This article introduces 4 national standards for the infrastructure construction and operation management of the biobank, GB／T 39766-2021Management specification for human biobank, GB／T 39767-2021 Management specification for human biomaterial, GB／T40364-2021 Fundamental terminology for human biobank and GB／T 39768-2021 Classification and coding for human biomaterial.We systematically describe the standardized organizational structure, personnel management, environmental facilities, reagents and consumables, information systems, sample management, etc. Through the 4 standards, we can strengthen the constructional standard of biobank, promote the standardization of management and operation, so as to offer samples of high quality to down-stream scientific research.

Sepsis is one of the most common causes of acute kidney injury (AKI) in clnical. Sepsis-associated AKI (SA-AKI) is difficult to detect in the early stage, and has a high mortality rate and poor prognosis. Its pathogenesis is complicated, with multiple factors involved,and its clinical phenotypes,disease progression, and treatment response are all potentially heterogeneous. Currently, there is no definite and effective means of intervention. This review summarized the pathophysiology of SAAKI, in order to provide a reference for further research.

Alternative polyadenylation (APA), as a crucial post-transcriptional regulatory mechanism, has been reported to generate distinct transcripts with variable 3′UTR lengths by selecting different polyadenylation sites (polyA sites, PAS) in 3′UTR. The cis-regulatory elements in pre-mRNA, corresponding trans-acting factors, as well as various enzymes and protein factors in the nucleus are involved in the regulation of APA. Many cis-regulatory elements, such as microRNA (miRNA) or RNA-binding protein (RBP) binding sites, are embedded in the 3′UTR sequence, therefore, the presence or absence of these cis-acting elements conferred by 3′UTR-APA has far-reaching effects on the stability, translation rate, nuclear export, cellular localization of target mRNAs, as well as proteins localization. APA-associated genetic variants have been proposed to affect diverse physiological and pathological processes. Here, we will systematically elaborate the regulatory mechanism of APA and its research progress in diabetic nephropathy.

Chronic kidney disease (CKD) is widely recognized as one of the leading causes of death worldwide. In order to reduce the incidence of CKD and slow its progression, effective diagnosis, monitoring, treatment, and even preventive measures are urgently needed. In recent years, several large clinical studies have confirmed that sodium glucose transporter 2 inhibitors (SGLT2i) have a renoprotective effect in CKD patients, and can significantly improve the long-term prognosis of CKD patients, but the exact mechanism is still unclear. Exploring the intrinsic mechanism could provide new target strategies for delaying CKD progression. Here, we intend to discuss the mechanism of renal injury in CKD mediated by the Warburg effect related to fructose metabolism and explore the effects of SGLT2i on the Warburg effect.

Chronic kidney disease (CKD) has become a public health challenge worldwide and is closely associated with the risk of cardiovascular and other adverse events. How to identify the occurrence and progression of CKD early, stratify the risk of the population, and give early prevention and treatment is the focus of clinical attention. Metabolomics, a non-invasive, high-throughput assay elucidates the pathophysiological mechanisms of diseases by quantitative or semi-quantitative analysis of the abundance of metabolites and the metabolic pathways involved. Studies indicate that the metabolome of CKD patients can show significant differences with deteriorating renal function, and a variety of metabolites are closely associated with the deterioration of renal function, the risk of cardiovascular events, and even the risk of all-cause mortality in patients. Metabolomics has been successfully applied to improve the assessment of GFR and explore novel biomarkers for the risk of CKD and its complications. Furthermore, the application of metabolomics expands the understanding of the underlying pathophysiologic mechanisms of various complications during CKD and strengthens the understanding of the interactions of gut microbiome and their metabolites with CKD. This article reviews the research progression and application of metabolomics in CKD to provide novel directions for clinical diagnosis and treatment.

Ｏｂｊｅｃｔｉｖｅ：To explore the application of artificial intelligence-based analytic renal pathology system (ARPS) in patients with primary membranous nephropathy (PMN).
Ｍｅｔｈｏｄｏｌｏｇｙ：Patients with biopsy-proven PMN in our center from January 2018 to December 2019 were enrolled. Their clinical and pathological data were collected. ARPS was applied to identify glomeruli lesions including global glomerular sclerosis (GS), segmental glomerular sclerosis (SS), crescents (C), and none of the above (NOA), and then quantify intraglomerular features, including intrinsic glomerular cells (M: mesangial cells; E: endothelial cells; P: podocytes) and glomerular area. The performance of ARPS was evaluated. The relationships between ARPSbased intraglomerular features and prognosis were further analyzed.
Ｒｅｓｕｌｔｓ：A total of 123 patients (65.0％ males) were included. The average age was 47.1±14.0 years at biopsy. For the identification of glomerular lesions, ARPS achieved the best effect in NOA with 0.967 F1-score; followed by GS with 0.811 F1-score and SS with 0.545 F1-score. The F1-scores of ARPS on identifying intrinsic cells were greater than 0.950. The glomerular area was larger in no response (NR) patients than that in partial remission (PR) and complete remission (CR) patients; the number, density and ratio of podocytes were higher in CR patients than that in PR and NR patients. The remission rate of urinary protein was higher in patients with higher podocytes number or density than that in patients with lower podocytes number or density. Renal phospholipase A2 receptor (PLA2R) and podocyte number were independent predictors of urinary protein remission.
Ｃｏｎｃｌｕｓｉｏｎ：ARPS performed well in the identification of glomerular lesions and intrinsic cell types in PMN. The ARPSbased intraglomerular characteristics were significantly correlated with prognosis in PMN patients.

Autoimmune disease is a type of disease in which the immune system is unable to recognize autoantigens and tissues and produce immune response, resulting in damage or functional abnormalities of tissues and organs.Chimeric antigen receptor T cells (CAR-T) therapy is regarded as a promising new type of tumor immunotherapy, which has achieved great success in hematological malignancies such as B lymphoblastic leukemia, and also provides a new idea for the treatment of autoimmune diseases.

Innate immune cells that exhibit enhanced immune responses to the same or different stimuli after secondary stimulation are defined as trained immunity. Trained immunity is mediated by metabolic and epigenetic reprogramming caused by longterm adaptation of innate immune cells, mainly manifested as enhanced production of proinflammatory factors during secondary stimulation. Trained immunity may have a protective effect on host defense, but inappropriate induction of training immunity may also have deleterious effects. In autoimmune diseases such as systemic lupus erythematosus, chronic kidney disease or dialysis, trained immunity might lead to disease progression or enhancement of persistent inflammatory responses.

ＴThe incidence of diabetes is increasing in China. Diabetic ketoacidosis (DKA) is one of the most serious acute complications of diabetes, its incidence and hospitalization rate are also increasing.The occurrence of DKA is mostly caused by insulin deficiency or insufficient dose in diabetes patients, and the release of glucocorticoids such as adrenaline, cortisol and growth hormone leads to further increase of blood glucose.The high risk of acute kidney injury (AKI) due to the disturbance of acid-base balance, electrolyte balance and mass loss of body fluid in patients with DKA. AKI can cause acute of renal failure in a short time, endangering the lives of patients. The purpose of this study was to investigate the mechanism of DKA induced renal injury, so as to prevent or reverse AKI in diabetic patients with DKA.

Hemocompatibility is one category of biocompatibility to evaluate the quality of blood-contacting device applications and therapies. Criteria of hemocompatibility of hemodialysis focus on thrombogenicity, complement activation, cells activation and cytokine generation. Approaches to improve hemocompatibility include usage of membrane with improved profiles, improving design and adequate rinse of extracorporeal circuit, minimize blood-air interfaces, and individuation of anticoagulation, which can improve outcomes of hemo dialysis patients.

A 19-year-old female patient was referred to our hospital with asymptomatic proteinuria, normal renal function, hyperuricemia. Extrarenal manifestations included short stature, diabetes mellitus, hearing loss, pre-excitation syndrome. Her mother was also short stature. The kidney biopsy revealed focal segmental glomerulosclerosis (FSGS), with dysmorphic abnormal mitochondria in the podocytes, parietal epithelial cells and renal tubular epithelial cells on electron microscopy. Gene testing revealed a m.3243A>G mutation in mitochondrial MT-TL1 gene, maternal inherited. The final diagnosis was mitochondrial disease associated with m.3243A>G mutation.

Magnesium is an ion with important physiological and biochemical effects. The serum magnesium of hemodialysis patients with endstage kidney disease is affected by residual renal function, dialysis clearance, drug and nutritional status.Hypermagnesemia is common but rarely accompanied by clinical symptoms,well hypomagnesemia is associated with increased hypotension, arrhythmia, muscle spasm, cardiovascular mortality, and all-cause mortality in hemodialysis patients. Mild hypermagnesemia can improve the parathyroid hormone level, vascular calcification and bone health of hemodialysis patients to a certain extent. Proton pump inhibitors, high-dose loop diuretics and malnutrition can reduce the serum magnesium concentration. It is safe and convenient to use dialysate with higher magnesium concentration to supplement. Magnesium is related to the survival status and prognosis of dialysis patients. Although it is not clear whether the relationship between magnesium and the clinical outcome of dialysis patients is causal, serum magnesium slightly higher than the physiological level seems to benefit patients more. It is necessary to further study metabalism of magnesium in hemodialysis patients to explore whether there is an optimal range of serum magnesium, so as to optimize the bone health, cardiovascular outcomes and survival rate of patients.

Ｏｂｊｅｃｔｉｖｅ：To summarize the clinical and pathological characteristics of 3 patients with lupus nephritis who carried the missense variants of glucose 6-phosphate dehydrogenase (G6PD) and to discuss the clinical value of G6PD variants detection in systemic lupus erythematosus.
Ｍｅｔｈｏｄｏｌｏｇｙ：We collected the clinical and pathological data of 3 patients with lupus nephritis in Jinling Hospital. Pathogenic variants was screened by whole-exome sequencing (WES) in the patients and validated by Sanger sequencing in family members. G6PD／6PGD ratio method was used to test the G6PD enzyme activity. Quantitative polymerase chain reaction (qPCR) and cytometric bead array (CBA) was used to detect inflammatory signatures in two patients.
Ｒｅｓｕｌｔｓ：All the 3 patients had a history of pancytopenia. Coombs tests were negative, but the red blood cell fragments were all positive. WES identified two kinds of variants in G6PD gene. Two males had a hemizygous variant of c.G1466T (p.R489L) inherited from their mother, while the female patient had a compound heterozygous variant of c.G1466T (p.R489L) and c.G1478A (p.R493H) inherited from the parents. The G6PD enzyme activity were all deficient. We further identified hyperactivation signatures of type I interferon signaling and overproduction of pro-inflammatory cytokines in the blood of two patients. Compared with the normal controls, the expression of in terferon-stimulated genes IFIT1, IFI44L, OAS1 and MX2 in blood cells of two patients was significantly higher, as well as the serum IL-8 and IP-10 levels.
Ｃｏｎｃｌｕｓｉｏｎ：Lupus nephritis patients with G6PD gene variants may present with anemia as the first or prominent clinical manifestation, accompanied by excessive activation of type I interferon signal pathway and excessive production of inflammatory cytokines in the blood. Its role in the pathogenesis of SLE deserves further study.

Ｏｂｊｅｃｔｉｖｅ：To evaluate the feasibility of serum creatinine／cystatin C (SCr／CysC) ratio in the assessment of clinical events in peritoneal dialysis (PD) patients.
Ｍｅｔｈｏｄｏｌｏｇｙ：This was a cross-sectional study. Patients aged 16~65 years with maintained PD treatment were enrolled in this study. Baseline clinical characteristics such as gender, age, smoking, diabetes, blood pressure, body surface area(BSA), body mass index(BMI) were obtained. Serum Cr, CysC were measured by standard laboratory techniques. Body composition was examined with BIA. All patients were examined by transthoracic echocardiography to measure diameter of left ventricle. Left ventriclar mass index (LVMI) and relative wall thickness(RWT) were obtained by calculation. The relations between SCr／CysC ratio with body composition, left ventricular geometry remodeling and clinical events (mortality, technique failure, or rehospitalization)were evaluated by Logistic regression.
Ｒｅｓｕｌｔｓ：A total of 104 PD patients were enrolled in final analysis. The mean age of participants was 46.7 years. Among them, 51 (49％) were males. PD treatment modality was mainly DAPD, and mean PD vintage was 49.3 months. Dialysis adequacy data shown with total (renal+peritoneal) Kt／V was 1.85±0.71. Mean SCr／CysC ratio was 0.86±0.23. Variables with moderate to strong correlations with SCr／CysC ratio were body mass index (BMI), arm muscle circumference (AMC), lean body mass (LBM), intracellular water to total body water ratio (ICW／TBW), residual renal function (rGFR), normalized protein catabolic rate (nPCR), creatinine clearance rate (Ccr), left ventricular mass (LVM), subjective global nutritional assessment (SGA), extracellular water to total body water ratio (ECW／TBW). Logistic regression analyses showed that AMC (OR=0.969,95%CI 0.944~0.995), SCr／CysC (OR=0.116,95%CI 0.031~0.432) ratio, and LVMI (OR=1.067,95%CI 1.035~1.100) was independently factor related with clinical events during two years.
Ｃｏｎｃｌｕｓｉｏｎ：The SCr／CysC ratio is appropriate for evaluating body composition and is associated with the clinical events in PD patients with maintained PD treatment.

Alport syndrome (AS) is a kind of hereditary collagen disease with kidney, eye and hearing impairment as primary manifestations, which will lead to end-stage kidney disease (ESKD)in the early stage, and is included in the first batch of rare diseases list in China. At present, there is no cure for AS, but the research and development of drugs for the treatment of AS is accelerating. This article reviews the progress of drug research and clinical trials of AS at home and abroad in the past decade, hoping to provide strategies and ideas for the future drug development and treatment of AS, so as to benefit AS patients and their families.

Ｏｂｊｅｃｔｉｖｅ：To investigate the prevalence and risk factors of pruritus in maintenance hemodialysis (MHD) patients, and to explore risk factors of pruritus.
Ｍｅｔｈｏｄｏｌｏｇｙ：A questionnaire survey involving skin pruritus and clinical data was conducted among MHD patients from 24 hemodialysis centers which were selected by stratified sampling in Jiangsu Province from October 2018 to October 2019. The relevant factors of pruritus were analyzed.
Ｒｅｓｕｌｔｓ：A total of 3819 MHD patients were included. The prevalence of pruritus and moderate-to-extreme pruritus was 2755％ and 18.02％, respectively. The median duration of pruritus was 12.00 months. Among patients with pruritus, the proportion of occasional and slight scratching was the highest, and more than half of the patients had scratches. The effective rate of conventional drug treatment was 42.21％. Multivariate regression analysis showed that age≥45 years, increased body surface area, low diastolic blood pressure, smoking, drinking, history of secondary hyperparathyroidism, warfarin, muscle weakness, bone pain, metastatic calcification, osteoporosis, serum calcium and serum phosphorus levels are risk factors for pruritus in MHD patients.
Ｃｏｎｃｌｕｓｉｏｎ：The degree of pruritus in MHD patients is mild while the effective rate of treatment is 42.21％. Nephrologists should pay attention to pruritus in dialysis patients, and timely intervention should be given to patients' living habits, comorbidities, chronic kidney diseasemineral and bone disorders, so as to relieve pruritus and improve their quality of life.

Ｏｂｊｅｃｔｉｖｅ：To observe the clinical efficacy and safety of daratumumab in  treatment of relapse／refractory monoclonal gammopathy of renal significance(MGRS).
Ｍｅｔｈｏｄｏｌｏｇｙ：Patients suffered relapse／refractory MGRS and received the treatment of daratumumab from Septmeber 2021 to January 2023 were enrolled in the study. The clinical data was collected to evaluate hematological and renal responses, adverse reactions were analyzed.
Ｒｅｓｕｌｔｓ：Five patients were enrolled, with a median age of 51 (50, 57) years and a median disease duration of 58 (23, 59) months. Two patients were diagnosed light chain deposition disease (LCDD), two patients were diagnosed proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID) and the other one was monoclonal immunoglobulin-related C3 glomerulopathy. After receiving of daratumumab, one of the two patients with difference free light chain>50 mg／L achieved hematological complete response and the other one achieved partial response, while two of the three patients with difference free light chain between 20~50 mg／L decreased to <10 mg／L. After a follow-up of 10 (9, 12) months, all five patients were alive, and two achieved renal response. Infusion-related adverse reactions included cough, chills and throat discomfort, and all were classified to grade 1. In addition, one patient had low degree fever unrelated to infection, and mild neutropenia occured in one patient.
Ｃｏｎｃｌｕｓｉｏｎ：Daratumumab is effective and safe in treatment of relapse／refractory MGRS, the hematological response rate was high, and renal response was observed in some patients.

Ｏｂｊｅｃｔｉｖｅ：To investigate the clinical and genetic features of patients with COQ8B nephropathy.
Ｍｅｔｈｏｄｏｌｏｇｙ：We retrospectively analyzed the clinical and pathological manifestations, treatment and prognosis in patients diagnosed with COQ8B nephropathy by genetic test.
Ｒｅｓｕｌｔｓ：A total of nine patients with biallelic mutations of COQ8B were included, among which two patients carried homozygous mutation, and seven patients carried compound heterozygous mutation. The genetic pattern of all patients was consistent with autosomal recessive inheritance pattern. Mutation analysis revealed seven sequence variants in COQ8B (NM_024876 c.748G>C、c.737G>A、c.1093C>G、c.532C>T、c.449G>A、c.1468C>T、c.1465C>T), among which c.737G>A missense mutation is a hotspot mutation in these patients. Patients showed a largely renal-limited phenotype. Of the nine patients, seven started with non-nephrotic proteinuria, and two presented with nephrotic syndrome. The median age at onset was 17 years. Five patients progressed to end-stage kidney disease with a median age at 19 years. Medullary nephrocalcinosis was detected in five patients. Six patients underwent renal biopsy, among which four showed focal segmental glomerulosclerosis. Electron microscopy examination revealed mitochondrial abnormalities. Patients did not respond to immunosuppressive therapy. Treatment with coenzyme Q10 (COQ10) in two patients didnt result in a significant clinical improvement, which may be due to the late initiation of treatment.
Ｃｏｎｃｌｕｓｉｏｎ：COQ8B mutation can lead to autosomal recessive genetic disease manifested by adolescent-onset proteinuria. The main pathological feature is focal segmental glomerulosclerosis. Mitochondrial abnormalities under electron microscope and medullary nephrocalcinosis may be clinical indicators for genetic test. Early genetic diagnosis and COQ10 supplementation can improve prognosis in these patients.

Circadian rhythm disorder can increase the risk of diseases including chronic kidney disease (CKD), cancers, diabetes, hypertension, neurological diseases and cardiovascular diseases. Although the treatment of CKD has made great progress,CKD remains a global public health problem and an important cause of cardiovascular death. Therefore, it is necessary to explore new treatments. As a major organ of the peripheral clock system, kidney participates in regulating the formation of circadian rhythms. This article reviews the association between circadian rhythm disruption and CKD, and related interventions such as circadian therapeutics, to provide new strategy for the prevention and treatment of CKD.