Clara Schott, Victoria Lebedeva, Cambrie Taylor, Saeed Abumelha, Pavel S Roshanov, Dervla M Connaughton
{"title":"Utility of Genetic Testing in Adults with CKD: A Systematic Review and Meta-Analysis.","authors":"Clara Schott, Victoria Lebedeva, Cambrie Taylor, Saeed Abumelha, Pavel S Roshanov, Dervla M Connaughton","doi":"10.2215/CJN.0000000000000564","DOIUrl":"10.2215/CJN.0000000000000564","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":"20 1","pages":"101-115"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142962510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Surrogate Clinical End Points in Studies of APOL1-Associated Kidney Disease: Just in Time.","authors":"Akinlolu O Ojo","doi":"10.2215/CJN.0000000628","DOIUrl":"10.2215/CJN.0000000628","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":"3-5"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142830201","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Dialysis for Decades, Then My Heart Breaks: A Patient Perspective.","authors":"Erich Ditschman","doi":"10.2215/CJN.0000000631","DOIUrl":"10.2215/CJN.0000000631","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":"1-2"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142814856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Peritoneal Dialysis Care for People with Diabetes, Polycystic Kidney Disease, or Advanced Liver Disease.","authors":"Shailesh Agarwal, Laura Gillis, Martin Wilkie","doi":"10.2215/CJN.0000000000000420","DOIUrl":"10.2215/CJN.0000000000000420","url":null,"abstract":"<p><p>People treated with peritoneal dialysis (PD) often have complicating conditions that require careful management. Three such conditions are reviewed in this article-diabetes mellitus, polycystic kidney disease, and chronic liver disease. Each of these conditions requires an understanding of both its effect on the delivery of the PD and the effect of the PD on the condition itself. In diabetes, glucose absorption from the dialysate complicates metabolic control and affects salt and water management and patient outcome. There is particular benefit in clinical care being delivered through a multidisciplinary team that involves both kidney and diabetes experts. In relation to polycystic kidney disease, a key issue is the potential for increased intraperitoneal pressure due to the combined effect of the enlarged polycystic organs and the presence of the dialysis solution, and therefore, the PD prescription requires to be managed with a particular focus on limiting that pressure. For patients with liver disease, key issues include nutritional support because PD can add to protein losses already consequent on the liver disease itself. Considered approaches are required to manage ascites and reduce infection risk and the potential for hernias and leaks to develop. Mortality in this group is unfortunately high-however, PD may present a better management option than hemodialysis in many patients-particularly in those where the liver disease is complicated by low BP, clotting abnormalities, or troublesome ascites. Overall, the choice to use PD in patients with these complicating conditions should be based on shared decision making with the patient and their family members informed by high-quality information in which risks, benefits, and management strategies are clearly presented.</p>","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":"139-146"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139378730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Improving Patient Selection in Clinical Trials: Should We Start Using the Pretrial eGFR Decline as Risk Enrichment Criterion?","authors":"Niels Jongs, Hiddo J L Heerspink","doi":"10.2215/CJN.0000000630","DOIUrl":"10.2215/CJN.0000000630","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":"6-8"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142814858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alix T Rosenberg, Carina Flaherty, Amanda H Anderson, Lawrence J Appel, Josef Coresh, Jiang He, James P Lash, Celina Liu, Panduranga S Rao, Jonathan Taliercio, Aditya Surapaneni, Morgan E Grams
{"title":"Surrogate End Points in Apolipoprotein L1 - Associated Kidney Disease : Evaluation in Three Cohorts.","authors":"Alix T Rosenberg, Carina Flaherty, Amanda H Anderson, Lawrence J Appel, Josef Coresh, Jiang He, James P Lash, Celina Liu, Panduranga S Rao, Jonathan Taliercio, Aditya Surapaneni, Morgan E Grams","doi":"10.2215/CJN.0000000000000575","DOIUrl":"10.2215/CJN.0000000000000575","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":"23-30"},"PeriodicalIF":8.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142584828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ultrafiltration Tolerance and Improving Outcomes with Continuous Renal Replacement Therapies.","authors":"Gonzalo Ramírez-Guerrero,Claudio Ronco,Mitchell Rosner","doi":"10.2215/cjn.0000000650","DOIUrl":"https://doi.org/10.2215/cjn.0000000650","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":"34 1","pages":""},"PeriodicalIF":9.8,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142887476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vincent Moolenaar,Rosanne H N Prins,Jarom Heijmans,Frederike J Bemelman,Rik Westland
{"title":"Proteinuria Remission in Recurrent Focal Segmental Glomerulosclerosis after Autologous Stem Cell Transplantation: a Patient and Clinicians' Perspectives.","authors":"Vincent Moolenaar,Rosanne H N Prins,Jarom Heijmans,Frederike J Bemelman,Rik Westland","doi":"10.2215/cjn.0000000651","DOIUrl":"https://doi.org/10.2215/cjn.0000000651","url":null,"abstract":"","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":"41 1","pages":""},"PeriodicalIF":9.8,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142887477","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yijiang Chen, Bangchen Wang, Dawit Demeke, Fan Fan, Celine Berthier, Laura Mariani, Kyle Lafata, Lawrence Holzman, Jeffrey Hodgin, Andrew Janowczyk, Laura Barisoni, Anant Madabhushi
{"title":"Clinical Relevance of Computational Pathology Analysis of Interplay Between Kidney Microvasculature and Interstitial Microenvironment.","authors":"Yijiang Chen, Bangchen Wang, Dawit Demeke, Fan Fan, Celine Berthier, Laura Mariani, Kyle Lafata, Lawrence Holzman, Jeffrey Hodgin, Andrew Janowczyk, Laura Barisoni, Anant Madabhushi","doi":"10.2215/CJN.0000000597","DOIUrl":"https://doi.org/10.2215/CJN.0000000597","url":null,"abstract":"<p><strong>Background: </strong>Interstitial fibrosis and tubular atrophy (IFTA), and density and shape of peritubular capillaries (PTCs), are independently prognostic of disease progression. This study aimed to identify novel digital biomarkers of disease progression and assess the clinical relevance of the interplay between a variety of PTC characteristics and their microenvironment in glomerular diseases.</p><p><strong>Methods: </strong>A total of 344 NEPTUNE/CureGN participants were included: 112 minimal change disease, 134 focal segmental glomerulosclerosis, 61 membranous nephropathy, and 37 IgA nephropathy. A PAS-stained whole slide image per patient was manually segmented for cortex, pre- and mature IFTA. Interstitial fractional space (IFS) was computationally quantified. A deep-learning model was applied to segment PTCs. Spatial and shape PTC pathomic features (230) were extracted from the cortex, IFTA, and non-IFTA sub-regions. Participants were divided into training and testing datasets (1:1). Univariate models incorporating IFTA subregions, and IFS-PTC density were constructed. LASSO regression models were used to identify the top PTC features associated with disease progression stratified by IFTA and non-IFTA sub-regions. Machine learning models were built using the top PTC features in IFTA and non-IFTA sub-regions to prognosticate disease progression.</p><p><strong>Results: </strong>PTC density in pre+mature IFTA and IFS, shape features in pre+mature IFTA, and spatial architecture features in the non-IFTA regions associated with disease progression. The machine learning generated risk scores showed a significant association with disease progression on the independent testing set.</p><p><strong>Conclusion: </strong>We uncovered previously underrecognized digital biomarkers of disease progression and the clinical relevance of the complex interplay between the status of the PTCs and the interstitial microenvironment.</p>","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":8.5,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142883178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Constantin A Wolff, Valeria Aiello, Elhussein A E Elhassan, Carlotta Cristalli, Sarah Lerario, Alexandro Paccapelo, Francesca Ciurli, Francesca Montanari, Amalia Conti, Katherine Benson, Marco Seri, Carolin B Brigl, Julia S Münster, Nicola Sciascia, Sebastian Kursch, Jonathan de Fallois, Gaetano La Manna, Kai-Uwe Eckardt, Nina Rank, Bernt Popp, Ria Schönauer, Peter J Conlon, Irene Capelli, Jan Halbritter
{"title":"Integrated use of Autosomal Dominant Polycystic Kidney Disease Prediction Tools and Risk Prognostication.","authors":"Constantin A Wolff, Valeria Aiello, Elhussein A E Elhassan, Carlotta Cristalli, Sarah Lerario, Alexandro Paccapelo, Francesca Ciurli, Francesca Montanari, Amalia Conti, Katherine Benson, Marco Seri, Carolin B Brigl, Julia S Münster, Nicola Sciascia, Sebastian Kursch, Jonathan de Fallois, Gaetano La Manna, Kai-Uwe Eckardt, Nina Rank, Bernt Popp, Ria Schönauer, Peter J Conlon, Irene Capelli, Jan Halbritter","doi":"10.2215/CJN.0000000625","DOIUrl":"https://doi.org/10.2215/CJN.0000000625","url":null,"abstract":"<p><strong>Background: </strong>Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of kidney failure. Specific treatment is indicated upon observed or predicted rapid progression. For the latter, risk stratification tools have been developed independently based on either total kidney volume or genotyping as well as clinical variables. This study aimed to improve risk prediction by combining both imaging and clinical-genetic scores.</p><p><strong>Methods: </strong>We conducted a retrospective multi-center cohort study of 468 patients diagnosed with ADPKD. Clinical, imaging, and genetic data were analyzed for risk prediction. We defined rapid disease progression as an estimated glomerular filtration rate (eGFR) slope ≥3 ml/min/1.73m2/year over two years, Mayo imaging classification (MIC) 1D-1E, or a Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD) score of ≥7 points. Using MIC, PROPKD, and Rare Exome Variant Ensemble Learner (REVEL) scores, several combined models were designed to develop a new classification with improved risk stratification. Primary endpoints were the development of advanced chronic kidney disease (aCKD) stages G4-G5, longitudinal changes in eGFR, and clinical variables such as hypertension or urological events. Statistically, logistic regression, survival, Receiver Operating Characteristic (ROC) analyses, linear mixed models, and Cox proportional hazards models were used.</p><p><strong>Results: </strong>PKD1-genotype (p <0.001), MIC class 1E (p <0.001), early-onset hypertension (p <0.001) and early-onset urological events (p =0.003) correlated best with rapid progression in multivariable analysis. While the MIC showed satisfactory specificity (77%), the PROPKD was more sensitive (59%). Among individuals with an intermediate risk in one of the scores, integration of the other score (combined scoring) allowed for more accurate stratification.</p><p><strong>Conclusions: </strong>The combined use of both risk scores was associated with higher ability to identify rapid progressors and resulted in a better stratification, notably among intermediate risk patients.</p>","PeriodicalId":50681,"journal":{"name":"Clinical Journal of the American Society of Nephrology","volume":" ","pages":""},"PeriodicalIF":8.5,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}