Objective To investigate the clinical and pathological features and prognosis of membranous nephropathy (MN) complicated with IgG4-related disease (IgG4?RD). Methods This was a retrospective analysis study. Patients diagnosed with MN by renal biopsy and clinically diagnosed with IgG4?RD at the First Affiliated Hospital of Zhengzhou University from January 2019 to June 2024 were selected as the study subjects, along with cases of MN complicated with IgG4-RD reported in Chinese, Japanese, and English databases up to June 2024. Patients diagnosed with primary MN (PMN) at our center from January to May 2023 were selected as the control group. Clinical, laboratory, pathological, therapeutic, and prognostic data were collected and analyzed. For each variable, only patients with complete data were included in the analysis. Complete remission or partial remission was defined as renal remission. Renal relapse was defined as 24-hour urinary protein ≥ 3.5 g with decreased serum albumin after achieving renal remission. Results A total of 42 patients with MN complicated with IgG4?RD were finally included, consisting of 6 patients from our center and 36 patients from the literature. There were 126 PMN patients. Extrarenal organ involvement was present in 83.3% (35/42) of patients with MN combined with IgG4?RD, and 19.0% (8/42) patients had involvement of three or more extrarenal organs. The most common sites of extrarenal involvement were lymph nodes and pancreas, each affecting 14 patients (33.3%). Compared with PMN patients, MN complicated with IgG4?RD patients had higher proportions of males [78.6% (33/42) vs. 58.7% (74/126), χ2=5.362, P=0.021], low serum complement C3 [55.6% (15/27) vs. 7.4% (9/122), χ2=34.493, P<0.001], complement C4 [33.3% (9/27) vs. 1.6% (2/122), χ2=28.008, P<0.001], simultaneous low complement C3 and C4 [29.6% (8/27) vs. 0.8% (1/122), χ2=27.454, P<0.001], elevated IgG [44.8% (13/29) vs. 1.7% (2/119), χ2=43.041, P<0.001], IgG4 >1.35 g/L [87.1% (27/31) vs. 4.5% (2/44), χ2=52.260, P<0.001] and ≥3 involved organs [19.0% (8/42) vs. 0(0/126), χ2=21.175, P<0.001], older age [(60.2±12.0) years vs. (47.5±12.3) years, t=5.824, P<0.001] and higher serum creatinine [107.8 (79.6, 213.2) μmol/L vs. 67.5 (53.0, 78.0) μmol/L, Z=4.664, P<0.001], and had lower serum albumin [(24.4±7.3) g/L vs. (27.3±7.0) g/L, t=-2.709, P=0.024], hemoglobin [(107.2±21.2) g/L vs. (130.1±16.2) g/L, t=-6.054, P<0.001] and proportion of serum anti?phospholipase A2 receptor antibody [12.9% (4/31) vs. 56.3% (71/126), χ2=18.822, P<0.001]. The incidence of storiform fibrosis [15.6% (5/32) vs. 0 (0/126), χ2=15.552, P<0.001], the proportions of electron-dense deposits in the tubular basement membrane (TBM) [41.7% (15/36) vs. 0 (0/126), χ2=53.004, P<0.001], glucocorticoid use [92.3% (36/39) vs. 29.4% (37/126), χ2=47.830, P<0.001], and renal relapse [19.4% (6/31) vs. 4.4% (5/114), χ2=5.801, P=0.016] in the MN with IgG4?RD group were significantly higher than those in the PMN group, and the proportions of immunosuppressant use [41.0% (16/39) vs. 86.5% (109/126), χ2=33.545, P<0.001] and rituximab use [15.4% (6/39) vs. 42.9% (54/126), χ2=9.714, P=0.002] were lower than those in the PMN group. There was no statistically significant difference in the proportion of renal remission between the two groups [79.5% (31/39) vs. 90.5% (114/126), χ2=2.424, P=0.120]. Conclusions Compared with PMN patients, patients with IgG4?RD-associated MN are older at diagnosis, have higher proportion of males, and exhibit more severe renal impairment and higher renal relapse rate, but have no obvious difference in the renal remission rate. Storiform fibrosis and electron-dense deposits along the TBM are characteristic pathological features of IgG4?RD-associated MN.
Objective To explore the prognostic value of systemic immune-inflammation index (SII) for the progression of type 2 diabetic kidney disease (DKD) to end-stage renal disease (ESRD). Methods It was a single-center retrospective study. The patients diagnosed with type 2 DKD in Guangdong Provincial People's Hospital from January 1, 2014 to January 1, 2025 and had a follow-up period of at least 3 months were enrolled. The observation endpoint was development of ESRD or initiation of renal replacement therapy with follow?up ending on January 1, 2025. According to albumin levels, the patients were divided into normal albumin group (≥35 g/L) and hypoalbuminemia group (<35 g/L), and the predictive value of SII for progression of DKD to ESRD was compared between the two albumin strata. The receiver-operating characteristic (ROC) curve was used to calculate the value of SII for predicting ESRD, and Youden index was utilized to identify the optimal cut-off value. Spearman correlation analysis was performed to examine the correlations between SII and key clinical parameters. Kaplan?Meier method was adopted to evaluate renal survival prognosis among patients stratified by different SII levels. Cox regression model was applied to assess the effect of SII on renal prognosis. Results A total of 773 patients were finally enrolled in this study, with age of 58 (50, 64) years and 524 males (67.79%). The follow-up duration was 22 (11, 42) months, and 281 patients (36.35%) reached ESRD. There were statistically significant differences between ESRD group and non-ESRD group in age, follow?up duration, proportion of hypertension, proportion of smoking history, proportion of lipid?lowering agents, proportion of antihypertensive agents, systolic blood pressure, diastolic blood pressure, hemoglobin, SII, serum creatinine, blood urea nitrogen, serum uric acid, total cholesterol, triglyceride, estimated glomerular filtration rate, total protein and albumin (all P<0.05). ROC curve analysis showed that the area under the curve (AUC) of SII for predicting ESRD was 0.655, with an optimal cut?off value of 632.64×109/L, sensitivity of 63.7% and specificity of 41.7% in all DKD patients; the AUC of SII for predicting ESRD was 0.678,with an optimal cut-off value of 834.49×109/L, sensitivity of 40.7%, and specificity of 87.8% in DKD patients with normal albumin; and the AUC of SII for predicting ESRD was 0.615, with an optimal cut-off value of 1 155.00×109/L, sensitivity of 24.3%, and specificity of 97.0% in DKD patients with low albumin. Correlation analysis showed that SII was positively correlated with diastolic blood pressure, serum creatinine and blood urea nitrogen, and negatively correlated with hemoglobin and estimated glomerular filtration rate among patients with different albumin levels (all P<0.05). Kaplan?Meier analysis revealed that elevated SII (SII≥834.49×109/L, SII≥1 155.00×109/L) was significantly correlated with adverse renal outcomes in normal albumin group and low albumin group, respectively (Log?rank test, χ2=43.964, P<0.001; χ2=36.988, P<0.001). Multivariate Cox regression analysis demonstrated that SII ≥834.49×109/L was an independent correlated factor for the progression of DKD to ESRD in normal albumin group [hazard ratio (HR)=1.674, 95% confidence interval (CI) 1.035-2.078, P=0.036], and SII≥1 155.00×109/L was an independent correlated factor for the progression of DKD to ESRD in the low albumin group (HR=2.976, 95% CI 1.985-4.462, P<0.001). Conclusions SII has a certain predictive value for the progression of type 2 DKD to ESRD. SII≥834.49×109/L is an independent risk factor for the progression of DKD to ESRD in type 2 DKD patients with normal albumin levels, while SII≥1 155.00×109/L is an independent risk factor for the progression of DKD to ESRD in patients with hypoalbuminemia.
Objective To explore the correlation between the triglyceride-glucose index (TyG) and the prevalence of chronic kidney disease (CKD) in elderly people aged ≥60 years and to analyze the mediating factors. Methods This study was a cross-sectional study. General information and laboratory test results from physical examinations of elderly people aged ≥60 years in Meilong Town, Minhang District, Shanghai in 2024 were collected. CKD was diagnosed if the estimated glomerular filtration rate was <60 ml·min-1·(1.73 m2)-1 or the urinary albumin-to- creatinine ratio was ≥30 mg/g. TyG was calculated using the formula TyG = ln[serum triglycerides (mg/dl) × serum fasting blood glucose (mg/dl)/2]. Multivariate logistic regression was used to analyze the correlation between TyG and the prevalence of CKD, and the percentile Bootstrap method was applied to analyze the mediating factors. Results A total of 19 372 elderly people aged ≥60 years were included in this study, with age of (71.02±6.17) years, including 8 664 males (44.72%). A total of 5 906 people were diagnosed with CKD, with a detection rate of 30.49%. Multivariate logistic regression analysis showed that after adjusting for various factors such as age, gender, history of diabetes, history of hypertension, blood lipids, and novel obesity indicators, continuous variable TyG was correlated with the prevalence of CKD in elderly people aged ≥60 years, with an odds ratio (OR) of 1.458 (95% confidence interval 1.350-1.575, P<0.001). TyG was significantly correlated with the prevalence of CKD in subgroups such as male/female, diabetic/non-diabetic, and hypertensive/non-hypertensive. Restricted cubic spline analysis indicated that the continuous variable TyG was non-linearly correlated with the prevalence of CKD in elderly people aged ≥60 years (χ2=5.940, df=1, P for overall=0.004, P for nonlinear=0.015). When TyG was >8.744, odds of CKD was increased. The mediation analysis revealed that systolic blood pressure, history of diabetes, high-density lipoprotein cholesterol, and overweight or obesity partially mediated the correlation between TyG and CKD. The mediation effect proportions were 10.258%, 9.173%, 6.963%, and 4.939% respectively; non-high-density lipoprotein cholesterol had a masking effect. Conclusions TyG index is correlated with the prevalence of CKD in the community population aged ≥60 years. Interventions targeting insulin resistance, diabetes, hypertension, lipid metabolism disorders, overweight or obesity, and other factors may help control the occurrence and development of CKD in this population.
Objective To explore the value of preoperative red blood cell distribution width-to-albumin ratio (RAR) for predicting acute kidney injury (AKI) after cardiac surgery with cardiopulmonary bypass (CPB). Methods It was a retrospective cohort study. Clinical data of patients undergoing cardiac surgery with CPB at the Heart Center of Henan Provincial People's Hospital from June to November 2024 were collected retrospectively, and RAR was calculated. According to the occurrence of AKI within 7 days postoperatively, the patients were divided into AKI and non-AKI groups, and baseline clinical data between the two groups were compared. Multivariate Logistic regression analysis was used to identify independent factors associated with AKI after cardiac surgery with CPB. The area under the receiver-operating characteristic curve (AUC) was used to evaluate the predictive performance of RAR and related indicators. Results A total of 543 patients were enrolled in this study, including 191 females (35.2%), with age of 56.00 (45.00, 62.00) years and RAR of 3.28 (3.06, 3.59). Postoperative AKI occurred in 175 patients (32.2%). Multivariate Logistic regression analysis showed that elevated RAR [odds ratio (OR)=6.472, 95% confidence interval (CI)3.596-11.650, P<0.001], increased age (OR=1.040, 95% CI 1.017-1.063, P<0.001), increased body mass index (BMI) (OR=1.199, 95% CI 1.122-1.281, P<0.001), elevated blood glucose (OR=1.210, 95% CI 1.033-1.417, P=0.018), elevated uric acid (OR=1.003, 95% CI 1.000-1.006, P=0.037), prolonged CPB duration (OR=1.007, 95% CI 1.003-1.011, P<0.001) and decreased lymphocyte count (OR=0.475, 95% CI 0.308-0.733, P<0.001) were independent factors associated with AKI after cardiac surgery with CPB. The receiver-operating characteristic curve analysis showed that RAR predicted AKI with an AUC of 0.711 (95% CI 0.663-0.759, P<0.001), and the optimal cut-off value was 3.20, with a sensitivity of 78.9% and a specificity of 51.9%. The combined model incorporating RAR, age, body mass index, blood glucose, lymphocyte count, uric acid, and CPB duration yielded an AUC of 0.843 (95% CI 0.808-0.878, P<0.001), with a sensitivity of 80.7% and a specificity of 78.8%, while the model without RAR yielded an AUC of 0.806 (95% CI 0.768-0.844, P<0.001), with a sensitivity of 85.4% and a specificity of 63.1%. DeLong test showed that the AUC of the combined model with RAR was significantly higher than that without RAR (Z=3.131, P=0.002). According to the optimal cut-off value of RAR, patients were divided into high (RAR>3.20) and low RAR (RAR≤3.20) groups. The high RAR group had a significantly higher incidence of postoperative AKI compared with the low RAR group [43.69% (135/309) vs. 17.09% (40/234), χ2=43.122, P<0.001]. Conclusion Preoperative high RAR is an independent factor associated with AKI after cardiac surgery with CPB, and RAR has a certain predictive value for postoperative AKI.
Objective To evaluate the efficacy and safety of telitacicept in the treatment of pediatric IgA vasculitis nephritis (IgAVN). Methods This was a single-center, retrospective, and case series study. The clinical data of pediatric patients with refractory IgAVN who received telitacicept at the Department of Nephrology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, between July 2023 and July 2024 were enrolled. All patients received renin? angiotensin system inhibitors combined with glucocorticoids and telitacicept, with a 24-week follow-up. Changes of urine protein-to-creatinine ratio (UPCR), urinary red blood cell count, estimated glomerular filtration rate (eGFR), peripheral blood lymphocyte subsets and glucocorticoid dosage were compared before and after treatment, and adverse events were recorded. Results Five patients (3 males, 2 females) with age of 9.8 (6.5, 16.6) years were enrolled. After 24 weeks, proteinuria remission was achieved in all five patients. The UPCR decreased from baseline 0.44 (0.28, 1.33) mg/mg to 0.09 (0.08, 0.16) mg/mg, representing a reduction of 79.5% (50.6%, 92.9%). The urinary red blood cell count fell from 149.69 (77.22, 214.50) cells/μl at baseline to 33.00 (10.56, 89.10) cells/μl. eGFR remained stable [121.0 (102.5, 148.5) ml·min-1·(1.73 m2)-1 vs. 141.9(120.1, 152.7) ml·min-1·(1.73 m2)-1]. There were no obvious changes in serum IgG, serum IgA, complement C3, CD4+ T-cell counts, and CD19+ B-cell counts after treatment. Glucocorticoids were discontinued in four patients within the 24-week follow-up period, with time to discontinuation of 16.0 (11.2, 23.0) weeks. In another case, the glucocorticoid dosage was reduced to 2.5 mg/d at the 24th week (equivalent to prednisone), and discontinued at the 26th week. There were no serious adverse events reported. Conclusions Adjunctive telitacicept therapy demonstrates significant urine protein reduction, glucocorticoid-sparing benefits, and a favorable safety profile in pediatric IgAVN.
Objective To investigate the role of farnesoid X receptor (FXR) in oxidative stress-induced acute kidney injury (AKI). Methods Eight-week-old male mice on a C57BL/6 background, weighing 20-25 g, were used for the animal experiments. Immunofluorescence staining was used to determine the localization of FXR expression in the kidney of wild-type normal mice. Subsequently, a renal ischemia?reperfusion (I/R) injury model was established to induce AKI in mice. Wild-type mice were divided into sham operation group and I/R group, with 10 mice in each group. Western blotting and real?time quantitative PCR were used to detect the protein and mRNA expression levels of FXR on postoperative day 2, respectively. Kidney tissues were harvested on postoperative days 2 and 6 for immunofluorescence staining to evaluate FXR expression. FXR knockout (FXR-/-) mice and their wild-type littermates were selected to establish renal I/R injury models and assigned to FXR-/- I/R group and wild-type I/R group, respectively, with 16 mice in each group. Blood samples were collected via the retro-orbital venous plexus on postoperative day 2 to measure blood urea nitrogen levels. Survival status was monitored for 14 days postoperatively, and Kaplan-Meier survival curve was plotted. Kidney tissues were also collected on postoperative days 2 and 7 for PAS staining to evaluate renal histopathological injury. Human renal proximal tubular epithelial cells (HK-2 cells) were infected with lentivirus carrying FXR short hairpin RNA (shRNA) to knock down FXR expression, followed by treatment with the FXR agonist GW4064 and H2O2-induced oxidative stress injury. Flow cytometry was used to detect apoptosis of HK-2 cells after oxidative stress injury. HK-2 cells were infected with lentivirus carrying sirtuin 1 (SIRT1) shRNA to knock down SIRT1 expression. Cells were divided into H2O2, H2O2+shSIRT1, H2O2+GW4064, and H2O2+GW4064+shSIRT1 groups, and real?time quantitative PCR was used to detect Caspase-3 (CASP3) mRNA expression. Results Animal experiments showed that FXR was predominantly expressed in renal proximal tubular epithelial cells of wild-type mice. Compared with the sham group, both FXR mRNA and protein levels of kidney tissues in the I/R group were lower (both P<0.05), and FXR mRNA level was significantly downregulated on day 2 after modeling. Immunofluorescence staining further showed that FXR protein expression was decreased on days 2 and 6 after modeling, especially in injured renal tubules. After I/R, the 14-day cumulative survival rate of FXR-/- mice was lower than that of wild-type mice. On postoperative day 2, blood urea nitrogen level in FXR-/- mice was significantly higher than those in wild-type mice (P<0.05). PAS staining further showed obvious tubular injury in FXR-/- mice on postoperative days 2 and 7, and the injury severity was greater than that in wild-type mice. In vitro experiments showed that FXR agonist treatment attenuated oxidative stress-induced apoptosis in HK-2 cells (P<0.05), whereas FXR knockdown aggravated apoptosis (P<0.05). In H2O2-treated HK-2 cells, SIRT1 knockdown upregulated CASP3 mRNA level; CASP3 mRNA level in the H2O2+GW4064+shSIRT1 group was significantly higher than that in the H2O2+GW4064 group (both P<0.05). Conclusions FXR is mainly expressed in renal proximal tubular epithelial cells of mice, and its expression decreases in I/R-induced AKI; FXR knockout can aggravate I/R-induced AKI. The protective effect of FXR against AKI may be related to the upregulation of SIRT1 expression.
The paper reports the long-term follow-up efficacy of budesonide enteric- coated capsules combined with optimized supportive therapy (including renin-angiotensin- aldosterone system inhibitors and symptomatic treatments) in two patients with stage 3 chronic kidney disease complicated with IgA nephropathy (IgAN). Case 1 was a newly diagnosed IgAN patient with mean baseline 24-hour urinary protein of 2.87 g and estimated glomerular filtration rate (eGFR) of 31.10 ml·min-1·(1.73 m2)-1. The patient received combination therapy for 9 months. At the end of treatment, 24-hour urinary protein reduced to 0.23 g (a 92.0% decline from baseline), and eGFR increased to 44.60 ml·min-1·(1.73 m2)-1, representing an increment of 13.50 ml·min-1·(1.73 m2)-1 versus baseline. During the 12-month follow-up after discontinuation of budesonide enteric-coated capsules, urinary protein remained stable, and renal function was markedly improved compared with pretreatment level. Case 2 had an 18-year history of IgAN. The patient had achieved long-term stability following optimized supportive therapy but recently developed recurrent disease activity, manifested as recurrent proteinuria combined with microscopic hematuria. Baseline 24-hour urinary protein was 3.69 g, with eGFR of 57.60 ml·min-1·(1.73 m2)-1. The patient received continuous combination therapy for 15 months. At month 9 of treatment, 24-hour urinary protein decreased to 0.17 g (95.4% reduction from baseline), and eGFR rose to 78.20 ml·min-1·(1.73 m2)-1, an increase of 20.60 ml·min-1·(1.73 m2)-1 from baseline. At month 15 of treatment, 24-hour urinary protein was 0.14 g (96.2% reduction from baseline), with eGFR of 75.00 ml·min-1·(1.73 m2)-1, representing an increment of 17.40 ml·min-1·(1.73 m2)-1 relative to baseline.Throughout the treatment and follow-up periods, both patients achieved prominent reductions in urinary protein from baseline alongside stabilized eGFR. Blood pressure, blood glucose and other parameters remained within steady ranges. No severe adverse events such as edema, infection, or other drug-related adverse reactions were documented. Long-term follow-up outcomes of these two patients indicate that budesonide enteric-coated capsules plus optimized supportive treatment yield favorable efficacy and safety in patients with stage 3 chronic kidney disease and IgAN. Nevertheless, larger-sample studies are warranted to verify its long-term renal protective effects and risk-benefit profile.
This paper presents a rare case of complete hydatidiform mole complicated with nephrotic syndrome. The patient was a woman of reproductive age who presented with irregular vaginal bleeding, hypertension, nephrotic syndrome, and acute kidney injury after 4 months of amenorrhea. A thorough diagnostic workup led to the diagnosis of hydatidiform mole. One week after uterine evacuation, the patient's nephrotic syndrome began to remit, with her blood pressure and renal function returning to normal. After excluding other potential causes such as infection, autoimmune diseases, and malignancies, the nephrotic syndrome was considered secondary to the molar pregnancy. This case suggests that hydatidiform mole can be complicated with nephrotic syndrome, highlighting the need for increased clinical awareness of this association. Early uterine evacuation is critical for the remission of nephrotic syndrome. The underlying mechanisms of nephrotic syndrome complicating hydatidiform mole remain to be further investigated.
Infection of the tunneled cuffed catheter and subsequent formation of right atrial septic thrombus and hematogenous pulmonary abscesses are serious complications in maintenance hemodialysis patients. This article reports a 44-year-old male maintenance hemodialysis patient who developed a large-size right atrial septic thrombus and multiple hematogenous pulmonary abscesses in both lungs due to infection with methicillin-resistant Staphylococcus aureus in the tunneled cuffed catheter of right internal jugular vein. The patient achieved transient improvement after early anti-infection treatment, but disease relapsed due to self-discharge without completing full-course therapy. Transthoracic echocardiography detected right atrial septic thrombus upon revisit. After multidisciplinary evaluation, high?risk catheter removal was performed under perioperative anticoagulation protection. Post-operative chest CT revealed multiple pulmonary abscesses in both lungs, and methicillin?resistant Staphylococcus aureus was isolated from bronchoalveolar lavage fluid culture, which confirmed the etiological diagnosis. This case fully demonstrates the clinical evolution process of such complications from local infection to intracardiac and distant dissemination, suggesting that early transthoracic echocardiography for screening of cardiac complications should be performed in patients with catheter-related bloodstream infections, especially those caused by Staphylococcus aureus infections; in the presence of large-sized active intracardiac septic thrombus, high-risk catheter removal may be safely performed through perioperative anticoagulation and individualized procedures, providing a reference strategy for clinical management of such high-risk cases.
This article summarizes and analyzes the clinical manifestations, renal pathology, and follow-up outcomes of three children with paired box gene 2(PAX2) variants, exploring the clinical characteristics and significance of glomerular pathological diversity. Case 1 (an 11-year-old male) and his elder sister, case 2 (a 15-year-old female), both carried a heterozygous PAX2 missensevariant (c.419G>A, p. Arg140Gln). Case 1 presented with isolated proteinuria; renal pathology showed IgA nephropathy (Oxford classification M0E0S1T0-C0), with positive staining for anti- galactose-deficient IgA1. He was treated with prednisone combined with tacrolimus and angiotensin-converting enzyme inhibitor (ACEI), and maintained normal renal function during 6 years of follow-up. Case 2 initially presented with proteinuria and impaired renal function, with an estimated glomerular filtration rate of 64.2 ml·min?1·(1.73 m2)?1. Renal pathology showed focal segmental glomerulosclerosis with negative immunofluorescence. After ACEI treatment, she remained at chronic kidney disease (CKD) stage 2 during 6 years of follow-up. Case 3 (an 8-year-old male) carried a de novo heterozygous deletion of exons 1-7 in the PAX2 gene. He presented with nephrotic-range proteinuria, impaired renal function (CKD 3b), a small right kidney and bilateral optic disc vascular abnormalities. Renal pathology revealed immune complex-mediated mesangial proliferative glomerulonephritis. His renal function remained stable after 1 year of follow-up with ACEI treatment. All three cases presented with proteinuria but exhibited distinct glomerular pathologies (IgA nephropathy, focal segmental glomerulosclerosis, and mesangial proliferative glomerulonephritis). Notably, the same PAX2 missense variant within one family corresponded to different pathological patterns, while the large?fragment deletion tended to be associated with more severe extrarenal manifestations. These findings highlight the marked heterogeneity of renal pathological phenotypes in PAX2?related nephropathy, suggesting that renal biopsy should be actively considered in PAX2 variant patients with proteinuria to guide individualized treatment, as genotype alone cannot reliably predict the pathological subtype.
Systemic light-chain amyloidosis is a systemic disease characterized by the deposition of amyloid proteins formed from misfolded monoclonal free light chains in multiple organs and systems throughout the body. Patients may develop structural and functional impairment in the affected organs. The kidney is the major affected organ; however, the underlying pathological mechanisms remain incompletely understood. Establishing stable and reproducible animal and cell models is essential for in-depth investigation of the pathogenesis of renal light-chain amyloidosis. This review summarizes current progress in the construction and study of in vivo and in vitro models of renal light-chain amyloidosis, including intraperitoneal and intravenous injection of monoclonal free light chains, subcutaneous injection of amyloid fibrils, transgenic models, and in vitro organoid models. The application of these models may facilitate a deeper understanding of the pathogenesis of renal light-chain amyloidosis and provide experimental conditions for the development of novel therapeutic strategies.
Polycystic kidney disease (PKD) is a hereditary kidney disorder characterized by progressive renal cysts, serving as a major cause of end-stage renal disease. The core pathogenesis lies in structural or functional defects of primary cilia. Functioning as cellular "signaling antennas", primary cilia are crucial for maintaining renal homeostasis. This review summarizes the fundamental functions of primary cilia and their pivotal role in PKD. It also outlines emerging therapeutic strategies for PKD, including modulating signaling pathways, intervening in ciliary structure and function, gene editing, activating ciliary autophagy, and nanomedicine-based targeted therapies. These approaches open up new therapeutic targets and avenues for PKD treatment.
