Browse Articles

  • Consensus Statement | | open

    This Consensus Statement developed on behalf of the Network for Early Onset Cystic Kidney Disease provides guidance on counselling, diagnosing and monitoring children with autosomal dominant polycystic kidney disease based on current evidence and a multi-stakeholder discussion of ethical issues.

    • Charlotte Gimpel
    • , Carsten Bergmann
    • , Detlef Bockenhauer
    • , Luc Breysem
    • , Melissa A. Cadnapaphornchai
    • , Metin Cetiner
    • , Jan Dudley
    • , Francesco Emma
    • , Martin Konrad
    • , Tess Harris
    • , Peter C. Harris
    • , Jens König
    • , Max C. Liebau
    • , Matko Marlais
    • , Djalila Mekahli
    • , Alison M. Metcalfe
    • , Jun Oh
    • , Ronald D. Perrone
    • , Manish D. Sinha
    • , Andrea Titieni
    • , Roser Torra
    • , Stefanie Weber
    • , Paul J. D. Winyard
    •  & Franz Schaefer
  • Review Article |

    Antimicrobial resistance is an emergent global problem and patients with chronic kidney disease have some of the highest rates of colonization and infection with antibiotic-resistant bacteria (ARB). This Review focuses on the epidemiology, prevention and treatment of infections with ARB.

    • Tina Z. Wang
    • , Rosy Priya L. Kodiyanplakkal
    •  & David P. Calfee
  • Consensus Statement | | open

    In this Evidence-Based Guideline on X-linked hypophosphataemia, the authors identify the criteria for diagnosis of this disease, provide guidance for medical and surgical treatment and explain the challenges of follow-up.

    • Dieter Haffner
    • , Francesco Emma
    • , Deborah M. Eastwood
    • , Martin Biosse Duplan
    • , Justine Bacchetta
    • , Dirk Schnabel
    • , Philippe Wicart
    • , Detlef Bockenhauer
    • , Fernando Santos
    • , Elena Levtchenko
    • , Pol Harvengt
    • , Martha Kirchhoff
    • , Federico Di Rocco
    • , Catherine Chaussain
    • , Maria Louisa Brandi
    • , Lars Savendahl
    • , Karine Briot
    • , Peter Kamenicky
    • , Lars Rejnmark
    •  & Agnès Linglart
  • News & Views |

    Cardiovascular disease (CVD) is a leading cause of death in young adults with incident end-stage renal disease (ESRD). Unlike children with ESRD, young adults with incident ESRD have high prevalence of diabetes, coronary artery disease and heart failure. These cardiovascular risk factors are associated with increased CVD-related hospitalizations and mortality in young adults.

    • Elaine Ku
    •  & Mark M. Mitsnefes
  • Review Article |

    The ubiquitin–proteasome system (UPS) maintains protein homeostasis through the selective degradation of proteins. In addition to degrading short-lived and misfolded proteins, the UPS regulates a multitude of cellular processes, including gene transcription and translation, cell survival and metabolism, and inflammation. This Review provides an overview of UPS-mediated proteostasis, focusing on the involvement of the UPS in renal physiology and pathophysiology.

    • Catherine Meyer-Schwesinger
  • Review Article |

    Hypertension is one of the most common comorbid conditions that is associated with obesity. Here, the authors discuss the complex interactions between renal, hormonal and nervous system factors that link excess adiposity with elevated blood pressure.

    • John E. Hall
    • , Jussara M. do Carmo
    • , Alexandre A. da Silva
    • , Zhen Wang
    •  & Michael E. Hall
  • News & Views |

    New data from the JAVELIN Renal 101 and KEYNOTE-426 trials provide evidence that immune-based combination therapy has superior efficacy to sunitinib monotherapy in patients with advanced renal cell carcinoma. The new findings raise important questions regarding the optimum choice of combination therapy for these patients.

    • Camillo Porta
    •  & Mimma Rizzo
  • Review Article |

    Autosomal dominant polycystic kidney disease (ADPKD) is caused by pathogenic mutations in the genes that encode polycystin 1 (PC1) and PC2. In this article, the authors discuss findings from structural and electrophysiological studies that give us insight into the function of polycystins and ADPKD pathogenesis.

    • Dominique Douguet
    • , Amanda Patel
    •  & Eric Honoré
  • News & Views |

    A new study used genome-wide association data and Mendelian randomization to investigate associations between the gut microbiome and metabolic traits. The researchers demonstrate that host genetic variants influence levels of the short-chain fatty acids butyrate and propionate in the gut, which in turn modulate host glycaemic metabolism.

    • Wei Ling Lau
    •  & Nosratola D. Vaziri
  • Review Article |

    Effective adjuvant therapies are needed to reduce the risk of recurrence of kidney cancer. Here, the authors discuss the results of adjuvant therapy trials, the potential of immune checkpoint inhibitors as adjuvant therapies and the need for multidisciplinary management of patients with resected kidney cancer.

    • Camillo Porta
    • , Laura Cosmai
    • , Bradley C. Leibovich
    • , Thomas Powles
    • , Maurizio Gallieni
    •  & Axel Bex
  • News & Views |

    Chronic kidney disease (CKD) is often clinically silent and traditional clinical data alone cannot differentiate disease subtypes. A recent study of the genetic basis of CKD in adults that examined the prevalence of monogenic kidney disease aetiologies supports the use of genetic analysis to improve diagnostics and treatment in CKD.

    • Asaf Vivante
    •  & Karl Skorecki
  • Review Article |

    This Review describes the current understanding of the role of epigenetics and epigenomics in diabetic kidney disease (DKD) and how epigenetic mechanisms might contribute to metabolic memory. The authors also discuss how epigenetic factors and non-coding RNAs could be used as biomarkers and drug targets for DKD diagnosis, prognosis and treatment.

    • Mitsuo Kato
    •  & Rama Natarajan
  • Review Article |

    Glycosylation refers to the addition of carbohydrate chains to proteins and lipids. In this Review, the authors discuss the broad role of glycans in immunity, cancer, xenotransplantation and glomerular filtration and the potential of ‘glycomedicine’.

    • Colin Reily
    • , Tyler J. Stewart
    • , Matthew B. Renfrow
    •  & Jan Novak
  • News & Views |

    Haemodialysis options for undocumented immigrants with end-stage renal disease range from standard of care thrice-weekly treatments to emergency-only haemodialysis. This latter approach is associated with poor patient outcomes and high costs. The time has come for the nephrology community to demand an end to the practice of emergency-only haemodialysis.

    • Rudolph A. Rodriguez
  • Review Article |

    This Review describes the reciprocal interactions between the immune system and the intestinal microbiota, focusing on components of the immune system that drive chronic diseases that involve the kidney. The authors also discuss limitations of current approaches to microbiota research and emphasize the need to move beyond studies of correlation to causation.

    • Felix Knauf
    • , J. Richard Brewer
    •  & Richard A. Flavell
  • News & Views |

    The PIVOTAL trial shows that proactive intravenous (i.v.) iron administration reduces cardiovascular events and deaths, transfusions and erythropoiesis-stimulating agent doses and does not increase infections in patients on haemodialysis. These findings upend the warnings of guidelines and experts about the dangers of i.v. iron and prove that maintaining low iron stores is harmful.

    • Daniel W. Coyne
  • Review Article |

    Pre-eclampsia is associated with substantial maternal and fetal morbidity and mortality worldwide. Here, the authors discuss the epidemiology, pathogenesis and current and future treatment of pre-eclampsia with a focus on the role of angiogenic imbalance.

    • Elizabeth A. Phipps
    • , Ravi Thadhani
    • , Thomas Benzing
    •  & S. Ananth Karumanchi
  • News & Views |

    A new study reports that human blood vessel organoids can be generated through the directed differentiation of human pluripotent stem cells. Use of these blood vessel organoids to model diabetic vasculopathy led to the identification of a new potential therapeutic target, suggesting that this system could have translational value for studies of diabetes complications.

    • Ryuji Morizane
  • News & Views |

    A reduction in proteinuria and albuminuria has long been proposed as a surrogate biomarker for clinically validated end points for interventional trials in patients with kidney disease. Taken together, the findings of two recent landmark meta-analyses present a formidable argument favouring such surrogacy but some uncertainty remains.

    • Richard J. Glassock
  • Review Article |

    This Review describes the main signalling pathways that are coordinated by primary cilia to control developmental processes, tissue plasticity and organ function and how defects in the output of ciliary signalling events are coupled to developmental disorders and disease progression.

    • Zeinab Anvarian
    • , Kirk Mykytyn
    • , Saikat Mukhopadhyay
    • , Lotte Bang Pedersen
    •  & Søren Tvorup Christensen
  • Review Article |

    The plasma accumulation of uraemic solutes seen in chronic kidney disease is associated with dysregulated homeostasis. In this Review, Nigam and Bush discuss uraemic syndrome in the context of the remote sensing and signalling hypothesis, which describes small-molecule-mediated communication between tissues and organs.

    • Sanjay K. Nigam
    •  & Kevin T. Bush
  • News & Views |

    The analyses in the 2017 Global Burden of Disease Study demonstrate the growing burden of chronic kidney disease (CKD), mainly driven by population ageing; absolute levels for every CKD metric considered rose significantly, whereas age-standardized rates were fairly stable. The prevalence of key metabolic CKD risk factors, particularly obesity, also show a worrying increase.

    • Simon D. S. Fraser
    •  & Paul J. Roderick
  • Review Article |

    Wilms tumour is the most common renal malignancy of childhood. Here, the authors review the genetic landscape of Wilms tumour and discuss how precision medicine guided by genomic information might lead to new therapeutic approaches and improve patient survival.

    • Taryn Dora Treger
    • , Tanzina Chowdhury
    • , Kathy Pritchard-Jones
    •  & Sam Behjati
  • Review Article |

    This Review presents our current understanding of C3 glomerulopathy. Smith et al. discuss the histopathological diagnosis and the crucial pathogenic role of complement dysregulation. Genetic and acquired drivers of C3 glomerulopathy, potential biomarkers and available treatments are highlighted.

    • Richard J. H. Smith
    • , Gerald B. Appel
    • , Anna M. Blom
    • , H. Terence Cook
    • , Vivette D D’Agati
    • , Fadi Fakhouri
    • , Véronique Fremeaux-Bacchi
    • , Mihály Józsi
    • , David Kavanagh
    • , John D. Lambris
    • , Marina Noris
    • , Matthew C. Pickering
    • , Giuseppe Remuzzi
    • , Santiago Rodriguez de Córdoba
    • , Sanjeev Sethi
    • , Johan Van der Vlag
    • , Peter F. Zipfel
    •  & Carla M. Nester
  • Review Article |

    Macrophages are versatile immune cells that protect the host against infection but can also promote chronic inflammation and fibrosis. In this Review, the authors discuss the diverse roles of macrophages in acute and chronic renal pathology as well as potential therapeutic targets.

    • Patrick Ming-Kuen Tang
    • , David J. Nikolic-Paterson
    •  & Hui-Yao Lan
  • News & Views |

    New findings demonstrate that endothelial nitric oxide synthase regulates major metabolic pathways in the kidney proximal tubule, which confers protection against oxidative stress during acute kidney injury (AKI). These findings give new insights into AKI pathophysiology and nitric oxide biology, and identify new targets for the treatment of AKI.

    • Pierre-Yves Martin
    •  & Sophie de Seigneux
  • News & Views |

    Scientists have long wondered how maternal diabetes, malnutrition and placental dysfunction impair fetal nephrogenesis. A new study discovered a link between prenatal metabolic stress and nephron deficit via dysregulation of DNA methylation — an epigenetic mechanism that is essential for the renewal and differentiation of nephron progenitors.

    • Samir S. El-Dahr
  • Review Article |

    Uraemic cardiomyopathy is the major phenotype of fatal cardiac disease in patients with end-stage renal disease. This Review focuses on the molecular mechanisms of uraemic cardiomyopathy, crosstalk between these mechanisms and implications for therapy

    • Xiaoliang Wang
    •  & Joseph I. Shapiro