The ability to generate 3D models of the developing human kidney via the directed differentiation of pluripotent stem cells – termed kidney organoids - has been hailed as a major advance in experimental nephrology. While these provide an opportunity to interrogate human development, model specific kidney diseases, facilitate drug screening and even deliver bioengineered tissue, most of these prophetic

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In the past decade, advances in the validation of surrogate end points for chronic kidney disease (CKD) progression have heightened interest in evaluating therapies in early CKD. In December 2020, the National Kidney Foundation sponsored a scientific workshop in collaboration with the US Food and Drug Administration (FDA) to explore patient, provider, and payor perceptions of the value of treating

Focal and segmental glomerulosclerosis (FSGS) is a histological lesion due to many causes including rare mutations of podocyte related genes. Recently, it has been found that TBC1D8B mutations can lead to early-onset FSGS and steroid-resistant nephrotic syndrome (NS) by influencing endocytic and recycling of nephrin. Here, we report a 19-year-old Chinese patient with NS and normal renal function. He

Calciphylaxis is a life-threatening complication most often associated with chronic kidney disease that occurs due to the deposition of calcium in dermal and adipose microvasculature. However, this condition may also be seen in patients with acute kidney injury. The high morbidity and mortality associated with calciphylaxis highlight the importance to correctly diagnose and treat this condition. However

Background Ciliogenesis-associated kinase 1 (CILK1) is a ciliary gene that localizes in primary cilia and regulates ciliary transport. Mutations in CILK1 cause various ciliopathies. However, the pathogenesis of CILK1-deficient kidney disease is unknown.Methods To examine whether CILK1 deficiency causes PKD accompanied by abnormal cilia, we generated mice with deletion of Cilk1 in cells of the renal

Background Secondary hyperparathyroidism (SHP) is a common complication of CKD that increases morbidity and mortality. In experimental SHP, increased parathyroid hormone (PTH) expression is due to enhanced PTH mRNA stability, mediated by changes in its interaction with stabilizing AUF1 and destabilizing KSRP. The isomerase Pin1 leads to KSRP dephosphorylation, but in SHP parathyroid Pin1 activity is

Background and objectives Recovery of kidney function after the start of maintenance dialysis can occur, but data on the incidence and risk factors for restarting dialysis after recovery of kidney function in this population are limited.Design, setting, participants, & measurements We conducted a retrospective study of adult Medicare beneficiaries who started dialysis between 2005 and 2015 according

The 13th International Podocyte Conference was held in Manchester and Online from the 28th-30th of July 2021. Originally planned for 2020, this biannual meeting was postponed by a year due to the COVID-19 pandemic and proceeded as an innovative hybrid meeting. In addition to in-person attendance, online registration was offered, and this attracted 490 conference registrations in total. As a Podocyte

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Background Mutations in SLC37A4, which encodes the intracellular glucose transporter G6PT, cause the rare glycogen storage disease type 1b (GSD1b). A long-term consequence of GSD1b is kidney failure, which requires KRT. The main protein markers of proximal tubule function, including NaPi2A, NHE3, SGLT2, GLUT2, and AQP1, are downregulated as part of the disease phenotype.Methods We utilized an inducible

Background Emerging evidence indicates that epigenetic modulation of gene expression plays a key role in the progression of autosomal dominant polycystic kidney disease (ADPKD). However, the molecular basis for how the altered epigenome modulates transcriptional responses, and thereby disease progression in ADPKD, remains largely unknown.Methods Kidneys from control and ADPKD mice were examined for

Background Compared with the general Medicare population, patients with ESKD have worse quality metrics for end-of-life care, including a higher percentage experiencing hospitalizations and in-hospital deaths and a lower percentage referred to hospice. We developed a Concurrent Hospice and Dialysis Program in which patients may receive palliative dialysis alongside hospice services. The Program aims

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The kidney is a structurally and functionally complex organ responsible for the control of water, ion and other solutes homeostasis. Moreover, the kidneys excrete metabolic waste products and produce hormones such as renin and erythropoietin. The functional unit of the kidney is the nephron, which is composed by a serial arrangement of a filter unit called the renal corpuscle and several tubular segments

Background and objectives The etiology of chronic kidney disease of unclear etiology, also known as Mesoamerican nephropathy, remains unclear. We investigated potential etiologies for Mesoamerican nephropathy in an immigrant dialysis population.Design, setting, participants, & measurements Migrants with Mesoamerican nephropathy kidney failure (n=52) were identified by exclusion of known causes of kidney

Rationale & Objective Patients with advanced chronic kidney disease (CKD) have been reported to experience profound psychosocial distress. Other work has established that patients with CKD from marginalized populations (including individuals who on the basis of race often face racism and related discrimination, termed “racialization”) experience health care inequities. Given limited information on

Rationale and Objective The safety and efficacy of long-term exercise training in reducing physical functional loss in older adults with advanced CKD and co-morbidity is uncertain. Study Design Multi-center, parallel group, randomized controlled trial. Settings and Participants Adults 55 years and older with CKD stage G3b-4 enrolled from centers in Baltimore and Boston. Intervention Twelve months of

Background Observational studies suggest that adequate dietary potassium intake (90–120 mmol/day) may be renoprotective, but the effects of increasing dietary potassium and the risk of hyperkalemia are unknown.Methods This is a prespecified analysis of the run-in phase of a clinical trial in which 191 patients (age 68±11 years, 74% males, 86% European ancestry, eGFR 31±9 ml/min per 1.73 m2, 83% renin-angiotensin

Background and objectives Daprodustat is a hypoxia-inducible factor prolyl hydroxylase inhibitor (HIF-PHI) being investigated for the treatment of anemia of CKD. In this noninferiority trial, we compared daprodustat administered three times weekly with epoetin alfa (epoetin) in patients on prevalent hemodialysis switching from a prior erythropoiesis-stimulating agent (ESA).Design, setting, participants

Background The embryonic renal stroma consists of multiple molecularly distinct cell subpopulations, the functional significance of which is largely unknown. Previous work has demonstrated that the transcription factors YAP and TAZ play roles in the development and morphogenesis of the nephrons, collecting ducts, and nephron progenitor cells.Methods In embryonic mouse kidneys, we identified a subpopulation

The glomerular vascular pole is the gate for the afferent and efferent arterioles and mesangial cells and a frequent location of peripolar cells with an unclear function. It has been studied in definitive detail for >30 years, and functionally interrogated in the context of signal transduction from the macula densa to the mesangial cells and afferent arteriolar smooth muscle cells from 10 to 20 years

Background: Ureteral obstruction is marked by disappearance of the vasopressin-dependent water channel aquaporin-2 (AQP2) in the renal collecting duct and polyuria upon reversal. Most studies of unilateral ureteral obstruction (UUO) models have examined late time points, obscuring the early signals that trigger loss of AQP2. Methods: We performed RNA-Seq on microdissected rat cortical collecting ducts

Rare cases of IgG-dominant immune complex mediated GN (ICGN) demonstrate immunoglobulin (Ig) subclass restriction without light chain restriction. Some of these cases may represent proliferative glomerulonephritis with monotypic immunoglobulin deposits (PGNMID) in which monotypic Ig is obscured by coexisting polytypic Ig. However, rigorous demonstration of this possibility is lacking to date. Here

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Background and objectives There are no standardized benchmarks to measure productivity and compensation of transplant nephrologists in the United States, and consequently, criteria set for general nephrologists are often used.Design, setting, participants, & measurements A web-based survey was sent to 809 nephrologists who were members of the American Society of Transplantation to gather data on measures

Kidney disease is an underappreciated medical complication of anorexia nervosa. Further research is needed to clarify the mechanisms that drive kidney disease in patients who restrict caloric intake or engage in purging. Education and awareness are also needed, because kidney disease is often under-recognized by these patients and their clinicians.

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Two recent randomized trials provide evidence to guide the management of sepsis. The CLASSIC trial reports that restrictive fluid therapy has no mortality benefit compared to a standard regimen in patients with septic shock, whereas the LOVIT trial reports that high-dose intravenous vitamin C might be harmful in patients with severe sepsis.