American Journal of Kidney Diseases最新文献

{"title":"A Patient With HIV and Nephrotic Range Proteinuria: A Quiz","authors":"Ali Shah , Meghan Gwinn , Sayna Norouzi , Zainab Obaidi","doi":"10.1053/j.ajkd.2025.05.006","DOIUrl":"10.1053/j.ajkd.2025.05.006","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"86 3","pages":"Pages A15-A18"},"PeriodicalIF":8.2,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144863496","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":"Missed Opportunities: Rethinking Primary Care Engagement in Dialysis-Dependent Populations.","authors":"Brittany N Watson, Clarissa J Diamantidis","doi":"10.1053/j.ajkd.2025.07.001","DOIUrl":"10.1053/j.ajkd.2025.07.001","url":null,"abstract":"","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144870851","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":"Autosomal Dominant Tubulointerstitial Kidney Disease: A Review","authors":"Anthony J. Bleyer MD MS, Kendrah O. Kidd PhD, Martina Živná PhD, Stanislav Kmoch PhD","doi":"10.1053/j.ajkd.2025.05.015","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.05.015","url":null,"abstract":"Autosomal dominant tubulointerstitial kidney disease (ADTKD) is an increasingly recognized rare condition with three primary characteristics: autosomal dominant inheritance, bland urinary sediment (absence of hematuria and proteinuria) and chronic kidney disease (CKD) leading to kidney failure (need for renal replacement therapy or kidney transplantation) between 20 and 80 years of age, with a mean age of kidney failure of approximately 45 years. Pathogenic variants in <ce:italic>UMOD</ce:italic>, <ce:italic>MUC1</ce:italic>, <ce:italic>REN</ce:italic>, and <ce:italic>APOA4</ce:italic> have been identified as causative in ADTKD families. Prior to 2000, ADTKD was only diagnosed clinically, and described in less than 50 families. However, with the advent of genetic testing and a better understanding of this condition, ADTKD is being increasingly recognized and is the third most common monogenic cause of kidney failure. The purpose of this review is to provide an understanding of the clinical characteristics of ADTKD, its subtypes and a practical approach to diagnosis and management for clinical nephrologists.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"85 2 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144899177","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":"Metabolic Bariatric Surgery in Kidney Transplant Candidates","authors":"Aleksandra Kukla MD, Ricardo V. Cohen MD, Allon N. Friedman MD","doi":"10.1053/j.ajkd.2025.06.015","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.06.015","url":null,"abstract":"Patients with obesity are the largest and fastest-growing demographic among individuals with renal failure in the United States. Transplant centers often reject patients with high body mass index due to risks of post-transplant complications. Metabolic bariatric surgery, the most efficient and durable weight loss method, can facilitate a timely kidney transplant listing and improve obesity-related comorbidities in a relatively safe manner. In this review, we identify kidney transplant candidates who are likely to benefit from MBS, discuss the potential risks of this procedure, and propose post-surgery monitoring and management to optimize safety and maximize the benefits of this therapy.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"107 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144899176","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":"Eculizumab Prophylaxis for Systematic Rechallenging Gemcitabine in Gemcitabine-Induced Thrombotic Microangiopathy: A Case Report.","authors":"Api Chewcharat, Mike Wang, Shruti Gupta, Harshabad Singh, Raad B Chowdhury","doi":"10.1053/j.ajkd.2025.06.014","DOIUrl":"10.1053/j.ajkd.2025.06.014","url":null,"abstract":"<p><p>Gemcitabine-induced thrombotic microangiopathy (GITMA) is a rare yet devastating complication in patients receiving gemcitabine, especially at cumulative doses above 20,000 mg/m². We report the case of a 72-year-old woman with metastatic pancreatic adenocarcinoma who developed severe thrombotic microangiopathy (TMA) during gemcitabine and nab-paclitaxel therapy. Her initial presentation included thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. A kidney biopsy confirmed TMA and given the high likelihood this was related to gemcitabine, therapy was discontinued, and the patient was transitioned to FOLFIRINOX. Although the patient stabilized with improved renal and hematologic parameters, her cancer progressed radiographically. Given her good performance status and the need for effective tumor control, a decision was made to rechallenge with gemcitabine under prophylactic terminal complement blockade. She received eculizumab 900 mg intravenously for four weekly doses, followed by reintroduction of gemcitabine and nab-paclitaxel. Over four treatment cycles given during a three-month period, the patient showed no signs of GITMA recurrence, maintaining stable renal function and normal hemolysis markers. This case demonstrates the potential role of eculizumab in preventing recurrent GITMA, allowing continued gemcitabine-based therapy for metastatic pancreatic adenocarcinoma. Further research is warranted to define optimal dosing and timing of eculizumab and long-term outcomes.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144870850","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":"Optimal Care to Promote Flourishing: Articulating the Purpose of Pediatric Nephrology.","authors":"Taylor R House, Ryan J Coller, Aaron Wightman","doi":"10.1053/j.ajkd.2025.06.013","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.06.013","url":null,"abstract":"<p><p>Pediatric nephrology is at a pivotal moment. As treatment advances have improved survival and clinical outcomes, families and clinicians increasingly call for a more holistic view of what it means to care well for children with kidney disease. Professional society mission statements, which emphasize a commitment to \"optimal care for children with kidney disease,\" offer a foundation upon which to articulate that broader vision. In this perspective, we discuss what we think optimal care isn't - an exclusive focus on cure, survival, and adult outcomes; and what optimal care is - promoting flourishing of children with kidney disease and their families. Rooted in bioethics and gaining traction in medicine, a shift to promoting flourishing has significant implications for pediatric nephrology in clinical care, research and advocacy, and professionalism. To navigate this critical juncture in pediatric nephrology and improve the outcomes most important to patients, families, and clinicians, optimal care must center on flourishing. Now is the time to expand our focus from kidney disease well-treated to lives well-lived.</p>","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144870852","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":"Exercise-Based Prehabilitation for Kidney Transplant Candidates: The FRAILMar Randomized Controlled Trial.","authors":"María José Pérez-Sáez,Elena Muñoz-Redondo,Andrea Morgado-Pérez,Lou Delcros-Forestier,Anna Bach,Anna Faura,Dolores Redondo,Betty Chamoun,Carla Burballa,Anna Buxeda,Marta Crespo,Ester Marco,Julio Pascual, ","doi":"10.1053/j.ajkd.2025.07.003","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.07.003","url":null,"abstract":"RATIONALE & OBJECTIVEKidney transplantation (KT) is the preferred treatment for kidney failure but carries significant post-transplant risks, particularly for frail patients. This study evaluated the effects of an exercise-based prehabilitation program on exercise capacity, muscle function, and muscle size among KT candidates.STUDY DESIGNAnalysis of functional outcomes within the FRAILMar study, a randomized controlled trial.SETTING & PARTICIPANTS121 KT candidates on the KT waiting list.INTERVENTIONThe intervention group participated in a prehabilitation program comprising 24 exercise sessions (1 hour, 3 times per week for 8 weeks), the control group received standard care. Randomization was stratified by frailty status.OUTCOMESThe primary outcome was exercise capacity assessed by maximal workload during a cardiopulmonary exercise test. Secondary outcomes included peripheral muscle function, respiratory muscle function, muscle size, and changes in frailty status.RESULTSThe mean age of the 121 individuals who were randomized was 63.4 years, 76% were men, and 40% were frail (Fried phenotype ≥ 2). 106 patients completed the prehabilitation program and, among them, compared to standard care, prehabilitation significantly improved exercise capacity (+12.8 watts; [95% CI: 3.4 to 22.2]; p=0.008), handgrip strength (+1.8 kg; [95% CI: 0.7 to 2.8]; p<0.001), and rectus femoris thickness (+1.2 mm; [95% CI: 0.3 to 2.0]; p=0.007). Frail patients showed significant improvements across most measures, demonstrating potential benefits for this subgroup.LIMITATIONSThis analysis was limited by a short follow-up period and the risk of type I error due to multiple comparisons, even though outcomes were pre-specified.CONCLUSIONSAn 8-week exercise-based prehabilitation program may improve KT candidates' exercise capacity, muscle function, and muscle size, effects also observed among frail patients. These findings may inform future research in this area and the evaluation of the value of standardized prehabilitation protocols.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"107 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857692","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":"Intronic and Coding Genetic Variants in Autosomal Recessive Polycystic Kidney Disease Among Israeli Bedouins of Arabian Peninsula Ancestry.","authors":"Nadav Agam,Ohad Wormser,Ari Biller,Noam Hadar,Vadim Dolgin,Ofek Freund,Matan M Jean,Amit Safran,Tomer Poleg,Bibi Kanengisser-Pines,Rebekka Kebesch-Assi,Masha Mazor-Oring,Osnat Cohen-Zontag,Michal Zmudjak-Olevson,Shlomit Ben-Menachem,Dror Ben-Ruby,Omer Shlomovitz,Asaf Vivante,Benjamin Dekel,Ohad S Birk,Ruth Schreiber","doi":"10.1053/j.ajkd.2025.06.011","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.06.011","url":null,"abstract":"RATIONALE & OBJECTIVEAutosomal recessive polycystic kidney disease (ARPKD) is typically caused by biallelic PKHD1 mutations. However, some clinically diagnosed patients remain without a molecular diagnosis. We aimed to identify cryptic pathogenic variants in Israeli Bedouin patients of Arabian Peninsula ancestry with ARPKD.STUDY DESIGNCase series.SETTING &PARTICIPANTSTwelve Bedouin patients of five unrelated families of Arabian Peninsula ancestry with ARPKD whose causative mutation was unknown despite standard genetic analyses.OBSERVATIONSWhole-genome sequencing (WGS), including short- and long-read platforms, followed by bioinformatics filtration and transcript analysis, identified a pathogenic PKHD1 variant confirmed by functional analysis: a deep-intronic PKHD1 variant (6:51,757,883 C>T (hg38), ENST00000340994.4: c.8643-2945 G>A) was identified in 12 patients with ARPKD within 5 families of Israeli Negev Bedouins originating from the Arabian Peninsula. Variant segregation within affected kindreds was consistent with autosomal recessive inheritance. cDNA analysis of urine-derived tubular kidney epithelial cells confirmed aberrant splicing with pseudo-exon inclusion of 121 bp, introducing a premature stop codon. This variant was found in 2 of 100 ethnically matched Bedouin controls (carrier rate 1:50). Among all known PKHD1 splicing-disrupting variants, this mutation received the lowest and most benign scores across splicing prediction tools. We also delineate 12 other PKHD1 variants in Negev Bedouins.LIMITATIONSFunctional studies were limited to urinary-derived epithelial cells, small cohort size.CONCLUSIONSWe describe a deep-intronic truncating PKHD1 variant, as a common founder mutation causing ARPKD in Israeli Bedouins of Arabian Peninsula ancestry. This PKHD1 mutation caused pseudo-exon inclusion through a donor-gain mechanism, without directly introducing a splice site. These findings highlight the diagnostic utility of WGS and transcript analysis in identifying pathogenic non-coding ARPKD variants.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"1221 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857694","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":"Can KDOQI Intervention Rate Target Be Met With Drug-Coated Balloons for Dysfunctional Arteriovenous Fistulas: Post-hoc Analysis of the IN.PACT AV Access Randomized Clinical Trial.","authors":"Anjana Gopal,Hiroaki Haruguchi,Andrew Holden,Robert Lookstein,Jeremiah Menk,Patrick Wang,Charmaine E Lok","doi":"10.1053/j.ajkd.2025.05.013","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.05.013","url":null,"abstract":"RATIONALE & OBJECTIVEA key Kidney Disease Outcomes Quality Initiative (KDOQI) 2019 Vascular Access Guidelines target is to have ≤3 percutaneous or surgical interventions per year to maintain arteriovenous fistula (AVF) patency. We hypothesized first, that the number of interventions to maintain long-term AVF patency conducted in a rigorous clinical trial could be adequately documented and compared against KDOQI targets, and second, that the use of drug-coated balloons (DCB) could reduce the rate of thrombosis and interventions to maintain AVF patency more than standard uncoated balloon angioplasty (PTA).STUDY DESIGNPost-hoc analysis of the IN.PACT AV Access randomized clinical trial.SETTING & PARTICIPANTSOutpatient adult hemodialysis patients with previously matured AVFs that had de novo or non-stented re-stenotic lesions enrolled between April 2017 and May 2018 in the United States, Japan and New Zealand.INTERVENTIONPatients were randomized to receive percutaneous transluminal angioplasty with the IN.PACT AV DCB or PTA.OUTCOMESRate of interventions to maintain access target lesion and access circuit patency and rate of access circuit thrombosis were calculated in both intervention groups and compared against the KDOQI 2019 guideline targets.RESULTSOf the 330 participants randomized, 133 patients completed their 3-year visit. The number of interventions per AVF-year to maintain access circuit patency through 36 months was 1.39 for the DCB group and 1.66 for the PTA group (rate difference -0.28 [-0.47, -0.08]; P = 0.01). The access circuit thrombosis rate was 0.041 in the DCB group and 0.069 in the PTA group (rate difference -0.028 [-0.065, 0.0082]; P=0.1).LIMITATIONSExclusion of AVF with prior thrombosis and small sample size at 36 months.CONCLUSIONSBoth DCB and PTA groups met the KDOQI targets of ≤3 percutaneous or surgical interventions per year to maintain AVF patency. The need for reinterventions to maintain patency and thrombosis rate was reduced with the use of DCB compared with PTA through 36 months.FUNDINGNone.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"9 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857695","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":"Delivery of Palliative Care in the Last Year of Life to Individuals Receiving Maintenance Dialysis: A Population-Level Cross-Sectional Study.","authors":"Michael J Bonares,Adrianna Bruni,Samantha Yoo,Lyndsay Harrison,Wenshan Li,Robert Talarico,Peter Tanuseputro,S Vanita Jassal","doi":"10.1053/j.ajkd.2025.06.012","DOIUrl":"https://doi.org/10.1053/j.ajkd.2025.06.012","url":null,"abstract":"RATIONALE & OBJECTIVELittle is known about physician-delivered palliative care and the sociodemographic and clinical factors associated with its utilization for individuals undergoing maintenance dialysis. This study described physician-delivered palliative care in the last year of life and evaluated the factors associated with utilization in this patient population.STUDY DESIGNPopulation-level cross-sectional study.SETTING & PARTICIPANTSIndividuals undergoing maintenance dialysis who died between April 2012 and March 2020 in Ontario, Canada.EXPOSURESSociodemographic factors (age, sex, immigration status, neighborhood-level income quintile, rurality, health region), comorbidities (heart failure, cirrhosis, pulmonary disease, dementia, malignancy), and kidney-specific factors (dialysis modality, access, duration; and prior kidney transplantation).OUTCOMEPhysician-delivered palliative care.ANALYTICAL APPROACHDescriptive statistics characterizing physician-delivered palliative care. Logistic regression evaluating the factors associated with utilization.RESULTS18,452 decedents who underwent maintenance dialysis were included (median age=71 years; 61.1% male). 52.2% received physician-delivered palliative care in the last year of life starting a median of 23 days before death. 65% died in hospital and 12% at home. Palliative care was initiated by a family physician for 68% of those receiving physician-delivered palliative care. It was delivered in the clinic setting for 44.8%, and through a generalist-only model for 46%. The odds of receiving palliative care were higher in the setting of a malignancy, dementia, or cirrhosis; and were lower among those who were recent immigrants, lived in lower-income neighborhoods, and resided in less dense/more rural regions. The odds of dying in hospital were lower among those who received palliative care, especially if it was delivered at home.LIMITATIONSExclusion of palliative care provided by non-physician providers; and inability to infer causal associations or to comment on the goal-concordance of end-of-life care.CONCLUSIONSOver half of decedents who underwent maintenance dialysis received physician-delivered palliative care in the last year of life, albeit typically starting 3 weeks before death. This may indicate a perception that palliative care is exclusively for end-of-life care. More research is required to identify barriers to delivering equitable palliative care.","PeriodicalId":7419,"journal":{"name":"American Journal of Kidney Diseases","volume":"45 1","pages":""},"PeriodicalIF":13.2,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857696","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}