Nephron最新文献

{"title":"Mutation Analysis of PKD1 and PKD2 Genes in a Large Italian Cohort Reveals Novel Pathogenic Variants Including a Novel Complex Rearrangement.","authors":"Silvia Orisio, Marina Noris, Miriam Rigoldi, Elena Bresin, Norberto Perico, Matias Trillini, Roberta Donadelli, Annalisa Perna, Ariela Benigni, Giuseppe Remuzzi","doi":"10.1159/000530657","DOIUrl":"10.1159/000530657","url":null,"abstract":"<p><strong>Background: </strong>Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited disease of the kidney. It occurs in adulthood but is also rarely diagnosed in early childhood. The majority of the disease-causing variants observed in ADPKD patients are in two genes: PKD1 and PKD2.</p><p><strong>Methods: </strong>237 patients from 198 families with a clinical diagnosis of ADPKD were screened for PKD1 and PKD2 genetic variants using Sanger sequencing and multiple ligation-dependent probe amplification analysis.</p><p><strong>Results: </strong>Disease-causing (diagnostic) variants were identified in 173 families (211 patients), 156 on PKD1 and 17 on PKD2. Variants of unknown significance were detected in 6 additional families, while no mutations were found in the remaining 19 families. Among the diagnostic variants detected, 51 were novel. In ten families, seven large rearrangements were found and the molecular breakpoints of 3 rearrangements were identified. Renal survival was significantly worse for PKD1-mutated patients, particularly those carrying truncating mutations. In patients with PKD1 truncating (PKD1-T) mutations, disease onset was significantly earlier than in patients with PKD1 non-truncating variants or PKD2-mutated patients.</p><p><strong>Conclusions: </strong>Comprehensive genetic testing confirms its utility in diagnosing patients with ADPKD and contributes to explaining the clinical heterogeneity observed in this disease. Moreover, the genotype-phenotype correlation can allow for a more accurate disease prognosis.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"273-291"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9893296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hospitalizations after Renal Transplantation in Children: Risk Factors, Causes, and Outcomes.","authors":"Songül Yılmaz, Zeynep Birsin Özçakar, Nilgün Çakar, Burcu Biral Coşkun, Fatma Fatoş Yalçınkaya","doi":"10.1159/000534787","DOIUrl":"10.1159/000534787","url":null,"abstract":"<p><strong>Introduction: </strong>The aims of this study were to evaluate the frequency and causes of hospitalizations in the posttransplant period of children, investigate the risk factors, and evaluate the relationship between hospitalizations and renal prognosis in the long term.</p><p><strong>Methods: </strong>We retrospectively reviewed the files of pediatric renal transplant patients, followed at least 6 months after kidney transplantation, in our center. Clinical information including age at transplantation, gender, primary disease, donor type, immuno-suppressive medication, hospitalization dates, and indications (infections and non-infectious) during follow-up period and graft outcomes was recorded.</p><p><strong>Results: </strong>A total of 74 children (46 males) were followed up for a median of 54 months. Among them, 69 patients (93.2%) were hospitalized 446 times. The most common cause of hospitalizations was infections (314 times, 70%). Urinary tract infections were the most important cause followed by upper respiratory tract infections. Forty (54%) patients were hospitalized 132 times (29.5%) for non-infectious reasons. The most common non-infectious reason was nonspecific graft dysfunction (19 patients, 30 times), followed by rejection (17 patients, 27 times). Younger age, use of induction therapy, and having congenital anomalies of kidney and urinary tract (CAKUT) were found to be risk factors for increased hospitalization rates (p &lt; 0.05). The number of hospitalizations was found to be negatively affecting the final glomerular filtration rate of transplant recipients (p: 0.04, r: -0.023).</p><p><strong>Conclusion: </strong>Patients with CAKUT, who received induction therapy, and small children were hospitalized more frequently after transplantation. Strategies to prevent hospitalizations will achieve a better graft prognosis.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"185-194"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71425224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tissue miRNA Profile Is Associated with Acute Tubular Necrosis, Rejection Phenotypes and BK Polyomavirus-Associated Nephropathy in Human Kidney Allografts.","authors":"Neva Bezeljak, Nika Kojc, Miha Arnol, Željka Večerić-Haler, Emanuela Boštjančič","doi":"10.1159/000534072","DOIUrl":"10.1159/000534072","url":null,"abstract":"<p><strong>Introduction: </strong>MicroRNAs (miRNAs), short noncoding RNAs, are involved in the modulation of gene expression, mainly by inhibiting the translation of mRNAs. Under physiological conditions, miRNAs are involved in viral infections and immune responses, among others; aberrant miRNA expression has been associated with kidney transplant pathologies, but a comprehensive comparison of later, particularly in tissue sections, is still pending.</p><p><strong>Methods: </strong>We used the genome-wide screening of miRNAs to identify those potentially involved in the disease processes after kidney transplantation. RNA was isolated from formalin-fixed paraffin-embedded kidney biopsy samples. Study included 8 patients with acute tubular necrosis (ATN), 8 patients with antibody-mediated rejection (ABMR), 10 patients with T-cell-mediated rejection (TCMR), 10 patients with BK polyomavirus-associated nephropathy (BKPyVAN), and 12 surveillance biopsies from patients with stable allograft function and no major abnormalities (normal allografts, CTRL).</p><p><strong>Results: </strong>We found 136 miRNAs differentially expressed in diseased kidney transplant tissue compared with normal allografts; of these, 74 miRNAs were differentially expressed in ABMR, 65 in ATN, 62 in BKPyVAN, 69 in TCMR, and 16 miRNAs were not associated with a specific disease phenotype. In addition, 29 miRNAs were differently expressed between ABMR and ATN, 39 between BKPyVAN and TCMR, and 20 between BKPyVAN and ABMR, and 38 between ABMR and TCMR.</p><p><strong>Conclusion: </strong>Our findings show that miRNA derived from kidney allograft biopsy samples represent an additional diagnostic tool to distinguish different disease phenotypes. This finding has the potential to assist clinicians in therapeutic decision-making and to translate to noninvasive monitoring of patients, e.g., blood samples.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"300-311"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71425225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Suspected Autosomal Recessive Polycystic Kidney Disease but Cerebellar Vermis Hypoplasia, Oligophrenia Ataxia, Coloboma, and Hepatic Fibrosis (COACH) Syndrome in Retrospect, A Delayed Diagnosis Aided by Genotyping and Reverse Phenotyping: A Case Report and A Review of the Literature.","authors":"Meenakshi Sambharia, Margaret E Freese, Francisco Donato, Girish Bathla, Ibrahim M M Abukhiran, Maisie I Dantuma, M Adela Mansilla, Christie P Thomas","doi":"10.1159/000527991","DOIUrl":"10.1159/000527991","url":null,"abstract":"<p><p>The clinical features of cerebellar vermis hypoplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis (COACH) characterize the rare autosomal recessive multisystem disorder called COACH syndrome. COACH syndrome belongs to the spectrum of Joubert syndrome and related disorders (JSRDs) and liver involvement distinguishes COACH syndrome from the rest of the JSRD spectrum. Developmental delay and oculomotor apraxia occur early but with time, these can improve and may not be readily apparent or no longer need active medical management. Congenital hepatic fibrosis and renal disease, on the other hand, may develop late, and the temporal incongruity in organ system involvement may delay the recognition of COACH syndrome. We present a case of a young adult presenting late to a Renal Genetics Clinic for evaluation of renal cystic disease with congenital hepatic fibrosis, clinically suspected to have autosomal recessive polycystic kidney disease. Following genetic testing, a reevaluation of his medical records from infancy, together with reverse phenotyping and genetic phasing, led to a diagnosis of COACH syndrome.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"264-272"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10863935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between the High-Sensitivity C-Reactive Protein/Albumin Ratio and New-Onset Chronic Kidney Disease in Chinese Individuals.","authors":"Zihao Zhang, Peipei Liu, Ling Yang, Naihui Zhao, Wenli Ou, Xiaofu Zhang, Yinggen Zhang, Shuohua Chen, Shouling Wu, Xiuhong Yang","doi":"10.1159/000534034","DOIUrl":"10.1159/000534034","url":null,"abstract":"<p><strong>Introduction: </strong>Inflammation is associated with development of chronic kidney disease (CKD). However, the association of the high-sensitivity C-reactive protein (hs-CRP)/albumin ratio (CAR) on the risk of CKD in the general population is unknown. This study explored the relationship between the CAR and CKD and the ability of this ratio to predict CKD in the general population.</p><p><strong>Methods: </strong>A total of 47,472 participants in the Kailuan study who met the inclusion criteria in 2010 were selected and grouped using the quartile method. A Cox proportional hazard regression model was used to evaluate the association of the CAR on the risk of CKD. The C-index, net reclassification index (NRI), and overall identification index (IDI) were calculated to evaluate the ability of the CAR to predict CKD.</p><p><strong>Results: </strong>During a follow-up of 378,383 person-years, CKD events occurred in 6,249 study participants (13.16%). The Cox proportional hazard regression model showed that the hazard ratio (95% confidence interval) for CKD events was 1.18 (1.10-1.28) in the Q3 group and 1.42 (1.32-1.53) in the Q4 group when compared with the Q1 group. Compared with the single index, the C-index, NRI, and IDI values were significantly improved when the CAR was added for prediction of risk of CKD.</p><p><strong>Conclusions: </strong>A higher CAR was an independent risk factor for CKD. The ability of the CAR to predict CKD was better than that of hs-CRP or albumin. The CAR provides an important reference index for predicting the risk of CKD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"160-170"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10911139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10224136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between Physical Frailty and Sleep Disturbances among Patients on Hemodialysis: A Cross-Sectional Study.","authors":"Shun Yoshikoshi, Shohei Yamamoto, Yuta Suzuki, Keigo Imamura, Manae Harada, Sachi Yamabe, Yusuke Matsunaga, Shiwori Osada, Hirokuni Tagaya, Atsuhiko Matsunaga","doi":"10.1159/000533418","DOIUrl":"10.1159/000533418","url":null,"abstract":"<p><strong>Introduction: </strong>Among patients on hemodialysis (HD), physical frailty and sleep disturbances are not only common but also associated with adverse outcomes. The aim of this study was to evaluate the association between physical frailty and sleep disturbances in patients on HD.</p><p><strong>Methods: </strong>This cross-sectional study was conducted from June 2017 to March 2021, with outpatients receiving HD 3 times a week at two dialysis facilities in Japan. Sleep disturbances were identified with the Athens Insomnia Scale (AIS). Physical frailty was defined using the Fried Frailty Phenotype. Patients were classified as \"non-frailty (number of frailty components: 0-2)\" or \"frailty (3-5).\" We examined the association of sleep disturbances with physical frailty and its components by performing a logistic regression analysis.</p><p><strong>Results: </strong>We analyzed 360 patients (mean age 65.6 years; 62% men). Eighty-one patients (23%) were classified into the group with frailty, and the mean AIS score was 5.2 ± 4.2 points. After adjusting for clinical characteristics, increasing the AIS score per 1 point was associated with higher odds of physical frailty (odds ratio, 1.12; 95% confidence interval, 1.05-1.20; p &lt; 0.01). As for the frailty components, exhaustion, low physical activity, and weak grip strength showed an association with sleep disturbances (all p &lt; 0.05).</p><p><strong>Conclusions: </strong>Sleep disturbances were independently associated with physical frailty in patients on HD. Future studies are warranted to investigate the causality between physical frailty and sleep disturbances in this population.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"152-159"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10389318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of Familial Hyperkalemia and Hypertension with Proximal Renal Tubular Acidosis and Epileptic Seizures.","authors":"Neta Shirin, Grace Rabinowitz, Ilan Blatt, Steven J D Karlish, Zvi Farfel, Haim Mayan","doi":"10.1159/000531868","DOIUrl":"10.1159/000531868","url":null,"abstract":"<p><strong>Introduction: </strong>Familial hyperkalemic hypertension (FHHt) is an inherited disease characterized by hyperkalemia, hypertension, and hyperchloremic acidosis (HCA). The primary defect is a hyperactive sodium chloride co-transporter, expressed in the renal distal tubule. FHHt is caused by mutation in either WNK1, WNK4, KLHL3, or Cul3. The mechanism of HCA is not completely understood.</p><p><strong>Methods: </strong>Clinical and genetic data were collected from the largest family with FHHt described in the literature. Urine ammonia was measured in 26 family members. Epilepsy was diagnosed clinically.</p><p><strong>Results: </strong>Of the 85 family members, 44 are affected by the Q565E WNK4 mutation, and 28 are newly described. In genetically engineered mice, urinary ammonium was decreased. In our study, urine ammonium did not change. In 11 unaffected subjects, urine ammonia per creatinine was 8.013 ± 3.620 m<sc>m</sc>/mm, and in 15 subjects affected by FHHt, it was 8.990 ± 4.300 m<sc>m</sc>/mm (p = 0.546, not significant). Due to the large family size and prolonged follow-up, rare conditions can be identified. Indeed, two children have genetic generalized epilepsy and one child has migraine. The prevalence of epilepsy is 4.545% (2/44) much higher than in the general population (0.681%). This difference is statistically significant (χ2 with Yates correction = 5.127, p = 0.023).</p><p><strong>Conclusions: </strong>We provide further evidence that the origin of HCA in FHHt lies in the proximal renal tubule. The association of FHHt with epilepsy leads us to speculate that the raised serum K in susceptible subjects may cause a rise in CSF K, and extracellular cerebral K, leading to epilepsy.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"179-184"},"PeriodicalIF":2.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10154778","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comorbidity and Medication Trends in Chronic Kidney Disease and Incident Atrial Fibrillation: A Nationwide Cohort Study.","authors":"Heini Jyrkilä, Kati Kaartinen, Leena Martola, Olli Halminen, Jari Haukka, Miika Linna, Pirjo Mustonen, Jukka Putaala, Konsta Teppo, Janne Kinnunen, Juha Hartikainen, K E Juhani Airaksinen, Mika Lehto","doi":"10.1159/000539603","DOIUrl":"10.1159/000539603","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic kidney disease (CKD) is associated with an increased incidence of atrial fibrillation (AF). Also, patients with AF are prone to adverse kidney outcomes. We examined comorbidities and medication use in patients with CKD and incident AF.</p><p><strong>Methods: </strong>The Finnish AntiCoagulation in Atrial Fibrillation (FinACAF) is a nationwide retrospective register-linkage study including data from 168,233 patients with incident AF from 2007 to 2018, with laboratory data from 2010 onwards. Estimated glomerular filtration rate (eGFR) was available for 124,936 patients. The cohort was divided into 5 CKD stages with separate groups for dialysis and kidney transplantation.</p><p><strong>Results: </strong>At AF diagnosis eGFR <60 mL/min/1.73 m2 was found in 27%, while 318 (0.3%) patients were on dialysis, and 188 (0.2%) had a functioning kidney transplant. Lowering eGFR yielded more comorbidities and medications. During 2010-2018 in patients with eGFR <60 mL/min/1.73 m2 prevalence of hypertension, dyslipidaemia, and diabetes increased from 82 to 88%, from 50 to 66% and from 25 to 33%, respectively (<0.001). Throughout the observation period, lipid-lowering medication was underused.</p><p><strong>Conclusion: </strong>More than one-fourth of patients with incident AF also had CKD stage 3-5 (eGFR <60 mL/min/1.73 m2). Both comorbidities and medication use increased with worsening kidney function. Prevalence of major cardiovascular (CV) risk factors increased during 2010-2018, but the use of survival-affecting medications, such as lipid-lowering medication, was suboptimal at all stages of CKD. More attention should be given to the optimal treatment of risk factors in this high CV risk population.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"755-767"},"PeriodicalIF":2.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141306380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Remote Surveillance Technology of Dialysis Arteriovenous Access: Retrospective Evaluation in a UK Renal Centre.","authors":"Alshymaa Rafiek Eltahan, Zulfikar Pondor, Rosemary L Donne, David Lewis, Maharajan Raman, Paul Hinchliffe, Jan Cowperthwaite, Dimitrios Poulikakos","doi":"10.1159/000538820","DOIUrl":"10.1159/000538820","url":null,"abstract":"<p><strong>Background: </strong>Early identification of dysfunctional arteriovenous haemodialysis (HD) vascular access (VA) is important for timely referral and intervention.</p><p><strong>Method: </strong>We retrospectively calculated VA risk score using Vasc-Alert surveillance software technology from HD treatment sessions in 2 satellite HD units over 18 months. We included in the analysis HD patients dialysing with arteriovenous fistula or graft (AVF/G) with available Vasc-Alert data for≥ 2 months. For group one (eventful) that included patients who developed vascular access thrombosis or stenosis over the study period, we collected Vasc-Alert risk score 2 months prior to the event and, for group two (uneventful), over 5 consecutive months. Vasc-Alert technology utilises routinely collected data during HD to calculate VA risk score and triggers an alert if the score is ≥7 in 3 consecutive dialysis sessions. Patients with &gt;2 alerts (vascular access score ≥7) per month were considered to have positive alerts.</p><p><strong>Results: </strong>From 140 HD patients, 81 patients dialysed via AVF/G. 77/81 had available Vasc-Alert data and were included in the final analysis. Out of 17 eventful patients, 11 (64.7%) had positive alerts 2 months prior to the vascular event. Out of the 60 patients without vascular events, 20 patients (33.3%) had positive alert. Vasc-Alert's sensitivity and specificity for vascular events were 64.7% and 66.6%, respectively. Within the 6 patients with thrombosed access, 2 patients (33.3%) detected by Vasc-Alert were not detected with clinical monitoring.</p><p><strong>Conclusion: </strong>Vascular access risk score can be a useful non-invasive vascular access surveillance method to assist clinical decision making.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"536-543"},"PeriodicalIF":2.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11332309/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140865356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunosuppressant Agents as Add-On Therapy Failed to Improve the Outcome of Immunoglobulin A Nephropathy with Crescent Score C1.","authors":"Xianjin Bi, Yanlin Yu, Siyan Zhou, Yue Zhou, Jinghong Zhao, Jiachuan Xiong","doi":"10.1159/000534788","DOIUrl":"10.1159/000534788","url":null,"abstract":"<p><strong>Introduction: </strong>The renoprotective benefits of adding immunosuppressant therapy to corticosteroid (CS) treatment for immunoglobulin A nephropathy (IgAN) patients with less than 25% crescent formation (C1) remain uncertain, warranting further research.</p><p><strong>Methods: </strong>A retrospective study was conducted on IgAN patients with crescent C1 lesions confirmed by renal biopsy at Xinqiao Hospital between May 1, 2017, and May 1, 2020. Patients were stratified into either the CS treatment group or the CS combined with an additional immunosuppressant therapy group. Follow-up assessments were conducted within 24 months. Propensity score analysis was used to match patients receiving CS and CS + immunosuppressant drug treatment in a 1:1 ratio. Primary outcomes included changes in estimated glomerular filtration rate (eGFR) and urine albumin-to-creatinine ratio (UACR). Subgroup analyses were performed to evaluate the benefits of different populations. Composite endpoint outcomes comprised a 30% eGFR decrease, end-stage kidney disease (ESKD) necessitating dialysis or transplant, or kidney disease-related mortality. Adverse events were also compared between the two groups.</p><p><strong>Results: </strong>296 IgAN patients with C1 lesions were included in the analysis. Baseline characteristics indicated that IgAN patients in the CS + immunosuppressant group exhibited poorer renal function and higher UACR levels. Propensity score analysis effectively minimized the influence of baseline clinical characteristics, including age, serum creatinine, initial eGFR, UACR, and 24-h proteinuria. Both treatment groups demonstrated continuous eGFR improvement and significant UACR reduction during follow-up, especially at 6 months. However, no significant differences in eGFR and UACR reduction rates were observed between the two groups throughout the entire follow-up period, both before and after matching. Subgroup analysis revealed improved eGFR in both treatment groups, notably among patients with an initial eGFR below 90 mL/min/1.73 m2. Conversely, IgAN patients with C1 lesions and a cellular crescent ratio exceeding 50% treated with CS and immunosuppressant therapy experienced a significant improvement in renal function and a decline in urinary protein creatinine ratio. Composite endpoint outcomes did not significantly differ between the two groups, while the incidence of adverse events was comparable.</p><p><strong>Conclusion: </strong>Our findings suggest that the addition of immunosuppressant therapy to corticosteroid monotherapy did not confer significant therapeutic advantages in patients with C1 lesions compared to CS monotherapy, although some specific patient populations appeared to derive modest benefits from this combined approach.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"587-600"},"PeriodicalIF":2.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140899077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}