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    3517 results match your criteria Clinical Journal of the American Society of Nephrology CJASN[Journal]

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    Prospective Evaluation of Kidney Disease in Joubert Syndrome.
    Clin J Am Soc Nephrol 2017 Nov 16. Epub 2017 Nov 16.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: Joubert syndrome is a genetically heterogeneous ciliopathy associated with >30 genes. The characteristics of kidney disease and genotype-phenotype correlations have not been evaluated in a large cohort at a single center.

    Design, Setting, Participants, & Measurements: We evaluated 97 individuals with Joubert syndrome at the National Institutes of Health Clinical Center using abdominal ultrasonography, blood and urine chemistries, and DNA sequencing. Read More

    Prevalence of Novel MAGED2 Mutations in Antenatal Bartter Syndrome.
    Clin J Am Soc Nephrol 2017 Nov 16. Epub 2017 Nov 16.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: Mutations in the MAGED2 gene, located on the X chromosome, have been recently detected in males with a transient form of antenatal Bartter syndrome or with idiopathic polyhydramnios. The aim of this study is to analyze the proportion of the population with mutations in this gene in a French cohort of patients with antenatal Bartter syndrome.

    Design, Setting, Participants, & Measurements: The French cohort of patients with antenatal Bartter syndrome encompasses 171 families. Read More

    Mesangial C4d Deposits in Early IgA Nephropathy.
    Clin J Am Soc Nephrol 2017 Nov 16. Epub 2017 Nov 16.
    Vall d'Hebron Institut de Recerca, Barcelona, Spain.
    Background And Objectives: The prognostic value of mesangial C4d deposits in IgA nephropathy has been analyzed in patients with reduced GFR but has not been analyzed in those with normal kidney function. The main objective of the study was to analyze the prognostic value of C4d deposits and association with response to treatment in patients with IgA nephropathy and normal GFR.

    Design, Setting, Participants, & Measurements: This retrospective cohort study included 190 patients with idiopathic IgA nephropathy diagnosed by kidney biopsy between 1988 and 2005. Read More

    Phenotypic Spectrum of Children with Nephronophthisis and Related Ciliopathies.
    Clin J Am Soc Nephrol 2017 Nov 16. Epub 2017 Nov 16.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: Genetic heterogeneity and phenotypic variability are major challenges in familial nephronophthisis and related ciliopathies. To date, mutations in 20 different genes (NPHP1 to -20) have been identified causing either isolated kidney disease or complex multiorgan disorders. In this study, we provide a comprehensive and detailed characterization of 152 children with a special focus on extrarenal organ involvement and the long-term development of ESRD. Read More

    Association between Urine Ammonium and Urine TGF-β1 in CKD.
    Clin J Am Soc Nephrol 2017 Nov 16. Epub 2017 Nov 16.
    Department of Internal Medicine, University of Utah Health, Salt Lake City, Utah.
    Background And Objectives: Urinary ammonium excretion increases in response to nonvolatile acids to maintain normal systemic bicarbonate and pH. However, enhanced ammonia production promotes tubulointerstitial fibrosis in animal models. Therefore, a subset of individuals with CKD and normal bicarbonate may have acid-mediated kidney fibrosis that might be better linked with ammonium excretion than bicarbonate. Read More

    Whole Exome Sequencing of Patients with Steroid-Resistant Nephrotic Syndrome.
    Clin J Am Soc Nephrol 2017 Nov 10. Epub 2017 Nov 10.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Read More

    Prognosis of Patients with Cirrhosis and AKI Who Initiate RRT.
    Clin J Am Soc Nephrol 2017 Nov 9. Epub 2017 Nov 9.
    Division of Nephrology and.
    Background And Objectives: Literature on the prognosis of patients with cirrhosis who require RRT for AKI is sparse and is confounded by liver transplant eligibility. An update on outcomes in the nonlisted subgroup is needed. Our objective was to compare outcomes in this group between those diagnosed with hepatorenal syndrome and acute tubular necrosis, stratifying by liver transplant listing status. Read More

    Residual Kidney Function and Peritoneal Dialysis-Associated Peritonitis Treatment Outcomes.
    Clin J Am Soc Nephrol 2017 Nov 7. Epub 2017 Nov 7.
    Leslie Dan Faculty of Pharmacy, University of Toronto, Toronto, Ontario, Canada; and.
    Background And Objectives: Residual kidney function contributes to the clearance of antibiotics excreted by the kidneys, lowering the antibiotic concentration, which may adversely affect the treatment of peritoneal dialysis-associated peritonitis. The objective of our study was to examine the relationship between residual kidneyfunction and peritonitis treatment outcomes.

    Design, Setting, Participants, & Measurements: Our study included 181 participants who experienced 339 episodes of Gram-positive, Gram-negative, and culture-negative peritoneal dialysis-associated peritonitis at a single centerfrom 2003 to 2010. Read More

    Dysproteinemias and Glomerular Disease.
    Clin J Am Soc Nephrol 2017 Nov 7. Epub 2017 Nov 7.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
    Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. Read More

    Randomized, Controlled Trial of TRC101 to Increase Serum Bicarbonate in Patients with CKD.
    Clin J Am Soc Nephrol 2017 Nov 4. Epub 2017 Nov 4.
    Department of Internal Medicine, Yale School of Medicine, New Haven, Connecticut.
    Background And Objectives: Metabolic acidosis is common in patients with CKD and has significant adverse effects on kidney, muscle, and bone. We tested the efficacy and safety of TRC101, a novel, sodium-free, nonabsorbed hydrochloric acid binder, to increase serum bicarbonate in patients with CKD and metabolic acidosis.

    Design, Setting, Participants, & Measurements: One hundred thirty-five patients were enrolled in this randomized, double-blind, placebo-controlled, multicenter, in-unit study (designated the TRCA-101 Study). Read More

    BP Reduction, Kidney Function Decline, and Cardiovascular Events in Patients without CKD.
    Clin J Am Soc Nephrol 2017 Nov 3. Epub 2017 Nov 3.
    Masters in Medicine, Department of Endocrinology, Diabetes and Metabolism, São João Hospital Center, Porto, Portugal; and.
    Background And Objectives: In the Systolic Blood Pressure Intervention Trial (SPRINT), intensive systolic BP treatment (target <120 mm Hg) was associated with fewer cardiovascular events and higher incidence of kidney function decline compared with standard treatment (target <140 mm Hg). We evaluated the association between mean arterial pressure reduction, kidney function decline, and cardiovascular events in patients without CKD.

    Design, Setting, Participants, & Measurements: We categorized patients in the intensive treatment group of the SPRINT according to mean arterial pressure reduction throughout follow-up: <20, 20 to <40, and ≥40 mm Hg. Read More

    Urine Anion Gap to Predict Urine Ammonium and Related Outcomes in Kidney Disease.
    Clin J Am Soc Nephrol 2017 Nov 2. Epub 2017 Nov 2.
    Division of Nephrology-Hypertension, Department of Medicine and.
    Background And Objectives: Low urine ammonium excretion is associated with ESRD in CKD. Few laboratories measure urine ammonium, limiting clinical application. We determined correlations between urine ammonium, the standard urine anion gap, and a modified urine anion gap that includes sulfate and phosphate and compared risks of ESRD or death between these ammonium estimates and directly measured ammonium. Read More

    Efficacy and Long-Term Safety of C.E.R.A. Maintenance in Pediatric Hemodialysis Patients with Anemia of CKD.
    Clin J Am Soc Nephrol 2017 Nov 2. Epub 2017 Nov 2.
    Pediatric Nephrology, Heidelberg University Hospital, Heidelberg, Germany.
    Background And Objectives: The study was conducted to identify a conversion factor for switching from previous erythropoiesis-stimulating agents (ESAs) to continuous erythropoietin receptor activator-methoxy polyethylene glycol-epoetin beta (C.E.R. Read More

    Randomized Clinical Trial of Sevelamer Carbonate on Serum Klotho and Fibroblast Growth Factor 23 in CKD.
    Clin J Am Soc Nephrol 2017 Oct 26. Epub 2017 Oct 26.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: Epidemiologic studies suggest that higher serum phosphaturic hormone fibroblast growth factor 23 levels are associated with increase morbidity and mortality. The aim of the FGF23 Reduction Efficacy of a New Phosphate Binder in CKD Trial was to evaluate the effect of sevelamer carbonate on serum C-terminal fibroblast growth factor 23 levels in normophosphatemic patients with CKD stage 3b/4.

    Design, Setting, Participants, & Measurements: Patients with CKD, eGFR between 45 and 15 ml/min per 1. Read More

    Association of Kidney Transplantation with Survival in Patients with Long Dialysis Exposure.
    Clin J Am Soc Nephrol 2017 Oct 26. Epub 2017 Oct 26.
    Division of Nephrology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada; and
    Background And Objectives: Evidence that kidney transplantation is associated with better survival compared to dialysis stems from data in populations with short durations of dialysis exposure. Recent changes in allocation policy increase access to transplantation for patients with longer dialysis exposure. The objective of this study was to determine the association of transplantation with survival in patients with ≥10 years of dialysis treatment. Read More

    Left Ventricular Assist Devices and the Kidney.
    Clin J Am Soc Nephrol 2017 Oct 25. Epub 2017 Oct 25.
    Division of Kidney Diseases and Hypertension, Department of Medicine,
    Left ventricular assist devices (LVADs) are common and implantation carries risk of AKI. LVADs are used as a bridge to heart transplantation or as destination therapy. Patients with refractory heart failure that develop chronic cardiorenal syndrome and CKD often improve after LVAD placement. Read More

    Diabetes and CKD in the United States Population, 2009-2014.
    Clin J Am Soc Nephrol 2017 Oct 20. Epub 2017 Oct 20.
    Kidney Research Institute, Division of Nephrology, and.
    Background And Objectives: Diabetes is an important cause of CKD. However, among people with diabetes, it is unclear to what extent CKD is attributable to diabetes itself versus comorbid conditions, such as advanced age and hypertension. We examined associations of diabetes with clinical manifestations of CKD independent of age and BP and the extent to which diabetes contributes to the overall prevalence of CKD in the United States. Read More

    APOL1 Risk Variants, Incident Proteinuria, and Subsequent eGFR Decline in Blacks with Hypertension-Attributed CKD.
    Clin J Am Soc Nephrol 2017 Nov 19;12(11):1771-1777. Epub 2017 Oct 19.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: The natural history of kidney disease among blacks who carry the APOL1 high-risk variants varies, with only a subgroup progressing to ESRD. We aimed to determine whether the APOL1 risk variants are associated with incident proteinuria in the context of hypertension-attributed CKD, and whether subsequent kidney function decline after the onset of proteinuria differs by APOL1 risk status.

    Design, Setting, Participants, & Measurements: Using Cox models, we studied the association between APOL1 risk status and incident proteinuria (defined as a doubling of urine protein-to-creatinine ratio to a level ≥0. Read More

    A Discrete Choice Study of Patient Preferences for Dialysis Modalities.
    Clin J Am Soc Nephrol 2017 Oct 19. Epub 2017 Oct 19.
    School of Public Health and.
    Background And Objectives: Improved knowledge about factors that influence patient choices when considering dialysis modality could facilitate health care interventions to increase rates of home dialysis. We aimed to quantify the attributes of dialysis care and the tradeoffs that patients consider when making decisions about dialysis modalities.

    Design, Setting, Participants, & Measurements: We conducted a prospective, discrete choice experiment survey with random parameter logit analysis to quantify preferences and tradeoffs for attributes of dialysis treatment in 143 adult patients with CKD expected to require RRT within 12 months (predialysis). Read More

    Sociodemographic, Psychologic Health, and Lifestyle Outcomes in Young Adults on Renal Replacement Therapy.
    Clin J Am Soc Nephrol 2017 Oct 19. Epub 2017 Oct 19.
    School of Social and Community Medicine, University of Bristol, Bristol, United Kingdom.
    Background And Objectives: Young adults receiving RRT face additional challenges in life. The effect of established kidney failure on young adulthood is uncertain. We aimed to establish the psychosocial and lifestyle status of young adults receiving RRT. Read More

    Health Care Costs Associated with AKI.
    Clin J Am Soc Nephrol 2017 Nov 19;12(11):1733-1743. Epub 2017 Oct 19.
    Department of Medicine, University of Alberta, Edmonton, Alberta, Canada;
    Background And Objectives: An understanding of the health care resource use associated with AKI is needed to frame the investment and cost-effectiveness of strategies to prevent AKI and promote kidney recovery.

    Design, Setting, Participants, & Measurements: We assembled population-based cohort of adults hospitalized in Alberta between November of 2002 and March of 2009 without ESRD or an eGFR<15 ml/min per 1.73 m(2). Read More

    Re-Establishing Brain Networks in Patients with ESRD after Successful Kidney Transplantation.
    Clin J Am Soc Nephrol 2017 Oct 18. Epub 2017 Oct 18.
    Department of Medical Imaging and
    Background And Objectives: Cognition in ESRD may be improved by kidney transplantation, but mechanisms are unclear. We explored patterns of resting-state networks with resting-state functional magnetic resonance imaging among patients with ESRD before and after kidney transplantation.

    Design, Setting, Participants, & Measurements: Thirty-seven patients with ESRD scheduled for kidney transplantation and 22 age-, sex-, and education-matched healthy subjects underwent resting-state functional magnetic resonance imaging. Read More

    Thrombotic Microangiopathy and the Kidney.
    Clin J Am Soc Nephrol 2017 Oct 17. Epub 2017 Oct 17.
    National Renal Complement Therapeutics Centre, Newcastle upon Tyne, Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK;
    Thrombotic microangiopathy can manifest in a diverse range of diseases and is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, including AKI. It can be associated with significant morbidity and mortality, but a systematic approach to investigation and prompt initiation of supportive management and, in some cases, effective specific treatment can result in good outcomes. This review considers the classification, pathology, epidemiology, characteristics, and pathogenesis of the thrombotic microangiopathies, and outlines a pragmatic approach to diagnosis and management. Read More

    Infrequent Provision of Palliative Care to Patients with Dialysis-Requiring AKI.
    Clin J Am Soc Nephrol 2017 Nov 17;12(11):1744-1752. Epub 2017 Oct 17.
    Division of Nephrology, Stanford University School of Medicine, Palo Alto, California; and.
    Background And Objectives: The use of palliative care in AKI is not well described. We sought to better understand palliative care practice patterns for hospitalized patients with AKI requiring dialysis in the United States.

    Design, Setting, Participants, & Measurements: Using the 2012 National Inpatient Sample, we identified patients with AKI and palliative care encounters using validated International Classification of Diseases, Ninth Revision, Clinical Modification codes. Read More

    FGF23 and Left Ventricular Hypertrophy in Children with CKD.
    Clin J Am Soc Nephrol 2017 Oct 12. Epub 2017 Oct 12.
    Division of Nephrology, Department of Pediatrics, University of California, San Francisco, San Francisco, California
    Background And Objectives: High plasma concentration of fibroblast growth factor 23 (FGF23) is a risk factor for left ventricular hypertrophy (LVH) in adults with CKD, and induces myocardial hypertrophy in experimental CKD. We hypothesized that high FGF23 levels associate with a higher prevalence of LVH in children with CKD.

    Design, Setting, Participants, & Measurements: We performed echocardiograms and measured plasma C-terminal FGF23 concentrations in 587 children with mild-to-moderate CKD enrolled in the Chronic Kidney Disease in Children (CKiD) study. Read More

    Brief Mindfulness Meditation for Depression and Anxiety Symptoms in Patients Undergoing Hemodialysis: A Pilot Feasibility Study.
    Clin J Am Soc Nephrol 2017 Oct 12. Epub 2017 Oct 12.
    McGill Meditation and Mind-Body Medicine Research Clinic and Geri-PARTy Research Group and
    Background And Objectives: Up to 50% of patients undergoing hemodialysis suffer from symptoms of depression and/or anxiety. Access to traditional pharmacotherapies and psychotherapies for depression or anxiety in this patient population has been inadequate. The objective of this study was to investigate the feasibility and effectiveness of brief mindfulness meditation intervention for patients on hemodialysis with depression and anxiety symptoms. Read More

    A Markov Analysis of Screening for Late-Onset Cytomegalovirus Disease in Cytomegalovirus High-Risk Kidney Transplant Recipients.
    Clin J Am Soc Nephrol 2017 Oct 12. Epub 2017 Oct 12.
    Department of Medicine, Section of Decision Sciences, University of Pittsburgh, Pittsburgh, Pennsylvania.
    Background And Objectives: Management strategies are unclear for late-onset cytomegalovirus infection occurring beyond 6 months of antiviral prophylaxis in cytomegalovirus high-risk (cytomegalovirus IgG positive to cytomegalovirus IgG negative) kidney transplant recipients. Hybrid strategies (prophylaxis followed by screening) have been investigated but with inconclusive results. There are clinical and potential cost benefits of preventing cytomegalovirus-related hospitalizations and associated increased risks of patient and graft failure. Read More

    Disparity between Nephrologists' Opinions and Contemporary Practices for Community Follow-Up after AKI Hospitalization.
    Clin J Am Soc Nephrol 2017 Nov 12;12(11):1753-1761. Epub 2017 Oct 12.
    Division of Nephrology, Department of Medicine,
    Background And Objectives: Recent guidelines suggest that patients should be evaluated after AKI for resolution versus progression of CKD. There is uncertainty as to the role of nephrologists in this process. The objective of this study was to compare the follow-up recommendations from nephrologists with contemporary processes of care for varying scenarios of patients hospitalized with AKI. Read More

    Pooled Analysis of Multiple Crossover Trials To Optimize Individual Therapy Response to Renin-Angiotensin-Aldosterone System Intervention.
    Clin J Am Soc Nephrol 2017 Nov 11;12(11):1804-1813. Epub 2017 Oct 11.
    Department of Clinical Pharmacy and Pharmacology and.
    Background And Objectives: In the treatment of CKD, individual patients show a wide variation in their response to many drugs, including renin-angiotensin-aldosterone system inhibitors (RAASi). To investigate whether therapy resistance to RAASi can be overcome by uptitrating the dose of drug, changing the mode of intervention (with drugs from similar or different classes), or lowering dietary sodium intake, we meta-analyzed individual responses to different modes of interventions.

    Design, Setting, Participants, & Measurements: Randomized crossover trials were analyzed to assess correlation of individual responses to RAASi and nonsteroidal anti-inflammatory drugs (NSAIDs; n=395 patients). Read More

    Randomized Crossover Trial of Blood Volume Monitoring-Guided Ultrafiltration Biofeedback to Reduce Intradialytic Hypotensive Episodes with Hemodialysis.
    Clin J Am Soc Nephrol 2017 Nov 10;12(11):1831-1840. Epub 2017 Oct 10.
    Departments of Medicine.
    Background And Objectives: Intradialytic hypotension (IDH) is associated with morbidity. The effect of blood volume-guided ultrafiltration biofeedback, which automatically adjusts fluid removal rate on the basis of blood volume parameters, on the reduction of IDH was tested in a randomized crossover trial.

    Design, Setting, Participants, & Measurements: We performed a 22-week, single blind, randomized crossover trial in patients receiving maintenance hemodialysis who had >30% of sessions complicated by symptomatic IDH in five centers in Calgary, Alberta, Canada. Read More

    The Lived Experience of "Being Evaluated" for Organ Donation: Focus Groups with Living Kidney Donors.
    Clin J Am Soc Nephrol 2017 Nov 9;12(11):1852-1861. Epub 2017 Oct 9.
    Sydney School of Public Health, The University of Sydney, Sydney, New South Wales, Australia.
    Background And Objectives: Comprehensive evaluations are required to safeguard voluntarism and minimize harm to living kidney donors. This process is lengthy, invasive, and emotionally challenging, with up to one fifth of potential donors opting out. We aimed to describe donors' experiences of the evaluation process. Read More

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