American Journal of Kidney Diseases

RATIONALE & OBJECTIVE: Alport syndrome (AS) is a common genetic kidney disease accounting for approximately 2% of patients receiving kidney replacement therapy (KRT). It is caused by pathogenic variants in COL4A3, COL4A4 or COL4A5 genes. The aim of this study was to evaluate the clinical and genetic spectrum of patients with autosomal dominant Alport syndrome (ADAS). STUDY DESIGN: Retrospective cohort study. SETTING & PARTICIPANTS: 82 families (252 patients) with ADAS were studied...

No abstract text is available yet for this article.

RATIONALE & OBJECTIVE: Patients with CKD are at elevated risk of metabolic acidosis due to impaired net acid excretion (NAE). Identifying early markers of acidosis may guide prevention in chronic kidney disease (CKD). This study compared NAE in participants with and without CKD, as well as the NAE, blood pressure (BP), and metabolomic response to bicarbonate supplementation. STUDY DESIGN: Randomized order, cross-over study with controlled feeding. SETTING & PARTICIPANTS: Participants consisted of 8 patients with CKD (estimated glomerular filtration rate 30-59mL/min/1...

OBJECTIVE: Regional citrate anticoagulation (RCA) is the preferred anticoagulation method for continuous kidney replacement therapy (CKRT) recommended by the KDIGO guidelines. Limited availability of calcium-free solutions often imposes challenges to the implementation of RCA for CKRT (RCA-CKRT). The principal purpose of this study was to characterize the outcomes of RCA-CKRT using calcium-containing solutions. STUDY DESIGN: Retrospective cohort study. SETTING & PARTICIPANTS: We evaluated the safety and efficacy of RCA-CKRT with calcium-containing dialysate and replacement fluid used for 128 patients...

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RATIONALE & OBJECTIVE: An early change in proteinuria is considered a reasonably likely surrogate endpoint in IgA nephropathy (IgAN), and can be used as basis for accelerated approval of therapies with verification in a post-marketing confirmatory trial. GFR slope is a recently validated surrogate endpoint for CKD progression and may be considered as the endpoint used for verification. We undertook a meta-analysis of clinical trials in IgAN to compare treatment effects on change in proteinuria to change in eGFR slope...

Osteoporosis is defined as "a skeletal disorder of compromised bone strength predisposing to an increased risk of fracture". However, based on bone histology, the term osteoporosis is only part of a spectrum of skeletal complications that includes osteomalacia and the various forms of renal osteodystrophy of chronic kidney disease-mineral and bone disorder (CKD-MBD). In addition, a label, "kidney-induced osteoporosis", has been proposed, despite the fact that CKD does not cause osteoporosis, as is diagnosed histologically...

There is increasing recognition of monoclonal gammopathy as a cause of proliferative glomerulonephritis (GN), including cases in which glomerular deposition of monoclonal immunoglobulin (mIg) is demonstrated. Recently, proliferative GN with mIg deposits (PGNMID) has incorporated a light chain (LC) variant of the disease (termed PGNMID-LC). Intriguingly, glomerular co-deposition of complement C3 is found in addition to monotypic LC, implying complement activation via the alternative pathway (AP). We present a unique case of proliferative GN in a 42-year old man who presented with nephrotic syndrome and was found to have κ-LC multiple myeloma...

Post-transplant lymphoproliferative disorder (PTLD) is one of the most feared complications following kidney transplantation. Over a 10-year period, the risk of PTLD in kidney transplant recipients is 12-fold higher than in matched non-transplanted population. Given the number of kidney transplantations performed, kidney transplant recipients experiencing PTLD outnumber other organ transplant recipients who develop PTLD. EBV infection is one of the most important risk factors for PTLD, although 40% of PTLDs are not EBV-associated in contemporary series...

Since maintenance hemodialysis (HD) first became available in the United States in 1962, there has been tremendous growth in the population of patients with kidney failure. HD has become a routine treatment carried out in outpatient clinics, hospitals, nursing facilities, and in patients' homes. Although it is a complex procedure, HD is quite safe. Serious complications are uncommon due to the use of modern HD machines and water treatment systems as well as the development of strict protocols to monitor various aspects of the HD treatment...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

RATIONALE AND OBJECTIVES: Interpersonal communication skills and professionalism competencies are difficult to assess among nephrology trainees. We developed a formative "Breaking Bad News" simulation and implemented a study in which nephrology fellows were assessed with regard to their skills in providing counseling to simulated patients confronting the need for kidney replacement therapy (KRT) or kidney biopsy. STUDY DESIGN: Observational study of communication competency in the setting of preparing for KRT for kidney failure, for KRT for acute kidney injury (AKI), or for kidney biopsy...

Evaluation of patients for kidney transplant candidacy is a comprehensive process that involves a detailed assessment of medical and surgical issues, psychosocial factors, and patients' physical and cognitive abilities with an aim of balancing the benefits of transplantation and potential risks of surgery and long-term immunosuppression. There is considerable variability among transplant centers in their approach to evaluation and decision-making regarding transplant candidacy. The 2020 KDIGO (Kidney Disease: Improving Guidelines Outcome) clinical practice guideline on the evaluation and management of candidates for kidney transplantation provides practice recommendations that can serve as a useful reference guide to transplant professionals...

Autosomal Dominant Polycystic Kidney Disease is the most common inherited cause of kidney failure accounting for 5-10% of cases. Predicting which patient would progress rapidly to kidney failure is critical to assess the risk-benefit ratio of any intervention and consider early initiation of long-term kidney protective measures which will maximize the cumulative benefit of slowing down the disease progression. Surrogate prognostic biomarkers are required to predict future decline in kidney function. Clinical, genetic, environmental, epigenetic and radiological factors have been studied as predictors of progression to kidney failure in ADPKD...

RATIONALE & OBJECTIVE: Percutaneous arteriovenous fistulae (pAVF) are created by establishing a proximal forearm anastomosis and offer a safe and reliable vascular access. This study compares the Ellipsys-pAVF with a proximal forearm Gracz-type surgical AVF (sAVF), chosen for comparison as it is constructed at the same anatomical site. STUDY DESIGN: Retrospective study of prospectively collected clinical data. SETTING: & Participants: All vascular access procedures conducted during a 34-month period were reviewed...

RATIONALE AND OBJECTIVE: The associations of glomerular markers of kidney disease (eGFR and albuminuria) with frailty and cognition are well established. However, the relationship of kidney tubular injury and dysfunction with frailty and cognition are unknown. STUDY DESIGN: Observational cross-sectional study; SETTING: & Participants: 2,253 participants with eGFR < 60 ml/min/1.73m2 in the Systolic Blood Pressure Intervention Trial EXPOSURES: Eight urine biomarkers: Interleukin-18 [IL-18, pg/mL], kidney injury molecule-1 [KIM-1, pg/mL], neutrophil gelatinase-associated lipocalin [NGAL, ng/mL], chitinase-3-like protein-1 [YKL-40, pg/mL], monocyte chemoattractant protein-1 [MCP-1, pg/mL], α-1 microglobulin [α1M mg/g], beta-2 microglobulin [β2M ng/mL], and uromodulin [Umod ng/mL]...

Kidney disease continues to manifest stark U.S. racial inequities, revealing the entrenchment of racism and bias within multiple facets of society including in our institutions, practices, norms, and beliefs. In this perspective, we synthesize theory and evidence to describe why an understanding of race and racism is integral to kidney care, providing examples of how kidney health disparities manifest interpersonal and structural racism. We then describe racialized medicine and color-blind approaches as well as their pitfalls, offering in their place suggestions to embed anti-racism and equity lens into our practice...

No abstract text is available yet for this article.

No abstract text is available yet for this article.