，根据中国临床诊疗实践特点，围绕钾代谢的机制、低钾血症、高钾血症及慢性肾脏病患者血钾的监测四部分内容制定了《中国慢性肾脏病患者血钾管理实践专家共识》。本共识是我国首个指导慢性肾脏病（CKD）患者血钾异常综合性管理的临床共识，旨在引起广大肾科医师重视，为我国CKD患者血钾管理的规范化诊断和治疗提供指导性建议。

为加强透析前期和初始透析慢性肾脏病（chronic kidney disease，CKD）患者的管理，《中国围透析期慢性肾脏病管理规范》专家组编写了本规范。规范首先提出“围透析期CKD”的概念及特征，其并发症发生率、病死率及治疗费用显著增高，同时肾小球滤过率快速降低，需要强化管理；接着本规范系统全面地阐述了围透析期CKD患者病情评估指标与频率、肾脏替代治疗时机与模式选择、透析充分性评估、围透析期常见并发症的诊断和处理；最后提出了围透析期CKD的未来研究方向。围透析期CKD管理一直是肾脏病临床的难点，本管理规范的制定、宣传及落实能够提高我国围透析期CKD患者的管理水平，从而对改善患者的存活率和生存质量、减轻医疗负担具有重要意义。

Objective To summarize the clinical features of 9 cases with mutations in PKHD1 gene for a better understanding of its phenotype. Methods Clinical data of nine cases with mutations in PKHD1 gene were summarized from January 2011 to December 2016 in our center, including clinical manifestations, laboratory findings, imaging data and family investigation. Next generation sequencing was used to screen 4000 genes in case 1 to 4 and whole exons in case 5 to 9. Significant variants detected by next generation sequencing were confirmed by conventional Sanger sequencing. Segregation analysis was performed using parental DNA samples. Relevant literature was reviewed. Results Among these 9 cases, 5 are male, 4 are female. The average age of onset was 2.6 years old (ranging from 0.5-5.2 years). Renal ultrasound revealed that all 9 cases had cysts in bilateral kidney, 7 cases with enlarged kidney, 1 case with normal size kidney, 1 case with normal size kidney, and 1 case with bilateral renal atrophy. Two cases with renal artery stenosis, 1 case with focal narrowing in left main branch and 1 case with vesico-ureteral reflux were found. Among the 9 cases, 3 cases had homozygous mutations, and 6 cases had compound heterozygous mutations, including 1 nonsense mutation, 1 frameshift mutation and 15 missense mutations. There were 2 cases with 3 heterozygous mutations, 2 c.5935C＞T mutations and 2 cases with C. 5869G＞A mutations. A total of 10 new mutations were identified. Conclusion Patients with mutations in the PKHD1 gene had normal size kidney, or even atrophic kidney. Renal artery stenosis, vesicoureteral reflux and bronchial stenosis were all first reported in patients with mutations in PKHD1 gene. The novel mutations, c.274C＞T, c.9059T＞C, c.8996delG, c.281C＞T, c.10424T＞A, c.7092T＞G, c.4949T＞C, c.5869G＞A, c.6197A＞G and c.1877A＞G further expanded the mutation spectrum of PKHD1 gene.

利妥昔单抗（rituximab，RTX）是一种作用于B细胞表面CD20分子的人鼠嵌合单克隆抗体，靶向诱导B细胞凋亡，减少自身抗体的产生，对系统性红斑狼疮、肾小球肾炎等与自身抗体致病相关的疾病治疗存在理论基础。改善全球肾脏病预后组织（Kidney Disease: Improving Global Outcomes，KDIGO）指南也建议把RTX用于治疗膜性肾病、微小病变肾病、狼疮肾炎等肾小球疾病。目前，RTX治疗肾小球肾炎在我国尚处于起步阶段，存在临床适应证不一、治疗方案多样等问题，导致疗效评估和分析存在困难。为此，中华医学会肾脏病学分会专家组通过阅读相关文献，并结合我国临床诊疗现状，对RTX治疗肾小球肾炎的适应证、治疗方案及随访管理注意事项等形成专家共识，以指导临床医生的工作实践，并为未来制定临床指南提供基础。

心力衰竭（heart failure）是透析（dialysis）患者的第二大心血管疾病。透析患者一旦合并心力衰竭，生存率显著下降。尽管国内外已颁布了多部普通人群心力衰竭相关的临床指南或专家共识，但由于透析患者肾功能极差甚至没有残余肾功能、慢性并发症多，加上血液透析（hemodialysis，HD）和腹膜透析（peritoneal dialysis，PD）治疗本身的特殊性、可变性及局限性，使透析患者与普通人群在心力衰竭诊断、治疗及管理等方面均存在很大的不同。故而现有国内外指南和共识并不完全适用于透析人群，迫切需要透析患者心力衰竭管理的指导性文件，以指导、规范心力衰竭的诊疗。鉴于此，中华医学会肾脏病学分会和中关村肾病血液净化创新联盟组织专家组制定了这部《中国透析患者慢性心力衰竭管理指南》。该指南基于循证医学证据及临床经验，就心力衰竭诊断、危险因素管理、HD管理、PD管理、药物管理及其他管理等问题做了系统的介绍，反映了当今透析患者心力衰竭诊疗的新观点及未来发展趋势，有利于进一步加强临床医生对透析患者心力衰竭的认识、规范透析患者心力衰竭的临床管理流程，对改善透析患者预后具有重要价值。

Objective To compare different equations for estimated glomerular filtration rate (eGFR) in patients with chronic kidney disease (CKD). Methods Hospitalized patients with CKD from the nephrology department of the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital) were recruited between December 2014 and May 2015. The calculations of eGFR and 24 h creatinine clearance rate (Ccr) were accomplished in three days after admission. The eGFRs were calculated separately using the 24 h creatinine clearance rate adjusted by the standard body surface area (Ccr_BSA), Cockcroft-Gault equation adjusted by the standard body surface area (eCcr_BSA), CKD-EPI creatinine equation (EPI_Cr), CKD-EPI cystatin C equation (EPI_CysC), CKD-EPI creatinine-cystatin C equation (EPI_Cr_CysC), simplified MDRD (MDRD) and China MDRD equations. The EPI_Cr_CysC equation was used as the standard and the precision and accuracy of the other six equations were compared and analyzed. Results A total of 403 CKD participants were enrolled in the study, with 228 male patients and a mean age of (54.9±18.4) years. The main primary diseases were chronic glomerulonephritis (43.7%) and diabetic nephropathy (13.2%). The median concentration of serum creatinine and cystatin C were 117.5 (69.7, 242.4) μmol/L and 1.80 (1.13, 3.31) mg/L, respectively. The median values of Ccr_BSA, eCcr_BSA, MDRD, China MDRD, EPI_Cr, EPI_CysC and EPI_Cr_CysC equations were 50.8 (21.1, 96.2), 51.9 (23.3, 93.2), 53.6 (23.0, 97.4), 52.2 (22.4, 94.1), 53.2 (22.1, 97.3), 35.1 (15.4, 67.0) and 49.1 (22.8, 82.3) ml?min-1?(1.73 m2)-1, respectively. There was well agreement among MDRD, China MDRD and EPI_Cr equations, while there were large differences between equations derived from CysC (EPI_Cr_CysC and EPI_CysC) and equations derived only from creatinine (EPI_Cr, MDRD, China MDRD, eCcr_BSA, Ccr_BSA equations). Compared with EPI_Cr_CysC equation (the reference equation), EPI_Cr equation showed the highest accuracy [percentage of other eGFR equation calculations that were ＞30% of the reference equation calculations (1-P30), 30.8%] while Ccr_BSA equation showed the lowest (1-P30, 42.4%). EPI_CysC equation showed the highest precision [inter-quartile range (IQR) of the difference, 11.7 ml?min-1?(1.73 m2)-1] while Ccr_BSA equation showed the lowest [IQR of the difference, 22.8 ml?min-1?(1.73 m2)-1]. Conclusions The agreement among equations derived only from creatinine is better; while it exhibits some differences between equations with cystatin C and equations derived only from creatinine. The accuracy of EPI_Cr equation is second only to EPI_Cr_CysC equation and it is currently the most suitable eGFR equation for clinical popularization of renal glomerular function assessment.

Objective To evaluate the prevalence of chronic kidney disease (CKD) in Chinese adult health check-up population, and to compare with the prevalence of CKD in the study of the general population as well as the large CKD cross-sectional study in China. Methods Epidemiological studies about CKD in Chinese adults health check-up population from January 2007 to December 2017 were searched in PubMed, SinoMed, CNKI, VIP and Wanfang Data. Meta-analysis of the prevalence of CKD was performed with software of Stata 12.0. Subgroup analyses of CKD staging, urban and rural, as well as geographical areas of the general population were executed. Results Twenty-two studies from adult health check-up population were included (238 349 persons). Egger's regression showed no publication bias (P＞0.05). The unstandardized prevalence rate of CKD was 12.49% (male 12.8%, female 12.5%). The respective unstandardized prevalences of proteinuria, hematuria and eGFR decline were 5.90%, 5.83% and 2.75%. The unstandardized prevalences of CKD in urban and rural population were 13.21% and 11.90%. The stages of CKD were mainly concentrated in the early stages. There was no significant difference in the non-standard detection rate of total eGFR decline among the adult medical examination population, the general population and the population studied cross-sectionally (P＞0.05). Furthermore, no significant difference in the non-standard detection rate of total hematuria and male hematuria was found between the adult health check-up population and the general population. In addition, the total proteinuric non-standard detection rate of the adult general population was similar with that of population studied cross-sectionally (P＞0.05). Conclusions The prevalence of CKD in Chinese adults is higher, the overall prevalence is however underestimated. The results of epidemiological investigation in adult health check-up population are similar to those of the general population, especially in men.

抗中性粒细胞胞质抗体（anti-neutrophil cytoplasmic antibody，ANCA）相关肾炎（ANCA-associated glomerulonephritis，AAGN）是造成中老年肾脏病患者急性肾功能减退的主要原因，病情严重，人肾存活率低。我国AAGN患者的ANCA类型、临床特征及预后与欧美国家的AAGN存在差异。基于我国对AAGN的临床研究证据并借鉴国外相关指南，中华医学会肾脏病学分会组织专家制定了ANCA相关肾炎的诊断和治疗中国指南，在AAGN的诊断和评估、随访管理、诱导期和维持期治疗、顽固性和复发性AAGN的治疗及感染的预防等方面提出了推荐或建议，为规范临床实践和个体化治疗决策的制定提供了指导意见。

Objective To compare the survival rates of elderly hemodialysis (HD) and peritoneal dialysis (PD) patients and identify their independent prognostic predictors. Methods Patients aging ＞60 years old who initiated dialysis between January 1, 2008 and December 31, 2014 were included. Propensity score method (PSM) was applied to adjust for selection bias. Kaplan-Meier method was used to obtain survival curves and a Cox regression model was used to evaluate risk factors for mortality. Results 447 eligible patients with maintenance dialysis were identified, 236 with hemodialysis and 211 with peritoneal dialysis. 174 pairs of patients were matched, with the baseline data [age, gender, Charlson comorbidity index (CCI) and the primary disease] between two groups showing no significant difference (P＞0.05). Cardiovascular events, cerebrovascular events and infection were major causes of death in both groups and there was no significant difference in the causes of death between two groups (P＞0.05). The overall survival rates at 1 and 5 year were 93.6% and 63.4% respectively in HD group, 91.9% and 61.5% in PD group. The differences of total survival rates between HD and PD patients were not significant (P＞0.05). Cox regression analysis showed age(≥80 year) (P＜0.001, HR=1.058, 95%CI 1.028-1.088), diabetic nephropathy (P=0.001, HR=2.161, 95%CI 1.384-3.373), CCI≥5 (P=0.007, HR=1.935, 95%CI 1.201-3.117) were independent prognostic risk predictors in HD patients; age(≥80 year) (P=0.022, HR=1.043, 95%CI 1.006-1.081), serum albumin level ＜ 35 g/L (P=0.025, HR=1.776, 95%CI 1.075-2.934), and prealbumin (P=0.012, HR=0.968, 95%CI 0.944-0.993) were independent prognostic predictors in PD patients. Conclusions The differences of total survival rates between aged HD and PD patients are not significant. Age, diabetic nephropathy, CCI≥5 and age, serum albumin＜35 g/L, prealbumin＞30 g/L respectively influence the survival of elderly HD and PD patients.

Objective      To evaluate the effects of high glucose on autophagy and apoptosis of podocyte and explore the signaling pathway in high glucose - induce podocyte autophagy.    Methods       Differentiated mouse podocytes were exposed to high glucose(30 mmol/L) or rapamycin (autophagy enhancer, 1 μg/L) or LY294002 (a selective PI3K inhibitor, 50 mmol/L) for 24 h. The formations of autophagy were observed by electron microscopy and acridine orange staining. Apoptosis was evaluated by flow cytometry. The expression of autophagy protein LC3 - II/I and Beclin - 1 as well as the phosphorylation of AKT and mTOR were examined by Western blotting analysis.   Results       High glucose induced podocytes apoptosis，increased autophagy and the expression of autophagy-associated proteins (all P＜0.05). Rapamycin further increased the expression of LC3-II and Beclin-1 protein (all P＜0.05)，but LY294002 inhibited partialiy the protein expression of LC3-II and Beclin-1 induced by high glucose (both P＜0.05). Treatment with rapamycin increased the phosphorylation of AKT, but reduced that of mTOR in podocytes. Moreover, LY294002 inhibited phosphorylation of both AKT and mTOR (both P＜0.05).   Conclusions       High glucose promotes podocyte autophagy and apoptosis. High glucose-induced autophagy is mediated partly through PI3K-AKT-mTOR signaling pathway.

终末期糖尿病肾脏病患者常见，其包含两种情况：一是由糖尿病引起的终末期肾病（end-stage renal disease，ESRD），又称之为糖尿病肾病；二是慢性肾脏病合并糖尿病，但糖尿病不是ESRD的病因，称之为合并糖尿病的ESRD。终末期糖尿病肾脏病患者行肾替代治疗的心脑血管事件发生率高、外周血管条件差、感染等并发症的风险高，治疗时血糖波动大，管理难度大。专家组对终末期糖尿病肾脏病肾替代治疗的时机、方式选择、腹膜透析、血液透析、肾移植治疗要点进行总结并提出有关建议，希望能引起广大肾科医师重视，不断提高治疗水平。

糖尿病（diabetes mellitus，DM）是全球关注的重大慢性病之一，目前我国糖尿病患病人数居全球首位。糖尿病肾病（diabetic kidney disease，DKD）是糖尿病最常见的严重微血管并发症之一，近年来患病率逐年攀升，已经超越原发性肾小球疾病，成为慢性肾脏病的首位病因。由于DKD发生机制复杂，其防治策略一直是国内外研究的热点，研究表明多种疗法在减少DKD患者尿蛋白排泄、延缓肾脏疾病进展中发挥重要作用。本文将从DKD基础治疗及新兴疗法两方面对DKD治疗的研究进展作一综述。

随着影像技术的不断发展，碘对比剂在介入治疗、血管造影等领域的应用日益增多。虽然碘对比剂的临床应用大大提高了疾病的诊疗水平，但其引起的急性肾损伤不容忽视。为降低碘对比剂诱导的急性肾损伤的发生率，中华医学会临床药学分会、中国药学会医院药学专业委员会和中华医学会肾脏病学分会组织国内专家，成立《碘对比剂诱导的急性肾损伤防治的专家共识》编写委员会，在参考国内外相关指南、共识及研究进展的基础上，结合我国的实际情况，针对碘对比剂的结构和分类、已上市的碘对比剂品种及理化性质、碘对比剂的应用现状、碘对比剂诱导的急性肾损伤的定义、流行病学、生物标志物、病理特征、危险因素与风险评估、预防措施和治疗手段等方面进行证据检索和评价，并充分讨论，制定了本共识，为临床医、药、护、技更有效、更安全地合理使用碘对比剂提供指导建议。

Objective    To compared two classical rat models of nephrotic syndrome and to provide some reference data to researchers.    Methods    Thirty male SD rats were randomly divided into control group, puromycin aminonucleoside-induced nephrotic syndrome (PAN) group and adriamycin-induced nephrotic syndrome (ADR) group. The body weight, twenty four hour proteinuria level, serum albumin concentration, cholesterol concentration, creatinine and urea concentration were measured. The renal pathology change was evaluated. The drug toxic effects, administration methods and the costs were also compared.    Results    There was no significant difference in body weight and hair color between control group and PAN group. Compared to control group, the body weight of the rats significantly decreased at day 15 and day 21 in ADR group (P＜0.01), accompanied by epilation and diarrhea. Compared to control group, the 24-hour urinary protein levels increased significantly at day 10 (P＜0.01), day 15 (P＜0.01), and  reached the peak level at day 15 (P＜0.01), day 21 (P＜0.01) in PAN group and ADR group respectively. Compared to control group, the serum albumin concentration decreased significantly at day 10 (P＜0.01), and return to normal level at day 15. The serum cholesterol concentration was increased significantly at day 10 (P＜0.01) and return to normal at day 15 in PAN group. Compared to control group, the serum albumin concentration was decreased significantly at day 15 (P＜0.05) and return to normal at day 21 in ADR group. No significant difference of serum creatinine and serum urea nitrogen levels were found among three groups. Compared to control group, the width of foot process increased significantly at day10 (P＜0.01) and day 15 (P＜0.05) in PAN group and ADR group respectively. To successfully induce a nephrotic rat model (per 100 g), the cost of PAN group was 3.1 times of ADR group (578.10 yuan vs 186.94 yuan).    Conclusions    Nephrotic syndrome can be induced by both PAN and ADR. The administration of PAN via intraperitoneal injection is more convenient as compared to ADR via tail intravenous injection. Compared to ADR, PAN can induce nephrotic syndrome model more rapidly, with more consistent detection index, and less toxic effects, but its cost is more expensive.
  

Objective To clarify the clinicopathological features of renal amyloidosis in order to achieve early diagnosis and treatment. Methods Clinicopathological data of 26 biopsy-proven renal amyloidosis cases in Department of Nephrology, Zhongshan Hospital, Fudan University between 2006 and 2010 were analyzed retrospectively. Immunohistochemistry and immunofluorescence of amyloid A protein, immunoglobulin light chains such as ?资、?姿 were performed on renal specimens for further classification. Results Age of 26 patients ranged from 40 to 77 years old, average (58.54±10.07) years. Twenty-two out of 26 patients (84.62%) were treated in local hospital before admitted to our department, and 21 patients (95.45%) were misdiagnosed as chronic primary glomerulonephritis. The prominent clinical manifestations of renal amyloidosis were nephrotic syndrome (17 cases, 65.38%), decreased blood pressure (16 cases, 61.53%), organ enlargement (8 cases, 30.77%) and bodyweight loss (6 cases, 23.08%). Fourteen out of 25 patients (56.00%) were found to have monoclonal light chains in serum by immunofixation electrophoresis. Three patients with mild pathological changes who had no confirmable Congo red stain were confirmed by electron microscopy. Twenty-three (88.46%) patients were diagnosed as AL amyloidosis, one (3.85%) as AA amyloidosis, one was strongly suspected of hereditary amyloidosis, and one was undetermined. Conclusions Renal amyloidosis is frequently misdiagnosed. Middle-aged and old nephrotic patients with decreased blood presure, organ enlargement and bodyweight loss may be the most helpful clues of the disease. Most patients have monoclonal light chains in serum or urine. Renal biopsy, especially electronic microscopy plays a crucial role in the early diagnosis of renal amyloidosis. Immunohistochemistry is important for patients with renal amyloidosis in pathological classification and treatment.