American Journal of Kidney Diseases最新文献

{"title":"Lupus Nephritis Patterns and Response to Type I Interferon in Patients With DNASE1L3 Variants: Report of Three Cases.","authors":"Stefano Volpi, Maria L Angelotti, Giulia Palazzini, Giulia Antonelli, Fiammetta Ravaglia, Federica Garibotto, Anna Agrusti, Alice Grossi, Alberto Magnasco, Giovanni M Rossi, Carmela Errichiello, Francesco Peyronel, Elisa Buti, Lorenzo Lodi, Gian M Ghiggeri, Paola Romagnani, Augusto Vaglio","doi":"10.1053/j.ajkd.2024.05.014","DOIUrl":"10.1053/j.ajkd.2024.05.014","url":null,"abstract":"<p><p>DNASE1L3 is an extracellular nuclease that digests chromatin released from apoptotic cells. DNASE1L3 variants impair the enzyme function, enhance autoantibody production and type I interferon (IFN-I) responses, and cause different autosomal recessive phenotypes ranging from hypocomplementemic urticarial vasculitis syndrome to full-blown systemic lupus erythematosus (SLE). Kidney involvement in patients with DNASE1L3 variants is poorly characterized. Herein, we describe the clinical course of 3 children with monogenic SLE due to DNASE1L3 variants who developed refractory glomerulonephritis leading to kidney failure. They had different renal histopathological patterns (ie, membranous, endocapillary, and extracapillary glomerulonephritis and thrombotic microangiopathy), all belonging to the lupus nephritis (LN) spectrum. One patient had a mixed phenotype, showing an overlap between SLE and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Using immunofluorescence, we detected glomerular expression of the IFN-I-induced human myxovirus resistance protein 1 (MXA), which was particularly evident in glomerular endothelial cells. Two of the patients had increased expression of interferon-stimulated genes in the peripheral blood, and all 3 patients had reduced serum DNAse activity. Our findings suggest that DNASE1L3-related glomerulonephritis can be included in the spectrum of IFN-I-mediated kidney disorders and provide the rationale for IFN-I-directed therapies in order to improve the poor outcome of this rare condition.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141764749","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":"Sex and the Relationship Between Cardiometabolic Risk Factors and Estimated GFR Decline: A Population-Based Cohort Study.","authors":"Michael K Sullivan, Jennifer S Lees, Brenda M Rosales, Rachel Cutting, Melanie L Wyld, Mark Woodward, Angela C Webster, Patrick B Mark, Nicole De La Mata","doi":"10.1053/j.ajkd.2024.05.007","DOIUrl":"10.1053/j.ajkd.2024.05.007","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Rationale & objective: &lt;/strong&gt;Females have a higher prevalence of chronic kidney disease (CKD) than males but are less likely to be treated with kidney replacement therapy (KRT). We studied the interaction between sex and the association of cardiometabolic risk factors for the decline in kidney function over time.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study design: &lt;/strong&gt;A population-based cohort study.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting & participants: &lt;/strong&gt;1,127,731 adults living in Wales, United Kingdom, within the Secure Anonymised Information Linkage Databank.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Exposure: &lt;/strong&gt;Sex and risk factors including age, estimated glomerular filtration rate (eGFR), cardiometabolic conditions, smoking, and socioeconomic deprivation. These risk factors were defined using primary care records.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Outcome: &lt;/strong&gt;The yearly declines in eGFR and the risk of incident kidney failure defined as long-term KRT and/or sustained eGFR&lt;15mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt;.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Analytical approach: &lt;/strong&gt;Linear mixed effects models and Cox proportional hazards analysis.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The average decline in eGFR at age≤73 years was equal in males and females. After age 73 years, eGFR decline was faster in males than females, particularly for males with heart failure (males-1.22mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year [95% CI, -1.25 to-1.20] vs females-0.87mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year [95% CI, -0.89 to-0.85]) and current smokers (males-1.58mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year [95% CI, -1.60 to-1.55] vs females-1.27mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year [95% CI, -1.29 to-1.25]). Socioeconomic deprivation was one of the most impactful risk factors on eGFR decline among females aged&gt;73 years, whereas cardiometabolic risk factors were more important among males. Older females at baseline were less likely to develop incident kidney failure than older males (P for age&lt;0.001).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;Study of people who were almost exclusively White and who had blood laboratory test data. Reliance on creatinine-based eGFR. Albuminuria and body mass index data were incomplete.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;The eGFR decline was faster in males than in females, especially in the setting of heart failure and smoking. Socioeconomic deprivation was an important risk factor associated with eGFR decline, particularly for females. further work is required to explore less well-recognized risk factors, but these findings may inform clinical management strategies of CKD overall and within sex-specific groups.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Plain-language summary: &lt;/strong&gt;Kidney function is known to decline at a faster rate among males than females. This study incorporated blood laboratory test results from the routine care of 1.1 million adults living in the United Kingdom and found that the decline in kidney function associated with risk factors varied by sex. Before and at the age of 73 years, the decline in kidney function was similar between males and femal","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141756535","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":"New Insights into Vitamin D Metabolism in Kidney Disease and Transplant","authors":"","doi":"10.1053/j.ajkd.2024.06.003","DOIUrl":"10.1053/j.ajkd.2024.06.003","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0272638624008400/pdfft?md5=a41dd097a80b827684c2892548e01469&pid=1-s2.0-S0272638624008400-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141750872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation and Management of Resistant Hypertension: Core Curriculum 2024","authors":"","doi":"10.1053/j.ajkd.2024.04.009","DOIUrl":"10.1053/j.ajkd.2024.04.009","url":null,"abstract":"<div><p>Resistant hypertension is defined as blood pressure above goal despite confirmed adherence to 3 first-line antihypertensive agents or when blood pressure is controlled with 4 or more medications at maximal or maximally tolerated doses. In addition to meeting these criteria, identifying patients with true resistant hypertension requires both accurate in-office blood pressure measurement as well as excluding white coat effects through out-of-office blood pressure measurements. Patients with resistant hypertension are at higher risk for adverse cardiovascular events and are more likely to have a potentially treatable secondary cause contributing to their hypertension. Effective treatment of resistant hypertension includes ongoing lifestyle modifications and collaboration with patients to detect and address barriers to optimal medication adherence. Pharmacologic treatment should prioritize optimizing first-line, once daily, longer acting medications followed by the stepwise addition of second-, third-, and fourth-line agents as tolerated. Physicians should systematically evaluate for and address any underlying secondary causes. A coordinated, multidisciplinary team approach including clinicians with experience in treating resistant hypertension is essential. New treatment options, including both pharmacologic and device-based therapies, have recently been approved, and more are in the pipeline; their optimal role in the management of resistant hypertension is an area of ongoing research.</p></div>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0272638624007935/pdfft?md5=20bb34f3c168434ee3e8f3e3462314a6&pid=1-s2.0-S0272638624007935-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Association of Plasma and Urine Uromodulin With Cardiovascular Disease in Persons With Hypertension and CKD.","authors":"Jesse C Ikeme, Rebecca Scherzer, Pranav S Garimella, Stein I Hallan, Ronit Katz, Michelle M Estrella, Joachim H Ix, Michael G Shlipak","doi":"10.1053/j.ajkd.2024.05.012","DOIUrl":"10.1053/j.ajkd.2024.05.012","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733306","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":"More Exams, More Problems: Do We Really Need a New Accreditation System for Transplant Nephrology?","authors":"Samira S Farouk, Anshul Bhalla, Meera Harhay, Laila Lakhani, Luis Sanchez Russo, Scott Sanoff, Manpreet Samra, Matthew A Sparks, Niralee Patel, Fasika Tedla, Anju Yadav, Roslyn B Mannon","doi":"10.1053/j.ajkd.2024.05.009","DOIUrl":"10.1053/j.ajkd.2024.05.009","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733305","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":"Visceral Abdominal Adiposity and Autosomal Dominant Polycystic Kidney Disease Progression: One More Step Toward Identifying Useful Biomarkers and Characterizing the Disease Metabolic Links","authors":"","doi":"10.1053/j.ajkd.2024.05.005","DOIUrl":"10.1053/j.ajkd.2024.05.005","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0272638624008412/pdfft?md5=1a25a4393ec9a0ce70ae8ee2a11bc019&pid=1-s2.0-S0272638624008412-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiovascular and Kidney Outcomes of Non-Diabetic CKD by Albuminuria Severity: Findings From the CRIC Study.","authors":"Rachel Shulman, Wei Yang, Debbie L Cohen, Peter P Reese, Jordana B Cohen","doi":"10.1053/j.ajkd.2024.05.008","DOIUrl":"10.1053/j.ajkd.2024.05.008","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Rationale & objective: &lt;/strong&gt;The clinical trajectory of normoalbuminuric chronic kidney disease (CKD), particularly in the absence of diabetes, has not yet been well-studied. This study evaluated the association of kidney and cardiovascular outcomes with levels of albuminuria in a cohort of patients with nondiabetic CKD.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Study design: &lt;/strong&gt;Prospective cohort study.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting & participants: &lt;/strong&gt;1,463 adults with nondiabetic CKD without known glomerulonephritis and diagnosed with hypertensive nephrosclerosis or unknown cause of CKD participating in the Chronic Renal Insufficiency Cohort (CRIC) Study.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Exposure: &lt;/strong&gt;Albuminuria stage at study entry.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Outcome: &lt;/strong&gt;Primary outcome: Composite kidney (halving of estimated glomerular filtration rate [eGFR], kidney transplantation, or dialysis), Secondary outcomes: (1) eGFR slope, (2) composite cardiovascular disease events (hospitalization for heart failure, myocardial infarction, stroke, or all-cause death), (3) all-cause death.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Analytical approach: &lt;/strong&gt;Linear mixed effects and Cox proportional hazards regression analyses.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Lower levels of albuminuria were associated with female sex and older age. For the primary outcome, compared with normoalbuminuria, those with moderate and severe albuminuria had higher rates of kidney outcomes (adjusted hazard ratio [AHR], 3.3 [95% CI, 2.4-4.6], and AHR, 8.6 [95% CI, 6.0-12.0], respectively) and cardiovascular outcomes (AHR, 1.5 [95% CI, 1.2-1.9], and AHR, 1.5 [95% CI, 1.1-2.0], respectively). Those with normoalbuminuria (&lt;30μg/mg; n=863) had a slower decline in eGFR (-0.46mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year) compared with those with moderate (30-300μg/mg, n=372; 1.41mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year) or severe albuminuria (&gt;300μg/mg, n=274; 2.63mL/min/1.73m&lt;sup&gt;2&lt;/sup&gt; per year). In adjusted analyses, kidney outcomes occurred, on average, sooner among those with moderate (8.6 years) and severe (7.3 years) albuminuria compared with those with normoalbuminuria (9.3 years) whereas the average times to cardiovascular outcomes were similar across albuminuria groups (8.2, 8.1, and 8.6 years, respectively).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;Self-report of CKD etiology without confirmatory kidney biopsies; residual confounding.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Participants with normoalbuminuric nondiabetic CKD experienced substantially slower CKD progression but only modestly lower cardiovascular risk than those with high levels of albuminuria. These findings inform the design of future studies investigating interventions among individuals with lower levels of albuminuria.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Plain-language summary: &lt;/strong&gt;Diabetes and hypertension are the leading causes of chronic kidney disease (CKD). Urine albumin levels are associated with cardiovascular and kidney disease outcomes among individuals with CKD. However, previous studies o","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733303","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":"Advancing Genetic Testing in Kidney Diseases: Report From a National Kidney Foundation Working Group.","authors":"Nora Franceschini, David L Feldman, Jonathan S Berg, Whitney Besse, Alexander R Chang, Neera K Dahl, Rasheed Gbadegesin, Martin R Pollak, Hila Milo Rasouly, Richard J H Smith, Cheryl A Winkler, Ali G Gharavi","doi":"10.1053/j.ajkd.2024.05.010","DOIUrl":"10.1053/j.ajkd.2024.05.010","url":null,"abstract":"<p><p>About 37 million people in the United States have chronic kidney disease, a disease that encompasses multiple causes. About 10% or more of kidney diseases in adults and as many as 70% of selected chronic kidney diseases in children are expected to be explained by genetic causes. Despite the advances in genetic testing and an increasing understanding of the genetic bases of certain kidney diseases, genetic testing in nephrology lags behind other medical fields. More understanding of the benefits and logistics of genetic testing is needed to advance the implementation of genetic testing in chronic kidney diseases. Accordingly, the National Kidney Foundation convened a Working Group of experts with diverse expertise in genetics, nephrology, and allied fields to develop recommendations for genetic testing for monogenic disorders and to identify genetic risk factors for oligogenic and polygenic causes of kidney diseases. Algorithms for clinical decision making on genetic testing and a road map for advancing genetic testing in kidney diseases were generated. An important aspect of this initiative was the use of a modified Delphi process to reach group consensus on the recommendations. The recommendations and resources described herein provide support to nephrologists and allied health professionals to advance the use of genetic testing for diagnosis and screening of kidney diseases.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141733302","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":"The Impact of Obesity on Glomerular Diseases Remains to be Determined","authors":"","doi":"10.1053/j.ajkd.2024.06.004","DOIUrl":"10.1053/j.ajkd.2024.06.004","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":null,"pages":null},"PeriodicalIF":9.4,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0272638624008424/pdfft?md5=c6bd296b2dc76816268a052dcb34977b&pid=1-s2.0-S0272638624008424-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141632395","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}