Objective To evaluate the efficacy and safety of daratumumab in treating patients with monoclonal immunoglobulin deposition disease (MIDD) with renal injury. Methods A case-series analysis study was conducted in MIDD patients with renal injury who received daratumumab treatment at the Department of Nephrology, Ruijin Hospital, affiliated to Shanghai Jiao Tong University School of Medicine, from December 2021 to October 2023. The clinical data of patients at the time of diagnosis and during the follow-up period were collected. Hematological and renal responses were assessed and adverse reaction events were recorded. Results Seven patients diagnosed with MIDD were included in this study, with a male-to-female ratio of 5∶2 and age of 46 (43, 52) years. One patient was light?heavy chain deposition disease, and the remaining 6 patients were light chain deposition disease. Among them, 5 patients had received prior treatment (1-2 lines of treatment with the regimen of cyclophosphamide, bortezomib and dexamethasone), while 2 patients were newly treated, one of whom had already started hemodialysis at diagnosis. Prior to receiving monoclonal antibody treatment, difference of serum free light chain (dFLC) among the 7 patients was 52 (7, 295) mg/L. Excluding 1 patient on dialysis, the remaining 6 patients had 24-hour urinary protein of 1.1 (0.2, 4.7) g, serum creatinine of 178.5 (157.8, 279.8) μmol/L and estimated glomerular filtration rate of 33.9 (24.2, 41.1) ml·min^-1·（1.73 m²）^-1. The daratumumab treatment was 17 (10, 20) infusions, with treatment duration of 17 (9, 23) months and follow-up time of 24 (13, 32) months. After treatment, among 5 previously treated patients, hematological response evaluation showed that 1 patient with baseline dFLC <20 mg/L and minimal residual disease negativity upon re-examination, while the remaining 4 patients achieved hematological responses of complete response or better. Renal response evaluation revealed that, except for 1 patient with partial response, the other 4 patients achieved very good partial response (VGPR) or better. Among 2 newly diagnosed patients, both achieved hematological efficacy at least VGPR, with one achieving renal complete response, while the other one remaining dialysis- dependent. Overall, dFLC of 7 patients was 4.9 (2.1, 11.5) mg/L. Among 6 non-dialysis patients, 24-hour urinary protein was 0.19 (0.06, 0.42) g, serum creatinine was 153.0 (120.8, 188.0) μmol/L and estimated glomerular filtration rate was 40.4 (35.2, 57.3) ml·min^-1·(1.73 m²)^-1. No severe adverse reactions were observed during daratumumab treatment. Conclusion The application of daratumumab in the treatment of MIDD with renal injury is effective and well tolerated, achieving high-quality hematological responses, with high renal responses reaching or exceeding VGPR and improvement of renal function.

Objective To investigate the clinical features, treatment strategies and prognosis of adult thrombotic thrombocytopenic purpura (TTP) patients and improve the clinicians' understanding of TTP. Methods It was a case series analysis study. The clinical data of TTP patients admitted to ZhongDa Hospital affiliated to Southeast University from August 2013 to November 2024 were retrospectively collected. The clinical manifestations, laboratory tests, treatment methods and prognosis of TTP patients were analyzed. Kaplan-Meier method and multivariate Cox proportional hazards regression model were utilized to assess the association between rituximab treatment and survival outcomes. Results The study included 24 TTP patients, with age of (58.38±15.03) years (21 to 87 years), 14 females (58.33%) and 10 males (41.67%). The first symptoms were often neurological abnormalities (lethargy, coma, sudden glossolalia or unconsciousness (10 patients, 41.67%). Five patients (20.83%) had the quinary syndrome, including fever, microangiopathic hemolytic anemia, thrombocytopenia, renal insufficiency and neurological symptoms, and 13 patients (54.17%) had the triad syndrome, including neurological syndromes, microangiopathic hemolytic anemia and thrombocytopenia. Twenty-three patients (95.83%) had anemia. Twenty patients (83.33%) presented with neurological abnormalities, among which 10 patients died of neurological events. Renal insufficiency occurred in 14 patients (58.33%). Nine patients (37.50%) presented with large areas of skin ecchymosis. Except for 1 patient complicating with lung adenocarcinoma and 1 patients complicating with bone metastasis tumor, the other patients had no active tumors. All 24 patients had PLASMIC scores ≥ 4 points, of which 11 patients (45.83%) had PLASMIC scores ≥ 6 points. Fourteen patients (58.33%) received the treatment for plasma exchange, and 7 patients (29.17%) did not undergo plasma exchange and received component transfusion and glucocorticoids therapy with poor prognosis due to rapid disease progression, old age or severe disease. Furthermore, 3 patients (12.50%) were only treated with component transfusion and glucocorticoids therapy for economic reasons, and died shortly after hospital discharge. Eight patients received plasma exchange, glucocorticoids combined with rituximab, of which one died, four survived, and three were lost to follow-up. Finally, fifteen patients (62.50%) died, 4 patients survived, and 5 patients were lost to follow-up (still alive before hospital discharge). Kaplan-Meier survival analysis demonstrated that mortality in the rituximab group was significantly lower than that in the non-rituximab group (Log-rank test, χ²=13.185, P<0.001). Multivariate Cox proportional hazards regression analysis further confirmed that no receiving rituximab was an independent correlated factor of death (HR=10.453, 95% CI 1.309-83.436, P=0.027). Conclusions TTP usually starts with neurological symptoms, and can affect multiple systems. The patients with neurological abnormalities have a poor prognosis. The patients with TTP have a rapid disease progression and a high mortality rate. Rapid identification and timely treatment are crucial for improving the prognosis of TTP. Combining rituximab based on plasma exchange and glucocorticoids may reduce mortality of TTP patients.

Objective To investigate the serum sodium level and its fluctuation in patients with hospitalized acquired acute kidney injury (AKI) and explore their impacts on in-hospital mortality. Methods It was a single-center retrospective study. The adult patients developing hospital-acquired AKI and receiving at least twice serum sodium tests admitted to Peking University First Hospital from January 1, 2018, to December 31, 2020 were included. Dysnatremia included hyponatremia (< 135 mmol/L) and hypernatremia (>145 mmol/L). The patients were divided into hyponatremia group, normal serum sodium group and hypernatremia group, and the differences of clinical data among the three groups were compared. The fluctuation of serum sodium level was evaluated by coefficient of variation. A restricted cubic spline was applied to investigate the association between serum sodium level at AKI onset and mortality. Poisson regression analysis was used to explore the mortality risk of dysnatremia at AKI onset, dysnatremia at admission, and coefficient of variation of serum sodium, respectively. Results Among the enrolled 1 475 AKI patients, the age was 66.0 (55.0, 78.0) years, and 850 patients (57.6%) were males. The estimated glomerular filtration rate was 77.3 (50.4, 97.6) ml·min^-1·(1.73 m²)^-1. The time from admission to AKI onset was 8 (4, 15) days. The incidence of hyponatremia and hypernatremia at admission were 19.6% (289/1 475) and 2.6% (39/1 475), respectively, while the incidence at AKI onset was 24.0% (354/1 475) and 12.7% (188/1 475), respectively. There was statistically significant difference in terms of age, the initial classification distribution of AKI, serum sodium at admission, serum sodium at the occurrence of AKI, the lowest serum sodium at hospitalization, the highest serum sodium at hospitalization, the coefficient of variation of serum sodium, and the proportions of heart failure, stroke, disseminated intravascular coagulation, sepsis, acute respiratory distress syndrome, shock, prerenal causes, circle diuretics and aldosterone antagonists among hyponatremia group, normal serum sodium group and hypernatremia group (all P<0.05). The restricted cubic spline analysis showed a "U"-shaped correlation between serum sodium level at AKI onset and in-hospital mortality. Poisson regression analysis showed that after adjusting for age, gender, number of chronic comorbidities, initial classification of AKI, basal estimated glomerular filtration rate and number of acute disease state, with normal serum sodium as the reference, hyponatremia (RR=1.56, 95% CI 1.14-2.13) and hypernatremia (RR=1.71, 95% CI 1.23-2.39) at AKI onset were correlated with an increased risk of in-hospital mortality. Hyponatremia at admission was correlated with an increased risk of in-hospital mortality (RR=2.13, 95% CI 1.62-2.79), while there was no statistically significant association between hypernatremia and in-hospital mortality (RR=1.22, 95% CI 0.62-2.44). After further adjusting serum sodium levels at admission and at the occurrence of AKI, the coefficient of variation of serum sodium level was still correlated with an increased risk of in-hospital mortality (RR=1.23, 95% CI 1.14-1.33). Conclusions Dysnatremia is common in patients with hospital-acquired AKI. The serum sodium level at AKI onset is correlated with in-hospital death in a "U" shape. Dysnatremia and serum sodium fluctuation are associated with an increased risk of in-hospital mortality.

Objective To develop a predictive model for adverse pregnancy outcomes in patients with pregnancy-related acute kidney injury (Pr?AKI) using machine learning methods. Methods This study was a single-center retrospective study. Patients with Pr?AKI in the First Affiliated Hospital of Xinjiang Medical University from January 2013 to December 2020 were included. Demographic characteristics, laboratory parameters, and fetal outcomes for comparative analysis between adverse pregnancy outcome group and favorable pregnancy outcome group were collected. Adverse pregnancy outcomes were defined as the occurrence of any one or more of the following events: stillbirth, perinatal death, preterm birth (reaching 28 weeks but less than 37 weeks), and low birth weight (< 2.5 kg). Conversely, an ideal pregnancy outcome was defined as the absence of any adverse pregnancy outcome events. The dataset was randomly divided into a training set (70%) and a validation set (30%). Logistic regression, decision tree, random forest, K-nearest neighbor, support vector machine, and lightweight gradient boosting algorithms were employed on the training set to develop predictive models for adverse pregnancy outcomes in patients with Pr?AKI. Receiver operating characteristic curves were plotted, and the area under the curves (AUC) were calculated. Recall, precision, accuracy, and F1 scores were used to evaluate the predictive performance of each model. The optimal machine learning model was selected for subsequent analysis. Predictive model variables were screened and compressed by visualizing SHAP (SHapley additive exPlanations) with recursive feature regression. Furthermore, the efficacy of each model was evaluated through calibration curves and clinical decision curves. The optimal predictive model was selected for internal validation using the validation set, and data of in-hospital Pr-AKI patients (72 cases) in the hospital from January 2021 to June 2023 were collected for validation (time series validation set). Results A total of 458 pregnancies in 441 patients were included in the present analysis, among which 277 cases (60.5%) resulted in adverse pregnancy outcomes. Utilizing the training set, 21 feature variables were selected for model construction. Among the 6 models, the random forest model performed the best (AUC=0.860, recall=0.784, precision=0.813, F1-score=0.790, accuracy=0.806). With subsequent feature refinement proceeding, a total of 12 clinical indicators were selected to construct the model. Among them, proteinuria, systolic blood pressure, and the highest serum creatinine were the top three related factors, and the other related factors included: severe preeclampsia, baseline serum creatinine, serum albumin, diastolic blood pressure, aspartate aminotransferase, blood uric acid, white blood cell count, serum cystatin C, and cholesterol. Among various machine learning models, the random forest model demonstrated optimal net benefits and the widest clinical utility range, showing robust performance in both internal validation set (AUC=0.80) and the time series validation set (AUC=0.72). Conclusions In this study, different machine learning algorithms are successfully applied to develop predictive models for adverse pregnancy outcomes in patients with Pr-AKI. The random forest model is translated into a clinically applicable tool, providing a reference for the convenient and rapid identification of adverse pregnancy outcomes in Pr-AKI patients.

Objective To explore the correlation between serum nidogen-2 (NID?2) and thoracic aortic calcification in patients with chronic kidney disease (CKD), and construct a risk prediction model based on NID-2 to evaluate its value in predicting the risk of the severe thoracic aortic calcification and cardiovascular and cerebrovascular events in CKD patients. Methods It was a prospective cohort study. Patients with CKD at stage 3 to 5D in the Zhongda Hospital Affiliated to Southeast University from January 2022 to January 2023 were enrolled. Syngo.via software was used to evaluate the volume of thoracic aortic calcification, and enzyme-linked immunosorbent assay was employed to determine the level of serum NID?2. According to the volume of thoracic aortic calcification, the patients were divided into three groups: no calcification group, mild calcification group and severe calcification group. The top 25% of the patients were defined as no or mild calcification group, and the latter 75% were defined as severe calcification group. The follow-up period was one year. During the follow-up period, cardiovascular and cerebrovascular events, as well as all-cause death among the enrolled patients were recorded. Logistic regression analysis was used to screen the influencing factors of thoracic aortic calcification. Based on the results of logistic regression analysis, a nomogram prediction model was constructed. The receiver operating characteristic curve (ROC curve), calibration curve, and decision curve were employed to evaluate the discrimination, calibration and clinical practicality of the nomogram model. Results A total of 132 patients were included, with 91 males (68.94%) and age of (56.51±16.37) years. There were 60 CKD 3-5 stage patients (non-dialysis, 45.45%) and 72 CKD 5D patients (dialysis, 54.55%). Serum ND-2 levels differed significantly among healthy individuals, dialysis patients and non-dialysis patients (H=70.651, P<0.001). There was no statistically significant difference in serum NID-2 level between the no or mild calcification group and the severe calcification group in dialysis patients (Z=350.00, P=0.426). The serum NID-2 level in the severe calcification group was significantly higher than that in the no or mild calcification group in non-dialysis patients (Z=242.00, P=0.019). In non-dialysis patients, there was a statistically significant correlation between serum NID?2 level and volume of thoracic aortic calcification (r=0.40, P<0.001). In dialysis patients, there was no statistically significant correlation between serum NID?2 level and volume of each segment of thoracic aortic calcification (all P>0.05). The univariate logistic regression analysis showed that, age, hemoglobin, serum albumin, estimated glomerular filtration rate, NID?2, hypertension, type 2 diabetes mellitus and cerebral infarction were correlated factors of thoracic aortic calcification in non-dialysis patients (all P<0.05). Multivariate logistic regression analysis showed that age (OR=1.22, 95% CI 1.08-1.50, P=0.010) was an independent correlated factor of thoracic aortic calcification in non-dialysis patients. The above related variables of univariate logistic regression analysis were incorporated into a nomogram to construct a predictive model for severe vascular calcification in non-dialysis patients, yielding an AUC of 0.94 (95% CI 0.89-0.99) in ROC curve, with a sensitivity of 83% and a specificity of 95%. A nomogram model based on above variables for predicting cardiovascular and cerebrovascular events in non-dialysis patients demonstrated an AUC of 0.95 (95% CI 0.90-1.00) in ROC curve, with a sensitivity of 95% and a specificity of 87%. Conclusions In non-dialysis patients, serum NID?2 level in the severe calcification group is significantly higher than that in the no or mild calcification group. The serum NID?2 is a related factor of thoracic aortic calcification and cardiovascular and cerebrovascular events in non-dialysis patients. The nomogram prediction model constructed by combining NID?2 with age, hemoglobin, serum albumin, estimated glomerular filtration rate, hypertension, type 2 diabetes mellitus and cerebral infarction has a high predictive value for the risk of thoracic aortic calcification as well as cardiovascular and cerebrovascular events in non-dialysis patients.

This article reviews the diagnosis, therapeutic approaches, and subsequent care for a patient with a complex, long-standing history of encapsulating peritoneal sclerosis (EPS). A 40-year-old male, who had been on peritoneal dialysis (PD) for 11 years, encountered refractory peritonitis, leading to the removal of PD catheter and the subsequent diagnosis of EPS. The patient was transitioned to hemodialysis (HD) and prescribed tamoxifen to mitigate peritoneal fibrosis. After 4 months on HD, the patient underwent a kidney transplant, but acute rejection episode caused the transplanted kidney to fail 3 months postoperatively, necessitating a return to HD. Over the past 7 years, the patient has been repeatedly hospitalized due to recurrent bowel obstructions and infected abdominal fluid accumulation. A multidisciplinary approach, including anti-infective therapy, gastrointestinal intervention, nutritional support, and psychological care, has been instrumental in managing symptoms, and sustaining life. This case underscores the importance of recognizing EPS in long-term PD patients with peritonitis. While discontinuing PD, switching to HD, or receiving kidney transplantation do not halt the progression of EPS, optimized comprehensive management can extend the patient's survival.

The paper presents a case of 69-year-old male with marginal zone lymphoma (MZL) manifesting as acute kidney injury (AKI) requiring emergency hemodialysis. The patient presented with fever, fatigue and rapidly progressive renal dysfunction, and serum creatinine was 1 105 μmol/L. Emergency central venous catheterization and hemodialysis were performed. Serum immunofixation electrophoresis revealed monoclonal IgM and λ light chain positivity. Autoantibody testing showed positivity for both anti-SSA and anti-SSB antibodies. Renal biopsy demonstrated interstitial infiltration by atypical B lymphocytes with λ light chain restriction. The diagnosis of MZL was confirmed by bone marrow biopsy. Following hemodialysis and chemotherapy with bendamustine plus rituximab, renal function significantly improved and remained stable during follow-up. This case highlights that AKI may serve as the initial manifestation of lymphoma, underscoring the importance of considering hematologic malignancies in the differential diagnosis of unexplained renal failure.

A 15-year-old boy developed abdominal pain and melena two weeks after COVID-19 infection, which was followed by gross hematuria and proteinuria. Laboratory studies revealed significantly elevated inflammatory markers. Endoscopic examinations showed multiple jejunal and ileal ulcers. Renal biopsy suggested mesangial proliferative glomerulonephritis with crescent formation, necrosis of capillary loops, and mesangial IgA deposits. IgA vasculitis without purpura was considered as a possible diagnosis, and the treatment with prednisone led to remission of both gastrointestinal and renal diseases.

IgA nephropathy (IgAN) is the most common primary glomerulonephritis. Mesangial hyperplasia and deposition of IgA immune complex are typical pathological changes of IgAN. Animal model is an important tool to explore the pathogenesis, diagnosis and treatment plan, and evaluate the safety and efficacy of drugs. At present, there are many kinds of IgAN animal models, including humanized animal models and non-humanized animal models, and there are great differences in the modeling principle, method, time and pathological changes. The humanized animal model is closer to the pathogenesis of IgAN in humans and has gradually become a research hotspot. In this paper, common animal models of IgAN in detail from the aspects of modeling method and time, characteristics and significance of each model, and the research progress of anthropomorphic animal models are reviewed to provide reference and inspiration for the basic research of IgAN.

Central venous lesion represents one of the common complications affecting vascular access in hemodialysis patients, potentially compromising hemodialysis efficacy. The management of symptomatic central venous lesion remains a critical challenge in clinical practice. Current primary treatment strategies include percutaneous transluminal angioplasty and percutaneous transluminal stenting. Advances in techniques such as sharp recanalization and the mother-child platform approach, along with the development of high-pressure balloons, paclitaxel- coated balloons, and covered stents, have significantly improved procedural success rates. However, unresolved issues persist, including standardized treatment protocols, technical considerations for lesion traversal, and optimal stent selection criteria. This article comprehensively reviews the treatment principles, lesion passage techniques, treatment techniques, and recent advancements of central venous lesion.

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.