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  • Expert group of clinical practice guideline for delaying the progression of chronic kidney disease (2025)
    Abstract (16168) PDF (4404) RichHTML (18551)

    Chronic kidney disease (CKD) is a major disease that severely harms the public health. As the disease progresses, patients may experience systemic complications involving multiple organs and finally develop end-stage kidney disease (ESKD), which severely threatens the lives of patients. And they have to rely on renal replacement therapies such as hemodialysis, peritoneal dialysis, or kidney transplantation to survive, imposing a heavy burden on families and society. Delaying the progression of CKD to reduce the incidence of ESKD and the risk of various severe complications is the primary goal of CKD treatment. Based on the latest evidence-based data and integrating the clinical practice experience of experts, this guideline has focused on formulating the comprehensive strategies including risk assessment for disease progression, strategies for delaying the disease progression, and management of complications in CKD. It is the aim to provide clinical practitioners with guidance on CKD management practices based on the latest evidence and suitable national conditions.

  • Chinese Society of Nephrology
    Abstract (12372) PDF (3798) RichHTML (12976)

    Chronic kidney disease (CKD) is a common and frequently-occurring disease in China. When CKD develops to end stage, it requires renal replacement therapy. Hemodialysis and peritoneal dialysis are currently the main renal replacement therapies. As the "lifeline" of dialysis patients, the quality of dialysis access directly affects the hospitalization and survival rate of patients. Therefore, it is of great significance to strengthen the study of dialysis access. At present, a number of clinical guidelines and expert consensus on dialysis access have been issued at home and abroad. However, with the development of new medical technology, the knowledge about dialysis access is constantly updated, especially the clinical application of new devices, equipment and new evidence of evidence-based medicine, which expands the diagnosis and treatment strategy of the establishment and maintenance of dialysis access. Therefore, it is necessary to compile a guideline for the diagnosis and treatment of dialysis access suitable for the Chinese population. In view of this, the Chinese Society of Nephrology organized an expert group to compile the "China Guidelines for Dialysis Access (2024)". This guideline refers to relevant domestic and foreign guidelines and expert consensus, integrates the clinical experience of Chinese dialysis access experts, systematically introduces dialysis access establishment, maintenance, monitoring, follow-up, and training of access doctors. In the process of preparation, the expert group, in accordance with the principle of rigor and conciseness, referred to the format of international guidelines, put forward the main points of diagnosis and treatment, and illustrated them case by case. This guideline reflects latest trends and perspectives in diagnosis and treatment. It is of great value to further strengthen the understanding of dialysis access, standardize the diagnosis and treatment system of dialysis access, formulate reasonable treatment principles, prolong the use time of dialysis access, and improve the quality of patients' lives. This guideline can be used as a reference for medical workers in clinical practice.

  • Expert Group for the Development of Guidelines on Comprehensive Management of Chronic KidneyDisease in the Elderly, China Association of Gerontology and Geriatrics
    Abstract (11855) PDF (2028) RichHTML (13086)

    Chronic kidney disease (CKD) is one of the most important non-infectious chronic diseases in China, posting a serious threat to the health of older adults. Currently, there is still a lack of guidelines or consensus on the comprehensive management of CKD in older adults, particularly for primary care providers. Therefore, Chinese Society of Gerontology and Geriatrics collaborated with nephrology experts in China to develop a guideline specifically tailored for older CKD patients. This guideline clearly defines CKD in older adults, recommends using CKD Epidemiology Collaboration equation (CKD-EPI) combined serum creatinine-cystatin C (cr-cyst) formula to assess renal function in order to facilitate disease staging and stratified management in this population. It emphasizes the importance of a multidisciplinary team in the comprehensive assessment of the health status in older CKD patients. Regarding treatment strategies, the guideline advocates for first identifying the cause of CKD and then developing personalized precise treatment plans based on clinical and pathological diagnoses, as well as introduces the methods for rational medication use. In terms of patient management, the guideline suggests that older CKD patients should adopt healthy lifestyle and dietary habits, engage in regular physical activities, and receive standardized disease management. It also encourages the integration of traditional Chinese medicine for treatment based on syndrome differentiation in older CKD patients. Additionally, it provides recommendations on referrals and renal replacement therapy. The purpose of this guideline is to offer a comprehensive management strategy for older CKD patients to healthcare providers in primary medical institutions, aiming to enhance their overall management capabilities, postpone disease progression, and ultimately improve the quality of life for older adults in China.

  • Clinical practice guidelines for management of chronic kidney disease during peridialysis in China (2025) Working Group of the Kidney Disease Dialysis Special Committee of the China Association of Non-Public Medical Institutions
    Abstract (8144) PDF (1601) RichHTML (8110)

    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.

  • Chinese consensus expert group on ultrasound interventional therapy for hemodialysis vascular access
    Abstract (5037) PDF (1607) RichHTML (5337)

    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.

  • Huang Naya, Wen Qiong, Fan Yanping, Chen Wei
    Abstract (4979) PDF (752) RichHTML (4315)

    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.

  • Expert Committee of China Special Fund for Kidney Disease Prevention and Treatment, China International Medical Exchange Foundation
    Abstract (4251) PDF (1599) RichHTML (4463)

    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.

  • Committee of multidisciplinary expert consensus on the clinical use of finerenone (2025 edition)
    Abstract (4135) PDF (685) RichHTML (4421)

    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.

  • Chinese Society of Nephrology
    Abstract (3596) PDF (895) RichHTML (3689)

    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.

  • Zhang Ying, Wang Xiaoxu, Zhang Xiaoliang, Wang Bin
    Abstract (3588) PDF (464) RichHTML (3561)

    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.

  • Clinical practice guidelines working group of the Kidney Disease Dialysis Special Committee of the China Association of Non Public Medical Institutions
    Abstract (3456) PDF (1007) RichHTML (3497)

    IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide, with high heterogeneity and the risk of progression to end-stage kidney disease. To improve clinicians' diagnosis and treatment of IgAN, Clinical practice guidelines working group organized national nephrologists to revise and compile this article through several rounds of discussion on 60 common questions in clinical practice of IgAN. The content covered epidemiological features, pathogenesis, clinical manifestations, diagnostic approaches, therapeutic strategies, and prognostic evaluation of IgAN, with particular emphasis on the clinical application of targeted-release formulation of budesonide—the first targeted etiological treatment, marking a new era in IgAN management. Additionally, comprehensive discussions were provided on management strategies for IgAN patients in various clinical scenarios, such as blood pressure management, treatment of special populations, and post-transplant recurrence. This article aims to offer practical and standardized clinical guidance to optimize the precise treatment of IgAN and improve patients' prognosis.

  • Expert consensus working group on the clinical use of double-filtration plasmapheresis in kidney diseases
    Abstract (3385) PDF (561) RichHTML (2655)

    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.

  • Clinical Practice Guidelines Working Group, Nephrology and Dialysis Association of Chinese Non-government Medical Institution Association
    Abstract (3375) PDF (817) RichHTML (3319)

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. In China, approximately 700,000 individuals are affected by ADPKD, with about half progressing to end-stage kidney disease (ESKD) between the ages of 50 and 60 years. ADPKD is the fourth leading cause of ESKD. In addition to causing renal lesions, the disease also involves multiple extrarenal organs, including liver cysts, intracranial aneurysms, valvular heart disease, etc., bringing heavy disease burden to individuals, families and society. Therefore, ADPKD has always been a research hotspot in the field of nephrology worldwide. In recent years, the basic and clinical research of ADPKD has made continuous progress, which has brought new theories, new technologies and new therapies to the clinical management of ADPKD. In order to improve the understanding and management of ADPKD among medical staff in China, the Clinical Practice Guidelines Working Group, Nephrology and Dialysis Association of Chinese Non-government Medical Institution Association has developed this Clinical practice guideline for ADPKD in China (2026). Based on evidence-based medicine evidence and clinical experience, the guideline systematically expounds the diagnosis and evaluation of ADPKD, measures to delay disease progression, treatment of renal and extrarenal complications, management of special ADPKD patients and other contents, comprehensively reflecting the forefront of clinical diagnosis and treatment of ADPKD and patient management strategies. This will serve as an important reference for standardizing the diagnosis, evaluation and treatment of ADPKD.

  • Nephrology Professional Committee of Chinese Research Hospital Association
    Abstract (3278) PDF (850) RichHTML (3644)

    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.

  • Fan Xin, Li Luyao, Fang Lin, Wang Sha, Yang Xiangdong
    Abstract (3029) PDF (504) RichHTML (3092)

    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.

  • Huang Naya, Wen Qiong, Tan Li, Fan Yanping, Chen Wei
    Abstract (2926) PDF (474) RichHTML (3049)

    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.

  • Blood Purification Branch of China Association for Promotion of Health Science and Technology
    Abstract (2799) PDF (586) RichHTML (2271)

    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.

  • Yao Shenghua, Wang Hui, Han Zongyang, Ma Kunling
    Abstract (2599) PDF (534) RichHTML (2744)

    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.

  • Wu Binxin, Su Xiaole, Wang Lihua
    Abstract (2378) PDF (426) RichHTML (2577)

    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.

  • Li Xinming, Mao Zhiguo, Mei Changlin, Xue Cheng
    Abstract (2315) PDF (264) RichHTML (2153)

    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.

  • Pan Mingming, Liu Bicheng
    Abstract (2217) PDF (564) RichHTML (2277)

    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.

  • Zhang Yan, Liao Hui, Tan Zhicheng, Li Rongshan
    Abstract (2170) PDF (85) RichHTML (2078)

    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.

  • Feng Sheng, Yang Xiao
    Abstract (1963) PDF (306) RichHTML (1951)

    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.

  • Jiang Houhui, Wei Zhongping, Chen Zhaowei, Ding Guohua
    Abstract (1906) PDF (165) RichHTML (2026)

    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.

  • 2025, 41(1): 77-80.
    Abstract (1892) PDF (65) RichHTML (1840)
  • Li Ziyi, Wang Jianqin
    Abstract (1839) PDF (154) RichHTML (1779)

    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.

  • Wu Han, Li Ying, Gu Mengru, Dai Chunsun
    Abstract (1807) PDF (153) RichHTML (1627)

    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.

  • Wang Fengmei, Wei Qing, Yang Yan, Zhang Xiaoliang, Wang Bin
    Abstract (1720) PDF (273) RichHTML (1742)

    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.

  • Feng Yuhua, Dou Jingyu, Zhang Zhen, Wen Lu, Li Qianqian, Su Yan, Cheng Genyang
    Abstract (1520) PDF (121) RichHTML (1719)

    A total of 269 non-diabetic chronic kidney disease (CKD) patients were enrolled in this study. Among them, 175 patients (65.1%) were assigned to the control group and received conventional therapy with maximally tolerated doses of renin-angiotensin-aldosterone system inhibitors, while 94 patients (34.9%) were assigned to the dapagliflozin group and received oral dapagliflozin 10 mg/day in addition to the conventional therapy. The results showed that the urine protein quantity in the dapagliflozin group was lower than those in the control group at 3, 6, 12, 18, and 24 months of follow-up (all P<0.05), and the blood albumin level was higher than those in the control group at 18 and 24 months of follow-up (all P<0.05). The Kaplan-Meier survival curve analysis results showed that the cumulative renal survival rate of the dapagliflozin group was significantly higher than that of the control group (Log-rank test, χ 2=5.078, P=0.024). Multivariable Cox regression analysis results revealed that using dapagliflozin was independently associated with a reduced risk of the composite endpoint in non-diabetic CKD patients (HR=0.400, 95%CI 0.163-0.983, P=0.046). There was no statistical difference in adverse reactions between the two groups (all P>0.05). It is indicated that dapagliflozin has a renal protective effect independent of hypoglycemic action and good safety.

  • Expert group of expert recommendations on renal biopsy evaluation of monoclonal gammopathy of renal significance
    2025, 41(8): 642-650.
    Abstract (1470) PDF (404) RichHTML (1558)

    Monoclonal gammopathy of renal significance (MGRS) refers to a spectrum of kidney disorders caused directly or indirectly by monoclonal immunoglobulin or its components. Early and precise diagnosis of MGRS, along with targeted therapies against pathogenic B-cell or plasma cell clones, is critical for improving renal prognosis. Renal biopsy remains the gold standard for diagnosis of MGRS, and which is not only essential for definitive diagnosis but also indispensable for precise classification of MGRS. To assist clinicians in the rational and standardized application of renal biopsy for early and accurate diagnosis of MGRS, the expert group of expert recommendations on renal biopsy evaluation of MGRS developed the expert recommendations on renal biopsy evaluation of monoclonal gammopathy of renal significance. This consensus elaborates on updated MGRS classifications, indications for renal biopsy, pathological evaluation, as well as the techniques for monoclonal immunoglobulin detection and clone identification. It further formulates six clinically actionable recommendations, aiming to provide an evidence-based guidance for clinical practice and improve the diagnosis and treatment of MGRS.

  • Li Weiwei, Chen Zhaowei, Ding Guohua
    Abstract (1460) PDF (73) RichHTML (1497)

    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.

  • Liu Qianling, Chen Wei
    Abstract (1432) PDF (211) RichHTML (1739)

    The research advancements of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in chronic kidney disease (CKD) were a central theme at the 2024 American Society of Nephrology (ASN) Kidney Week. Clinical studies reaffirmed the cardio-renal benefits of SGLT2i in CKD patients, including those with advanced-stage disease and elderly populations. Notably, these benefits persisted for up to 12 months after treatment discontinuation. Furthermore, CKD management has evolved from a traditional "three-pillar" approach to a "four-pillar" strategy, with combination therapies demonstrating significant synergistic potential for cardio-renal protection. Mechanistic studies revealed novel pathways through which SGLT2i exert renoprotective effects, including promoting renal regeneration, modulating epigenetic modifications, regulating immune cell activity, and restoring proximal tubule metabolic homeostasis. Further research is warranted to optimize therapeutic outcomes and maximize clinical benefits for patients.

  • Zhu Nan, Cai Leiming, Yuan Weijie, Chen Yunfei
    Abstract (1398) PDF (167) RichHTML (1250)

    Acute kidney injury during pregnancy (P?AKI) threatens the health of both the mother and fetus, which should be given sufficient attention. During pregnancy, the kidneys undergo a series of physiological changes to adapt to the demands from the mother and infant, leading to the kidneys more susceptible to damage than non pregnant individuals. This article analyzes the pathophysiological changes, causes, diagnostic criteria, related diseases, and therapeutic measures of P?AKI. It also points out that for severe preeclampsia, thrombocytopenia syndrome, and acute fatty liver disease, the pregnancy should be terminated upon diagnosis. While for thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, plasma exchange should be the main treatment. Further analysis of the dialysis indications for P?AKI is aimed at providing reference for accurate clinical diagnosis and effective treatment, thereby improving the survival rates of pregnant women and fetuses.

  • Xue Zhuorui, Zheng Ke
    Abstract (1381) PDF (144) RichHTML (1299)

    Anti-vascular endothelial growth factor (VEGF) drugs inhibit tumor angiogenesis by blocking the VEGF-VEGF receptor signaling pathway and serve as a key therapeutic option for rat sarcoma viral oncogene homolog wild-type metastatic colorectal cancer. However, these drugs may induce kidney damage through mechanisms such as excessive activation of the local complement system, reduced production of prostaglandin E2 and abnormal endothelial cell-podocyte crosstalk. The diagnosis of kidney damage associated with anti-VEGF drugs is based on pathological findings, with thrombotic microangiopathy being a characteristic feature. Given that cancer patients are frequently treated with multiple potentially nephrotoxic drugs, a renal biopsy is recommended when proteinuria or other signs of kidney damage are detected. After confirmation, anti-VEGF drugs should be promptly discontinued, and individualized strategies based on proteinuria severity should be implemented. Since renal impairment may affect cancer therapy, early recognition and intervention are crucial. The current understanding of this disease in China remains limited. This article reviews its pathogenesis, pathological features, and treatment strategies, aiming to provide a reference basis for clinical practice.

  • Xu Lingyi, Tang Linger, Xue Shuo, Zhou Qingqing, Jiang Lei, Yang Li, Zheng Xizi
    Abstract (1373) PDF (112) RichHTML (1272)

    Objective To summarize the clinicopathologic characteristics of malignant hypertension (MHT) patients with acute kidney injury (AKI) and application of renin?angiotensin?aldosterone system inhibitor (RAASi). Methods It was a retrospective cohort study. The adult patients with MHT and AKI admitted to Peking University First Hospital from January 1, 2012 to July 14, 2022. The patients were categorized into RAASi group and non-RAASi group based on RAASi administration from AKI onset to discharge. The clinicopathological data between the two groups were compared, and application of RAASi was analyzed. Results A total of 179 patients were enrolled with age of 31 (26, 37) years and 148 males (82.7%). Ninety-five patients (53.1%) received dialysis treatment. The common causes of MHT were essential hypertension (125 patients, 69.8%), renal hypertension (39 patients, 21.8%) and endocrine hypertension (7 patients, 3.9%). AKI severity distribution showed 41 patients (22.9%) in stage 1, 1 patient (0.5%) in stage 2 and 137 patients (76.5%) in stage 3. Among MHT patients, 94 patients (52.5%) had been treated with RAASi before AKI, and 13 patients (7.3%) discontinued RAASi after AKI. Among 85 patients (47.5%) without receiving RAASi treatment before AKI, 68 new patients (38.0%) received RAASi treatment after AKI, and 40 patients (22.3%) were treated with the support of dialysis. Compared with non-RAASI group (n=30), proportions of chronic kidney disease (χ2=6.324, P=0.012) and post-AKI hyperkalemia (χ2=4.048, P=0.044) in RAASi group (n=149) were lower, and the proportion of dialysis treatment (χ2=5.638, P=0.018), admission diastolic blood pressure (Z=-3.609, P<0.001) and maximum diastolic blood pressure during hospitalization (Z=-1.978, P=0.048) were higher. There were no statistically significant differences in the rates of target blood pressure control and renal function recovery between the two groups during hospitalization (all P>0.05). During hospitalization, 64 patients received renal biopsies, of which 50 patients (78.1%) had typical MHT vascular lesions such as "onion skin" in renal arterioles. Twenty-seven patients (42.2%) were complicated with glomerular diseases, and IgA nephropathy was the most common type (85.2%, 23/27). The proportions of glomerular ischemia and sclerosis, endothelial cell proliferation and acute renal tubular injury in RAASi group (n=54) were lower than those in non-RAASi group (n=10), and proportions of thrombosis and "onion skin" change were higher than those in RAASi group (n=10), but the differences were not statistically significant (all P>0.05). Renal function recovery occurred in 47 patients (26.3%) by discharge. Among 95 dialysis patients, 26 patients (27.4%) achieved dialysis independence at discharge. Conclusions MHT patients with AKI exhibit severe renal pathology and short-term poor prognosis. RAASi is primarily prescribed to those with relatively better kidney function or those receiving dialysis support.

  • Zhao Bin, Zhang Lihong, Zhan Shen, Liu Lifang, Liu Wei, Guo Shanshan, Xiao Guanghui, Wang Yuzhu
    Abstract (1356) PDF (143) RichHTML (1277)

    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.

  • Chen Si, Pan Ying, Lu Yifei, Qian Li, Li Qing, Xu Yili, Duan Suyan, Wu Lin, Zhang Bo, Xing Changying, Mao Huijuan, Yuan Yanggang
    Abstract (1332) PDF (216) RichHTML (1353)

    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.

  • Wan Ling, Chen Chaoying, Tu Juan, Li Huarong, Sun Jinshan, Xia Hua
    Abstract (1313) PDF (125) RichHTML (1494)

    Objective To evaluate the efficacy and safety of rituximab (RTX) in children with steroid resistant nephrotic syndrome (SRNS). Methods The was a retrospective observational study. A retrospective analysis was conducted on the clinical data of 14 children with SRNS who received RTX treatment in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from July 2021 to August 2023. The changes in urinary protein content, renal function, serum albumin, immunoglobulin and other indicators before and after RTX medication were compared to evaluate the clinical efficacy and safety of RTX treatment for SRNS. Results A total of 14 children with SRNS were selected for this study, with a male to female ratio of 6:8. The age of onset of kidney disease was (4.36±3.12) years, and 8 of them underwent kidney biopsy. Among them, 6 cases showed focal segmental glomerulosclerosis in renal pathology, and 2 cases showed minimal change disease. The age of 14 children who first used RTX was (8.45±3.98) years old, with a dose of 375 mg/m2 and a maximum dose of 500 mg. The number of children who had used 2, 3, 4, and 5 doses of RTX was 6, 6, 1, and 1, respectively. RTX was administered orally with compound sulfamethoxazole to prevent infection. Glucocorticoids and immunosuppressants were discontinued 4.0(2.5, 6.5) months after the first dose of RTX. The median follow-up time was 10.5(6.0, 18.3) months. By the end of the 3-month, 6-month, and follow-up endpoints, the complete remission rates of kidney disease in the children were 100.0%(14/14), 85.7%(12/14), and 64.3%(9/14), respectively. Five children experienced kidney disease recurrence. Compared with before the first dose of RTX treatment, the serum albumin and height significantly increased, while body mass index significantly decreased at the end of follow-up (all P<0.05). There was no statistically significant difference in urinary protein content, renal function, and IgG (all P>0.05). During the RTX treatment, all 14 children did not experience any infusion reaction, and there were no serious infections during follow-up. One case was diagnosed with hypogammaglobulinemia. Conclusions RTX can improve the remission rate and recurrence rate of SRNS children, reduce the dosage of glucocorticoids and related drug untoward reaction, significantly improve patient height and BMI, with minimal side effects. Especially for SRNS patients who cannot be relieved by the combination of glucocorticoids and immunosuppressants, RTX may be considered.

  • Huang Haocheng, Li Jun, Yang Xiaobing
    Abstract (1297) PDF (202) RichHTML (1152)

    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.

  • CKIA Innovation Research Working Group, Society of Nephrology, Chinese Research Hospital Association
    Abstract (1257) PDF (166) RichHTML (1143)

    Peritoneal dialysis is becoming the preferred dialysis mode for more and more patients with end-stage renal disease. However, the current peritoneal dialysis products cannot meet the ideal peritoneal dialysis treatment needs. Based on the analysis of the incoPat patent database, the Innovation Research Working Group of the China Kidney Innovation Association (CKIA) summarized the peritoneal dialysis patent applications of the past ten years from 46 representative medical institutions in the field of nephrology in 19 provinces and 4 municipalities in China. A total of 271 patents related to peritoneal dialysis were screened, mainly utility model patents (80.1%), and the top three needs included peritoneal dialysis manual fluid exchange, peritoneal dialysis catheter or peritoneal dialysis transfer set, drainage fluid disposal and sampling. The subdivision of patent was summarized, which fully reflects the urgent need to improve the effectiveness, safety and convenience of peritoneal dialysis treatment, improve the achievement ratio of peritoneal dialysis-related surgery and reduce complications, and improve the whole chain of peritoneal dialysis related products to enhance the quality of treatment and meet the personalized needs of patients. At the same time, the report shows that the new diagnosis and treatment methods involved in the patent application for peritoneal dialysis are seriously insufficient, and there is still a considerable gap with the cutting-edge scientific and technological level in the field of kidney. By condensing the technological innovation needs of the whole chain of peritoneal dialysis related products, the working group provides track for the future innovation and transformation of peritoneal dialysis treatment, in order to promote the progress of peritoneal dialysis technology in China.

  • Ye Ziyang, Duan Qi, Wu Feng, Li Xiaotong, Zhang Sijie, Li Yafeng
    Abstract (1253) PDF (81) RichHTML (1097)

    Objective To explore the role of curcumin (Cur) in improving IgA nephropathy (IgAN) and its related mechanisms. Methods Fifty 7-month-old miR-23b knockout (miR-23b-/-) mice weighing (25±5) g were used to establish an IgAN disease model, and were randomly divided into IgAN group, IgAN+Cur (150 mg/kg) group and IgAN+Cur (300 mg/kg) group using simple randomisation. Sixteen healthy 7-month-old weighing (25±3) g C57BL/6J wild-type mice served as the normal control group. IgAN+Cur (150 mg/kg) and IgAN+Cur (300 mg/kg) groups were respectively gavaged continuously with 150 mg/kg Cur and 300 mg/kg Cur for 8 weeks, and the normal control and IgAN groups were gavaged continuously with an equal dose of 0.9% sodium chloride solution for 8 weeks. The samples of urine, serum, intestinal fluid, intestinal tissues, kidney tissues and liver tissues were collected from each group. In vitro experiments, human cloned colon adenocarcinoma (Caco-2) cells were divided into blank control (Ctrl), Ctrl+Cur (10 μmol/L), Ctrl+ Cur (60 μmol/L), tumor necrosis factor?α (TNF‐α), TNF‐α+Cur (10 μmol/L) and TNF‐α+Cur (60 μmol/L) groups. Enzyme-linked immunosorbent assay was used to detect serum alanine transaminase, aspartate transaminase, secretory IgA (sIgA), creatinine, blood urea nitrogen, 24 h urine microalbumin, as well as sIgA, TNF-α, interleukin (IL)-6 and IL-1β in the intestinal fluids. HE staining was used to observe the effect of Cur on liver tissues, the hyperplasia of glomerular mesangial zone in kidney tissues and the morphological and structural changes of intestinal epithelial barrier, and the histopathological damage scores were performed respectively. PAS staining was used to observe the changes of glomerular basement membrane and mesangial matrix. Immunofluorescence was used to observe the deposition of immune complexes in the glomerular mesangial zone. Real-time quantitative PCR was used to detect the mRNA expression levels of B?cell activating factor (BAFF)and a proliferation inducing ligand (APRIL). Western blotting was used to detect the protein expression levels of tight junction proteins zonula occluden?1 (ZO-1) and occludin in the mouse intestinal tissues. The potential targets of Cur in IgAN were predicted. Western blotting was used to detect the protein expression levels of tight junction proteins, as well as Toll?like receptor 9 (TLR9), myeloid differentiation primary response protein (MyD88), nuclear factor?κB p65 (NF-κB p65) and p-NF-κB p65. Results Genetic identification results revealed that all IgAN model mice exhibited the miR-23b-/- genotype, confirming successful model establishment. Seven-month-old mice were subsequently selected for Cur treatment. Histopathological analysis demonstrated no significant differences in hepatic tissue morphology across groups, with comparable liver histopathological injury scores and unaltered liver function parameters, thereby validating the safety of Cur administration. Compared with the normal control group, IgAN mice displayed elevated levels of serum sIgA, serum creatinine, blood urea nitrogen and 24 h urine microalbumin (all P<0.05). Renal pathological results revealed severe mesangial hypercellularity in glomeruli, higher glomerular injury scores, and notable glomerular mesangial deposits of IgA, IgG and complement C3 in IgAN mice (all P<0.05). Additionally, intestinal pathological alterations were observed, including structural changes in intestinal epithelium and Peyer's patches, accompanied by significantly higher intestinal histopathological injury scores in IgAN mice (P<0.05). Intestinal epithelial expression levels of ZO-1 and occludin were significantly reduced, while sIgA, TNF-α, IL-1β and IL-6 in intestinal fluid were elevated (all P<0.05). Serum FITC fluorescence intensity was markedly increased, and intestinal tissue exhibited upregulated mRNA expression of BAFF and APRIL (all P<0.05). Following Cur treatment, serum sIgA level and renal function indices in mice showed partial recovery (all P<0.05). Renal pathological improvements included alleviated mesangial hypercellularity, reduced glomerular injury scores, and diminished glomerular immune complex deposition (all P<0.05). Intestinal pathologies, including epithelial and Peyer's patch lesions, were mitigated, with decreased intestinal histopathological injury scores (P<0.05). Additionally, intestinal tight junction protein expression levels were upregulated, intestinal fluid sIgA level was reduced, inflammatory markers were attenuated, serum FITC fluorescence intensity was declined, and intestinal BAFF and APRIL mRNA expression levels were downregulated (all P<0.05). In vitro experiments demonstrated that TNF-α exposure reduced tight junction protein expression in Caco-2 cells, whereas Cur treatment reversed the effect (all P<0.05). Target prediction analysis revealed that Cur effectively bound to TLR9 structural domain in IgAN. Experimental validation confirmed that Cur treatment suppressed the upregulated protein expression levels of TLR9, MyD88, NF-κB p65 and p-NF-κB p65 in intestinal tissues of IgAN mice (all P<0.05). Conclusion Cur has a significant effect in the treatment of IgAN and can regulate intestinal mucosal immunity by inhibiting the TLR9/MyD88/NF-κB signaling pathway, thereby reducing renal injury and protecting the kidneys.

  • Wang Congfei, Pan Bin, Xie Xishao, Xu Chunping, Chen Jianghua, Zhang Ping
    Abstract (1193) PDF (192) RichHTML (1097)

    Objective To analyze the impact of post?dialysis blood pressure (Post?BP) on the long-term survival prognosis of maintenance hemodialysis (MHD) patients and the related risk factors. Methods It was a retrospective cohort study. The data of patients who underwent their first hemodialysis (HD) from January 1, 2007, to June 30, 2021, as recorded in the dialysis registration system of the Kidney Disease Center, the First Affiliated Hospital, Zhejiang University School of Medicine was retrospectively analyzed. The mean Post?BP was calculated for each HD session 4-6 months after hemodialysis. According to the mean value of post?dialysis diastolic pressure (Post?DBP) at 4-6 months after dialysis, patients were divided into 3 groups (Post?DBP<80 mmHg, 80 mmHg≤Post?DBP<90 mmHg, Post?DBP≥90 mmHg). According to whether the mean value of post?dialysis systolic pressure (Post?SBP) was ≥140 mmHg and whether the mean value of Post?DBP was ≥80 mmHg, patients were divided into 4 groups (Post?SBP<140 mmHg, Post?DBP≥80 mmHg; Post?SBP≥140 mmHg, Post?DBP≥80 mmHg; Post?SBP<140 mmHg, Post?DBP<80 mmHg; Post?SBP≥140 mmHg, Post?DBP<80 mmHg). Patients' first dialysis time was used as the starting point of follow-up, and the end point of follow-up was death or conversion to peritoneal dialysis or kidney transplantation or up to December 31, 2021. Kaplan?Meier survival analysis, Log?rank test, and multivariate Cox regression model were used to analyze the relationship between Post?BP and survival rate and the related factors of prognosis in MHD patients. Results According to inclusion criteria, a total of 1 213 patients were included. Kaplan?Meier survival curve showed that the long-term survival rate had statistically significant differences among Post?DBP<80 mmHg, 80 mmHg≤Post?DBP<90 mmHg and Post?DBP≥90 mmHg groups (Log?rank test, χ2=58.838, P<0.001), and Post?DBP<80 mmHg group was the lowest. Further comparing the cardiovascular diseases (CVD) mortality among the three groups, the curve showed a statistically significant difference (Log?rank test, χ2=27.926, P< 0.001), and the highest CVD mortality was found in the Post?DBP<80 mmHg group. Multivariate Cox regression model analysis showed that Post?DBP<80 mmHg was an independent associated factor for death in MHD patients (with Post?DBP mmHg≥90 group as reference, HR=4.197, 95% CI 1.452-12.197, P=0.008). When patients were divided into 4 groups according to whether the mean value of Post?SBP was ≥140 mmHg and whether the mean value of Post?DBP was ≥80 mmHg, Kaplan?Meier survival analysis showed a statistically significant difference in long-term survival rate among the four groups (Log?rank test, χ2=65.636, P<0.001), among which Post?SBP≥140 mmHg, Post?DBP<80 mmHg group had the lowest long-term survival rate. Further comparing the CVD mortality rate among the four groups, the curve showed a statistically significant difference (Log?rank test, χ2=29.784, P<0.001), and the highest CVD mortality rate was found in the Post?SBP≥140 mmHg, Post?DBP<80 mmHg group. Multivariate Cox regression analysis revealed that regardless of whether the average Post?SBP was ≥140 mmHg, Post?DBP<80 mmHg was an independent associated factor for death in MHD patients(with Post?SBP<140 mmHg, Post?DBP≥80 mmHg group as reference, Post?SBP≥140 mmHg, Post?DBP<80 mmHg group: HR=3.416, 95% CI 1.294-9.019, P=0.013; Post?SBP<140 mmHg, Post?DBP<80 mmHg group: HR=3.574, 95% CI 1.451-8.802, P=0.006). Conclusions The long-term survival rate of the group with Post?SBP≥140 mmHg and Post?DBP<80 mmHg is significantly lower. Post?DBP<80 mmHg is an independent risk factor for death in MHD patients regardless of whether the average Post?SBP is ≥140 mmHg.

  • Zhang Xumei, Wang Haiyan, Yue Zhihui, Wei Haixia, Sun Liangzhong
    Abstract (1187) PDF (65) RichHTML (954)

    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.

  • Sun Xinyu, Guo Wenfeng, Wang Tingting, Li Huirong, Zhou Lu, Liu Hongbao
    Abstract (1145) PDF (74) RichHTML (1039)

    Acute kidney injury (AKI), a life-threatening clinical syndrome, lacks effective therapeutic interventions beyond supportive care such as hemodialysis and kidney transplantation. Pathologically, excessive reactive oxygen species -mediated oxidative stress is recognized as a pivotal contributor to AKI progression, positioning reactive oxygen species scavenging as a critical therapeutic target. Emerging nanozymes, nanomaterials with enzyme-mimetic activities, have shown promise in addressing AKI by targeting renal oxidative stress. The article mainly reviewed the content of classification of nanozymes, research findings on nanozymes in treating AKI along with discussions on their mechanisms of action, as well as the current challenges and existing issues in nanozymes-based AKI therapy, to provide enhanced research perspectives for advancing nanozyme applications in the prevention and treatment of AKI.

  • Sun Jinshan, Chen Chaoying, Tu Juan, Geng Haiyun, Li Huarong, Wan Ling, Zhou Lin
    Abstract (1119) PDF (77) RichHTML (1103)

    Objective To investigate the distribution and drug resistance of common pathogens causing urinary tract infection (UTI) in children in Beijing, so as to provide reference for clinical rational use of antibiotics. Methods It was a retrospective cohort study. The results of clinical data, urine culture and drug sensitivity in children with urinary infection treated in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from June 2018 to June 2018 were retrospectively analyzed. According to the diagnostic criteria of "Chinese expert consensus on the diagnosis and treatment of UTI (2015 edition) - Complicated urinary tract infection", the children were divided into complex group and simple group according to whether they had complicated factors, and the pathogenic factors of the complex group were analyzed. The χ2 test was used to compare the distribution of pathogenic bacteria in urine culture and the resistance rate of Escherichia coli to common antibiotics between the two groups. Results A total of 187 children with UTI were enrolled in this study. The age ranged from 1 month after birth to 17 years old, and the median age was 8 months. There were 88 males (47.1%) and 99 females (52.9%), and the male/female ratio was 1:1.125. Male infants accounted for 79.5% (70/88) of male infants and female infants accounted for 48.5% (48/99) of female infants. There were 45 cases (24.1%) in the simple UTI group and 142 cases (75.9%) in the complicated UTI group. A total of 216 strains of pathogens were isolated, mainly Gram-negative bacteria (151/216, 69.9%), of which Escherichia coli was the most common (86/216, 39.8%). The second was gram-positive bacteria (57/216, 26.4%), among which Enterococcus faecium (37/216, 17.1%) was the most common. The positive rate of Escherichia coli infection in the simple UTI group was significantly higher than that in the complicated UTI group [71.1% (32/45) vs. 31.6% (54/171), χ2=23.234, P<0.001], and the positive rate of Klebsiella pneumoniae, Pseudomonas aeruginosa, Enterococcus faecium and fungal infection in the simple UTI group was significantly lower than those in the complicated UTI group. However, the differences were not statistically significant (all P>0.05). The resistance rate of Escherichia coli to ampicillin was the highest in children with UTI [91.9% (79/86)], and it was sensitive to amikacin, meropenem, imipenem, piperacillin/tazobactam, cefepime, piperacillin, cefazolin, cefoperazone/sulbactam. The drug resistance rates were 5.8% (5/86), 5.8% (5/86), 9.3% (8/86), 10.5% (9/86), 14.0% (12/86), 15.1% (13/86), 18.6% (16/86) and 18.6% (16/86), respectively. The resistance rate of Escherichia coli infection to ceftriaxone in the complicated UTI group was significantly higher than that in the simple UTI group [59.3% (32/54) vs. 24.4% (11/32), χ2=4.977, P=0.026]. Eight fungi (3.7%) were susceptible to fluconazole, voriconazole, itraconazole and amphotericin B. Conclusions The main pathogens of UTI in children are Gram-negative bacteria, among which Escherichia coli is the most common pathogen, but the proportion of infection has a downward trend in recent years. The resistance rate of ceftazidime and ceftriaxone is relatively low, which can be used as empirical drugs for children with UTI in this region.

  • Lan Qin, Long Hongyan, Da Jingjing, Yu Fuxun, Zha Yan
    Abstract (1091) PDF (71) RichHTML (1106)

    Extracellular vesicles (EVs) are communication vectors between cells and organs, which have been demonstrated new potential role in the pathogenesis, diagnosis, and treatment of kidney diseases. This paper describes the role of EVs in intercellular and inter organ communication in diabetic nephropathy by reviewing the biological properties of EVs, EVs-mediated organ-organ crosstalk, cell-cell crosstalk in diabetic nephropathy, and the role of EVs in the diagnosis and treatment of renal diseases. These insights aim to provide a theoretical basis for a more comprehensive understanding of the role of EVs in the pathogenesis and progression of diabetic nephropathy.

  • Ao Guangyu, Li Jing, Tang Yuanyuan, Chen Min
    Abstract (1090) PDF (119) RichHTML (933)

    Chronic kidney disease (CKD) patients with concurrent renal anemia and thalassemia present complex pathogenesis involving various iron metabolism disorders, which are among the key factors contributing to suboptimal therapeutic outcomes. This paper reports the clinical experience of treating five CKD stage 3-4 patients with thalassemia using roxadustat. The treatment outcomes demonstrated that all patients experienced significant improvements in anemia, and no cases of iron overload were observed throughout the follow-up period. These findings suggest that roxadustat effectively increases hemoglobin levels in patients with renal anemia and thalassemia while maintaining a low risk of iron overload, providing a potential therapeutic option for these challenging cases.

  • Wang Xin, Zhou Yun
    Abstract (1088) PDF (153) RichHTML (1068)

    Membranous nephropathy (MN) is an organ-specific autoimmune disease, which is the main cause of nephrotic syndrome in adults. In 70%-80% of patients,MN occurs without an identifiable underlying cause. Regarding primary MN (PMN), 70% of which are associated with M-type phospholipase A2 receptor (PLA2R). At present, the treatment of MN primarily involves non-specific immunotherapy, such as immunosuppressants and B cell depletion. This review focused upon the mechanism of immune response in PLA2R-associated MN, aiming to provide theoretical basis and direction for specific immunosuppressive therapies in MN.

  • Guzailinuer·Sailaiajimu, Zou Guming, Qi Xinxin, Niu Peiyuan, Huang Xuan, Liu Zhen, Li Suhua, Lu Chen
    Abstract (1083) PDF (56) RichHTML (970)

    Objective To screen bile acid-related characteristic genes in IgA nephropathy (IgAN) based on the feature gene selection algorithm in the machine learning method, aiming to exploring the molecular biological mechanisms and biomarkers of IgAN. Methods The gene expression data and sample grouping information of GSE93798, GSE116626 and GSE35487 were downloaded from the Gene Expression Omnibus (GEO). Bile acid-related gene sequences were obtained from the Molecular Signatures Database (MSigDB). R language was used to identify differentially expressed genes between IgAN samples and healthy control samples. Candidate genes were obtained by intersecting differentially expressed genes and bile acid-related genes. The least absolute shrinkage and selection operator (LASSO) algorithm in machine learning was used to screen the feature genes in the candidate genes as biomarkers, and the feature genes in the training set and validation set were analyzed by the rate of change index. Receiver operating characteristic curve (ROC) method was used to evaluate the diagnostic value of identified bile acid related characteristic genes for IgAN. Gene set enrichment analysis (GSEA) was used to analyze the Spearman correlation between the characteristic genes and all other genes and their related metabolic pathways. The expression of disease-characteristic genes in the kidney tissues of IgAN rats was validated by real-time PCR. Results Gene expression information from kidney tissue samples of 20 IgAN cases and 22 healthy controls were obtained from GEO database. A total of 204 bile acid-related genes including 24 pathways were obtained from MSigDB. The results of gene differential expression analysis showed that 333 genes in the kidney tissues of IgAN patients were differentially expressed compared with those of healthy controls, including 102 up-regulated genes and 231 down-regulated genes, among which 12 differentially expressed genes were related to bile acid genes, as follows: NR1H4,SLC23A1, ALDH8A1, FABP1, ALB, SLC27A2, DIO1, CYP8B1, BBOX1, PIPOX, AKR1C1 and SLC10A2. Five characteristic genes (NR1H4, SLC23A1, FABP1, ALB and AKR1C1) were screened by LASSO regression algorithm.ROC analysis results showed that in GSE93798 cohort genes, the AUC of NR1H4, SLC23A1, FABP1 and ALB genes with differential expression was >0.95 respectively in diagnosing IgAN, and that of AKR1C1 genes with differential expression was >0.85 in diagnosing IgAN. The gene expression data of SLC23A1 in GSE35487 cohort was missing. ROC analysis results of other four genes showed that the AUC of differential expression of ALB gene for IgAN was >0.95 respectively, that of NR1H4 gene was >0.70, and that of both FABP1 and AKR1C1 gene was >0.60. In the GSE116626 cohort genes, the AUC of five disease characteristic genes (NR1H4, SLC23A1, FABP1, ALB, AKR1C1) for diagnosing IgAN was >0.60, respectively. These results suggested that 5 characteristic genes have certain distinguishing ability between IgAN group and control group. GSEA results were displayed that the characteristic genes were related to butyric acid metabolism, propionic acid metabolism, arginine and proline metabolism, valine leucine and isoleucine degradation, fatty acid metabolism, etc. These results suggested that five characteristic genes might be related to IgAN through the above metabolic mechanisms. The verification results of five bile acid characteristic genes in the rat model of IgAN in the kidney tissue showed that the expressions of four genes, NR1H4, SLC23A1, FABP1 and ALB, were higher than those of the control group, and there was no statistical significance in the expression of AKR1C1 gene between the two groups. Conclusions The expression of bile acid-related characteristic genes is abnormal in the kidney tissue of IgAN patients. Four bile acid-related differentially expressed genes, NR1H4, SLC23A1, FABP1 and ALB, are expected to be biomarkers for non-invasive diagnosis and therapeutic targets.

  • Liao Panli, Zhu Gaohong, Yang Huihui, Huang Lin, Wang Daojing, Wang Jia, Liu Heng, Jiang Qianqian, Wang Xiaowen
    Abstract (1082) PDF (75) RichHTML (1081)

    Objective To observe the efficacy of eculizumab in children with atypical hemolytic uremic syndrome. Methods It was a single-center observational study. The clinical data of children diagnosed with atypical hemolytic uremic syndrome and treated with eculizumab in Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2023 to May 2024 were retrospectively collected. Eculizumab was used at the conventional dose based on the children 's weight. Event-free survival (no death or end-stage renal disease) rate, complete remission rate and recurrence rate of thrombotic microangiopathy in children with atypical hemolytic uremic syndrome after eculizumab treatment were analyzed. The complete remission time of estimated glomerular filtration rate, hemoglobin, platelet, lactic dehydrogenase, urine routine and the adverse reactions during the treatment were observed. Whole exome sequencing was used to conduct genetic testing based on blood samples of the children and their parents. Results There were 4 children enrolled in the study. Four children were all Han Chinese, including 3 males and 1 female. The median age of onset was 8 years (ranging from 7 to 10 years). Two patients had complement gene abnormalities, both of which were homozygous deletions of complement factor H-related 1 and complement factor H-related 3. All the patients were free of plasma exchange or perfusion after treatment with eculizumab, and the 6-month event-free survival rate and thrombotic microangiopathy complete remission rate were both 4/4. The complete remission time was 19 (14-28) days. The time for the complete recovery of platelets, lactate dehydrogenase, estimated glomerular filtration rate and hemoglobin in 4 children was 4 (1-5), 19 (14-28), 10 (5-14) and 29 (20-42) days, respectively. Except for 1 patient whose urine routine fluctuated between negative and weakly positive expression, the other 3 patients had normal urine routine. All the patients discontinued eculizumab. Two patients without gene mutations discontinued eculizumab after 7 doses, and there was no recurrence during the 1-year follow-up after drug withdrawal. Two patients with genetic abnormalities discontinued eculizumab after 26 weeks of treatment, and no recurrence was found during the 3-month follow-up after drug withdrawal. One patient developed rash approximately 7 days after receiving the third dose of eculizumab. The rash was relieved after anti-allergic treatment, and there was no recurrence after the continued use of eculizumab. Conclusion Eculizumab is effective and safe in the treatment of children with atypical hemolytic uremic syndrome. Discontinuation of eculizumab can be considered in patients without gene mutations when their condition is stable, but close monitoring and follow-up are needed after drug withdrawal.