KEY POINTS:Among 72 patients with childhood-onset ANCA-associated vasculitis, kidney transplant survival was good (86%). ANCA-associated vasculitis relapse occurred in 8 patients (11%), a median of 71 months after transplantation and resulted in graft failure in only one case. Positive ANCA at the time of transplantation did not predict graft failure but was associated with a higher risk of relapse and worse graft function. BACKGROUND:ANCA-associated vasculitis (AAV) is rare in children, and results in kidney failure in up to one third of cases. There is very limited knowledge on kidney transplantation in childhood-onset AAV. We assessed kidney transplantation outcomes and prognostic factors in a multicenter cohort of patients with childhood-onset AAV. METHODS:Patients diagnosed with AAV during childhood (≤18 years) who received a kidney transplant were included in this retrospective study. We determined patient and graft survival, rates of chronic graft dysfunction (defined as eGFR <60 ml/min per 1.73 m 2 for ≥3 months) and AAV relapse, and assessed determinants of outcome with logistic regression models. Patients were matched 1:2 for age, sex, and era of transplantation with non-AAV recipients from the Hospital for Sick Children in Toronto, Canada, and their graft survival was compared. RESULTS:We included 72 patients, of whom 53 (74%) had microscopic polyangiitis and 19 (26%) granulomatosis with polyangiitis. Their median age (interquartile range at the time of diagnosis and transplantation was 12 (9-14) and 14 (12-16) years, respectively. After a median post-transplant follow-up of 53 months (interquartile range, 25-97), 70 patients (97%) were alive, 62 (86%) had a functioning graft, 28 (39%) had developed chronic graft dysfunction, and 8 (11%) had experienced AAV relapse. Graft survival was comparable between AAV and non-AAV recipients. Acute rejection was the only independent predictor of graft failure (hazard ratio [HR], 12.11; 95% confidence interval [CI], 1.19 to 122.49). Positive ANCA at the time of transplantation was significantly associated with a chronic graft dysfunction (HR, 4.16; 95% CI, 1.71 to 10.13) and AAV relapse (HR, 23.1; 95% CI, 2.67 to 200.28). CONCLUSIONS:Patients with childhood-onset AAV show good overall and graft survival after kidney transplantation and a low rate of post-transplant relapse. Further studies are warranted to confirm whether positive ANCA at the time of transplantation is associated with poorer graft outcomes.

Key Points Among 72 patients with childhood-onset ANCA-associated vasculitis, kidney transplant survival was good (86%). ANCA-associated vasculitis relapse occurred in 8 patients (11%), a median of 71 months after transplantation and resulted in graft failure in only one case. Positive ANCA at the time of transplantation did not predict graft failure but was associated with a higher risk of relapse and worse graft function. Background ANCA-associated vasculitis (AAV) is rare in children, and results in kidney failure in up to one third of cases. There is very limited knowledge on kidney transplantation in childhood-onset AAV. We assessed kidney transplantation outcomes and prognostic factors in a multicenter cohort of patients with childhood-onset AAV. Methods Patients diagnosed with AAV during childhood (≤18 years) who received a kidney transplant were included in this retrospective study. We determined patient and graft survival, rates of chronic graft dysfunction (defined as eGFR <60 ml/min per 1.73 m 2 for ≥3 months) and AAV relapse, and assessed determinants of outcome with logistic regression models. Patients were matched 1:2 for age, sex, and era of transplantation with non-AAV recipients from the Hospital for Sick Children in Toronto, Canada, and their graft survival was compared. Results We included 72 patients, of whom 53 (74%) had microscopic polyangiitis and 19 (26%) granulomatosis with polyangiitis. Their median age (interquartile range at the time of diagnosis and transplantation was 12 (9–14) and 14 (12–16) years, respectively. After a median post-transplant follow-up of 53 months (interquartile range, 25–97), 70 patients (97%) were alive, 62 (86%) had a functioning graft, 28 (39%) had developed chronic graft dysfunction, and 8 (11%) had experienced AAV relapse. Graft survival was comparable between AAV and non-AAV recipients. Acute rejection was the only independent predictor of graft failure (hazard ratio [HR], 12.11; 95% confidence interval [CI], 1.19 to 122.49). Positive ANCA at the time of transplantation was significantly associated with a chronic graft dysfunction (HR, 4.16; 95% CI, 1.71 to 10.13) and AAV relapse (HR, 23.1; 95% CI, 2.67 to 200.28). Conclusions Patients with childhood-onset AAV show good overall and graft survival after kidney transplantation and a low rate of post-transplant relapse. Further studies are warranted to confirm whether positive ANCA at the time of transplantation is associated with poorer graft outcomes.

Anti-glomerular basement membrane (anti-GBM) disease is a rare, aggressive autoimmune disorder that seldom coexists with IgA nephropathy (IgAN). The development of anti-GBM disease secondary to IgAN in pediatric patients—specifically with confirmed seroconversion—is exceptionally rare. We present a case of a 14-year-old boy who developed anti-GBM disease shortly after an initial diagnosis of IgAN. A 14-year-old male presented with a 10-day history of gross hematuria. Initial evaluation showed normal renal function and negative anti-GBM antibodies. The initial renal biopsy confirmed IgAN (Lee grade V; Oxford classification M1E0S1T0-C2). Despite receiving continuous Nefecon monotherapy (16 mg/day) combined with irbesartan 150 mg/day, his renal function deteriorated rapidly four months later, with serum creatinine (Scr) surging from 65 to a peak of 665 µmol/L. Repeat serological testing revealed a significant seroconversion of anti-GBM antibodies from negative to strongly positive (> 8.0 AI). A second renal biopsy demonstrated devastating progression, with 100

Chronic Kidney Disease-Mineral and Bone Disorder (CKD-MBD) in children refers to the systemic mineral and bone metabolism disorders caused by CKD, including biochemical abnormalities, abnormalities in bone turnover, mineralization, quality, and ectopic calcification. Like the adult patients, mineral metabolism and bone structure abnormalities are universally present in children with CKD. But there have been no guidelines specifically for the children in the past. The medication treatment and clinical practices for children are usually based on those for adults, lacking targeted guidance. On the one hand, the children are in the process of growth and development, and the adult guidelines do not take into account the characteristics of children. On the other hand, the medication range for children is different from that of adults, and new drugs have been verified and tested in children. Hence, based on the developmental characteristics of children, combined with expert opinions from adults, children, kidney, nutrition, and medicine, this guideline was initiated by the Subspecialty Group of Nephrology, the Society of Pediatrics, Chinese Medical Association. It answered 15 important clinical questions related to the diagnosis and treatment of pediatric CKD-MBD, aimed to provide individualized treatment plans considering the clinical characteristics of CKD children and their treatment goals, while also addressing the needs of their growth and development.

Chronic Kidney Disease-Mineral and Bone Disorder (CKD-MBD) in children refers to the systemic mineral and bone metabolism disorders caused by CKD, including biochemical abnormalities, abnormalities in bone turnover, mineralization, quality, and ectopic calcification. Like adult patients, mineral metabolism and bone structure abnormalities are universally present in children with CKD. However, there have been no guidelines specifically for children in the past. The medication treatment and clinical practices for children are usually based on those for adults, thus lacking targeted guidance. Children are in the process of growth and development, and adult guidelines do not take into account the characteristics of children. In addition, the medication range for children is different from that of adults, and new drugs have been verified and tested in children. Therefore, based on the developmental characteristics of children, combined with expert opinions about adults, children, kidney, nutrition, and medicine, this guideline was initiated by the Subspecialty Group of Nephrology, the Society of Pediatrics, Chinese Medical Association. It answers many important clinical questions related to the diagnosis and treatment of pediatric CKD-MBD, aims to provide individualized treatment plans considering the clinical characteristics of children with CKD and their treatment goals, while addressing the needs of their growth and development.

Growth disorders are one of the common complications of chronic kidney disease (CKD) in children, adversely affecting both the quality of life and survival time of CKD patients. Recombinant human growth hormone (rhGH) is an effective treatment for growth disorders in children with CKD. This article reviews the mechanisms underlying growth disorders in children with CKD, the therapeutic effects, safety, and precautions of rhGH, and long-term management of diagnosis and treatment of this disorder.

m6A regulators were demonstrated to modulate the functions of intestinal epithelial and immune cells in the ulcerative colitis. This study aimed to elucidate whether and how the m6A reader heterogeneous nuclear ribonucleoprotein C (HNRNPC) regulates macrophage function in the colitis. We observed elevated HNRNPC in the inflammatory Raw264.7 cells and macrophages in the dextran sodium sulfate (DSS)-induced colitis. Knocking down HNRNPC can mitigate LPS-induced activation of macrophages in vitro. Furthermore, adoptive transfer of macrophages with HNRNPC knockdown significantly alleviated colitis compared to those transfected with negative control siRNA. Additionally, RNA sequencing illuminated that HNRNPC regulated functions of macrophages by inhibiting alternative mRNA slicing, involving adjusting acute inflammatory response, and promoting cell chemotaxis and migration. Besides, HNRNPC can govern the stability of Itgb7, and Itgb7 might be an effective target for HNRNPC in macrophages. Our findings highlight the crucial role and therapeutic potential of HNRNPC inhibition in macrophages in alleviating colitis.

Dent disease (DD) is a rare monogenic, X-linked recessive disorder characterized by proximal renal tubular dysfunction. The hallmark features include low-molecular-weight proteinuria (LMWP), hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive renal failure. A notable feature of Dent disease is the progressive decline in renal function, with 30% to 80% of male patients advancing to end-stage renal disease (ESRD) between the ages of 30 and 50. However, there is limited research detailing the progression of chronic kidney disease (CKD) in pediatric Dent disease patients. To date, European and Chinese cohorts have reported 44 and 4 cases, respectively, of childhood CKD associated with Dent disease. Though these cases lack detailed descriptions. In this study, we retrospectively analyzed the clinical characteristics, genetic variant spectrum, and follow-up data of 25 children with Dent disease. The aim was to explore the causes of CKD occurring during childhood in Dent disease and to raise awareness among pediatricians about this condition. We conducted a retrospective analysis of 25 patients diagnosed with Dent disease between 2012 and 2024 at the Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology. The study analyzed the genetic backgrounds, clinical phenotypes, and laboratory findings of these 25 unrelated patients. All pediatric patients in this cohort were male. Of the 25 patients, 19 (76.0%) were diagnosed with DD1, while 4 (16.0%) were diagnosed with DD2. The specific type of 2 patients (8.0%) could not be determined and were classified as Dent-not identified (Dent-NI). Low-molecular-weight proteinuria was present in all patients. There was no difference in clinical manifestations between DD1 patients and DD2 patients. All 25 patients underwent genetic testing. Variants in CLCN5 were detected in 19 patients, and OCRL variants were identified in 4 patients. No pathogenic variants were detected in the remaining 2 patients. For CLCN5, most variants were missense variants (31.6%), followed by frameshift (26.3%), nonsense (26.3%), deletion (10.5%) and splicing mutation (5.3%). Of these, 13 variants were novel. For OCRL, detected variants included missense variants (75.0%) and frame shift (25.0%), with 3 being previously unreported. The median follow-up duration was 4.7 ± 3.02 years and the median age of patients at the last follow-up was 9 ± 4.91 years. During follow-up, 7 patients progressed to CKD, with 4 in CKD stage II, 2 in stage III, and 1 in stage V, all these CKD patients had a history of acute kidney injury (AKI). Among DD1 patients, clinical phenotypes and genotypes were compared between those who developed chronic renal failure and those who did not. Significant differences were noted in the incidence of hypercalciuria (37.5% vs 100%, P = 0.031), nephrolithiasis (37.5% vs 100%, P = 0.031) and acute kidney injury (12.5% vs 83.33%, P = 0.026) between the two groups. Our study expands the genetic spectrum of Dent disease and highlights the potential for CKD progression during childhood. Nephrolithiasis and AKI are critical risk factors for CKD progression, emphasizing the need for early diagnosis and proactive management. Further research on genotype-phenotype correlations and the development of targeted therapies is essential to improving outcomes for patients with Dent disease.

The efficacy and safety of unfractionated heparin (UFH) and regional citrate anticoagulation (RCA) in continuous renal replacement therapy (CRRT) are subjects of controversy across paediatric patients. We conducted a search of the PubMed, Embase and Cochrane Library databases from inception to 2024. The Newcastle-Ottawa Scale was used to assess the quality of the included studies. Sensitivity analysis confirmed the stability of results; publication bias was investigated by Egger test. We conducted a search of the PubMed, Embase and Cochrane Library databases from inception to 2024, and 12 studies were included. Compared with UFH, RCA significantly prolonged circuit survival time (WMD 12.09, 95% CI: [4.48, 19.71], p = 0.002), reduced the risk of filter clotting (RR = 0.60, 95% CI: [0.42-0.85], p = 0.004) and bleeding (RR = 0.42, 95% CI: [0.23-0.79], p = 0.007). Although citrate anticoagulation was more likely to cause metabolic alkalosis (RR = 3.22, 95% CI: [1.34-7.75], p = 0.009) and hypocalcemia (RR = 2.92, 95% CI: [1.93-4.41], p < 0.001), metabolic complications were manageable and mild. Further subgroup analysis of paediatrics showed that, compared with children with an average weight > 10 kg, citrate anticoagulation significantly extended the circuit survival time of children with an average weight ≤ 10 kg (p < 0.001). Sensitivity analysis confirmed that the results were generally robust. RCA could be the first choice for anticoagulation in paediatrics, especially for infants weighing ≤ 10 kg.

Children with refractory nephrotic syndrome (NS) often experience frequent relapses despite combination immunosuppressive therapy. This study evaluated the efficacy and safety of scheduled rituximab (RTX) maintenance therapy in children with refractory NS who failed multi-target therapy. We retrospectively analyzed 48 children under 18 years old with steroid-dependent or steroid-resistant NS who had ≥ 2 relapses within 6 months despite corticosteroids plus at least two other immunosuppressants (multi-target therapy failure). Relapse rates before and after RTX, medication reduction, adverse events, and kidney outcomes were assessed. Of 51 patients treated, 48 met inclusion criteria (three excluded for severe infusion reactions or loss to follow-up). RTX maintenance therapy significantly reduced the annual relapse rate from a mean of 2.1 relapses/year before RTX to 0.2 relapses/year after RTX (p < 0.0001). Over half of patients (56

Importance:Both tacrolimus (TAC) and mycophenolate mofetil (MMF) are recommended for children with frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). However, their comparative effectiveness and safety have not been evaluated through randomized clinical trials. Objective:To compare the effectiveness and safety of TAC and MMF in children with FRNS or SDNS. Design, Setting, and Participants:In this multicenter, open-label randomized clinical trial conducted at 12 pediatric nephrology centers across China, 270 children aged 2 to 18 years with FRNS or SDNS were allocated at a 1:1 ratio to treatment with either TAC or MMF. The study was conducted from November 2019 to July 2023, and data analysis was completed from July 2023 to March 2024. Intervention:Patients received either TAC (0.025-0.050 mg/kg, orally twice daily) or MMF (10-15 mg/kg, orally twice daily) for 1 year, along with a tapering regimen of steroids. Main Outcomes and Measures:The primary end point was 1-year relapse-free survival. Relapse frequency, cumulative steroid dosage, and safety profiles were also evaluated. Results:A total of 292 patients from 12 care centers were assessed for eligibility, and 270 patients were randomized to receive either TAC (n = 135) or MMF (n = 135). Among 270 patients, median (IQR) age was 6.91 (4.25-9.96) years, and 70 patients (25.9%) were female. Compared with MMF, the 1-year relapse-free survival rate in the TAC group was 1.86-fold higher (hazard ratio [HR], 2.86; 95% CI, 1.79-4.76; P < .001) in the intention-to-treat analysis. This difference was also significant after adjusting for the per-protocol analysis (HR, 2.78; 95% CI, 1.72-4.55; P < .001). The mean (SD) time to first relapse was significantly longer in the TAC group (323.99 [98.33] days) compared to the MMF group (263.21 [132.84] days). Furthermore, the TAC group showed a lower annual relapse rate than the MMF group (17.78% vs 41.48%) and required a significantly lower mean (SD) cumulative steroid dose (0.22 [0.10] mg/kg/day vs 0.34 [0.22] mg/kg/day). The safety profile was similar in both groups. Conclusions and Relevance:In this randomized clinical trial, compared with MMF, a 1-year course of TAC therapy significantly extended the period of relapse-free survival in children with FRNS or SDNS. Trial Registration:ClinicalTrials.gov Identifier: NCT04048161.

Hemodialysis, a common renal replacement treatment for end-stage kidney disease, exacerbates thrombosis and oxidative stress by exposing the blood to low-biocompatibility dialysis membranes. Here, we developed a functionalized hemodialysis membrane with excellent anticoagulant and antioxidant properties, and simultaneously explored the mechanisms of antioxidation and anticoagulation of gallic acid carbon dots (GA-CDs). This study utilized a traditional Chinese medicine extract, gallic acid, as the main raw material and synthesized non-metallic nitrogen-doped GA-CDs through a hydrothermal reaction with ethylenediamine. The antioxidant and anticoagulant performance of GA-CDs was evaluated by measuring reactive oxygen species(ROS) clearance rate and antioxidant enzyme expression, and macrophage procoagulation experiments and tissue factor expression respectively. Subsequently, GA-CDs were co-deposited with polydopamine and heparin-like polymer on the polysulfone (PES) membrane surface to produce functionalized membrane. Finally, the bio-compatibility of the functionalized membrane was assessed by detecting cytotoxicity, ROS clearance rate, anticoagulant effect, hemolysis rate and complement activation. One-way ANOVA was used for statistical significance analysis between multiple groups and statistical analyses were performed by GraphPad Prism 9.5 and Origin 2021. The synthesized GA-CDs possessed superior cytocompatibility and antioxidative cytoprotection, 100μg/ml GA-CDs could effectively eliminate 94.7% ROS and 37.2% O2− production, increasing the cell survival rate in the oxidative stress state from 76.9% to 90.9% (P = 0.0304). Mechanistic investigations revealed that GA-CDs predominantly activated the nuclear factor erythroid 2-related factor2 (Nrf2) signaling cascade, enhancing the expression of antioxidant proteins, most notably heme oxygenase-1 (Ho-1), to induce cytoprotective effects. After inhibiting Nrf2 expression, GA-CDs could only eliminate 39.3% of ROS production, indicating that the activated Nrf2-Ho-1 pathway is crucial for the ROS clearance ability conferred by GA-CDs. Besides, GA-CDs also upregulated the mRNA expression of the antioxidant enzymes Cat (P < 0.0001) and Sod1 (P = 0.0042), facilitating the clearance of intracellular ROS. On the other hand, the GA-CDs exerted anticoagulant effects by inhibiting the activation and up-regulation of tissue factors on the surface of endothelial cells and macrophages caused by oxidative stress. Endothelial cell tissue factors mRNA expression was upregulated after the co-culture with macrophages under oxidative stress state (P = 0.0014), and the pro-coagulation time of endothelial cells was shortened by 50.0 seconds, corroborating our hypothesis that oxidatively stressed macrophages activate endothelial cells. And when oxidatively damaged macrophages and activate endothelial cells were treated with GA-CDs, the level of tissue factors mRNA expression declined (the P-values were 0.0405 and 0.0034 respectively), and the coagulation time was prolonged by 16.6 seconds and 53.4 seconds respectively compared to that of the control group. Finally, the functional PES membrane by GA-CDs-supported heparin-like polymer exhibited excellent antioxidant and anticoagulant properties. The DPPH and ABTS+ radical scavenging rates of the modified membrane were 83% and 97%, respectively, and the APTT can reach 420 seconds. In addition, the hemolysis rate of modified membrane was also much lower than 5%, and the rate of complement C3 activation was significantly inhibited (P = 0.0002), substantially increasing the biocompatibility of the blood dialysis membrane. We developed a highly biocompatible hemodialysis membrane with superior anticoagulant and antioxidant capabilities by surface modification using a GA-CDs-supported heparin-like polymer, offering considerable potential for clinical blood purification applications.

IgA nephropathy (IgAN) is a common glomerular disease in children that may progress to chronic kidney disease (CKD) and kidney failure. Identifying reliable prognostic markers is important for guiding clinical management. This study investigated independent predictors of poor prognosis in pediatric IgAN and their impact on eGFR slope, as well as the dynamic patterns of complete kidney remission and relapse, predictive factors associated with remission, and the influence of remission on poor prognosis. A retrospective cohort study of 224 children (aged 3–18 years) with biopsy-proven IgAN and ≥ 3 years of follow-up was enrolled. Poor prognosis was defined as persistent eGFR < 90 mL/min/1.73 m2 for ≥ 3 months. Multivariate logistic regression, receiver operating characteristic (ROC) curve analysis, and linear mixed-effects model were used to identify predictive factors and evaluate eGFR slope. Complete kidney remission was defined as the absence of hematuria and proteinuria with eGFR ≥ 90 mL/min/1.73 m2 for ≥ 1 year. Cumulative incidence function and Fine-Gray competing risk regression model were applied to analyze remission dynamic patterns. Over a median follow-up of 5.41 years, 12.05

The hemodialysis membrane is crucial for maintaining the lives of patients with end-stage renal disease. However, during use, the membrane is prone to issues such as clotting and oxidative stress. Of particular concern is the relatively low removal efficiency of the most destructive reactive oxygen species, the hydroxyl radical (center dot OH). In this study, the in situ growth of Prussian blue nanoperoxidase on polysulfone membranes was used to enhance the anticoagulation and antioxidant performance of the membranes. These functionalized membranes demonstrated clearance capacity of 54%, 77%, and 52% for 1,1-diphenyl-2-trinitrophenylhydrazine radicals, 2,2'azinobis(3-ethylbenzothiazoline-6-sulfonic acid ammonium salt), and hydrogen peroxide, respectively. Specifically, the clearance capacity for center dot OH was 83% (2.82 mu mol/cm2), which is the second highest value reported for membranes. Furthermore, the activated partial thromboplastin time reached 95 s. The antioxidant properties of the modified membrane remained stable after 12 h of cleaning.

BackgroundLupus nephritis (LN) is one of the most common secondary glomerulonephritis in children, and may progress to end-stage kidney disease if remission induction is not successful. Currently, the recommended induction treatment for lupus nephritis class Ⅲ/Ⅳ/V are corticosteroids combined with cyclophosphamide (CYC). Although tacrolimus has demonstrated comparable efficacy to CYC in adult patients with LN, its effectiveness and safety in the treatment of childhood lupus nephritis (cLN) have not been extensively investigated, particularly in comparison to cyclophosphamide (CYC). The objective of this study is to retrospectively assess the effect of calcineurin inhibitors (CNIs) compared to CYC as the initial induction therapy for class Ⅲ/Ⅳ/Ⅴ cLN.MethodscLN of class Ⅲ/Ⅳ/V treated initially with intravenous CYC or CNIs from January 2009 to January 2022 were included. The collected clinical, pathological, and treatment data were analyzed to assess and compare the efficacy and safety of CNIs versus CYC.ResultsA total of 75 LN patients were eligible. 46 patients received corticosteroids combined with intravenous CYC for induction remission, while 29 patients with oral CNIs, mainly tacrolimus. The CNI group had a significantly higher complete remission (CR) rate (55.2%) compared to the CYC group (17.4%) after 3 months of treatment, and this difference remained at 6 months (62.1% vs 26.1%). The reduction of proteinuria was also more significant in the CNI group compared to the CYC group at 3-month induction. The overall incidence of adverse effects was significantly lower in CNI than in CYC (17% vs 63%, p = 0.032). Multiple logistic regression analysis identified edema, anti-dsDNA, and CYC as risk factors for non-remission after 6-month induction therapy.ConclusionsThis study demonstrates that CNIs are safer and more effective than CYC for quickly reducing proteinuria and achieving higher CR rate in initial induction treatment for lupus nephritis class III/IV/V.Clinical Trial RegistrationChiCTR2300075587.

A prominent feature of Dent disease (DD) is the progressive decline in renal function, with 30

Background:Childhood IgA nephropathy (IgAN) and IgA Vasculitis-associated nephritis (IgAVN) are same in pathogenesis and still a challenge to pediatricians. Purpose:To evaluate the efficacy and safety of Telitacicept in pediatric patients with IgAN or IgAVN. Patients and Methods:This was a retrospective single-centre study of pediatric IgAN or IgAVN with urine protein-to-creatinine ratio (UPCR) over 500 μg/mg who were treated with subcutaneous administration of Telitacicept and followed for at least 6 months. The effects on induction of remission and side effects were evaluated. Results:13 pediatric patients including 7 with IgAN and 6 with IgAVN were enrolled in the study. After 6-month treatment, 6 of 13 (46.15%) patients achieved remission, 4 (30.77%) patient achieved complete remission. The median percent reduction in UPCR was 90.74% (84.38%, 94.76%). The median percent reduction in urine red blood cells count was 96.21% (75.33%, 98.99%). Compared with baseline, serum albumin increased significantly [P < 0.001, 95% CI (-10.30, -4.04)], and estimated glomerular filtration rate (eGFR) remained stable and within the normal range. Significant reductions were observed in levels of IgA [P < 0.001; 95% CI (1.15, 2.49)], IgM [P = 0.002; 95% CI (0.53, 1.08)], as well as CD19+ lymphocytes [P = 0.012; 95% CI (6.63, 34.67)]. The average follow-up was 6.5 ± 0.8 months, 5 patients achieved glucocorticoid discontinuation. No severe adverse events were observed. Conclusion:Telitacicept was safe in pediatric IgAN/IgAVN patients and could significantly induce renal remission through reducing proteinuria and hematuria.