Clément Triaille, Kevin Côté, Jérôme Coulombe, Maité Daroux, Clémence David, Marie-Louise Frémond, Elie Haddad, Naseer Khan, Victor Kokta, Anne-Laure Lapeyraque, Céline Lebas, María Luisa Matoses Ruipérez, Berta López Montesinos, Héloise Reumaux, Tiphanie P. Vogel, Marie-Paule Morin, Véronique Phan
{"title":"Kidney Transplant Outcomes in Coatomer Protein Complex Subunit Alpha (COPA) Syndrome: Report of Five Patients","authors":"Clément Triaille, Kevin Côté, Jérôme Coulombe, Maité Daroux, Clémence David, Marie-Louise Frémond, Elie Haddad, Naseer Khan, Victor Kokta, Anne-Laure Lapeyraque, Céline Lebas, María Luisa Matoses Ruipérez, Berta López Montesinos, Héloise Reumaux, Tiphanie P. Vogel, Marie-Paule Morin, Véronique Phan","doi":"10.1053/j.ajkd.2025.12.007","DOIUrl":"10.1053/j.ajkd.2025.12.007","url":null,"abstract":"<div><div>COPA (coatomer protein complex subunit α) syndrome is a rare inborn error of immunity associated with constitutive activation of type I interferon signaling. Organ inflammation and damage (eg, lungs, kidneys, joints) usually develops early in life. Kidney involvement occurs in a subset of COPA syndrome patients, with the potential for rapid progression to kidney failure. Through an international collaboration, we identified COPA syndrome patients who developed kidney failure and underwent a kidney transplantation. The aim of the present report was to describe (1) the kidney involvement at presentation, (2) the immunosuppressive strategies used before and after transplant, (3) the kidney graft outcomes, and (4) the evolution of the underlying COPA disease after transplantation. Five COPA syndrome patients were included. Kidney failure manifested at young age (range, 5-40 years). Kidney involvement was heterogenous between patients (ANCA-associated vasculitis–like disease, lupus or immune complex–mediated glomerulonephritis or overlapping phenotypes), and all had advanced histological damage at clinical presentation. Pulmonary disease of variable severity was also present in all patients. Kidney disease responded poorly to multiple lines of immunosuppression, justifying kidney transplantation between the ages of 7.5 and 42 years. After a follow-up of 10 months to 12 years, we report favorable graft outcomes in all patients (CKD stage 2-3b), although 1 patient developed polyomavirus infection (skin, kidney graft) and another died of progressive lung disease.</div></div>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"88 1","pages":"Pages 170-176"},"PeriodicalIF":9.4,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147615519","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":"Family Physicians’ Perspectives on Providing Living Kidney Donor Care","authors":"Chloe Wong-Mersereau , Saly El Wazze , Kathleen Gaudio , Katya Loban , Shaifali Sandal","doi":"10.1053/j.ajkd.2025.09.018","DOIUrl":"10.1053/j.ajkd.2025.09.018","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Living kidney donors (LKDs) perceive gaps in care along their donation journeys, such as poor coordination and communication between providers. Better integration of their primary care providers (PCPs) in their donation journey can help address these gaps. We captured the perspectives of Canadian PCPs to understand the challenges they perceive when caring for LKDs and their recommendations to address them.</div></div><div><h3>Study Design</h3><div>Qualitative descriptive design.</div></div><div><h3>Setting & Participants</h3><div>Semistructured in-depth interviews with 19 family physicians conducted until data saturation.</div></div><div><h3>Analytical Approach</h3><div>Thematic analysis.</div></div><div><h3>Results</h3><div>Participants highlighted several challenges with the current living kidney donation processes: (1) limited scope of LKD care in primary practice; (2) poor clarity surrounding roles and responsibilities before donation (perceived as a facilitator) and after donation (perceived as low-barrier and high-access resource, and providing soft psychosocial support); (3) variable understanding of the living kidney donation process; and 4) lack of support and resources (patient accessibility to primary care, communication challenges between providers, poor health record transmission, limited availability of mental health services, limited financial support for patients and providers). The participants had 3 key recommendations: (1) support PCPs with pragmatic approaches and on-demand resources; (2) empower LKDs to be active participants in their care; and (3) implement strategies to address LKD care provision deficiencies (primary care accessibility, developing a collaborative care model, improving communication between all stakeholders, dedicated and prolonged psychosocial support, and governmental prioritization).</div></div><div><h3>Limitations</h3><div>About half the sample had limited experience with LKD care; inability to recruit some PCPs such as nurse practitioners.</div></div><div><h3>Conclusions</h3><div>As co-providers of patient care during the predonation phase and the primary drivers of care in the postdonation period, PCPs are positioned to deliver high-quality care to LKDs, contribute to long-term data collection, and potentially improve LKD outcomes. This study outlines challenges PCPs perceive and possible approaches to address them.</div></div><div><h3>Plain-Language Summary</h3><div>Living kidney donors (LKDs) have reported gaps in their care, such as poor communication and a lack of coordination between doctors. Because family physicians play a key role in supporting donors before and after surgery, we interviewed 19 Canadian family doctors to hear their perspectives. Participants reported challenges such as unclear roles, limited scope of seeing LKDs in their practices, poor communication with transplant centers, and a lack of mental health and financial services. The","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 376-387.e1"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145696694","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}
Song Bai , Xin Yang , Qiuju Sheng , Qing Zhang , Li Liu , Shaomei Sun , Xing Wang , Ming Zhou , Qiyu Jia , Kun Song , Kaijun Niu , Yang Ding , Yang Xia
{"title":"Association of Fatty Liver Disease and the Risk of Nephrolithiasis: Findings From Two Prospective Cohort Studies","authors":"Song Bai , Xin Yang , Qiuju Sheng , Qing Zhang , Li Liu , Shaomei Sun , Xing Wang , Ming Zhou , Qiyu Jia , Kun Song , Kaijun Niu , Yang Ding , Yang Xia","doi":"10.1053/j.ajkd.2025.09.012","DOIUrl":"10.1053/j.ajkd.2025.09.012","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Evidence is limited regarding the associations of nonalcoholic fatty liver disease (NAFLD) and metabolic dysfunction–associated fatty liver disease (MAFLD) with the development of nephrolithiasis. This study assessed the associations of NAFLD and MAFLD with the risk of incident nephrolithiasis using data from 2 cohort studies conducted in the People’s Republic of China and the United Kingdom.</div></div><div><h3>Study Design</h3><div>Prospective cohort study.</div></div><div><h3>Setting & Participants</h3><div>26,490 participants without nephrolithiasis at baseline in the Tianjin Chronic Low-grade Systemic Inflammation and Health Cohort Study (TCLSIH) and 294,577 participants in the UK Biobank.</div></div><div><h3>Exposure</h3><div>Fatty liver diagnosed by abdominal ultrasonography in the TCLSIH and by the Hepatic Steatosis Index in the UK Biobank. NAFLD and MAFLD were defined according to standard clinical criteria in both cohorts.</div></div><div><h3>Outcome</h3><div>Nephrolithiasis was confirmed by ultrasonography in the TCLSIH and identified through ICD-10 and OPCS-4 in the UK Biobank.</div></div><div><h3>Analytical Approach</h3><div>Cox proportional hazards regression analysis was used to assess the relationship between exposures and incident nephrolithiasis.</div></div><div><h3>Results</h3><div>The TCLSIH and the UK Biobank recorded 806 and 2,743 new cases of nephrolithiasis during a median follow-up period of 4 and 12 years, respectively. Participants in both cohorts showed a significantly increased risk of nephrolithiasis in the setting of NAFLD (TCLSIH: HR, 1.69 [95% CI, 1.46-1.95]; UK Biobank: HR, 1.66 [95% CI, 1.53-1.79]) and MAFLD (TCLSIH: HR, 1.79 [95% CI, 1.55-2.08]; UK Biobank: HR, 1.54 [95% CI, 1.42-1.66]) after multivariable adjustments.</div></div><div><h3>Limitations</h3><div>Observational nature limits causal inferences; generalizability limited outside of the cohorts studied; limited diagnostic approaches to detect nephrolithiasis; unavailability of stone composition data.</div></div><div><h3>Conclusions</h3><div>Both NAFLD and MAFLD are associated with a higher risk of nephrolithiasis. The results suggest that NAFLD/MAFLD and their associated metabolic conditions may represent modifiable risk factors for nephrolithiasis.</div></div><div><h3>Plain-Language Summary</h3><div>Evidence on the association between nonalcoholic fatty liver disease (NAFLD) or metabolic dysfunction–associated fatty liver disease (MAFLD) and the risk of nephrolithiasis remains limited. In this study, we assessed whether NAFLD or MAFLD were associated with an increased risk of developing nephrolithiasis by analyzing data from 2 large prospective cohorts in the People’s Republic of China and the United Kingdom. Our findings demonstrated that individuals with NAFLD or MAFLD had a significantly higher risk of incident nephrolithiasis later in life. This increased risk remained consistent across different age ","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 364-375.e1"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145516260","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}
Carolyn T. Thorpe , Ryan P. Hickson , Xinhua Zhao , Sherrie L. Aspinall , Vimal K. Derebail , Binxin Cao , Alexa Ehlert , Joshua M. Thorpe , Ronald J. Falk , Susan L. Hogan
{"title":"Antimicrobial Prophylaxis in US Medicare Beneficiaries Receiving Immunosuppressants for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis","authors":"Carolyn T. Thorpe , Ryan P. Hickson , Xinhua Zhao , Sherrie L. Aspinall , Vimal K. Derebail , Binxin Cao , Alexa Ehlert , Joshua M. Thorpe , Ronald J. Falk , Susan L. Hogan","doi":"10.1053/j.ajkd.2025.08.019","DOIUrl":"10.1053/j.ajkd.2025.08.019","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 393-396"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145746719","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}
Jia Wei Teh , Sinead Stoneman , Michelle M. O’Shaughnessy
{"title":"Complement Inhibition in Immunoglobulin A Nephropathy: A Mini-Review","authors":"Jia Wei Teh , Sinead Stoneman , Michelle M. O’Shaughnessy","doi":"10.1053/j.ajkd.2025.07.023","DOIUrl":"10.1053/j.ajkd.2025.07.023","url":null,"abstract":"<div><div>IgA nephropathy (IgAN) is the most common immune-mediated glomerular disease worldwide. Advanced understanding of the role of complement in IgAN pathogenesis has motivated the development of complement inhibition as a therapeutic strategy. Iptacopan, a complement factor B inhibitor, is the first approved complement inhibitor for IgAN. Several other complement inhibitors are being studied in phase 2/3 clinical trials. How best to integrate complement inhibition into the evolving treatment paradigm for IgAN remains a challenge. This review provides an overview of the role of complement in the pathogenesis and progression of IgAN and summarizes current and emerging complement-targeted IgAN therapies.</div></div>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 412-421"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145801332","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}
Susan F. Massengill , Kenneth A. Andreoni , Keith A. Bellovich , Cheryl Courtlandt , Vimal K. Derebail , David L. Feldman , Gia J. Oh , John W. Sleasman , Kristina A. Bryant
{"title":"Improving Vaccination in People With CKD: Report From a National Kidney Foundation Working Group","authors":"Susan F. Massengill , Kenneth A. Andreoni , Keith A. Bellovich , Cheryl Courtlandt , Vimal K. Derebail , David L. Feldman , Gia J. Oh , John W. Sleasman , Kristina A. Bryant","doi":"10.1053/j.ajkd.2025.10.014","DOIUrl":"10.1053/j.ajkd.2025.10.014","url":null,"abstract":"<div><div>People with chronic kidney disease (CKD) are particularly vulnerable to vaccine-preventable infections. Therefore, the National Kidney Foundation organized a multidisciplinary working group, including people with CKD and family care partners, to develop recommendations to improve vaccination rates, vaccination effectiveness, and health care for the CKD population. A modified Delphi process was used to achieve consensus on the recommendations. Recommendations on vaccination in CKD patients were organized into these categories: (1) patient preferences; (2) assessing the vaccination status of patients with CKD; (3) assessing vaccine response in patients with CKD; (4) vaccination of immunocompromised patients; (5) vaccination in posttransplant patients; (6) vaccinations in patients with CKD before international travel; (7) vaccination of household contacts of patients with CKD; (8) assessing vaccine efficacy and safety in clinical trials with patients with CKD; (9) training and resources for health care teams; (10) training of health care teams for discussions with patients; (11) role of technology in promoting and improving vaccination; and (12) advocacy and policy considerations for promoting and improving vaccination rates. The working group’s recommendations should improve communication between patients and health care clinicians, inclusion of people with CKD in vaccine trials, and use of existing clinical guidelines as well as generate educational resources and training materials for CKD patients of all ages and health care professionals.</div></div>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 402-411"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145823890","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":"Anticoagulation Decisions in Patients With Kidney Failure Requiring Hemodialysis: Caught Between a Clot and a Hard Place","authors":"An S. De Vriese","doi":"10.1053/j.ajkd.2026.01.004","DOIUrl":"10.1053/j.ajkd.2026.01.004","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 292-294"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146776031","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}
Susan L. Ziolkowski , Jin Long , Thomas L. Nickolas , Shaun Bender , Simin Goral , Babette S. Zemel , Chamith S. Rajapakse , Thomas Dienemann , Mary B. Leonard
{"title":"Corticosteroid, Parathyroid Hormone, and Body Composition Associations With Bone Density and Structure Following Kidney Transplantation","authors":"Susan L. Ziolkowski , Jin Long , Thomas L. Nickolas , Shaun Bender , Simin Goral , Babette S. Zemel , Chamith S. Rajapakse , Thomas Dienemann , Mary B. Leonard","doi":"10.1053/j.ajkd.2025.10.005","DOIUrl":"10.1053/j.ajkd.2025.10.005","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Kidney transplant (KT) ameliorates the underlying abnormalities contributing to skeletal fragility in chronic kidney disease, but fracture rates increase after transplant. This study sought to assess the impact of changes in body composition and mineral metabolism on bone mineral density (BMD) and structure following kidney KT.</div></div><div><h3>Study Design</h3><div>24-month prospective cohort study.</div></div><div><h3>Setting & Participants</h3><div>60 incident KT recipients and 361 healthy controls aged 20-60 years.</div></div><div><h3>Outcomes</h3><div>Tibia volumetric BMD (vBMD) and cortical dimensions measured using peripheral quantitative computed tomography and areal BMD (aBMD), appendicular lean mass index (ALMI), and fat mass index measured using dual-energy X-ray absorptiometry.</div></div><div><h3>Analytical Approach</h3><div>Analytical Approach: Outcomes were converted to sex-specific <em>z</em> scores for age and compared with those in 361 controls. Quasi–least-squares regression models identified correlates of change.</div></div><div><h3>Results</h3><div>At baseline, ALMI, trabecular and cortical vBMD, cortical thickness, and total hip, femoral neck, and ultradistal radius aBMD were lower in KT recipients versus controls (<em>P</em> ≤ 0.003). During the first 6 months after KT, ALMI, fat mass index, and body mass index (BMI) increased (<em>P</em> < 0.001), whereas tibia trabecular vBMD and lumbar spine aBMD decreased (<em>P</em> ≤ 0.005). Cortical vBMD increased from 6 months onward (<em>P</em> < 0.001) in association with decreasing parathyroid hormone levels (<em>P</em> = 0.004). Cortical thickness decreased between 6 and 24 months (<em>P</em> = 0.002) because of a loss of endocortical bone. Total hip and femoral neck aBMD remained stable for 6 months, then improved through 24 months (<em>P</em> ≤ 0.02). Ultradistal radius aBMD decreased throughout the study (<em>P</em> ≤ 0.003). Higher BMI and ALMI were associated with gains in hip and spine aBMD and trabecular vBMD (all <em>P</em> ≤ 0.02). Corticosteroid dose was negatively associated with changes in spine and hip aBMD and cortical and trabecular vBMD (all <em>P</em> ≤ 0.02). Higher bone turnover marker levels and loss of cortical vBMD and thickness were associated with increases in serum calcium concentrations (<em>P</em> ≤ 0.03).</div></div><div><h3>Limitations</h3><div>Not generalizable to older KT recipients.</div></div><div><h3>Conclusions</h3><div>Skeletal deficits in KT recipients largely stabilize or improve beyond 6 months, with the exception of progressive cortical thinning. Strategies are needed to preserve cortical bone before and after KT. Gains in BMI and ALMI may improve bone health in the weight-bearing skeleton following KT.</div></div><div><h3>Plain-Language Summary</h3><div>We enrolled 60 adults at the time of kidney transplant (KT) and compared measures of bone and muscle mass versus those in 361 healt","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 298-312.e1"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145472770","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}
Kevin Wang , Zoe Bamber , Lonnie Pyne , Arrti A. Bhasin , Michael Walsh , Rathika Krishnasamy , Glenn M. Chertow , Roberto Pecoits-Filho , Scott Klarenbach , Stephanie Thompson , David Collister
{"title":"Representation of Older Patients Receiving Maintenance Dialysis in Randomized Controlled Trials: A Meta-epidemiologic Study","authors":"Kevin Wang , Zoe Bamber , Lonnie Pyne , Arrti A. Bhasin , Michael Walsh , Rathika Krishnasamy , Glenn M. Chertow , Roberto Pecoits-Filho , Scott Klarenbach , Stephanie Thompson , David Collister","doi":"10.1053/j.ajkd.2025.09.023","DOIUrl":"10.1053/j.ajkd.2025.09.023","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"87 3","pages":"Pages 397-401"},"PeriodicalIF":8.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145746700","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}