Given the remarkable growth in the prevalence of kidney disease, its deficient diagnosis and treatment and poor clinical outcomes with heavy medical burden, the China Kidney Innovation Association (CKIA) summarized and refined the "Top Ten Innovative Directions" in the field of kidney disease, aiming to address urgent clinical pain points and conduct in-depth analysis in the diagnosis, treatment, and research of kidney disease. This work is based on innovative, prospective, scientific and feasible principles. Through extensive collection, in-depth discussions, and rigorous research, the expert group have condensed ten innovative research directions covering innovative diagnostic technologies, precision treatment methods and cutting-edge research approaches, in an effort to provide clear guidance and direction for future innovative research and transformation in kidney disease.

Chronic kidney disease (CKD) during the peridialysis period (CKD-PeriDialysis) includes two stages: predialysis and initial dialysis. During this period, patients have a high incidence of complications and comorbidities, a high mortality rate, high treatment costs, and a rapid decrease in glomerular filtration rate. In the early stage of dialysis, due to the particularity, variability, and limitations of dialysis technology, the patients' internal environment undergoes drastic changes, and the mortality rate increases instead of decreasing. Therefore, there is an urgent need for guiding documents for the management of CKD patients during the peridialysis period in clinical practice. In view of this, the Clinical Practice Guidelines for the Management of CKD during Peridialysis in China (2025) Working Group of the Kidney Disease Dialysis Special Committee of the China Association of Non-Public Medical Institutions has developed this clinical practice guidelines for management of CKD during peridialysis in China (2025). This guideline is based on evidence-based medicine and clinical experience, comprehensively elaborating on the disease evaluation indicators and frequency, the timing and mode selection of renal replacement therapy, the assessment of dialysis adequacy, and the diagnosis and management of common complications during the peridialysis in CKD patients during the peridialysis. This guideline reflects new perspectives and future development trends in the diagnosis and treatment of CKD patients during the peridialysis, which is conducive to further strengthening the understanding of nephrologists on CKD during the peridialysis, standardizing clinical management processes, thereby improving survival rates and quality of life of the patients, and reducing medical burden.

In recent years, ultrasound-guided percutaneous transluminal angioplasty (PTA) has been widely used for the treatment of stenotic or occlusive lesions in hemodialysis vascular access. However, there is currently a dearth of clinical guidelines or expert consensus for this technology. Therefore, it becomes an urgent problem in the field of hemodialysis vascular access how to further standardize the clinical procedures of ultrasound-guided PTA to ensure its efficacy and safety in clinical diagnosis and treatment. For this purpose, Chinese consensus expert group on ultrasound interventional therapy for hemodialysis vascular access has formulated this "Expert consensus on ultrasound interventional therapy for hemodialysis vascular access in China (2024)". The present consensus encompasses several key aspects, including preoperative assessment, ultrasound diagnostic procedures, interventional treatment standards, surgical operating procedures, and strategies for managing complications. It aims to provide practical guidance for clinicians to improve treatment outcomes, mitigate the risk of complications, and enhance patients' quality of life. The promotion of this consensus is expected to facilitate the standardized application and popularization of ultrasound interventional techniques in the treatment of arteriovenous dialysis access.

IgA nephropathy is the most common primary glomerular disease globally, with the highest incidence in the Asian region, and has a high risk of progressing to end-stage renal disease even in patients with low proteinuria. The treatment paradigm for IgA nephropathy has undergone significant changes. Treatment should aim to reduce pathogenic IgA and IgA immune complex formation, including intestinal mucosal B cell immune modulators such as budesonide enteric-coated capsules, targeted APRIL and BAFF agents, and B cell depletors; it should also manage glomerular inflammation, including corticosteroids, mycophenolate mofetil, hydroxychloroquine, and targeting complement therapy; and it should manage general responses to nephron loss, including lifestyle interventions, renin-angiotensin system inhibitors, sodium-glucose cotransporter-2 inhibitors, and endothelin receptor antagonists. This article provides a comprehensive overview of the treatment paradigm and drug advancements for IgA nephropathy, aiming to provide more rational treatment options for IgA nephropathy patients and improve their outcomes.

Rituximab (RTX) is a human mouse chimeric monoclonal antibody that acts on CD20 molecules on the surface of B cells. Currently, its application in the treatment of immune glomerular diseases is becoming increasingly widespread. However, there are still many problems in the application of RTX by clinical physicians. In response to this, we organized multiple rounds of discussions and revisions among national nephrologists on the 50 clinical issues that everyone is most concerned about and formed this article. The content covers the scope of application, evidence-based evidence, therapeutic regimen, drug usage methods and precautions, medication for special populations, and drug safety of rituximab in the treatment of membranous nephropathy, minimal change nephropathy, antineutrophil cytoplasmic antibody (ANCA) associated vasculitis and other immune glomerular diseases. In particular, specific answers are provided to typical questions raised by clinical doctors, aiming to provide practical guidance and reference for the broader clinicians.

Chronic kidney disease (CKD)-associated pruritus (CKD?aP) is also known as uremic pruritus. It is one of the common complications in patients with end-stage renal disease. CKD?aP can occur in CKD people of all ages, with a high prevalence, seriously impairing quality of life and physical/mental health, increasing long-term mortality risk, and imposing substantial burdens on healthcare systems. It is worth noting that there is a lack of effective drugs for the treatment of CKD?aP in clinical work, and it is often overlooked by medical workers. Therefore, in order to improve clinicians' understanding of CKD?aP and provide standardized clinical practice guidance, the Expert Committee of China Special Fund for Kidney Disease Prevention and Treatment has reached this consensus on the definition, diagnostic procedures, evaluation criteria, and treatment principles of CKD?aP, aiming to provide reference for clinicians in nephrology and other related disciplines to standardize clinical diagnosis and treatment.

Common complications related to nutritional metabolism and somatic function in chronic kidney disease patients include protein-energy wasting, sarcopenia, and frailty. These three complications are different and closely related. This article reviews recent research progress on the definitions, epidemiology, diagnosis and evaluation, underlying causes, intervention measures and their differences and connections of chronic kidney disease in conjunction with protein-energy wasting, sarcopenia and frailty, to help clinicians identify them and personalize interventions.

Chronic kidney disease (CKD) is a significant global public health issue. In recent years, several new drugs have shown substantial efficacy in CKD treatment. Sodium-glucose cotransporter 2 inhibitors, nonsteroidal mineralocorticoid receptor antagonists, and glucagon-like peptide-1 receptor agonists improve renal outcomes in CKD patients through various mechanisms, and also demonstrate favorable effects on cardiovascular outcomes. Novel agents, including endothelin receptor antagonists, are accumulating clinical evidence for delaying kidney disease progression, with ongoing development of new therapeutic targets. This article reviewed the latest research progress of these drugs in CKD treatment, offering new perspectives and reference points for comprehensive CKD management.

Membranous nephropathy (MN) is a type of glomerular disease characterized by diffuse thickening of glomerular basement membrane with subepithelial immune complex deposition, and traditional diagnosis of MN mainly relies on the pathological results of renal biopsy. In recent years, the emergence of biomarkers related to MN such as phospholipase A2 receptor and thrombospondin type-1 domain-containing 7A has changed the diagnosis and treatment mode of MN, providing a new basis for the diagnosis, treatment and prognosis of MN. MN patients with positive specific target antigens exhibit different clinical manifestations and prognoses. Specific target antigens can not only guide diagnosis, but also has predictive value for prognosis. Immunosuppressive therapy is a common treatment for idiopathic MN patients, and the emergence of novel medications such as biologics represents a advance in the treatment of MN, providing a broader array of options for managing the condition. Conversely, the treatment approach for secondary MN primarily targets the management of the primary disease. Based on multiple and new literature, we reviewed the researches progress of target antigens and immunosuppressive therapy related to MN, so as to provide references for clinical diagnosis and treatment of MN.

Immune checkpoint inhibitors (ICI) are a recently emerged class of antitumor therapeutic agents which have shown good respond in the treatment of various tumors. The accompanying immune-related adverse events (irAE) are a new class of adverse drug reactions that are closely related to ICI therapy and can affect multiple organs. Although the incidence of renal irAEs is approximately 1.4%-5.8%, the number of patients is increasing significantly as the base population using the ICI drug continues rapidly expanding. If severe renal irAE are not recognized and treated in time, they may rapidly progress to renal failure, which will bring adverse consequences to the treatment of kidney and tumor. In view of this, the Chinese Society of Nephrology organized an expert group to prepare this consensus basing on relevant domestic and international consensus, guidelines, and research. The propose of this consensus is to introduce the diagnosis, treatment, and management of renal irAE, and provide guidance and suggestions to clinical practitioners on standardization of diagnosis and treatment of renal irAE.

Patients with chronic kidney disease (CKD), whether undergoing dialysis treatment or not, are at a high risk of hyperkalemia. Recurrent episodes of hyperkalemia can, significantly increase the risks of cardiovascular events and mortality, hospitalization, and renal replacement therapy in CKD patients, and severely affect the long-term survival benefits of CKD patients. Potassium ion binders play an important role in controlling hyperkalemia in CKD patients, reducing complications, and avoiding the reduction or discontinuation of renin-angiotensin- aldosterone system inhibitors. To guide the normative use of potassium ion binders, the Nephrology Branch of Chinese Research Hospital Association has organized a group of expert renal disease specialists. They have formulated this consensus based on the latest domestic and international management guidelines/consensus and research findings, combined with domestic clinical practice, to improve the long-term prognosis of hyperkalemia in Chinese CKD patients.

Objective To elucidate the prevalence of hyperphosphatemia and utilization rate of phosphorus-lowering drugs in adult non-dialysis chronic kidney disease (CKD) patients in China, and explore the relationship between hyperphosphatemia, phosphate-lowering therapy, and clinical outcomes. Methods It was a large retrospective and multicenter cohort study. The study subjects were sourced from the China Renal Data System. The first-time hospitalized patients aged 18 years old and above and diagnosed with CKD who did not enter dialysis and having serum phosphorus test results from 2013 to 2020 were included. Hyperphosphatemia was defined as an initial serum phosphorus level exceeding 1.45 mmol/L during hospitalization, and normal serum phosphorus was defined as 0.97 mmol/L ≤ serum phosphorus ≤ 1.45 mmol/L. Cox regression model was employed to assess the association of hyperphosphatemia with all-cause mortality, cardiovascular mortality, and CKD progression as well as the association of phosphorus-lowering therapy with all-cause mortality and cardiovascular mortality. Results A total of 157 987 adult non-dialysis CKD patients with serum phosphorus test results were included in the study, with age of 60 (47, 72) years old and 91 453 males (57.89%). The estimated glomerular filtration rate was 63.49 (39.12, 92.90) ml·min^-1·(1.73 m²)^-1. The common comorbidities included hyperlipidemia (53.48%, 84 497/157 987), hypertension (37.23%, 58 818/157 987) and heart failure (27.02%, 42 686/157 987). The prevalence of hyperphosphatemia was 14.83% (23 431/157 987). Among CKD stage 3-5 patients with hyperphosphatemia, the utilization rate of phosphate-lowering medications was 13.34% (3 962/29 705). Multivariate Cox regression analysis showed that among 64 662 patients with ≥ 90 days of follow-up [3.3 (1.5, 5.4) years], hyperphosphatemia was significantly correlated with an increased risk of all-cause mortality and cardiovascular mortality (hyperphosphatemia/normal serum phosphorus, HR=1.13, 95% CI 1.06-1.22, P=0.001; HR=1.28, 95% CI 1.04-1.56, P=0.018, respectively). Among 47 581 patients with ≥ 90 days of follow-up [1.8 (0.9, 3.2) years] and baseline estimated glomerular filtration rate > 30 ml · min^-1 ·(1.73 m²)^-1, hyperphosphatemia was significantly correlated with an increased risk of CKD progression (hyperphosphatemia/normal serum phosphorus, HR=1.08, 95% CI 1.00-1.17, P=0.038). Among 8 856 patients with follow-up data of death [3.3 (1.5, 5.4) years] and hyperphosphatemia,phosphate-lowering medication use was significantly correlated with a reduced risk of all-cause mortality (HR=0.88, 95% CI 0.82-0.95, P<0.001) and cardiovascular mortality (HR=0.84, 95% CI 0.72-0.97, P=0.022). Conclusions The prevalence of hyperphosphatemia is high among Chinese adult non-dialysis CKD patients, but the utilization rate of phosphate-lowering medications remains low. Hyperphosphatemia is an independent risk factor of all-cause mortality, cardiovascular mortality, and kidney function progression, while phosphate-lowering medications may mitigate these risks in non-dialysis CKD patients. Regular monitoring of serum phosphorus level for non-dialysis CKD patients, and initiating phosphate-lowering therapy for hyperphosphatemia patients may positively improve clinical outcomes.

Diabetes, kidney disease, and cardiovascular disease often coexist and mutually exacerbate one another. Substantial evidence has demonstrated that finerenone, a novel nonsteroidal mineralocorticoid receptor antagonist, provides significant cardiorenal benefits—on top of onventional blood pressure and glucose control—in patients with type 2 diabetes-associated chronic kidney disease, as well as in those with mildly reduced or preserved ejection fraction, and even in non-diabetes-related chronic kidney disease. In order to make the clinical application of finerenone more reasonable and standardized, a multidisciplinary panel of experts in endocrinology, cardiology, and nephrology has developed the multidisciplinary expert consensus on the clinical application of finerenone (2025 edition). Building upon the 2023 Chinese expert consensus, this updated document integrates the latest evidence and practical experience from a multidisciplinary perspective. It puts forward 19 recommendations aimed at serving as a practical reference for the rational use of finerenone across relevant clinical disciplines.

Cognitive impairment is a common complication in patients with chronic kidney disease (CKD), which is characterized by high incidence, difficulty in control, difficulty in treatment, significant impact and poor prognosis, but clinicians generally lack understanding of it. The Blood Purification Branch of China Association for Promotion of Health Science and Technology collaborates with multidisciplinary experts such as nephrology and neurology, reviews and summarizes the epidemiology, risk factors, pathogenesis, clinical manifestations, assessment, diagnosis and treatment of CKD-related cognitive impairment, and forms the first Chinese expert consensus, aiming to enhance clinicians' understanding of cognitive impairment in CKD, strengthen the management of cognitive impairment, and improve patients' prognosis.

With the advancement of blood purification technology in China, double- filtration plasmapheresis (DFPP), a form of plasma exchange therapy, has been widely used and popularized in the fields of nephrological, rheumatological, gastroenterological and neurological diseases. The key advantages of DFPP lie in its selective removal of pathogenic substances from plasma, effective control of disease progression, and significant conservation of exogenous plasma, thereby reducing the risk of blood-borne infections. Currently, there are no unified guidelines or consensus regarding the clinical application of DFPP in kidney diseases, either domestically or internationally. To address this gap, the Expert Group of the Dialysis and Transplantation Branch of the Chinese Society of Bioengineering has developed the "Experts consensus on the clinical application of DFPP in Kidney Diseases (2025)". This consensus provides comprehensive guidance on the indications, contraindications, technical considerations, specific application recommendations for kidney diseases, and management of complications associated with DFPP. Its primary aim is to standardize the clinical use of DFPP in kidney diseases and offer practical recommendations for medical professionals in the field.

In recent years, rituximab has been gradually used in the treatment of idiopathic membranous nephropathy (IMN). Compared with traditional treatments, the safety and effectiveness of rituximab in the treatment of IMN have been confirmed, which induces remission in 60%-80% of patients. For the remaining 20%-40% patients, several mechanisms can explain rituximab resistance: decreased rituximab bioavailability; internalized by targeted B cells; the generation of anti-rituximab antibody; chronic and irreversible damage to the glomerular filtration barrier; autoreactive B-cell clones in secondary lymphoid organs that cannot be effectively eliminated. The treatment of patients with rituximab-refractory IMN remains controversial and challenging. The recognition of IMN as an antibody-mediated autoimmune disease has rationalized the use of immunosuppressive drugs such as B cell-targeted therapies, plasma cell-targeted therapies, and complement inhibitors. This review mainly summarizes recent advances in the understanding of the physiological mechanisms of rituximab resistance, and in the management of rituximab-refractory IMN, aiming to aid in the clinical management of IMN.

The α?Klotho protein is an anti-aging protein encoded by the Klotho gene and mainly exists in the renal tubules in the form of type 1 membrane protein (membrane-boundform). The extracellular domain of the membrane-bound α?Klotho protein can be cleaved and secreted into the circulation, which is called soluble α?Klotho. Soluble α?Klotho is the main form that can be detected in the body fluid. This paper mainly reviews the biological functions of soluble α?Klotho in the kidney and its possible molecular biological mechanisms, and also summarizes the role of soluble α?Klotho in various kidney diseases and the effects of α?Klotho on the other organs.

Ferroptosis is an iron-dependent programmed cell death. Iron overload and lipid reactive oxygen accumulation play a core role in the occurrence and development of ferroptosis. Any factors affecting the balance of iron metabolism and redox system may induce and aggravate ferroptosis. Ferroptosis is involved in the pathological process of acute kidney injury, diabetic nephropathy, renal interstitial fibrosis and some other kidney diseases, and inhibiting ferroptosis has potential renoprotective benefits. This article reviewed the pathophysiological mechanism of ferroptosis and its research progress in renal diseases, and discussed the application prospect of targeted ferroptosis in the treatment of renal diseases.

Objective To analyze the disease burden of chronic kidney disease (CKD) in the Belt and Road countries and its change trend. Methods It was a cross-sectional epidemiological study based on surveillance data. Data on age-standardized prevalence rate (ASPR) and age-standardized mortality rate (ASMR) of CKD were derived from the 2019 Global Burden of Disease Study. The annual percentage change (APC) was calculated to evaluate the ASPR trend of CKD from 1990 to 2019. Results In 2019, the number of CKD cases and deaths in the Belt and Road countries was 426 million and 798 000, respectively, accounting for 61.1% and 55.9% of CKD cases and deaths worldwide. The ASPR and ASMR of CKD in China were 8.1% and 11.2 per 100 000 population, slightly lower than the global average. Countries in North America and Oceania had a higher burden of CKD, and European countries had a lower burden. In the etiology, hypertension and diabetes-related CKD morbidity and mortality accounted for 23.7% and 68.8% of total CKD morbidity and mortality, respectively. From 1990 to 2019, the ASPR of CKD increased in 150 countries (98.0%) and the fastest increase was observed in Morocco (APC=1.52%). The hotspots with high ASPR of CKD were located in Belt and Road countries from Asia, South/North America and Oceania, and the hotspots with high ASMR were distributed in countries from Africa, South/North America and Oceania. The sociodemographic index and life expectancy were positively correlated with the ASPR of CKD (r=0.409, P<0.001; r=0.361, P<0.001) , and negatively correlated with the ASMR of CKD (r=-0.317, P<0.001; r=-0.391, P<0.001). Conclusions Belt and Road countries have substantial disease burdens of CKD, and the prevalence rate of CKD is rising fast. Health cooperation among member states should be strengthened to jointly address the challenges posed by chronic diseases such as CKD.

As the most important component of renal parenchyma, tubular epithelial cells (TECs) play numerous regulatory roles in the occurrence and development of chronic kidney disease(CKD). Exosomes, double-membrane vesicles that are naturally secreted by cells, contain various bioactive molecules, including proteins, nucleic acids, and lipids, and serve as mediators for intercellular communications. This article reviewed the sources and characteristics of TECs-derived exosomes, the roles of TECs-derived exosomes affecting renal interstitial fibrosis in CKD, and the roles of TECs-derived exosomes in the diagnosis of CKD, to provide new ideas for the prevention and treatment in CKD.

Chronic kidney disease-associated pruritus (CKD?aP) is a common complication in patients with end-stage renal disease, which strongly reduces the quality of life. The pathogenesis of CKD?aP is complex, with unclear etiology, and there is no recognized treatment method. This paper reviews the research progress of the pathogenesis of CKD?aP, including the hypotheses of toxin deposition, peripheral neuropathy, immune and inflammatory system disorder, and opioid receptor imbalance, and the treatment of CKD?aP, including adequate dialysis, local skin medication, systemic medication, nutrition, ultraviolet B, and acupuncture.

Polycystic kidney disease (PKD) is a hereditary kidney disease characterized by the formation of numerous cysts in the kidneys, which progressively impairs renal function over time. PKD is primarily divided into two types: autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), with ADPKD being more prevalent. Current treatments primarily focus on symptom relief and disease progression delay, lacking a curative approach. However, the development of gene editing technologies such as clustered regularly interspaced short palindromic repeats (CRISPR) and CRISPR-associated protein 9 (CRISPR-Cas9) and adeno-associated virus (AAV) vectors has offered new therapeutic possibilities for ADPKD and ARPKD. These include approaches like antisense oligonucleotides (ASO), adenovirus-mediated gene knockdown, CRISPR- Cas9, Pkd1 gene enhancement therapy, and the use of induced pluripotent stem cells (iPSCs), which have shown potential efficacy in animal models and early clinical studies. Despite facing technological challenges, ethical and legal issues, and high costs, gene therapy presents an unprecedented hope for PKD treatment. Future interdisciplinary collaboration and international cooperation are essential for developing more effective treatment strategies for PKD patients.

IgA nephropathy (IgAN) is the most common primary glomerular disease and main reason of progression to end?stage renal disease in patients with kidney diseases. Various clinical, pathological, demographic and new factors discovered by researchers in recent years play important roles in the prognosis assessment of IgAN. Furthermore, developing prognostic evaluation models based on several different factors to identify patients at high risk of progression in the early disease stage is clinically significant. This article reviews the researches progress of influencing factors and relevant evaluation models for prognosis of IgAN.

Objective To evaluate the efficacy and safety of sacubitril?valsartan (SV) in reducing blood pressure in patients with non-dialysis-dependent chronic kidney disease(NDD-CKD) stage 3-5 and concomitant hypertension. Methods It was a retrospective study. Adult NDD-CKD stage 3-5 patients with hypertension treated with SV alone or in combination with SV on the basis of 2 to 4 antihypertensive drugs having unsatisfactory effects from March 1, 2022 to June 30, 2023 in Zhongda Hospital Affiliated to Southeast University were included. SV doses ranged from 25 mg once daily to 200 mg twice daily. The primary outcome was blood pressure control. The changes of blood pressure and laboratory examination indexes between baseline and 1 to 2 months after SV treatment were compared, and adverse events were also recorded. Results A total of 37 NDD-CKD stages 3-5 patients with hypertension were included in the study, with age ranging from 29 to 85 years old, and 15 males (40.5%). The SV duration of medication was 1 (1, 1) month. At the study endpoint, there was a significant decrease in mean systolic blood pressure, diastolic blood pressure, and pulse pressure from baseline, with reduction of 20.4 (9.6, 28.8) mmHg (1 mmHg=0.133 kPa, Z=-5.243, P<0.001), (6.9±7.6) mmHg (t=5.532, P<0.001), and 13.0 (8.0, 18.8) mmHg (Z=-4.941, P<0.001), respectively. During the study period, 8 patients (21.6%) experienced worsening of renal function, and there were no statistically significant differences in the changes of serum creatinine and estimated glomerular filtration rate before and after treatment (Z=-0.487, P=0.626; Z=-0.110, P=0.912, respectively). No adverse drug reactions such as hyperkalemia, symptomatic hypotension, angioedema, hepatic impairment, and decreased hemoglobin level were observed during the study period. Conclusion SV effectively lowers blood pressure in NDD-CKD stage 3-5 patients with hypertension and exhibits good safety.

Kidney is a highly energy-demanding organ rich in mitochondria. Numerous studies have indicated that mitochondria play a crucial role in maintaining normal kidney function and in the pathogenesis of various kidney diseases. Mitochondrial DNA is the exclusive genome of mitochondria. Damage to mtDNA not only leads to mitochondrial dysfunction and degradation of mitochondrial quality, but also acts as an endogenous inflammatory molecule, activating various inflammatory pathways, which contribute to cellular damage and the progression of kidney diseases. This article reviews the mechanisms of mitochondrial DNA damage and its significant role in triggering inflammatory injury in kidney diseases. Additionally, it summarizes the current research progress on various intervention strategies targeting this type of damage.

Objective To explore the prevalence and independent associated factors of vascular calcification (VC) in non-dialysis chronic kidney disease (CKD) patients of stage 3-5. Methods It was a single-center cross-sectional observational study. Non-dialysis stage 3-5 CKD patients ≥18 years old who were admitted to the Department of Nephrology, the First Affiliated Hospital of Sun Yat-sen University from May 1, 2022 to December 31, 2022 with VC evaluation were enrolled. The patients' general information, laboratory examination and imaging data were collected. Coronary artery calcification (CAC), thoracic aorta calcification (TAC), abdominal aorta calcification (AAC), carotid artery calcification and aortic valve calcification (AVC) were evaluated by cardiac-gated electron-beam CT (EBCT) scans, lateral lumbar x-ray, cervical macrovascular ultrasound and echocardiography, respectively. The differences in clinical data and the prevalence of VC at different sites of patients with different CKD stages were compared, and the prevalence of VC at different sites of patients in different age groups [youth group (18-44 years old), middle-aged group (45-64 years old) and elderly group (≥65 years old)] and patients with or without diabetes were compared. Multivariate logistic regression analysis was used to analyse the independent associated factors of VC for different areas. Results A total of 206 patients aged (51±14) years were included, including 129 (62.6%) males. There were 44 patients with CKD stage 3 (21.4%), 51 patients with CKD stage 4 (24.8%), and 111 patients with CKD stage 5 (53.9%). CKD was caused by chronic glomerulonephritis [104 cases (50.5%)], diabetic kidney damage [35 cases (17.0%)], hypertensive kidney damage [29 cases (14.1%)] and others [38 cases (18.4%)]. Among 206 patients, 131 (63.6%) exhibited cardiovascular calcification, and the prevalence of CAC, TAC, AAC, carotid artery calcification, and AVC was 37.9%, 43.7%, 37.9%, 35.9% and 9.7%, respectively. The overall prevalence of VC in young, middle-aged and elderly patients was 24.6%, 73.6% and 97.4%, respectively. With the increase of age, the prevalence of VC in each site gradually increased, and the increasing trend was statistically significant (all P<0.001). The overall prevalence of VC in CKD patients with diabetes was 92.5% (62/67), and the prevalence of VC at each site in the patients with diabetes was significantly higher than that in the patients without diabetes (all P<0.001). Multivariate logistic regression analysis revealed that age (every 10 years increase, OR=2.51, 95%CI 1.77-3.56, P<0.001), hypertension (OR=5.88, 95%CI 1.57-22.10, P=0.009), and diabetes (OR=4.66, 95%CI 2.10-10.35, P<0.001) were independently correlated with CAC; Age (every 10 years increase, OR=6.43, 95%CI 3.64-11.36, P<0.001) and hypertension (OR=6.09, 95%CI 1.33-27.84, P=0.020) were independently correlated with TAC; Female (OR=0.23, 95%CI 0.07-0.72,P=0.011), age (every 10 years increase, OR=3.90, 95%CI 2.42-6.29, P<0.001), diabetes (OR=5.37, 95%CI 2.19-13.19, P<0.001) and serum magnesium (OR=0.01,95%CI 0-0.35, P=0.014) were independently correlated with AAC. Moreover, age and diabetes were independently correlated with carotid artery calcification, AVC and overall VC Conclusions The prevalence of VC in non-dialysis CKD patients of stage 3-5 is 63.59%, of which CAC reaches 37.9%, TAC is the most common one (43.7%), while AVC is the least one (9.7%). Age and diabetes are the independent associated factors for VC of all sites except TAC, while hypertension is an independent associated factor for both CAC and TAC.

Autogenous arteriovenous fistula (AVF) is the preferred vascular access of hemodialysis in maintenance hemodialysis patients. However, due to the influence of various factors, new AVF may show immaturity, which will affect the use and dialysis effect of vascular access. AVF maturation is an important clinical concern. It is important to clarify the physiological and biochemical factors, and biological mechanisms of AVF immaturation. Based on the literature, this review focuses on the biological mechanisms of AVF maturation and the physiological and biochemical factors affecting AVF maturation, including vascular conditions, gender and age, and underlying diseases, to provide reference for improving the clinical maturation rate of AVF and the treatment effect in hemodialysis patients.

The peritoneal equilibration test is primarily used to assess peritoneal function, guide the prescription of peritoneal dialysis, and determine the prognosis of peritoneal dialysis patients. This article overviews the peritoneal equilibration testing methods, peritoneal transport function classification, peritoneal membrane dysfunction, and their clinical significance. It also examines the factors that may influence the results of the peritoneal equilibration test and the precautions to be taken when applying it in clinical practice.

The identification of pathogenic antigens in membranous nephropathy (MN) is a hot topic in the research field of kidney diseases. In recent years, the widespread application of mass spectrometry has brought a breakthrough in the identification of MN-pathogenic antigens. As the antigen spectrum continues to be refined, the diagnosis of MN has evolved from morphological level to molecular level. This article reviewed the research progress of currently identified antigens of MN, such as phospholipase A2 receptor (a major pathogenic antigen of primary MN), thrombospondin type 1 domain-containing 7A (a potential tumor-associated antigen), neural epidermal growth factor-like protein 1 (an antigen associated with various secondary factors), semaphorin 3B (an antigen specific to pediatric MN) and so on, and the pathogenic mechanisms and clinical significance of these antigens.

Renal biopsy has been an essential part of the diagnosis and management of kidney disease. In recent years, the rapid development of artificial intelligence (AI) technology based on convolutional neural networks has significantly facilitated its utilization in nephrology. This article focuses on the research of AI in the recognition of tissue structure and pathological diagnosis of kidney biopsy. It elaborates on the identification and segmentation of kidney tissue structure and pathological features, as well as its auxiliary role in disease diagnosis across three dimensions: light microscopy, immunofluorescence, and electron microscopy. The aim is to provide a reference for the application of AI in renal pathology research and precision medicine.

Objective To evaluate the clinical application value of iohexol plasma clearance assay in assessing glomerular filtration rate (GFR) in patients with chronic kidney disease (CKD), and identify alternative methods of ^99mTc-diethylene triamine pentaacetic acid renal kinetic imaging (Gates) method for measuring GFR (^99mTc?mGFR). Methods It was a cross-sectional study. The CKD patients hospitalized in the Department of Nephrology at the Second Affiliated Hospital of Nanjing Medical University between October 2022 and September 2023 were enrolled. Iohexol plasma clearance was determined by collecting blood samples at 2 and 4 hours after intravenous administration of 5 ml iohexol, and high performance liquid chromatography was used to measure the plasma concentration of iohexol. Br?chner-Mortensen and Jacobsson formulas were used to calculate the double and single plasma iohexol clearance, respectively. CKD-epidemiology collaboration equation based on serum creatinine concentration was used to calculate the estimated GFR (eGFR). Pearson's coefficient was performed to analyze the correlation of iohexol dual plasma clearance assay-measured GFR (iohexol?DS?mGFR), iohexol single plasma 4 hours clearance assay-measured GFR (iohexol?SS_4h?mGFR), iohexol single plasma 2 hours clearance assay-measured GFR (iohexol?SS_2h?mGFR) and eGFR with ^99mTc?mGFR as the gold standard. Bland?Altman analysis, 95% limits of agreement, and intra-correlation coefficient were used to compare the diagnostic concordance of Iohexol?DS?mGFR, Iohexol?SS_4h?mGFR, Iohexol?SS_2h?mGFR and eGFR with ^99mTc?mGFR. Results The study enrolled 64 CKD patients, aged (58.91±13.08) years old, comprising of 38 males and 26 females. The distribution of patients across CKD stages based on ⁹⁹Tc?mGFR was as follows: 12 patients (18.8%) in stage 1, 14 patients (21.9%) in stage 2, 26 patients (40.6%) in stage 3, 10 patients (15.6%) in stage 4, and 2 patients (3.1%) in stage 5. The Pearson correlation analysis revealed that the correlation coefficients of iohexol?DS?mGFR, iohexol-SS_4h?mGFR, iohexol?SS_2h?mGFR and eGFR with ^99mTc?mGFR were 0.925, 0.867, 0.820 and 0.894 (all P<0.001), respectively. The median deviation of absolute value of iohexol?DS?mGFR, iohexol-SS_4h?mGFR, iohexol?SS_2h?mGFR and eGFR in the total study population were 6.66, 9.63, 11.47 and 9.59 ml·min^-1·(1.73 m²)^-1, respectively. The proportions of iohexol?DS?mGFR, iohexol-SS_4h?mGFR, iohexol?SS_2h?mGFR and eGFR located in the gold-standard GFR ± 10% interval (P10) were 35.9%, 29.7%, 26.6% and 29.7%, respectively, and the proportions located in the gold-standard GFR ± 30% interval ( P30 ) were 87.5%, 68.8%, 60.9% and 73.4%, respectively. Conclusions Iohexol dual plasma clearance is well correlated and consistent with ^99mTc?mGFR. It is a safe and easy alternative to isotope ^99mTc?mGFR for clinical use in determining GFR.

Ferroptosis is a type of programmed cell death characterised by iron-dependent accumulation of lipid reactive oxygen species, which is closely related to intracellular metabolism of amino acids, lipids, and iron, and regulation of ferroptosis can intervene and treat certain diseases. Diabetic kidney disease (DKD) is a chronic microvascular complication of diabetes.Although the exact pathogenesis of DKD is not clear, the results of the existing studies have proved that ferroptosis participates in the development of diabetic kidney injury, and plays an important role in kidney tubular injury in particular, and the inhibition of ferroptosis may be one of the directions for the treatment of DKD. In recent years, researchers have conducted a lot of studies on ferroptosis through animal models of DKD, but the specific pathogenesis and therapeutic effects regarding ferroptosis have not been fully revealed. This article introduces ferroptosis and its connection with apoptosis, autophagy and other forms of cell death, as well as the main mechanisms regulating the development of ferroptosis through systematic Xc-GSH-GPX4 axis, NADPH-FSP1-CoQ10 axis, GCH1-BH4- phospholipid axis and various regulatory factors, and provides an overview of its role in kidney tubular injury of DKD. Through the review of these contents, the possibility of ferroptosis as a therapeutic target for DKD is discussed, in order to provide reference for the basic research and clinical treatment of DKD.

Objective To investigate the efficacy of rituximab (RTX) monotherapy in the treatment of adult minimal change disease (MCD). Methods This study was a case series analysis. The clinical data of 10 MCD patients who received RTX monotherapy at the Department of Nephrology, Zhongda Hospital, Southeast University, from January 2021 to October 2023 were retrospectively conducted. Results The onset age of the 10 patients was (48.4±21.2) years old, including 7 males and 3 females. Four patients (4/10) were aged 60 or above. One patient (1/10), 6 patients (6/10) and 3 patients (3/10) were treated with rituximab once, 2 times and 3 times, respectively. The follow-up duration was (327.1±141.6) d. All 10 patients achieved complete remission within 3 months, with 4 cases (4/10) achieving complete remission within 1 month. The mean time to remission was 35.7 d. During the follow-up period, none of the 10 patients experienced relapse. No significant adverse reactions were observed during treatment and follow-up. Conclusion Rituximab monotherapy demonstrates effective treatment outcomes in patients with MCD, but multi-center, large sample clinical validation is required. Long-term follow-up is necessary to assess its sustained efficacy and safety.

The 2025 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guideline for IgA nephropathy represented a comprehensive update to the 2021 edition, emphasizing early diagnosis, risk stratification, and precision therapy. The guideline recommended that patients with proteinuria ≥0.5 g/d and no contraindications should undergo timely renal biopsy to confirm pathological type and assess risk. Proteinuria ≥0.5 g/d was defined as the threshold for progressive renal risk, warranting treatment initiation. For the first time, the new guideline advocated for simultaneous initiation of interventions targeting the two key pathological processes of IgA nephropathy: (1) etiological intervention to suppress the formation of galactose-deficient IgA1 (Gd-IgA1) and its immune complexes, and (2) management of the secondary responses following nephron loss. Budesonide enteric capsules are currently the only agent proven to significantly reduce Gd-IgA1 and related immune complexes by targeting gut mucosal immunity and decreasing pathogenic IgA production, thereby interrupting the "four-hit" pathogenesis at its source. Clinical studies have demonstrated that budesonide markedly reduces proteinuria, slows estimated glomerular filtration rate decline, and can be safely re-administered, providing evidence for its role in long-term maintenance therapy. Sodium-glucose cotransporter-2 inhibitors and sparsentan are recommended to mitigate compensatory injury following nephron loss. A comprehensive management approach combining etiological and symptomatic therapies, along with lifestyle modification and blood pressure control, is essential to improve the long-term prognosis of patients with IgA nephropathy.

Objective To explore the value of combined detection of urinary kidney injury markers in the diagnosis of early-stage diabetic kidney disease (DKD), and to provide evidence for early-stage DKD screening. Methods It was a retrospective study. The clinical data of patients with type 2 diabetes mellitus (T2DM) from the Second Affiliated Hospital of Hainan Medicine University from January 2022 to August 2023 were collected. According to urinary microalbumin/creatinine ratio (UACR), the patients were divided into three groups: isolated diabetes group (UACR < 30 mg/g), early-stage DKD group (30 mg/g ≤ UACR < 300 mg/g) and clinical DKD group (UACR ≥ 300 mg/g), and the differences of clinical data among three groups were compared. Glomerular injury markers urinary microalbumin, transferrin, immunoglobulin (Ig) and α2 macroglobulin, and renal tubule injury markers α1 microglobulin (α1?MG), β2 microglobulin (β2?MG), retinol-binding protein (RBP), N-acetyl-β-D-glucosidase (NAG), neutrophil gelatinase-associated lipid carrier protein (NGAL) were measured. Spearman correlation method was used to analyze the correlation between urinary kidney injury markers and clinical indicators. Multivariate logistic regression analysis method was used to analyze the risk factors of DKD occurrence (UACR > 300 mg/g). Receiver-operating characteristic curve was used to analyze the value of individual and combined detection of urinary renal injury markers in the diagnosis of early-stage DKD (30 mg/g ≤ UACR < 300 mg/g). Results A total of 116 T2DM patients were enrolled in this study, aged (61.99±12.56) years old (30 to 91 years old), with 79 males (68.1%). There were 44 (37.9%) isolated diabetes patients, 27 (23.3%) early-stage DKD patients, and 45 (38.8%) clinical DKD patients. Serum creatinine (Scr, H=34.183, P<0.001) and blood urea nitrogen (BUN, H=34.082, P<0.001) in clinical DKD group were higher than those in isolated diabetes group and early-stage DKD group. Spearman correlation analysis showed that glomerular injury markers urinary microalbumin, transferrin, Ig and α2 macroglobulin were positively correlated with Scr, BUN and UACR, and negatively correlated with estimated glomerular filtration rate and serum albumin (all P<0.05). Renal tubule injury markers urinary α1?MG, β2?MG, NAG, RBP, and NGAL were positively correlated with Scr, BUN and UACR, and negatively correlated with estimated glomerular filtration rate and serum albumin (all P<0.05). Multivariate logistic regression analysis indicated that systolic blood pressure ≥ 140 mmHg (OR=1.033, 95%CI 1.008-1.060, P=0.010), high urinary microalbumin (OR=1.018, 95%CI 1.007-1.030, P=0.001), high urinary RBP (OR=1.309, 95%CI 1.086-1.577, P=0.005), high urinary NGAL (OR=1.004, 95%CI 1.000-1.008, P=0.037), low serum albumin (OR=0.833, 95%CI 0.749-0.926, P=0.001) and low urinary Ig (OR=0.994, 95%CI 0.990-0.999, P=0.018) were independent influencing factors of DKD occurrence. Receiver-operating characteristic curve revealed that the area under the curve (AUC) was the largest for diagnosing early-stage DKD when urinary microalbumin was detected alone (AUC=0.976, 95%CI 0.955-0.997, P<0.001), with sensitivity and specificity of 95.6% and 90.1%, respectively. The combined detection of urinary microalbumin + Ig + transferrin + α2 macroglobulin + α1-MG + β2-MG + NAG + RBP + NGAL had an AUC of 0.986 (95%CI 0.971-1.000, P<0.001), with sensitivity and specificity of 93.3% and 98.5%, respectively, which was better than each single index. Further optimized detection combination was urinary microalbumin combined with β2-MG and NGAL, which had the best diagnostic efficacy (AUC=0.978, 95%CI 0.958-0.999, P<0.001), with sensitivity and specificity of 95.6% and 93.0%, respectively. Conclusions Compared with the single detection of each index, the combined detection of urinary glomerular injury and renal tubule injury markers has higher value in diagnosing early-stage DKD. The combined detection of urinary microalbumin combined with β2-MG and NGAL has the highest value in diagnosing early-stage DKD.

The study was a prospective observational study. A total of 24 patients who underwent maintenance hemodialysis (MHD) at Haidian Hospital in Beijing from May 2024 to June 2024 were included as the study subjects. The safety and efficacy of a new single-needle dialysis in MHD patients were evaluated. The reasons for using single-needle dialysis included waiting for the maturity of internal fistula(7 cases, 29.17%), autogenous arteriovenous fistula thrombosis occurred (6 cases, 25.00%), puncture difficulty occurred (7 cases, 29.17%), and pain sensitivity or elderly (4 cases, 16.67%). The results showed that the average blood flow was (155.65±5.90) ml/min, total blood volume was (35.92±2.65) L during single-needle dialysis. One patient had slight puncture leakage, and the puncture success rate was 95.83%. Relevant indicators of dialysis adequacy showed that the average urea clearance (Kt/V) was 0.90±0.42, urea reduction ratio was 58.31%±7.93%, and online real-time Kt/V monitoring average value was 0.98±0.55. The results suggest that the application of the new improved single-needle dialysis mode in MHD patients is safe and effective.

Acute kidney injury (AKI) is a common clinical acute and critical condition. There is a lack of clinical interventions or therapeutic drugs that can significantly improve AKI outcomes. Lipids, including fatty acids, triglycerides, sphingolipids, phospholipids and cholesterol play crucial roles in energy metabolism, cell membrane composition, cell signaling, and cell homeostasis and survival. Recent lipidomics studies have revealed significant alterations in the content and composition of renal lipids during AKI, highlighting their important roles in the onset, progression, and outcomes of the disease. A common feature of AKI across multiple etiologies is altered lipid metabolism, characterized by insufficient energy generation due to mitochondrial damage and deposition of excess lipids in the kidney. The article summarizes the characteristics of renal lipid metabolism in physiological state, alterations of renal lipid metabolism in AKI and molecular mechanisms related to lipid metabolism disorders that aggravate AKI through mitochondrial damage, oxidative stress, autophagy dysfunction, activation of inflammatory and immune responses and regulated cell death, to provide new ideas and therapeutic targets for the clinical treatment of AKI.

Primary membranous nephropathy (PMN) is one of the common causes of nephrotic syndrome in adults and is characterized by the deposition of immune complexes, resulting in thickening of basement membrane. With the identification of more autoantibodies, the understanding of the pathogenesis of PMN has expanded. Currently, supportive therapy and immunosuppressive therapy are the primary treatments for PMN, which mainly consists of cyclophosphamide, calcineurin inhibitors, rituximab, and so on. With the advent and application of an increasing number of biological agents, a subset of refractory or drug-resistant PMN patients can be effectively treated and achieve remission. This article reviews recent advances in the management of PMN. It covers pretreatment assessment, immunosuppressive therapy, complement- targeted therapy, and anticoagulant therapy and deliberates on the management of rituximab- resistant patients. The review aims to provide clinicians with an up-to-date basis for individualized clinical decision-making.

Objective To investigate the correlation between complement C3 and urine protein level and proteinuria remission status in patients with primary membranous nephropathy (PMN), and better guide individualized clinical treatment. Methods It was a single-center retrospective study. The clinical data of PMN patients who underwent renal biopsy in the First Affiliated Hospital of Nanjing Medical University from January 2017 to June 2022 were collected. Patients with 24 h urinary protein ≥ 3.5 g were followed up after receiving standard treatment, and the last outpatient or inpatient review was used as the end point of follow-up. 24 h urine protein was collected to evaluate the remission status of proteinuria. Kaplan-Meier method was used to analyze the correlation between serum and renal complements and proteinuria remission. Cox regression analysis method was used to analyze the correlation between serum C3 level and renal tissue C3 deposition and proteinuria remission. Results This study included 507 PMN patients with 312 (61.54%) males, aged 54 (43, 64) years old. Compared with 24 h urinary protein < 3.5 g group, proportion of males (χ²=22.479, P<0.001), age (Z=-2.521, P=0.012), systolic blood pressure (Z=-4.148, P<0.001), diastolic blood pressure (Z=-4.084, P<0.001), serum anti-phospholipase A2 receptor (PLA2R) antibody titer (Z=-7.019, P<0.001), total cholesterol (Z=-8.796, P<0.001), triglyceride (Z=-6.158, P<0.001), low density lipoprotein cholesterol (Z=-8.716, P<0.001), serum creatinine (Z=-7.368, P<0.001), serum C3 (Z=-3.663, P<0.001), serum C4 (Z=-6.560, P<0.001), proportion of glucocorticoid use (χ²=116.417, P<0.001) and proportion of immunosuppressant use (χ²=53.839, P<0.001) were all higher, while serum albumin (Z=12.518, P<0.001), estimated glomerular filtration rate (Z=6.345, P<0.001) and serum IgG (Z=7.321, P<0.001) were all lower in 24 h urinary protein ≥3.5 g group. There were 268 patients included in the follow-up cohort with baseline 24 h urinary protein of 7.15 (5.14, 10.24) g, serum anti-PLA2R antibody titer of 61.44 (14.35, 193.24) RU/ml, serum C3 of 1.005 (0.864, 1.150) g/L, and serum C4 of 0.260 (0.214, 0.317) g/L. Kaplan-Meier survival curve showed that the incomplete remission rate of proteinuria in serum C3 > 1.005 g/L group was lower than that in serum C3 ≤ 1.005 g/L group (log-rank χ²=4.757, P=0.029). There was no significant difference in the incomplete remission rate of proteinuria between serum C4 ≤ 0.260 g/L group and serum C4 > 0.260 g/L group (log-rank χ²=3.543, P=0.060). Renal C1q (log-rank χ²=0.167, P=0.683) and C4 (log-rank χ²=1.927, P=0.165) deposition had no significant effects on proteinuria remission in PMN patients. The incomplete remission rate of proteinuria in patients with renal C3 deposition was higher than that in patients without renal C3 deposition (log-rank χ²=7.018, P=0.008). Univariate Cox regression analysis showed that serum C3 level and C3 deposition in renal tissues were influencing factors of incomplete remission of proteinuria (both P<0.05), while adjusting for gender, age, mean arterial pressure, serum anti-PLA2R antibody, serum albumin and 24 h urinary protein, serum C3 ≤ 1.005 g/L (HR=1.374, 95% CI 1.021-1.849, P=0.036), C3 deposition in renal tissues (HR=1.949, 95% CI 1.098-3.460, P=0.023), and serum C3 ≤ 1.005 g/L combined with C3 deposition in renal tissues (HR=1.472, 95% CI 1.093-1.983, P=0.011) were independent influencing factors of incomplete remission of proteinuria. Conclusions The serum C3 level and C3 deposition in renal tissues are closely related to urinary protein level and proteinuria remission status in PMN patients. The patients with higher urinary protein have higher serum C3. For patients with massive proteinuria, serum C3 ≤ 1.005 g/L, C3 deposition in renal tissues, serum C3 ≤ 1.005 g/L combined with C3 deposition in renal tissues are independent risk factors of incomplete remission of proteinuria.

Objective To analyze the mutation pathogenicity of the novel compound heterozygous mutation in the PKHD1 gene causing autosomal recessive polycystic kidney disease (ARPKD) family, expand the PKHD1 gene mutation database, and explore the genotype-phenotype correlations of PKHD1 gene mutation causing ARPKD. Methods Clinical data and peripheral blood of a patient with ARPKD caused by the novel compound heterozygous mutation in the PKHD1 gene and their family members were collected. High-throughput sequencing was used to detect pathogenic mutations in the proband, and PCR amplification and Sanger sequencing were used to verify the pathogenic mutations in the family. AlphaFold software was applied to predict changes in protein structure in the present or absent mutations, and the pathogenicity of mutations was analyzed. Results The patient was a young male who underwent splenectomy due to liver cirrhosis and hypersplenism at age 7. He developed end-stage renal disease at age 22, requiring maintenance peritoneal dialysis, and died of severe pneumonia and septic shock at age 24. Genetic testing revealed three compound heterozygous mutations in the PKHD1 gene inherited from his parents: a missense mutation (c.5935G>A) inherited from the father and a missense mutation (c.1187G>A) and a novel splice mutation (c.6332+1_6332+2insG) from the mother. The single missense mutation allele likely contributed to the prolonged survival. c. 6332+1_ 6332+2insG is a novel splicing mutation that has not been reported in the past, which can lead to early termination of protein translation. This discovery expands the PKHD1 gene mutation database. c. 1187G>A (p.S396N) and c.5935G>A (p.G1979R) occur in the PA14 and G8 domains of the protein, respectively, and are associated with early and severe liver phenotypes in patients. Conclusions The mutation types and amino acid localization of the PKHD1 gene are associated with the heterogeneity of clinical phenotypes in ARPKD patients. Analyzing structural changes in proteins before and after mutations can help understand the pathogenicity at a molecular level, establishing genotype-phenotype correlations and providing valuable insights for assessing prognosis and identifying high-risk ARPKD patients early.

Objective To explore the clinical feature and genetic variation of NPHS1 variant-associated nephropathy (NPHS1-VAN) in Chinese patients. Methods This study was a case-series analysis. Patients with NPHS1-VAN, who were treated and/or followed in the Department of Pediatrics, Nanfang Hospital, Southern Medical University between 2018 and 2023 were recruited into this study. Genotype, phenotype and their relationship were analyzed. Results Nine NPHS1-VAN patients from 8 non-consanguineous Chinese families were collected, including 5 males and 4 females. There were 7 cases with an onset age within 3 months and 2 cases with an onset age of 6 months and 13 years, respectively. Seven patients harbored compound heterozygous variants, two had homozygous variants, including 8 missense variations,3 frameshift variants, and 1 splicing site variant. Four patients in 3 families harbored missense variant c.928G>A, two of them experienced spontaneous remission of proteinuria at the age of 1 year and 2 years, respectively, another one had persistent proteinuria and entered end stage renal disease (ESRD) at 11 years old. The other one had an onset age of 6 months with no response to steroids initially. She got complete remission by tacrolimus administered, but relapse frequently and partially responded to steroids later. Two patients of this group died, one of them died of respiratory failure 3 days after birth. Excessive amniotic fluid and fetal edema were acknowledged at 28 weeks of gestational age. He harbored compound heterozygous variants of NPHS1, c.1135C>G (R379G) and c.1339G>A (E447K). His mother previously experienced fetal death at 28 weeks gestational age for her first pregnant and stillborn at 36 weeks of gestational age for her second pregnant, respectively. One patient in this study who harbored homozygous variant of c.1339G>A (E447K) presented with a mild phenotype, onset age was 13 years old and didn't progress to ESRD yet at 21 years. Thus, variant E447K was hypothesized to be weakly pathogenic, while R379G may be strongly pathogenic with a risk of death. Five novel variants were identified in this group of patients, 3 missense variants (c.1135C>G, c.1157A>T, c.3197T>A) and 2 frameshift variants (c.709_710delCT, c.3193delG). Renal biopsy was performed in 4 cases, of whom two were focal segmental glomerular sclerosis and another two were minimal change disease. Conclusions NPHS1-VAN possesses remarkable clinical and genetic heterogeneity. Five novel variants were identified. Missense variant is the most common variant type and c.928G>A is the most common one in this group of patients, in consistent with previous report in China. Children harbor c.928G>A may have a mild phenotype with possible spontaneous remission and may be response to steroids and calcineurin inhibitor. Variant c.1135C>G (R379G) may have a strong pathogenicity, and patient who harbors this variant may have a severe phenotype.

Piezo is a newly discovered mechanosensitive ion channel (MSC) in mammals, characterized by a unique homotrimeric three-leaf propeller-shaped structure that converts mechanical signals into biological and electrical signals, thus participating in the regulation of various physiological and pathological processes. In recent years, an increasing number of studies have demonstrated the crucial role of Piezo channel in renal physiology and pathophysiology. This article aims to provide new perspectives and targets for the prevention and treatment of renal diseases by reviewing the recent research advances in the structure, kinetics and pharmacology of Piezo channel, especially their expression and physiopathologic roles in the kidney.

Anti-glomerular basement membrane (GBM) nephritis and anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis are both autoimmune diseases that can involve multiple systems throughout the body, and both can cause crescent glomerulonephritis. At present, cases of such "double-positive" antibodies have been reported at home and abroad, but the cases of acute progressive glomerulonephritis (RPGN) with IgA nephropathy that are both positive for GBM and ANCA are relatively rare. This case was a middle-aged female patient, whose clinical manifestations were consistent with rapidly progressive glomerulonephritis, positive for anti-GBM antibodies and anti-myeloperoxidase. After renal puncture biopsy, she was diagnosed as anti-GBM nephritis complicated with ANCA associated vasculitis and IgA nephropathy.After treatment with plasma exchange, hemodialysis, glucocorticoids, cyclophosphamide, etc., the condition improved and the patient was discharged. But the patient did not follow the doctor's instructions to take medication and continue hemodialysis treatment after discharge, and the condition worsened, ultimately leading to death.

Anemia is a significant influencing factor of peritoneal dialysis (PD) patients to suffer from cardiovascular diseases, worse therapeutic effect and death. However, compared with hemodialysis patients, there are fewer clinical studies and epidemiological investigations concerning anemia in PD patients. The influencing factors of anemia in PD patients are numerous and complex, but there is a lack of strong evidence on the specific degree and direction of each factor. Further understanding of the influencing factors of anemia in PD patients is more conducive to the targeted correction of anemia status. This article reviewed the epidemiological status of anemia in PD patients, and the influencing factors of anemia from the aspects of physiological factors, therapeutic factors, nutritional factors and other factors, to provide references for the treatment and related studies of anemia in PD patients.

Objective To explore the incidence and risk factors of severe bleeding after percutaneous renal biopsy (PRB) in patients with advanced chronic kidney disease (CKD). Methods The study was a retrospective cohort analysis. The data were collected from patients with advanced CKD who were hospitalized in the Department of Nephrology, Nanfang Hospital, Southern Medical University and underwent PRB between January 2010 and December 2020. Severe bleeding after PRB was defined by any of the following criteria: a postoperative hemoglobin decrease of ≥20 g/L within 48 hours, a maximum diameter of perirenal hematoma ≥5 cm postoperatively, or the need for posterior pituitary hormone, blood transfusion, or renal vascular intervention post-surgery. The occurrence of severe bleeding following PRB served as the primary endpoint for this study. Logistic regression model was used to analyze the risk factors associated with severe bleeding in patients with advanced CKD undergoing PRB. Results A total of 895 patients aged (46.1±14.1) years were encompassed in the study. Among them, 60.1%(538/895) were male, 15.9%(142/895) were afflicted with diabetes, and 57.9%(518/895) suffered from hypertension. The estimated glomerular filtration rate (eGFR) was (40.1±13.2) ml?min^-1?(1.73 m²)^-1, and the 24-hour urine protein excretion was 2.5(1.1, 4.9) g. After PRB, 22.9%(205/895) of the patients encountered severe bleeding, including 30 patients (14.6%) who received postoperative somatostatin, 10 patients (4.9%) who underwent postoperative blood transfusion, 1 patient (0.5%) who underwent postoperative renal vascular intervention for hemostasis, and no fatalities occurred. Compared to the non-severe bleeding group, patients in the severe bleeding group after PRB exhibited a higher proportion of hypertension [64.4%(132/205) vs. 55.9%(386/690), χ2=4.627, P=0.031]. Additionally, preoperative serum creatinine levels and mean arterial pressure were significantly elevated [(193.9±106.6) μmol/L vs. (180.8±102.6) μmol/L, t=-2.559, P=0.011; (95.8±10.9) mmHg vs. (93.9±11.0) mmHg, t=-2.134, P=0.033]. Furthermore, platelet counts were lower in the severe bleeding group [(227.5±70.3) ×10⁹/L vs. (247.5±74.8) ×10⁹/L, t=-3.788, P<0.001]. No statistically significant differences were observed between the two groups regarding age, gender distribution, prevalence of diabetes mellitus, as well as preoperative serum albumin level, hemoglobin concentration, other coagulation function indicators and pathological histological type (all P>0.05). Multivariate logistic regression analysis indicated that body mass index (OR=0.936, 95% CI 0.891–0.984, P=0.010), eGFR (OR=0.985, 95% CI 0.971–0.999, P=0.034), serum albumin level (OR=1.041, 95% CI 1.011–1.072, P=0.007), 24 hours urinary protein excretion (OR=1.092, 95% CI 1.030–1.158, P=0.003), and platelet count (OR=0.996, 95% CI 0.994–0.999, P=0.002) were independently associated with the severe bleeding following PRB in patients with advanced CKD. In the PRB cohort analyzed, the six most prevalent renal histological types were as follows: IgA nephropathy (46.3%, 414/895), membranous nephropathy (11.1%, 99/895), focal segmental glomerulosclerosis (8.5%, 76/895), diabetic nephropathy (7.6%, 68/895), sclerotic kidney disease (6.9%, 62/895), and vascular sclerosis of the kidneys (4.9%, 44/895). Conclusions Patients with advanced CKD exhibit a heightened risk of severe bleeding following PRB, estimated at approximately 22.9%. Independent risk factors for the occurrence of severe bleeding complications in these patients include low body mass index, reduced eGFR, decreased platelet count, elevated serum albumin, and increased urinary protein level.