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Nested Case-Control Study of Intervention in Early Infancy to Prevent Tuberous Sclerosis Complex–Associated Renal Angiomyolipoma 婴儿早期干预预防结节性硬化症相关肾血管平滑肌脂肪瘤的巢式病例对照研究
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.021
Shuo Dun , Lin Wan , Yang-Yang Wang , Qian Lu , Qi Zhang , Qiu-Hong Wang , Jia Wang , Hai-Qing Zhao , Li-Ping Zou
{"title":"Nested Case-Control Study of Intervention in Early Infancy to Prevent Tuberous Sclerosis Complex–Associated Renal Angiomyolipoma","authors":"Shuo Dun ,&nbsp;Lin Wan ,&nbsp;Yang-Yang Wang ,&nbsp;Qian Lu ,&nbsp;Qi Zhang ,&nbsp;Qiu-Hong Wang ,&nbsp;Jia Wang ,&nbsp;Hai-Qing Zhao ,&nbsp;Li-Ping Zou","doi":"10.1016/j.ekir.2025.03.021","DOIUrl":"10.1016/j.ekir.2025.03.021","url":null,"abstract":"<div><h3>Introduction</h3><div>In current studies, treatment of tuberous sclerosis complex (TSC)–related renal angiomyolipoma (RAML) was initiated only after clinical progression or the onset of symptoms, rather than at an earlier stage in the disease process. However, the previous case report indicated that early mammalian target of rapamycin (mTOR) inhibition in patients with TSC might prevent the development of TSC lesions. We performed this nested case-control study to evaluate whether prophylactic sirolimus initiation in early infancy prevented TSC-related RAML (TSC-RAML).</div></div><div><h3>Methods</h3><div>A nested case-control study design was used, based on the Efficacy and Safety of Sirolimus in Pediatric Patients With Tuberous Sclerosis (ESOSIPT) study cohort. Children with TSC initiating sirolimus at age ≤ 3 months and without RAML at baseline comprised the case group (treatment group). Propensity score matching (1:3) was used to select the control group from the first visit patients who were aged &gt; 3 months without mTOR inhibitor exposure. The incidence of RAML was compared via Kaplan-Meier method with log-rank testing.</div></div><div><h3>Results</h3><div>Twenty-seven eligible children entered the study as a case group, and 81 patients who had not used mTOR inhibitors were matched. The log-rank test showed that the incidence of RAML between the 2 groups was statistically significant (<em>P</em> = 0.047). Short-term adverse events (AEs) were reported in 55.6% (15/27) of early-treated infants, predominantly grade 1 or 2 according to the common terminology criteria for AEs.</div></div><div><h3>Conclusion</h3><div>The use of sirolimus in early infancy might have had potential benefits in preventing TSC-RAML, and the short-term AEs were usually mild or moderate.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1960-1970"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Tuberous Sclerosis Complex–Associated Tubulointerstitial Kidney Disease 结节性硬化症复合物相关的小管间质肾病
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.04.001
Hamza Sakhi , Pierre Isnard , Idris Boudhabhay , Jean-Michel Correas , Stephane Burtey , Dominique Joly , Bertrand Knebelmann , Aude Servais
{"title":"Tuberous Sclerosis Complex–Associated Tubulointerstitial Kidney Disease","authors":"Hamza Sakhi ,&nbsp;Pierre Isnard ,&nbsp;Idris Boudhabhay ,&nbsp;Jean-Michel Correas ,&nbsp;Stephane Burtey ,&nbsp;Dominique Joly ,&nbsp;Bertrand Knebelmann ,&nbsp;Aude Servais","doi":"10.1016/j.ekir.2025.04.001","DOIUrl":"10.1016/j.ekir.2025.04.001","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 2049-2053"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144221451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Gut Microbiome Diversity and Uric Acid in Serum and Urine 血清和尿液中肠道微生物群多样性与尿酸
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.040
Cecilie Ness , Dmitri Svistounov , Marit D. Solbu , Natalia Petrenya , Neoma Boardman , Kirsti Ytrehus , Trond G. Jenssen , Andrew Holmes , Stephen J. Simpson , Svetlana N. Zykova
{"title":"Gut Microbiome Diversity and Uric Acid in Serum and Urine","authors":"Cecilie Ness ,&nbsp;Dmitri Svistounov ,&nbsp;Marit D. Solbu ,&nbsp;Natalia Petrenya ,&nbsp;Neoma Boardman ,&nbsp;Kirsti Ytrehus ,&nbsp;Trond G. Jenssen ,&nbsp;Andrew Holmes ,&nbsp;Stephen J. Simpson ,&nbsp;Svetlana N. Zykova","doi":"10.1016/j.ekir.2025.03.040","DOIUrl":"10.1016/j.ekir.2025.03.040","url":null,"abstract":"<div><h3>Introduction</h3><div>An increasing body of evidence has shown the importance of the gut microbiota in modulating serum uric acid (SUA) levels. In this study, we aimed to determine the association between gut microbiome diversity, diet, SUA, and fractional excretion of uric acid (FEUA) in the kidney.</div></div><div><h3>Methods</h3><div>A cross-sectional study was conducted in 53 adults with normal or elevated SUA and estimated glomerular filtration rate (eGFR) range from 37 to 124 ml/min per 1.73 m<sup>2</sup>. Fecal microbiome composition was analyzed using 16S ribosomal RNA sequencing; and alpha diversity was expressed as reverse Simpson, Shannon, and Richness indices. Dietary data were collected, and dietary patterns were identified using principal component analysis. Unadjusted linear regression and models adjusted for sex, waist-hip ratio (WHR), and eGFR were used to study the association between gut microbial diversity, dietary pattern scores, and SUA/FEUA.</div></div><div><h3>Results</h3><div>Shannon index was negatively associated with SUA after multiple adjustment (β −36.4, 95% CI [−66.2 to −6.7], <em>P</em> = 0.017; adjusted R<sup>2</sup> = 0.62, <em>P</em> &lt; 0.001). Sex (standardized β = 0.52) and WHR (standardized β = 0.35) had the highest effect on SUA, followed by Shannon diversity index (standardized β = −0.22). We found that Shannon index (standardized β = 0.49, <em>P</em> &lt; 0.001) was positively associated with FEUA after adjustment for sex and “sweet” dietary pattern. This model explained 40% of the variability in FEUA (<em>P</em> &lt; 0.001). None of the dietary patterns were associated with SUA or FEUA.</div></div><div><h3>Conclusion</h3><div>A higher gut microbial diversity was associated with lower SUA and more efficient elimination of uric acid by the kidneys. There is a need for studies assessing efficacy and safety of interventions on the gut microbiome as a treatment of hyperuricemia.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1683-1693"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144222947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Community-Specific Differences in Kidney Function in Rural and Urban India 印度农村和城市肾功能的社区特异性差异
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.04.006
Nivetha Subramanian , Shuchi Anand , Xue Yu , Maria E. Montez-Rath , Prashant Jarhyan , Sheril Rajan , Nikhil Srinivasapura Venkateshmurthy , Adeera Levin , Dorairaj Prabhakaran , Peter Craig , Poornima Prabhakaran , Sailesh Mohan , Lindsay Jaacks
{"title":"Community-Specific Differences in Kidney Function in Rural and Urban India","authors":"Nivetha Subramanian ,&nbsp;Shuchi Anand ,&nbsp;Xue Yu ,&nbsp;Maria E. Montez-Rath ,&nbsp;Prashant Jarhyan ,&nbsp;Sheril Rajan ,&nbsp;Nikhil Srinivasapura Venkateshmurthy ,&nbsp;Adeera Levin ,&nbsp;Dorairaj Prabhakaran ,&nbsp;Peter Craig ,&nbsp;Poornima Prabhakaran ,&nbsp;Sailesh Mohan ,&nbsp;Lindsay Jaacks","doi":"10.1016/j.ekir.2025.04.006","DOIUrl":"10.1016/j.ekir.2025.04.006","url":null,"abstract":"","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 2035-2040"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144222970","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Corticosteroid Effects in IgA Nephropathy by Baseline Proteinuria and Estimated GFR 基线蛋白尿和估计GFR对IgA肾病的皮质类固醇作用
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.008
Dana Kim , Brendon L. Neuen , Jicheng Lv , Michelle A. Hladunewich , Vivekanand Jha , Lai Seong Hooi , Helen Monaghan , Robert A. Fletcher , Laurent Billot , Vlado Perkovic , Hong Zhang , Muh Geot Wong
{"title":"Corticosteroid Effects in IgA Nephropathy by Baseline Proteinuria and Estimated GFR","authors":"Dana Kim ,&nbsp;Brendon L. Neuen ,&nbsp;Jicheng Lv ,&nbsp;Michelle A. Hladunewich ,&nbsp;Vivekanand Jha ,&nbsp;Lai Seong Hooi ,&nbsp;Helen Monaghan ,&nbsp;Robert A. Fletcher ,&nbsp;Laurent Billot ,&nbsp;Vlado Perkovic ,&nbsp;Hong Zhang ,&nbsp;Muh Geot Wong","doi":"10.1016/j.ekir.2025.03.008","DOIUrl":"10.1016/j.ekir.2025.03.008","url":null,"abstract":"<div><h3>Introduction</h3><div>Higher proteinuria and lower estimated glomerular filtration rate (eGFR) are predictors of kidney failure in IgA nephropathy (IgAN); however, it is uncertain whether these markers modify response to corticosteroids. This <em>post hoc</em> analysis of the TESTING trial assessed the effects of methylprednisolone on kidney and safety outcomes by baseline proteinuria and eGFR.</div></div><div><h3>Methods</h3><div>A total of 503 participants with IgAN and proteinuria ≥ 1 g/d were randomized to oral methylprednisolone (full-dose 0.6–0.8 mg/kg/d or reduced-dose 0.4 mg/kg/d) versus placebo. Participants were categorized according to baseline proteinuria (1–&lt; 1.5, 1.5–&lt; 3, ≥ 3 g/d) and eGFR (20–&lt; 30, 30–&lt; 45, 45–&lt; 60, 60–120 ml/min per 1.73 m<sup>2</sup>).</div></div><div><h3>Results</h3><div>Over a mean follow-up of 4.2 years, methylprednisolone lowered the risk of the primary outcome (≥ 40% decline in eGFR, kidney failure, or death because of kidney disease) by 47% (hazard ratio: 0.53, 95% confidence interval [CI]: 0.39–0.72), with consistent effects regardless of baseline proteinuria (<em>P</em>-interaction = 0.53) or eGFR (<em>P</em>-interaction = 0.68). Similarly, methylprednisolone improved the decline in total eGFR slope and reduced proteinuria, regardless of baseline proteinuria or eGFR (all <em>P</em>-interaction &gt; 0.10). The number of serious adverse events (SAEs) was higher with methylprednisolone than with placebo across all proteinuria and eGFR levels. Absolute benefits and harms varied by eGFR, such that for those with eGFR &lt; 30 ml/min per 1.73 m<sup>2</sup>, absolute risk of SAEs may outweigh potential advantages. However, this subgroup was small, predominantly received full-dose methylprednisolone, and was older than those with eGFR ≥ 30 ml/min per 1.73 m<sup>2</sup>.</div></div><div><h3>Conclusion</h3><div>Methylprednisolone improves kidney outcomes in IgAN at high risk of progression, irrespective of proteinuria or eGFR, although the risk-benefit balance may be less favorable in those with advanced disease plus other risk factors for corticosteroid-related toxicities.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1886-1895"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Clinicopathologic Characteristics of Recurrent Lupus Nephritis Post-Transplant Using Surveillance and Indication Biopsies 移植后复发性狼疮性肾炎的临床病理特征监测及指征活检
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.044
Maria Lourdes Gonzalez Suarez , Wen Jiqiu , Andrea G. Kattah , Loren P. Herrera Hernandez , Carrie A. Schinstock , Mireille El Ters , Leticia Rolon , Mary E. Fidler , Samih H. Nasr , Sanjeev Z. Sethi , Martin Mai , Hasan Khamash , Mark D. Stegall , Sam T. Albadri , Ali Duarte-Garcia , Maxwell Smith , Hatem Amer , Lynn D. Cornell , Ladan Zand , Timucin Taner , Andrew J. Bentall
{"title":"The Clinicopathologic Characteristics of Recurrent Lupus Nephritis Post-Transplant Using Surveillance and Indication Biopsies","authors":"Maria Lourdes Gonzalez Suarez ,&nbsp;Wen Jiqiu ,&nbsp;Andrea G. Kattah ,&nbsp;Loren P. Herrera Hernandez ,&nbsp;Carrie A. Schinstock ,&nbsp;Mireille El Ters ,&nbsp;Leticia Rolon ,&nbsp;Mary E. Fidler ,&nbsp;Samih H. Nasr ,&nbsp;Sanjeev Z. Sethi ,&nbsp;Martin Mai ,&nbsp;Hasan Khamash ,&nbsp;Mark D. Stegall ,&nbsp;Sam T. Albadri ,&nbsp;Ali Duarte-Garcia ,&nbsp;Maxwell Smith ,&nbsp;Hatem Amer ,&nbsp;Lynn D. Cornell ,&nbsp;Ladan Zand ,&nbsp;Timucin Taner ,&nbsp;Andrew J. Bentall","doi":"10.1016/j.ekir.2025.03.044","DOIUrl":"10.1016/j.ekir.2025.03.044","url":null,"abstract":"<div><h3>Introduction</h3><div>The incidence of recurrent lupus nephritis (RLN) after kidney transplantation (KTx) varies with higher rates of RLN reported in surveillance biopsy-based studies (vs. clinically indicated biopsies).</div></div><div><h3>Methods</h3><div>We present a multisite retrospective study evaluating surveillance and clinically indicated biopsies in 209 first KTx recipients who had native lupus disease.</div></div><div><h3>Results</h3><div>Of the 112 patients with satisfactory material for comprehensive histology, RLN was observed in 40 (35.7%). We describe the pathology of histologic RLN (HRLN; 40%) and clinical RLN (CRLN; 60%). African Americans had the highest recurrence rate (48.3%) of whom 50% had CRLN. HRLN was noted as early as 18 days, with early diagnosis (&lt; 3 months of follow-up time) using surveillance biopsies. Mesangioproliferative pattern of glomerular injury (<sup>˷</sup> class II lupus nephritis [LN] by International Society of Nephrology/Renal Pathology Society) was the most frequent pattern of RLN. IgG dominance or codominance was the most frequent Ig staining pattern. A full-house pattern of staining was only seen in 6% of HRLN and 37% of CRLN. C4d stain, as a stand-alone immunofluorescence (IF) test, was performed in the index renal allograft biopsy in 37.5% of patients, with RLN, prompting evaluation with a full IF panel. Electron microscopy (EM) confirmed the findings on IF. Graft loss because of lupus-associated pathology was observed in 50% of RLN subjects, of which thrombotic microangiopathy was seen in 25%.</div></div><div><h3>Conclusion</h3><div>Our study demonstrates that RLN is frequent and may be clinically quiescent. Sequential biopsy evaluation provided an opportunity to study the natural evolution of the disease.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1829-1842"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Patients With Mild ADPKD by Kidney Imaging but Low Estimated GFR 肾显像显示轻度ADPKD但估计GFR低的患者
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.045
Seung Heyck Lee , Mauricio Miranda Cam , Taher Dehkharghanian , Fatemah Nasri , Saima Khowaja , Amirreza Haghighi , Xuewen Song , Korosh Khalili , York Pei
{"title":"Patients With Mild ADPKD by Kidney Imaging but Low Estimated GFR","authors":"Seung Heyck Lee ,&nbsp;Mauricio Miranda Cam ,&nbsp;Taher Dehkharghanian ,&nbsp;Fatemah Nasri ,&nbsp;Saima Khowaja ,&nbsp;Amirreza Haghighi ,&nbsp;Xuewen Song ,&nbsp;Korosh Khalili ,&nbsp;York Pei","doi":"10.1016/j.ekir.2025.03.045","DOIUrl":"10.1016/j.ekir.2025.03.045","url":null,"abstract":"<div><h3>Introduction</h3><div>High height-adjusted total kidney volume (HtTKV) and low estimated glomerular filtration rate (eGFR) typically indicate high cystic burden by imaging and low kidney function, respectively, identifying high-risk patients for disease progression in autosomal dominant polycystic kidney disease (ADPKD). Here, we report the prevalence, clinical characteristics, and causes of mild ADPKD in patients using imaging and low eGFR, an ill-defined clinical scenario.</div></div><div><h3>Methods</h3><div>We studied 473 patients with kidney function measurements, <em>PKD1</em> and <em>PKD2</em> genetic screen, and total kidney volume (TKV) measurements by magnetic resonance imaging (MRI) or computed tomography. Mayo Clinic Imaging Classification (MCIC) based on age and HtTKV was used to assess cystic disease severity. Patients with a discordant phenotype were defined as those with MCIC 1A/B and eGFR &lt; 80 ml/min per 1.73 m<sup>2</sup>. We reviewed medical records to compare patients with and without the discordant phenotype, examining clinical characteristics such as second kidney disease(s), nephrotoxic exposure, diabetes mellitus, and metabolic syndrome-related traits.</div></div><div><h3>Results</h3><div>Of 473 patients, 55 (12%) displayed a discordant phenotype. Among these patients, 13 (24%) had normal kidney functions by 24-h creatinine clearance (CrCl<sub>24</sub>) (&gt; 80 ml/min per 1.73 m<sup>2</sup>) and high urinary creatinine excretion rates, indicating underestimation of their kidney function by eGFR likely because of high muscle mass. In addition, discordant patients showed a higher prevalence of hypertension (82% vs. 57%, <em>P</em> &lt; 0.001), dyslipidemia (58% vs. 15%, <em>P</em> &lt; 0.001), diabetes mellitus (15% vs. 3%, <em>P</em> &lt; 0.05), and a second kidney disease (16% vs. 1%, <em>P</em> &lt; 0.001).</div></div><div><h3>Conclusion</h3><div>Mild ADPKD by imaging with low eGFR represents a significant clinical scenario with conflicting prognostic indicators, underscoring the need for delineating underlying causes and providing more appropriate management.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1855-1863"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Prediction Model of Disease Progression in X-Linked Alport syndrome Based on Clinical Characteristics and Genetic Variants 基于临床特征和遗传变异的x连锁Alport综合征疾病进展预测模型
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.006
Mengyao Zeng , Hongling Di , Jie Ding , Yanqin Zhang , Hong Xu , Jingyuan Xie , Jianhua Mao , Aihua Zhang , Guisen Li , Jiahui Zhang , Erzhi Gao , Dandan Liang , Qing Wang , Ling Wang , Yu An , Chunxia Zheng , Zhihong Liu
{"title":"A Prediction Model of Disease Progression in X-Linked Alport syndrome Based on Clinical Characteristics and Genetic Variants","authors":"Mengyao Zeng ,&nbsp;Hongling Di ,&nbsp;Jie Ding ,&nbsp;Yanqin Zhang ,&nbsp;Hong Xu ,&nbsp;Jingyuan Xie ,&nbsp;Jianhua Mao ,&nbsp;Aihua Zhang ,&nbsp;Guisen Li ,&nbsp;Jiahui Zhang ,&nbsp;Erzhi Gao ,&nbsp;Dandan Liang ,&nbsp;Qing Wang ,&nbsp;Ling Wang ,&nbsp;Yu An ,&nbsp;Chunxia Zheng ,&nbsp;Zhihong Liu","doi":"10.1016/j.ekir.2025.03.006","DOIUrl":"10.1016/j.ekir.2025.03.006","url":null,"abstract":"<div><h3>Introduction</h3><div>Alport syndrome (AS) is an inherited kidney disease with significant clinical heterogeneity. Prognosis prediction and risk assessment are important to assist patient care. However, a predictive tool of disease progression is still lacking.</div></div><div><h3>Methods</h3><div>The prediction model was developed in 363 patients (124 kidney failure events) with X-linked AS (XLAS) from a single-center retrospective cohort study and validated in 2 external cohorts, including 193 (27 events) and 125 patients (33 events) with XLAS from 6 centers and the literature database, respectively. Cox proportional hazards regression analysis with stepwise selection was used to select the important variables related to the progression to kidney failure, by using the baseline demographic, clinical, and genetic data. The performance of the prediction model was evaluated and compared using receiver-operating characteristic (ROC) curve and calibration plot.</div></div><div><h3>Results</h3><div>There were 4 variables identified that were significantly associated with the progression to kidney failure in the final model, namely sex, proteinuria, estimated glomerular filtration rate (eGFR), and pathogenic variants in <em>COL4A5.</em> Based on the model risk stratification, the median age at kidney failure was 23, 30, and 61 years in the low-, intermediate-, and high-risk groups, respectively. This model shows the best discrimination in predicting the progression to kidney failure before the age of 30 years, with areas under the curve (AUCs) &gt; 0.80 in both development and external cohorts.</div></div><div><h3>Conclusion</h3><div>A prediction model of progression to kidney failure based on clinical characteristics and genetic variants was developed and validated in patients with XLAS.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 2024-2034"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144222969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of Sleep Characteristics on Rapid Estimated GFR Decline in Adults with Normal Kidney Function 睡眠特征对肾功能正常成人GFR快速下降的影响
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.042
Mohammadreza Akbari , Seyed Alireza Mirhosseini , Kimia Falamarzi , Hossein Molavi Vardanjani , Zahra Rahimian , Azizallah Dehghan , Hiva Alipanah , Jamshid Roozbeh
{"title":"Effect of Sleep Characteristics on Rapid Estimated GFR Decline in Adults with Normal Kidney Function","authors":"Mohammadreza Akbari ,&nbsp;Seyed Alireza Mirhosseini ,&nbsp;Kimia Falamarzi ,&nbsp;Hossein Molavi Vardanjani ,&nbsp;Zahra Rahimian ,&nbsp;Azizallah Dehghan ,&nbsp;Hiva Alipanah ,&nbsp;Jamshid Roozbeh","doi":"10.1016/j.ekir.2025.03.042","DOIUrl":"10.1016/j.ekir.2025.03.042","url":null,"abstract":"<div><h3>Introduction</h3><div>This study was conducted to evaluate the association between self-reported sleep characteristics and rapid decline of estimated glomerular filtration rate (eGFR) in adults with initially normal kidney function.</div></div><div><h3>Methods</h3><div>Data from participants in the reevaluation phase of the Fasa Adult Cohort Study (FACS) were analyzed. Rapid eGFR decline was defined as an annual decrease &gt; 3 ml/min per 1.73 m<sup>2</sup> and rapid eGFR percent decline was defined as an annual decrease &gt; 10th percentile of study sample. Daily sleep duration, daytime napping, sleep onset latency (SOL), the use of sleeping pills and daytime sleepiness were assessed at baseline through interviews conducted by trained nurses. Poisson regression models were applied to evaluate the risk of outcomes across sleep characteristics.</div></div><div><h3>Results</h3><div>A total of 2810 participants, of whom 53.7% were female, with a mean age of 49.8 (± 8.3) yrs and a baseline eGFR of 88.5 (± 10.6) were studied. Regular daytime napping was associated with a reduced risk of rapid eGFR decline in individuals aged &gt; 50 yrs (adjusted relative risk [RR]: 0.64, 95% confidence interval [CI]: 0.48–0.86). Specifically, naps exceeding 90 minutes daily were associated with a 49% reduced risk compared with nonnappers (adjusted RR: 0.51, 95% CI: 0.33–0.81). Among participants aged &lt; 50 yrs, sleeping &lt; 7 h/d was linked to a higher likelihood of rapid eGFR percent decline (adjusted RR:1.78, 95% CI: 1.12–2.82).</div></div><div><h3>Conclusion</h3><div>Regular daytime napping, especially in older adults, may be protective against kidney function decline, whereas short sleep duration may accelerate eGFR decline in younger adults. Addressing sleep habits may aid in preventing chronic kidney disease (CKD).</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1733-1741"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223003","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Patient and Health Care Provider Perspectives of Cognitive Difficulties in Dialysis 透析患者和卫生保健提供者认知困难的观点
IF 5.7 2区 医学
Kidney International Reports Pub Date : 2025-06-01 DOI: 10.1016/j.ekir.2025.03.056
Frederick H.F. Chan , Phoebe X.H. Lim , Xiaoli Zhu , Jimmy Lee , Sabrina Haroon , Titus Wai Leong Lau , Allen Yan Lun Liu , Behram A. Khan , Jason C.J. Choo , Andrew Davenport , Konstadina Griva
{"title":"Patient and Health Care Provider Perspectives of Cognitive Difficulties in Dialysis","authors":"Frederick H.F. Chan ,&nbsp;Phoebe X.H. Lim ,&nbsp;Xiaoli Zhu ,&nbsp;Jimmy Lee ,&nbsp;Sabrina Haroon ,&nbsp;Titus Wai Leong Lau ,&nbsp;Allen Yan Lun Liu ,&nbsp;Behram A. Khan ,&nbsp;Jason C.J. Choo ,&nbsp;Andrew Davenport ,&nbsp;Konstadina Griva","doi":"10.1016/j.ekir.2025.03.056","DOIUrl":"10.1016/j.ekir.2025.03.056","url":null,"abstract":"<div><h3>Introduction</h3><div>Cognitive impairments (CIs) are common among patients on dialysis, compromising functional capacity, decision-making, and quality of life. Previous work is dominated by quantitative studies, leaving the everyday experience of these impairments and their practical implications poorly understood. This qualitative study explored patients’ and providers’ perspectives on the lived experience of cognitive difficulties, impacts on daily living and care delivery, and associated needs.</div></div><div><h3>Methods</h3><div>Semistructured interviews and focus groups were conducted with 29 patients on hemodialysis (HD) and 27 renal health care providers (HCPs), including nephrologists, renal nurses, and allied health professionals. Interview transcripts were analyzed using reflexive thematic analysis.</div></div><div><h3>Results</h3><div>The following 6 themes emerged: (i) manifestation of cognitive difficulties, (ii) perceived risk factors, (iii) impacts, (iv) attitudes toward cognitive difficulties, (v) strategies to manage cognitive difficulties, and (vi) unmet needs. Participants acknowledged dialysis patients’ CIs across multiple domains that compromised independence, increased caregiving burden, and interfered with routine care. HCPs highlighted health and safety risks associated with CIs, whereas patients viewed these impairments with varied attitudes, including normalization and denial. Patients and providers employed diverse strategies to manage cognitive difficulties, and identified a need for societal awareness, social support, and guidelines and tools to assess and manage cognitive difficulties.</div></div><div><h3>Conclusion</h3><div>CIs is a major issue in renal care that affects the well-being of patients and their families and complicates care provision. This study identified modifiable barriers at the individual and systemic levels to the identification and management of CIs in renal settings, suggesting potential targets for intervention.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"10 6","pages":"Pages 1771-1783"},"PeriodicalIF":5.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144223006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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