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.

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.

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.

The physiological function of kidneys in the elderly population decreases with aging. Meanwhile, the aging population is highly susceptible to acute kidney injury (AKI) during the progression and diagnosis and treatment of other diseases. At present, the clinical diagnosis of renal lesions mainly depends on pathology, but renal biopsy is difficult to repeat and sampling is one-sided, and for elderly patients, there are existing comorbidities that make perioperative management difficult. As one of the highlight topics in the research of medical imaging, multimodal functional magnetic resonance imaging is of great significance in distinguishing the pathological and physiological characteristics of kidney diseases and predicting prognosis. This article reviews the research progress of multimodal functional magnetic resonance imaging in AKI in the elderly.

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：This retrospective study was to evaluate the clinical characteristics and prognosis of chronic kidney disease (CKD) patients complicated with novel coronavirus pneumonia (NCP).
Ｍｅｔｈｏｄｏｌｏｇｙ：We retrospectively analyzed the clinical and imaging features before and after coronavirus disease 2019 (COVID-19) of CKD patients complicated with NCP who were admitted to the National Clinical Research Center of Kidney Diseases, Jinling Hospital from December 2022 to June 2023.
Ｒｅｓｕｌｔｓ：Among the 72 CKD patients with NCP, the median age at COVID19 was 64 (39, 76) years old, and the median duration of CKD course was 36 (7, 120) months. The top four original diseases for CKD were diabetic nephropathy (22.22％), lupus nephritis (12.50％), membranous nephropathy (9.72％) and ANCA associated glomerulonephritis (9.72％), and the coverage rate of COVID-19 vaccine was 18.06％. 51.39％ of NCP were medium, 20.83％ were severe and 27.78％ were critical. The in-hospital mortality rate was 19.44％. The inflammatory index, proportion of fungal infection, heart failure, hepatic injury, and pneumothorax in the death group were higher than those in the survival group, and the lymphocyte subsets were lower than those in the survival group (P<0.05). Multivariate COX model showed that age, lactic dehydrogenase, CD4+T lymphpocyte and C-reactive protein during COVID-19 were independent risk factors for death in CKD patients complicated with NCP.
Ｃｏｎｃｌｕｓｉｏｎ：CKD patients with NCP have a high proportion of DN, a low vaccination rate and more concomitant diseases. Patients with advanced age, hypoxia, immunodeficiency and strong inflammatory response have a poor prognosis. Coinfections or superinfections of fungus and multiple organ failure are common during NCP treatment.

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.

Membranous nephropathy (MN) is a glomerular disease closely associated with immune system dysregulation. The pathogenic cornerstone of MN lies in the generation of autoantibodies targeting specific antigens located on podocytes, culminating in the formation of immune complexes. Recent advancements in high-resolution liquid chromatography-tandem mass spectrometry (LC-MS／MS), laser microdissection-mass spectrometry (LMD-MS), immunohistochemistry, and immunofluorescence microscopy have enabled the identification and validation of a series of target antigens from complex biological samples. In addition, the levels of certain proteins, metabolites and ncRNA in urine and blood samples of MN were different from those in healthy controls, and these molecular biomarkers were of significant significance in the diagnosis, disease monitoring and prognosis assessment of MN.

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.

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.

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 curative effect and safety of artificial expanded polytetrafluorethylene (ePTFE) protective external vascular sheath for kinked transplanted renal artery.
Ｍｅｔｈｏｄｏｌｏｇｙ：A total of 286 kidney transplants were performed between January 2019 and June 2022 at the First Affiliated Hospital of Soochow University, of which 8 cases presented with excessive anastomotic artery length and arterial kinking after placement in the iliac fossa, affecting the blood supply of transplanted kidney. An artificial ePTFE external vascular sheath was then used to wrap and immobilize the anastomotic artery during the operation. Demographics, preoperative, and postoperative recovery data of those patients were retrospectively analyzed, and the efficacy and safety of the method were evaluated.
Ｒｅｓｕｌｔｓ：Among the 8 patients, 6 were male and 2 were female, with an average age of 43.1±8.5 years. All 8 cases had a kinked anastomotic artery when the right donor kidney with a single branch artery was anastomosed end-to-end with the recipient internal iliac artery. Two cases exhibited a weakened pulsation of transplant kidney and a darkening of its color immediately after the kidney was placed in the right iliac fossa. No deterioration of color or tension was found in the remaining 6 cases. The application of an artificial ePTFE external vascular sheath significantly corrected the kinked artery and improved blood supply of the transplanted kidney. Postoperative ultrasonography showed that the arterial velocity was 146.7±10.4 cm／s, and the resistance index (RI) was 0.68±0.06. All of the patients recovered normally after surgery except for one case that developed delayed graft function (DGF), with a gradual increase in urine output on the fifth postoperative day and a return to normal renal function in the second week. No rejection, arterial thrombosis, or infection occurred, and no abnormalities were observed under the ultrasound or CT. The flow velocity and RI in the third month after the operation were 151.3±7.8 cm／s and 0.67±0.07, respectively. There were no significant differences compared to the immediate postoperative data (P>005). All 8 cases survived without any manifestations of renal artery stenosis, infection and discomfort in the surgical area.
Ｃｏｎｃｌｕｓｉｏｎ：The ePTFE protective external sheath effectively corrected the kinked anastomotic artery, improved the blood supply, and reduced the possibility of longterm stenosis in the transplanted renal artery. It demonstrated good stability, high safety and few complications.

Thrombotic microangiopathy (TMA) is a lethal complication of renal transplantation that severely affects the long-term survival of the renal transplant recipient and the transplanted kidney. Transplant renal thrombotic microangiopathy can be divided into de novo TMA and recurrent atypical haemolytic uremic syndrome (aHUS). De novo TMA is more common and has a worse prognosis than recurrent aHUS. At this stage, the clinical manifestations of TMA in transplanted kidneys are varied and specific tests are lacking. Therefore, the diagnosis of TMA in transplanted kidneys relies heavily on kidney tissue biopsy. No precise individualised treatment protocols with proven efficacy have been studied. This paper reviews the recent research progress in the pathogenesis and treatment of TMA in transplanted kidneys, providing new ideas for the accurate diagnosis and effective treatment of TMA in transplanted kidneys in the future.

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.

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.

An 11-year-old boy presented with massive proteinuria and hematuria. The patient had a history of immunorelated pancytopenia, acquired hemophilia and cerebral infarction. Renal biopsy revealed membranoproliferative glomerulonephritis with occasional thrombotic micro-angiopathy-like lesions. On electron microscopy,there were a lot of microtubules in mesangial and subendothelial region,ranging in size from 8 to 12 nm. The final diagnosis was membranoproliferative glomerulonephritis, which was related to immune complex and thrombotic micro-angiopathy lesions.