Objective To explore the correlation between the intensity of net ultrafiltration in continuous renal replacement therapy (CRRT) and the survival prognosis in critically ill patients with acute kidney injury (AKI), and provide evidence-based references for establishing optimal net ultrafiltration target during CRRT. Methods This was a retrospective observational study. Demographic and clinical data of critically ill AKI patients who received CRRT in the Intensive Care Unit of West China Hospital, Sichuan University from May 2021 to September 2023 were collected. Net ultrafiltration was defined as the hourly fluid clearance volume in the 72 hours prior of CRRT. This variable was converted into a categorical variable, including low net ultrafiltration <1.01 ml·kg^-1·h^-1, moderate net ultrafiltration 1.01-1.38 ml·kg^-1·h^-1 and high net ultrafiltration >1.38 ml·kg^-1·h^-1, and the differences of baseline characteristics and clinical treatment conditions among the three groups were compared. Kaplan-Meier survival curve and log-rank test were used to compare the survival conditions among the three groups in patients at 28 days and 60 days after CRRT. Logistic regression analysis method was used to analyze the related factors of mortality in patients 28 days and 60 days after CRRT. Results This study included a total of 661 critically ill AKI patients who underwent CRRT for more than 72 hours. The age was 56.00 (43.00, 68.00) years, and 488 patients (73.83%) were males. The net ultrafiltration rate was 1.36 (0.94, 1.89) ml·kg^-1·h^-1. Among them, 188 patients (28.44%) were in the low net ultrafiltration group, 152 patients (23.00%) were in the medium net ultrafiltration group, and 321 patients (48.56%) were in the high net ultrafiltration group. There were statistically significant differences among the three groups in terms of gender distribution (χ²=17.81, P<0.001), body mass index (H=32.37, P<0.001), urine volume 24 hours before admission (H=9.41, P=0.009), fluid overload (H=6.02, P=0.049), platelets (H=13.49, P=0.001), pro?B type natriuretic peptide (H=14.18, P<0.001), serum creatinine (H=9.66, P=0.008), lactate (H=9.83, P=0.007), AKI stage distribution (χ²=15.51, P=0.004), admission indication (P<0.001), total CRRT duration (H=8.45, P=0.015), ultrafiltration (H=456.10, P<0.001), net ultrafiltration (H=561.20, P<0.001), and vasoactive?inotropic score at 72 hours of CRRT treatment (H=10.42, P=0.005). Kaplan-Meier survival analysis showed that there were statistically significant differences in the 28-day (Log-rank test, χ2=10.89, P=0.004) and 60-day (Log-rank test, χ2=8.55, P=0.014) survival rates among the three groups in patients after CRRT. Multivariate logistic regression analysis showed age (OR=1.03, 95% CI 1.02-1.04, P<0.001), mean arterial pressure (OR=0.98, 95% CI 0.97-1.00, P=0.011), bilirubin (OR=3.02,95% CI 1.39-5.59, P=0.006), 72-hour vasoactive?inotropic score (OR=1.01, 95% CI 1.00-1.02, P=0.004), low net ultrafiltration group (medium net ultrafiltration group as a reference, OR=1.66, 95% CI 1.02-2.72, P=0.042), and high net ultrafiltration group (medium net ultrafiltration group as a reference, OR=1.78, 95% CI 1.14-2.78, P=0.011) were independent correlated factors of 28-day mortality after CRRT. Age (OR=1.02，95% CI 1.01-1.04， P<0.001), mean arterial pressure (OR=0.98， 95% CI 0.97-1.00， P=0.016), fluid overload (OR=1.10, 95% CI 1.02-1.19, P=0.012), bilirubin (OR=4.96， 95% CI 1.00-17.80， P=0.013), 72-hour vasoactive?inotropic score (OR=1.02， 95% CI 1.01-1.03， P=0.003), and high net ultrafiltration group (medium net ultrafiltration group as a reference, OR=1.91，95% CI 1.22-3.00， P=0.005) were independent correlated factors of 60-day mortality after CRRT. Conclusions During the first 72 hours of CRRT, net ultrafiltration > 1.38 ml·kg^-1·h^-1 and net ultrafiltration < 1.01 ml·kg^-1·h^-1 are associated with a higher mortality rate at 28 days or 60 days after CRRT. Net ultrafiltration of 1.01-1.38 ml·kg^-1·h^-1 may be a relatively safe range.

Objective To conduct a systematic review of reported cases of autosomal dominant tubulointerstitial kidney disease (ADTKD) caused by uromodulin (UMOD) gene mutations (ADTKD-UMOD) in China, summarize the clinical, pathological and genetic variation characteristics, and analyze the genotype-phenotype correlation. Methods It was a retrospective systematic analysis study. The search terms "UMOD", "ADTKD-UMOD", "uric acid kidney disease (UAKD)", "medullary cystic kidney disease type 2 (MCKD2)", and "familial juvenile hyperuricemic nephropathy type 1 (FJHN1)" were used, and relevant literature on ADTKD-UMOD cases in China were retrieved from PubMed, CNKI, Wanfang Data and Chinese Medical Journal Full-text Database. The data of the cases involved in the literature were collected and summarized, and the clinical and pathological characteristics and genetic variation features of Chinese ADTKD-UMOD patients were analyzed. The patients were divided into exon 3 mutation group and non-exon 3 mutation group based on the mutation sites, and the differences of clinical phenotypes between the two groups were compared. The patients were also divided into domain 8 cysteine (D8C) mutation group and non-D8C mutation group based on the mutation regions, and the differences of clinical phenotypes between the two groups were compared. Results A total of 17 relevant articles on ADTKD-UMOD cases in China were retrieved, involving 57 patients from 34 families. The age at first diagnosis was 24.0 (20.0, 39.5) years. Fifty-three patients (93.0%) had a family history of nephropathy or hyperuricemia. Among the 48 patients with recorded blood uric acid levels, 36 patients (75.0%) had hyperuricemia, with age of 24.0 (20.3, 37.3) years. Fifty-four patients had chronic kidney disease assessment records, among which 46 patients (85.2%) developed chronic kidney disease, and 21 patients (38.9%) developed end-stage renal disease. The age of end-stage renal disease was 39.0 (24.0, 46.0) years, with age of 33.0 (21.0, 46.5) years in males and 39.5 (25.5, 45.5) years in females (Z=-0.649, P=0.516). Twenty patients underwent renal biopsies, and 19/20 patients had tubular or interstitial lesions, and 9/20 patients had glomerular lesions, mainly manifested as focal segmental or global glomerulosclerosis. Forty patients had renal ultrasound examination records, among which 36 patients (90.0%) had abnormal results, with renal cysts being the most common type (12 patients, 30.0%). Among the 34 family cases, no ADTKD-UMOD hotspot mutation was found in the UMOD gene mutations. Thirty-two families (94.1%) were missense mutations, 26 families (76.5%) had mutation sites in exon 3, and 16 families (47.1%) had mutation regions in D8C. The proportion of hypertension in the non-exon 3 mutation group was higher than that in the exon 3 mutation group (χ²=9.84, P=0.002). The proportion of males in the non-D8C mutation group was higher than that in the D8C mutation group (χ²=4.97, P=0.026). Conclusions The main clinical manifestations of Chinese ADTKD-UMOD patients are hyperuricemia, and the main renal histopathological changes are tubular and interstitial lesions. Some patients have glomerular lesions, which need to be differentiated from focal segmental glomerulosclerosis. Renal cysts detected by renal ultrasound can suggest the diagnosis of the disease. Missense mutation is the main type of UMOD gene mutations. The gene mutation region may be correlated with hypertension and gender.

Objective To explore the efficacy and its related factors of rituximab (RTX) in the treatment of children with frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS). Methods It was a single-center retrospective study. The clinical data of FRNS/SDNS children first treated with RTX in the First Affiliated Hospital of Sun Yat-sen University from November 1, 2016 to September 1, 2023 were collected. The number of relapse within 1 year before and after RTX treatment, the time to first relapse after RTX treatment, and the time to B-cell reconstitution were analyzed. At the first treatment, a single dose of RTX was given at 375 mg/m², with a maximum dose of 500 mg, once a week, for 1 to 4 doses. The count of CD19⁺ lymphocytes in the peripheral blood of the children was continuously monitored. If B-cell reconstruction was performed, the decision on whether to proceed to the next course of RTX treatment was made based on clinical manifestations. Kaplan-Meier method was used to analyze relapse-free survival rate after receiving RTX. Cox proportional hazards regression model was used to analyze the related factors of relapse after RTX treatment. Results A total of 98 FRNS/SDNS children receiving RTX treatment were enrolled, including 75 males (76.5%). The age at onset was 4.0 (1.9, 7.1) years and age of receiving RTX was 11.3 (8.5, 13.5) years. There were 90 children (91.8%) achieving complete remission, while 8 patients (8.2%) did not respond to RTX treatment, and 3 patients (3.1%) progressed to end-stage kidney disease after receiving RTX. The relapse-free survival rates at 6 months and 1 year after RTX treatment were 83.3% (75/90) and 57.9% (22/38), respectively. The frequency of relapse 1 year after RTX treatment decreased compared to 1 year before RTX treatment (Z=-7.398, P<0.001). Compared with children without relapse during the period of B-cell depletion, relapsed children had a higher number of relapse within one year after RTX treatment (Z=5.246, P<0.001). The time to first relapse after RTX treatment was 8.3 (4.6, 13.9) months in 51 relapse patients. Compared with children receiving 1 dose of RTX in the first course, those receiving 2 or more doses had a longer time to the first relapse (Z=2.983, P=0.003). There was no statistically significant difference in time to the first relapse between children who received mycophenolate mofetil therapy after RTX treatment and those who didn't (P>0.05). The reconstruction time of B cells after the first course of RTX was 6.9 (5.3, 9.0) months. Compared to children receiving one dose of RTX in the first course, those receiving two or more doses had a longer B-cell reconstitution time (Z=2.739, P=0.006). There was no statistically significant difference in B-cell reconstitution time between children who received mycophenolate mofetil therapy after RTX treatment and those who didn't (P>0.05). Univariate Cox regression analysis showed that recurrence after calcineurin inhibitor (CNI) treatment before RTX treatment and the number of recurrence in one year before RTX treatment were correlated factors of recurrence after RTX treatment (both P<0.05). Multivariate Cox regression analysis showed that recurrence after CNI treatment before RTX treatment was an independent correlated factor of relapse after RTX therapy (HR=3.496, 95% CI 1.245-9.818, P=0.018). Infusion reactions occurred in 10 patients (10.2%) and infections were observed in 24 patients (24.5%) during B cell depletion. No serious adverse events occurred. Conclusions RTX is well tolerated and effective in treating FRNS/SDNS. Recurrence after CNI treatment before RTX treatment may be an independent related factor of relapse after RTX treatment.

Objective To investigate the protective effect and its mechanism of proscillaridin A (PSD?A) on podocyte injury in lupus nephritis (LN). Methods Molecular docking and surface plasmon resonance techniques were used to analyze the binding status of PSD?A to signal transducer and activator of transcription 1 (STAT1). The immortalized human podocyte injury model in the lupus group was induced by the serum of systemic lupus erythematosus patients, and the control and PSD?A intervention (2 nmol/L, 4 nmol/L) groups were also set up. Six female 12-week-old C57BL/6 mice were designated as the control group, and 12 female 12-week-old MRL/lpr lupus mice were randomly divided into lupus group and PSD?A intervention group by random number table method. The PSD?A intervention group was intraperitoneally injected with 5 mg/kg PSD?A, once per week for 6 consecutive weeks. While the control group and the lupus group were intraperitoneally injected with the same volume of the solvent without PSD?A. Western blotting and real-time quantitative PCR were employed to detect the relative protein and mRNA expression levels of podocin, STAT1, and interferon?induced protein with tetratricopeptide repeat 1 (IFIT1) in podocytes of each group. Enzyme-linked immunosorbent assay was used to detect the levels of serum anti-double strand DNA antibody and interferon-α in mice. Coomassie brilliant blue was used to detect the urinary protein level. HE, PAS, Masson and PASM staining and transmission electron microscopy were used to observe the pathological changes of renal tissues. Immunohistochemistry was used to examine the protein expression of podocin, STAT1 and IFIT1 in renal tissues. Results Molecular docking and surface plasmon resonance techniques proved that PSD?A could bind to STAT1 protein and they exhibited a robust binding affinity. The podocyte experiments showed that, compared with the lupus group, the relative expression levels of podocin protein and mRNA in the PSD?A intervention group were upregulated, while the relative expression levels of STAT1 and IFIT1 protein and mRNA were downregulated (all P<0.05). The animal experiments showed that, compared with the lupus group, the serum levels of anti-double strand DNA antibody, interferon-α, and urinary protein in PSD?A intervention group were decreased, the pathological damage of renal tissues was alleviated, and the injury of renal podocytes was reduced. Immunohistochemical staining showed that the relative protein expression levels of STAT1 and IFIT1 of renal tissues in the PSD?A intervention group were lower than those in the lupus group (all P<0.05). Conclusion PSD?A can play a protective role in podocyte injury in LN, and its mechanism may be related to the inhibition of the STAT1 signaling pathway.

Pheochromocytoma is a neuroendocrine tumor that produces catecholamines, leading to elevated blood pressure and metabolic changes in patients. It can result in serious complications affecting the heart, brain, kidneys, and blood vessels, potentially becoming a primary cause of death. Most pheochromocytoma patients present with atypical symptoms, making misdiagnosis or missed diagnosis common. This article reports a case of pheochromocytoma crisis misdiagnosed as severe viral myocarditis and includes a review of the relevant literature.

The paper reports a 32-year-old female acute myeloid leukemia patient who developed graft-versus-host disease after paternal hematopoietic stem cell transplantation, which subsequently led to renal thrombotic microangiopathy. She subsequently required a kidney transplant from the same donor 5 years later due to renal failure. Considering that both the bone marrow and kidney were from the same donor and the recovery of renal function was favorable, immunosuppressive therapy was discontinued after a short course of anti-rejection treatment, with maintained stable kidney function. This case suggests that under the condition of high chimerism, allogeneic hematopoietic stem cell transplantation and kidney transplantation from the same donor can achieve immune tolerance, potentially improving solid organ transplantation success rate. The findings provide a novel therapeutic approach for solid organ transplantation following allogeneic hematopoietic stem cell transplantation.

This article reports a rare case of thrombotic microangiopathy (TMA) with renal involvement complicated by allogeneic hematopoietic stem cell transplantation (allo?HSCT). The patient appeared increased serum creatinine 20 d after allo?HSCT, and gradually appeared hypertension, oliguria and edema. Despite discontinuing suspected medications, serum creatinine level did not decrease. Treatment with basiliximab and mycophenolate mofetil was initiated to prevent rejection, leading to gradual normalization of urine output and serum creatinine level. However, after stopping mycophenolate mofetil, the patient experienced recurrent increased blood pressure and decreased pulse oximetry, responding well to prednisone but recurring upon cessation, with gradually increased serum creatinine level. Renal pathology indicated that chronic TMA after allo?HSCT caused renal injury, primarily affecting the glomeruli. The renal function achieved long-term stability through low-dose prednisone and symptomatic treatment. By reviewing relevant literature, we discussed the clinical manifestations, laboratory tests, pathological features and treatment strategies of TMA with renal involvement complicated by allo?HSCT.

Protein-energy wasting and loss of muscle mass are prevalent in chronic kidney disease (CKD) patients and are strongly associated with adverse clinical outcomes, necessitating the development of simple and efficient assessment tools. Cystatin C, a cathepsin protease inhibitor, is produced consistently by nucleated cells and exhibits less dependence on muscle mass compared to creatinine. Emerging evidence suggests that combining serum cystatin C with serum creatinine enhances the evaluation of muscle mass and strength in CKD patients, while also demonstrating significant clinical prognostic utility. This article synthesizes current knowledge on biological characteristics and metabolic pathways of cystatin C, recent advances on its combined use with serum creatinine for assessing muscle mass, and its predictive value for clinical prognosis in CKD. The review aims to inform future research directions and clinical applications.

Diabetic kidney disease (DKD) is one of most common microvascular complications of diabetes mellitus. A subset of patients with DKD may experience rapid disease progression, ultimately developing end-stage renal disease. The timing of hemodialysis initiation is a critical influencing factor of the survival outcomes in end-stage renal disease patients undergoing dialysis. However, a unified standard for the optimal timing of hemodialysis initiation in DKD patients is currently lacking, and the issue remains controversial in clinical practice. This article reviews research progress on the timing of hemodialysis in patients with DKD, covering aspects such as the current status of hemodialysis treatment, advances in research on hemodialysis timing, relevant guidelines, intentional delayed dialysis strategies, and evaluation criteria for dialysis timing to provide a reference for scientifically developing hemodialysis initiation plans and strategies in clinical practice.

IgA nephropathy (IgAN) is a glomerulonephritis characterized by diffuse deposition of immune complexes mainly composed of IgA in the mesangial area of the glomerulus. However, some patients show monotypic IgA deposits in the immunofluorescence examination, and its clinicopathological significance is not yet clear. The renal pathological changes of IgAN with monotypic IgA deposition are similar to those of proliferative glomerulonephritis with monoclonal IgA deposit (IgA-PGNMID), which has a risk of progressing to hematological malignancies and a worse clinical prognosis. It is necessary to differentiate them based on clinical pathological manifestations and hematological examinations. Based on previous literature reports and the research results of our research group, this review summarizes and analyzes the mechanism, clinical and pathological characteristics, and prognosis of IgAN with monotypic IgA deposition, and the relationship between IgAN with monotypic IgA deposition and IgA-PGNMID, to improve clinical doctors' understanding of IgAN with monotypic IgA deposition, reduce missed diagnosis and misdiagnosis, and improve patients' prognosis.

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.