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Color pulsed-wave Doppler ultrasound (CPWD-US) emerges as a pivotal tool in intensive care units (ICUs) for diagnosing acute kidney injury (AKI) swiftly and non-invasively. Its bedside accessibility allows for rapid assessments, making it a primary imaging modality for AKI characterization. Furthermore, CPWD-US serves as a guiding instrument for key diagnostic-interventional procedures such as renal needle biopsy and percutaneous nephrostomy, while also facilitating therapy response monitoring and AKI progression tracking. This review shifts focus towards the integration of renal ultrasound into ICU workflows, offering contemporary insights into its utilization through a diagnostic standard-oriented approach. By presenting a flow chart, this review aims to provide practical guidance on the appropriate use of point-of-care ultrasound in critical care scenarios, enhancing diagnostic precision, patient management and safety, albeit amidst a backdrop of limited evidence regarding long-term outcomes., (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Emergencies in nephrology are mainly acute life-threatening situations for patients. Furthermore, one needs to consider constellations that are so damaging to the kidneys that the need for permanent dialysis develops within a short period of time. Acute kidney failure as an immediate consequence is categorized using the Acute Kidney Injury Network (AKIN) stages and is pathophysiologically subdivided into pre-, intra- and post-renal. This leads to changes in volume status, acid base and electrolytes, while the terms nephrotic and nephritic describe the amount of kidney damage and help to choose diagnostic steps wisely. Patients that are already undergoing dialysis treatment or have received a kidney transplant are a further specific group in the case of emergencies., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Neonatal acute kidney injury (AKI) is a common yet underdiagnosed condition in neonates with significant implications for long-term kidney health. Lack of timely recognition and documentation of AKI contributes to missed opportunities for nephrology consultation and follow-up, potentially leading to adverse outcomes., Methods: We conducted a quality improvement (QI) project to address this by incorporating an automated real-time electronic medical record (EMR)-AKI alert system in the Neonatal Intensive Care Unit (NICU) at Le Bonheur Children's Hospital. Our primary objective was to improve documentation of neonatal AKI (defined as serum creatinine (SCr) > 1.5 mg/dL) by 25% compared to baseline levels. The secondary goal was to increase nephrology consultations and referrals to the neonatal nephrology clinic. We designed an EMR-AKI alert system to trigger for neonates with SCr > 1.5 mg/dL, automatically adding AKI diagnosis to the problem list. This prompted physicians to consult nephrology, refer neonates to the nephrology clinic, and consider medication adjustments., Results: Our results demonstrated a significant improvement in AKI documentation after implementing the EMR-AKI alert, reaching 100% compared with 7% at baseline (p < 0.001) for neonates with SCr > 1.5 mg/dL. Although the increase in nephrology consultations was not statistically significant (p = 0.5), there was a significant increase in referrals to neonatal nephrology clinics (p = 0.005)., Conclusions: Integration of an EMR alert system with automated documentation offers an efficient and economical solution for improving neonatal AKI diagnosis and documentation. This approach enhances healthcare provider engagement, streamlines workflows, and supports QI. Widespread adoption of similar approaches can lead to improved patient outcomes and documentation accuracy in neonatal AKI care., (© 2024. The Author(s).)

Background: Probiotics are living microorganisms that may confer health benefits to their host if administered in sufficient quantities. However, data on the use of probiotics in transplant recipients are scarce., Method: This multi-center survey of pediatric nephrologists aimed to examine variations in practice regarding the use of probiotics in pediatric kidney transplant recipients. The survey was conducted via a 10-item questionnaire (developed in Survey Monkey) administered to pediatric nephrologists participating in the Pediatric Nephrology Research Consortium meeting in April 2023., Results: Sixty-four pediatric nephrologists completed the survey. Twenty-seven (42.2%) respondents reported having prescribed probiotics to pediatric kidney transplant recipients. The primary reason for probiotic use was the treatment of antibiotic-associated diarrhea (n = 20), with other reasons including recurrent Clostridium difficile infection (n = 15), general gut health promotion (n = 12), recurrent urinary tract infections (n = 8), and parental request (n = 1). Of those who prescribed probiotics, 48.1% held them during periods of neutropenia and 14.8% during central venous line use. Of the 64 respondents, 20 reported the lack of safety data as a concern for using probiotics in kidney transplant recipients., Conclusion: Pediatric nephrologists are increasingly prescribing probiotics to pediatric kidney transplant recipients; nevertheless, substantial practice variations exist. The paucity of safety data is a significant deterrent to probiotic use in this population., (© 2024 The Author(s). Pediatric Transplantation published by Wiley Periodicals LLC.)

In recent decades, nephropathology has developed worldwide as a subspeciality of pathology, which requires special methodological and technical equipment to process the material and specific clinical and pathological expertise to interpret the findings. These special requirements mean that nephropathology is not available at all pathology institutes, but is carried out on a large scale in a few highly specialised centres. The history of nephropathology, or in a narrower sense the specialised histopathological examination of kidney biopsies, began in 1958 with the first use or performance of a kidney biopsy [1]. It thus replaced the practice of urinalysis, which had been common since the Middle Ages, as a diagnostic tool for kidney diseases. Specialised techniques such as immunofluorescence or immunohistology but also electron microscopy are required to assess specific renal changes, for which the examination of renal biopsies is one of the few remaining routine applications today. In Germany and German-speaking countries, the discipline developed thanks to the work of outstanding people in the field of pathology who were primarily involved in this discipline and had the necessary technical and human resources in their laboratories to ensure that these biopsies could be analysed., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Dialysis might not benefit all older patients with kidney failure, particularly those with multimorbid conditions and frailty. Patients' and healthcare professionals' awareness of the presence of geriatric impairments could improve outcomes by tailoring treatment plans and decisions for individual patients., Objective: We aimed to explore the perspectives of patients and healthcare professionals on nephrology-tailored geriatric assessment to fuel decision-making for treatment choices in older patients with kidney failure., Design: In an exploratory qualitative study using focus groups, participants discussed perspectives on the use and value of nephrology-tailored geriatric assessment for the decision-making process to start or forego dialysis., Participants and Measurements: Patients (n = 18) with kidney failure, caregivers (n = 4), and professionals (n = 25) were purposively sampled from 10 hospitals. Interviews were audio-recorded, transcribed verbatim and inductively analysed using thematic analysis., Results: Three main themes emerged that supported or impeded decision-making in kidney failure: (1) patient psycho-social situation; (2) patient-related factors on modality choice; (3) organisation of health care. Patients reported feeling vulnerable due to multiple chronic conditions, old age, experienced losses in life and their willingness to trade longevity for quality of life. Professionals recognised the added value of nephrology-tailored geriatric assessment in three major themes: (i) facilitating continual holistic assessment, (ii) filling the knowledge gap, and (iii) uncovering important patient characteristics., Conclusions: nephrology-tailored geriatric assessment was perceived as a valuable tool to identify geriatric impairments in older patients with kidney failure. Integration of its outcomes can facilitate a more holistic approach to inform choices and decisions about kidney replacement therapy., (© 2023 The Authors. Journal of Renal Care published by John Wiley & Sons Ltd on behalf of European Dialysis & Transplant Nurses Association/European Renal Care Association.)

Kidney diseases, both acute and chronic, are a substantial burden on individual and public health, and they continue to increase in frequency. Despite this and an intense focus on the study of disease mechanisms, few new therapeutic approaches have extended to the clinic. This is in part due to poor pharmacology of many, if not most, therapeutics with respect to the sites of kidney disease within the glomerulus or nephron. Considering this, within the past decade, and more pointedly over the past 2 years, there have been substantial developments in nanoparticle systems to deliver therapeutics to the sites of kidney disease. Here, we provide a broad overview of the various classes of nanomaterials that have been developed to improve therapeutic development for kidney diseases, the strategy used to provide kidney accumulation, and briefly the disease models they focused on, if any. We then focus on one specific system, polymeric mesoscale nanoparticles, which has broadly been used over 13 publications, demonstrating targeting of the tubular epithelium with 26-fold specificity compared with other organs. While there have been several nanomedicines that have advanced to the clinic in the past several decades, including mRNA-based coronavirus disease vaccines and others, none have focused on kidney diseases specifically. In total, we are confident that the rapid advancement of nanoscale-based kidney targeting and a concerted focus by clinicians, scientists, engineers, and other stakeholders will push one or more of these technologies into clinical trials over the next decade., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Nephrology.)

Background: Chronic kidney disease (CKD) affects around 10% of the global population and has been estimated to affect around 50% of individuals with type 2 diabetes and 50% of those with heart failure. The guideline-recommended approach is to manage with disease-modifying therapies, but real-world data suggest that prescribing rates do not reflect this in practice., Objective: To develop a cross-specialty consensus on optimal management of the patient with CKD using a modified Delphi method., Design: An international steering group of experts specialising in internal medicine, endocrinology/diabetology, nephrology and primary care medicine developed 42 statements on aspects of CKD management including identification and screening, risk factors, holistic management, guidelines, cross-specialty alignment and education. Consensus was determined by agreement using an online survey., Participants: The survey was distributed to cardiologists, nephrologists, endocrinologists and primary care physicians across 11 countries., Main Outcomes and Measures: The threshold for consensus agreement was established a priori by the steering group at 75%. Stopping criteria were defined as a target of 25 responses from each country (N=275), and a 4-week survey period., Results: 274 responses were received in December 2022, 25 responses from Argentina, Australia, Brazil, Guatemala, Mexico, Singapore, South Korea, Taiwan, Thailand, Turkey and 24 responses from Egypt. 53 responses were received from cardiologists, 52 from nephrologists, 55 from endocrinologists and 114 from primary care physicians. 37 statements attained very high agreement (≥90%) and 5 attained high agreement (≥75% and <90%). Strong alignment between roles was seen across the statements, and different levels of experience (2-5 years or 5+ years), some variation was observed between countries., Conclusions: There is a high degree of consensus regarding aspects of CKD management among healthcare professionals from 11 countries. Based on these strong levels of agreement, the steering group derived 12 key recommendations focused on diagnosis and management of CKD., Competing Interests: Competing interests: MA has received payment honoraria for lectures, presentations, speaker bureaus, publication writing or educational events from: Amgen, AstraZeneca, Bayer, Boehringer Ingelheim, Menarini, MSD, Novo Nordisk, Sandoz and Sanofi. MA has also participated in a Data Safety Monitoring Board or Advisory Boards for: AstraZeneca, Bayer, Boehringer Ingelheim and Novo Nordisk. MCB has worked as speaker for: Aché, AstraZeneca, Bayer, Boehringer-Ingelheim, Novo-Nordisk, and Servier. JC has consulted for, or been on advisory boards for: AstraZeneca, ATC, Bayer, GSK, Nitto Denko and Pfizer. JC has been a speaker for: Abbott, AstraZeneca, Bayer and Boehringer Ingelheim. JC has been on Steering Committees or Data Safety Monitoring Boards for: AstraZeneca, Boehringer Ingelheim and Novartis, and has received scientific grant funding from AstraZeneca, ATC, and Nitto Denko. Y-JL has been a speaker and Advisory Board member for: AstraZeneca Taiwan. MM was the International Society for Nephrology Deputy Chair for Latin America and Caribbean (2019–2023) and is currently the on the council for the International Society for Peritoneal Disease (2022–2024). MM has been a consultant for: AstraZeneca, Bayer, and Boehringer Ingelheim, and provided expert testimony for: AstraZeneca, Bayer and Boehringer Ingelheim. MM has received grants from or has pending grants with: AstraZeneca*, Bayer*, Boehringer, Renal Research Institute* and Tricida*. MM has received travel grants from: AstraZeneca and Medcom. GJRD has been a speaker or Advisory Board member for Astra Zeneca, Boehringer Ingelheim, Bayer, Fresenius and Medtronic. SC has received honoraria as a speaker or advisory board member from AstraZeneca, Boehringer Ingelheim, Daewon, Daewoong, Eli Lilly, Korea Pharma, Myoung Poom Medical, Otsuka, Sanofi-Aventis and Yuhan. CP has been a speaker or participated in advisory boards for Astra Zeneca, Boehringer Ingelheim, Eli Lilly, GlaxoSmithKline, Otsuka, Vifor-CSL. SHA-K, VSP and TT declare no conflict of interest in regard to this study., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: This study aims to characterize the current status of the nephrology workforce in China and evaluate its optimal capacity based on real-world patient mobility data., Methods: Data on nephrologists in China were collected from two prominent online healthcare platforms using web crawlers and natural language processing techniques. Hospitalization records of patients with chronic kidney disease (CKD) from January 2014 to December 2018 were extracted from a national administrative database in China. City-level paths of patient mobility were identified. Effects of nephrology workforce on patient mobility were analyzed using multivariate Poisson regression models., Results: Altogether 9.13 nephrologists per million population (pmp) were in practice, with substantial city-level variations ranging from 0.16 to 88.79. The ratio of nephrologists to the estimated CKD population was 84.57 pmp. Among 6 415 559 hospitalizations of patients with CKD, 21.3% were cross-city hospitalizations and 7441 city-level paths of patient mobility with more than five hospitalizations were identified. After making adjustment for healthcare capacity, healthcare insurance, economic status, and travel characteristics, the Poisson regression models revealed that the number of nephrologists in both the source city (incidence rate ratio [IRR] 0.99, per 1 pmp increase) and destination city (IRR 1.07, per 1 pmp increase) were independently associated with patient mobility. An IRR plateau was observed when the number of nephrologists exceeded 12 pmp in the source city, while a rapidly increasing IRR was observed beyond 20 pmp in the destination city., Conclusions: The nephrology workforce in China exhibits significant geographic variations. Based on local healthcare needs, an optimal range of 12-20 nephrologists pmp is suggested., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Introduction: Renal events are common in cancer patients and malignancy is a prevalent complication in both patients transplanted and under kidney replacement therapy (KRT). In recent years, onco-nephrology has been developed as a subspecialty whose scope has not been well established yet. The aim of our study was to assess resident and senior physicians' knowledge and expectations about onco-nephrology., Methods and Materials: Two anonymous self-administered online questionnaires were developed by a multidisciplinary team and distributed to French residents and senior physicians., Results: Two hundred twenty-eight physicians answered the survey, including 128 (56%) nephrologists, of which 98 (43%) were senior physicians and 130 (57%) were residents. Nephrologists rated their confidence in their ability to face onco-nephrological situation at 6/10 (interquartile range (IQR) 4.0-7.0) and oncologists at 6.0/10 (5.0-7.0). Managing cancer drugs in patients on KRT or in transplanted patients and discussion about introducing dialysis in cancer patients were designated as the most challenging topics. Asking if they had received appropriate learning, residents' median agreement was ranked at 3.0/10 (2.0-4.0). Forty-six percent of the respondents considered available resources as not appropriate. Specialized onco-nephrology consultations were accessible for 21% of the respondents. Finally, respondents thought there is a strong need for a national working group (8.3/10) with 87% of them expecting new reliable guidelines., Conclusion: The present survey revealed physicians' expectations about onco-nephrology implementation in France. An appropriate answer could be the creation of a national working group. Therefore, GRIFON (Groupe de Recherche Interdisciplinaire en OncoNéphrologie) has recently been created., (© 2022. The Author(s) under exclusive licence to American Association for Cancer Education.)

Purpose of Review: This review will summarize and synthesize recent findings in regard to monogenic kidney disorders, including how that evidence is being translated into practice. It will add to existing key knowledge to provide context for clinicians in consolidating existing practice and approaches., Recent Findings: Whilst there are long established factors, which indicate increased likelihood of identifying a monogenic cause for kidney disease, these can now be framed in terms of the identification of new genes, new indications for genomic testing and new evidence for clinical utility of genomic testing in nephrology. Further, inherent in the use of genomics in nephrology are key concepts including robust informed consent, variant interpretation and return of results. Recent findings of variants in genes related to complex or broader kidney phenotypes are emerging in addition to understanding of de novo variants. Phenocopy phenomena are indicating a more pragmatic use of broader gene panels whilst evidence is emerging of a role in unexplained kidney disease. Clinical utility is evolving but is being successfully demonstrated across multiple domains of outcome and practice., Summary: We provide an updated framework of evidence to guide application of genomic testing in chronic kidney disease (CKD), building upon existing principles and knowledge to indicate how the practice and implementation of this can be applied today. There are clearly established roles for genomic testing for some patients with CKD, largely those with suspected heritable forms, with these continuing to expand as new evidence emerges., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

This article describes the articulation, development, and deployment of a machine learning (ML) model-driven value solution for chronic kidney disease (CKD) in a health system. The ML model activated an electronic medical record (EMR) trigger that alerted CKD patients to seek primary care. Simultaneously, primary care physicians (PCPs) received an alert that a CKD patient needed an appointment. Using structured checklists, PCPs addressed and controlled comorbid conditions, reconciled drug dosing and choice to CKD stage, and ordered prespecified laboratory and imaging tests pertinent to CKD. After completion of checklist prescribed tasks, PCPs referred patients to nephrology. CKD patients had multiple comorbidities and ML recognition of CKD provided a facile insight into comorbid burden. Operational results of this program have exceeded expectations and the program is being expanded to the entire health system. This paradigm of ML-driven, checklist-enabled care can be used agnostic of EMR platform to deliver value in CKD through structured engagement of complexity in health systems.

Studies have shown that as many as 1 in 10 adults with chronic kidney disease has a monogenic form of disease. However, genetic services in adult nephrology are limited. An adult Kidney Genetics Clinic was established within the nephrology division at a large urban academic medical center to increase access to genetic services and testing in adults with kidney disease. Between June 2019 and December 2021, a total of 363 patients were referred to the adult Kidney Genetics Clinic. Of those who completed genetic testing, a positive diagnostic finding was identified in 27.1%, a candidate diagnostic finding was identified in 6.7% of patients, and a nondiagnostic positive finding was identified in an additional 8.6% of patients, resulting in an overall yield of 42.4% for clinically relevant genetic findings in tested patients. A genetic diagnosis had implications for medical management, family member testing, and eligibility for clinical trials. With the utilization of telemedicine, genetic services reached a diverse geographic and patient population. Genetic education efforts were integral to the clinic's success, as they increased visibility and helped providers identify appropriate referrals. Ongoing access to genomic services will remain a fundamental component of patient care in adults with kidney disease., (© 2022 Wiley Periodicals LLC.)

Introduction: Acute kidney injury (AKI) affects nearly 20% of all hospitalised patients and is associated with poor outcomes. Long-term complications can be partially attributed to gaps in kidney-focused care and education during transitions. Building capacity across the healthcare spectrum by engaging a broad network of multidisciplinary providers to facilitate optimal follow-up care represents an important mechanism to address this existing care gap. Key participants include nephrologists and primary care providers and in-depth study of each specialty's approach to post-AKI care is essential to optimise care processes and healthcare delivery for AKI survivors., Methods and Analysis: This explanatory sequential mixed-methods study uses survey and interview methodology to assess nephrologist and primary care provider recommendations for post-AKI care, including KAMPS (kidney function assessment, awareness and education, medication review, blood pressure monitoring and sick day education) elements of follow-up, the role of multispecialty collaboration, and views on care process-specific and patient-specific factors influencing healthcare delivery. Nephrologists and primary care providers will be surveyed to assess recommendations and clinical decision-making in the context of post-AKI care. Descriptive statistics and the Pearson's χ 2 or Fisher's exact test will be used to compare results between groups. This will be followed by semistructured interviews to gather rich, qualitative data that explains and/or connects results from the quantitative survey. Both deductive analysis and inductive analysis will occur to identify and compare themes., Ethics and Dissemination: This study has been reviewed and deemed exempt by the Institutional Review Board at Mayo Clinic (IRB 20-0 08 793). The study was deemed exempt due to the sole use of survey and interview methodology. Results will be disseminated in presentations and manuscript form through peer-reviewed publication., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Hyperuricemia, an independent risk factor for ensuing chronic kidney disease (CKD), contributed to tubulointerstitial fibrosis and insufficiency of renal fatty acid oxidation. Many studies have shown that renal fatty acid oxidation dysfunction is related to the TGF- β 1/Smad3 signaling pathway. We experimented the effects of Zishen Qingre Tongluo Formula (ZQTF) on the adenine/yeast-induced HN rats and uric acid-induced renal mouse tubular epithelial cells (mTECs), determined whether this effect was related to the TGF- β 1/Smad3 signaling pathway, and further investigated the relationship between this effect and renal fatty acid oxidation. Rats were given intraperitoneally with adenine (100 mg/kg) and feed chow with 10% yeast for 18 days and then received ZQTF (12.04 g/kg/day) via intragastric gavage for eight weeks. The TGF- β 1/Smad3 signaling pathway and renal fatty acid oxidation protein were detected by using western blotting, real-time PCR, and immunohistochemistry staining. mTECs induced by UA were used to investigate the relationship between the TGF- β 1/Smad3 signaling pathway and renal fatty acid oxidation. After treatment with ZQTF, levels of UA, 24 h UTP, BUN, and Scr were significantly decreased and histologic injuries were visibly ameliorated in HN rats. Western blotting, real-time PCR, and immunohistochemistry staining revealed that PGC-1 α , PPAR γ , and PPAR α significantly increased, and fibronectin, collagen 1, and P-Smad3 significantly decreased in HN rats and UA-induced mTECs after ZQTF treatment. SIS3 (a specific inhibitor of Smad3) treatment significantly increased the expressions of PGC-1 α , PPAR γ , and PPAR α and decreased the expressions of fibronectin, collagen 1, and P-Smad3 in UA-induced mTECs. Our study demonstrated that ZQTF exhibited renoprotective effects by promoting renal fatty acid oxidation via the regulation of the TGF- β 1/Smad3 signaling pathway. Thus, the present results indicated that ZQTF was a novel antifibrotic strategy for hyperuricemic nephropathy., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2021 Peng Liu et al.)

Competing Interests: Competing interests: We have read and understood BMJ policy on declaration of interests and declare EP was supported by a career development grant K08AG055670 from the National Institute on Aging and is investigator of an investigator initiated grant to the Brigham and Women’s Hospital from Boehringer Ingelheim, not related to the topic of the submitted work.

In this single-center prospective study of 20 patients receiving maintenance hemodialysis (HD), we compared the therapeutic effects of medium cut-off (MCO) and high flux (HF) dialyzers using metabolomics and proteomics. A consecutive dialyzer membrane was used for 15-week study periods: 1st HF dialyzer, MCO dialyzer, 2nd HF dialyzer, for 5 weeks respectively. 1 H-nuclear magnetic resonance was used to identify the metabolites and liquid chromatography-tandem mass spectrometry (LC-MS/MS) analysis was used to identify proteins. To compare the effects of the HF and MCO dialyzers, orthogonal projection to latent structure discriminant analysis (OPLS-DA) was performed. OPLS-DA showed that metabolite characteristics could be significantly classified by 1st HF and MCO dialyzers. The Pre-HD metabolites with variable importance in projection scores ≥ 1.0 in both 1st HF versus MCO and MCO versus 2nd HF were succinate, glutamate, and histidine. The pre-HD levels of succinate and histidine were significantly lower, while those of glutamate were significantly higher in MCO period than in the HF period. OPLS-DA of the proteome also substantially separated 1st HF and MCO periods. Plasma pre-HD levels of fibronectin 1 were significantly higher, and those of complement component 4B and retinol-binding protein 4 were significantly lower in MCO than in the 1st HF period. Interestingly, as per Ingenuity Pathway Analysis, an increase in epithelial cell proliferation and a decrease in endothelial cell apoptosis occurred during the MCO period. Overall, our results suggest that the use of MCO dialyzers results in characteristic metabolomics and proteomics profiles during HD compared with HF dialyzers, which might be related to oxidative stress, insulin resistance, complement-coagulation axis, inflammation, and nutrition., (© 2021. The Author(s).)

Chronic kidney diseases (CKD) are a major health problem affecting approximately 10% of the world's population and posing increasing challenges to the healthcare system. While CKD encompasses a broad spectrum of pathological processes and diverse etiologies, the classification of kidney disease is currently based on clinical findings or histopathological categorizations. This descriptive classification is agnostic towards the underlying disease mechanisms and has limited progress towards the ability to predict disease prognosis and treatment responses. To gain better insight into the complex and heterogeneous disease pathophysiology of CKD, a systems biology approach can be transformative. Rather than examining one factor or pathway at a time, as in the reductionist approach, with this strategy a broad spectrum of information is integrated, including comprehensive multi-omics data, clinical phenotypic information, and clinicopathological parameters. In recent years, rapid advances in mathematical, statistical, computational, and artificial intelligence methods enable the mapping of diverse big data sets. This holistic approach aims to identify the molecular basis of CKD subtypes as well as individual determinants of disease manifestation in a given patient. The emerging mechanism-based patient stratification and disease classification will lead to improved prognostic and predictive diagnostics and the discovery of novel molecular disease-specific therapies., (© 2021. The Author(s).)

Background and Objectives: Survivors of AKI are at higher risk of CKD and death, but few patients see a nephrologist after hospital discharge. Our objectives during this 2-year vanguard phase trial were to determine the feasibility of randomizing survivors of AKI to early follow-up with a nephrologist or usual care, and to collect data on care processes and outcomes., Design, Setting, Participants, & Measurements: We performed a randomized controlled trial in patients hospitalized with Kidney Disease Improving Global Outcomes (KDIGO) stage 2-3 AKI at four hospitals in Toronto, Canada. We randomized patients to early nephrologist follow-up (standardized basket of care that emphasized BP control, cardiovascular risk reduction, and medication safety) or usual care from July 2015 to June 2017. Feasibility outcomes included the proportion of eligible patients enrolled, seen by a nephrologist, and followed to 1 year. The primary clinical outcome was a major adverse kidney event at 1 year, defined as death, maintenance dialysis, or incident/progressive CKD., Results: We screened 3687 participants from July 2015 to June 2017, of whom 269 were eligible. We randomized 71 (26%) patients (34 to nephrology follow-up and 37 to usual care). The primary reason stated for declining enrollment included hospitalization-related fatigue ( n =65), reluctance to add more doctors to the health care team ( n =59), and long travel times ( n =40). Nephrologist visits occurred in 24 of 34 (71%) intervention participants, compared with three of 37 (8%) participants randomized to usual care. The primary clinical outcome occurred in 15 of 34 (44%) patients in the nephrologist follow-up arm, and 16 of 37 (43%) patients in the usual care arm (relative risk, 1.02; 95% confidence interval, 0.60 to 1.73)., Conclusions: Major adverse kidney events are common in AKI survivors, but we found the in-person model of follow-up posed a variety of barriers that was not acceptable to many patients., Clinical Trial Registry Name and Registration Number: Nephrologist Follow-up versus Usual Care after an Acute Kidney Injury Hospitalization (FUSION), NCT02483039 CJASN 16: 1005-1014, 2021. doi: https://doi.org/10.2215/CJN.17331120., (Copyright © 2021 by the American Society of Nephrology.)

Among renal cells, podocytes (glomerular epithelial cells) are the most critical to prevent plasma proteins from excessive loss by forming their sophisticated foot processes (FP) and slit diaphragms (SD). A general finding in the glomeruli of patients with nephrotic syndrome is the foot processes "effacement" resulted from dysregulated actin cytoskeleton reorganization. Ultrastructural analysis in patients with nephrotic syndrome has demonstrated that such changes tend to be dynamic and can sometimes be reversible. In a more molecular sense, injured podocytes can no longer maintain their tight regulation and "retract" their FP, but not "efface" them. Past studies have revealed multiple exquisite mechanisms and arrays of proteins participating in the regulation of cytoskeletal rearrangement, and these mechanisms serve as potential targets to treat. A major challenge to develop specific therapies is the targeted mechanism has to be crucial and specific enough for podocyte-oriented kidney diseases, and it would be even better to manifest in most of the glomerulonephritis. Studies have shown many approaches targeting different mechanisms, but none of them has been proved to be effective in clinical medicine. Up to the present, Abatacept (Orencia) is the first (and the only) clinical targeted therapy demonstrating limited success. It inhibits the co-stimulatory response of B7-1 (CD80) induced in various types of glomerulonephritis. Future clinical studies have to be expanded to substantiate this highly specific targeted therapy because the Abatacept effect is not generally accepted even within the nephrology community. Nevertheless, there are ongoing searches for specific treatment targeting podocytes through various approaches., (© 2021 The Authors. The Kaohsiung Journal of Medical Sciences published by John Wiley & Sons Australia on behalf of Kaohsiung Medical University.)

Background: Artificial intelligence (AI) has had a significant impact on our lives and plays many roles in various fields. By analyzing the past 30 years of AI trends in the field of nephrology, using a bibliography, we wanted to know the areas of interest and future direction of AI in research related to the kidney., Methods: Using the Institute for Scientific Information Web of Knowledge database, we searched for articles published from 1990 to 2019 in January 2020 using the keywords AI; deep learning; machine learning; and kidney (or renal). The selected articles were reviewed manually at the points of citation analysis., Results: From 218 related articles, we selected the top fifty with 1188 citations in total. The most-cited article was cited 84 times and the least-cited one was cited 12 times. These articles were published in 40 journals. Expert Systems with Applications (three articles) and Kidney International (three articles) were the most cited journals. Forty articles were published in the 2010s, and seven articles were published in the 2000s. The top-fifty most cited articles originated from 17 countries; the USA contributed 16 articles, followed by Turkey with four articles. The main topics in the top fifty consisted of tumors (11), acute kidney injury (10), dialysis-related (5), kidney-transplant related (4), nephrotoxicity (4), glomerular disease (4), chronic kidney disease (3), polycystic kidney disease (2), kidney stone (2), kidney image (2), renal pathology (2), and glomerular filtration rate measure (1)., Conclusions: After 2010, the interest in AI and its achievements increased enormously. To date, AIs have been investigated using data that are relatively easy to access, for example, radiologic images and laboratory results in the fields of tumor and acute kidney injury. In the near future, a deeper and wider range of information, such as genetic and personalized database, will help enrich nephrology fields with AI technology., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rare diseases of phosphate/calcium metabolism correspond to a wide and heterogeneous spectrum of diseases. Recent knowledge in physiology and genetics has made it possible to better characterize them and to propose attractive therapeutic approaches based on the underlying pathophysiology. These diseases are often at the interface between nephrology and endocrinology. In this spirit of a multidisciplinary care, each specialty can bring its own critical point of view and its own specificities to improve patient care. The objective of this manuscript is to "read" with a nephrologist's point of view the main frameworks of diseases of phosphate/calcium metabolism, to illustrate the three crucial messages of nephro-protection sent to endocrinologists. First, calciuria must be interpreted both in absolute value (concentration hypercalciuria) and in ratio (flow hypercalciuria). Second, renal monitoring of therapies inducing hypercalciuria on kidneys with normal renal function (e.g. active vitamin D analogs or teriparatide) should be systematic. Last, hyperphosphatemia, often latent in hypoparathyroidism and pseudo-hypoparathyroidism, should be detected and at least benefit from dietary measures, in the context of Western diets rich in phosphate hidden in food additives., (Copyright © 2020. Published by Elsevier Masson SAS.)

Impact of gliflozines in the treatment of non-diabetic nephropathies and cardiac failure has lately been demonstrated. Tolvaptan has now been recognized in Switzerland as a treatment of hyponatremia. In hemodialysis, some progress has been made in the management of dysfunctional arterio-venous fistulas. A glimmer of hope in the treatment of uremic pruritus? Conservative management of a stable coronary heart disease is also advocated in patients with end-stage kidney disease. Therapy with immune cells may either minimize or remove the need for immunosuppression in renal transplant patients. A new predictive score combining several markers can predict long-term graft failure., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Onconephrology is a new subspecialty of nephrology that recognizes the important intersections of kidney disease with cancer. This intersection takes many forms and includes drug-induced nephrotoxicity, electrolyte disorders, paraneoplastic glomerulonephritis, and the interactions of chronic kidney disease with cancer. Data clearly demonstrate that, when patients with cancer develop acute or chronic kidney disease, outcomes are inferior, and the promise of curative therapeutic regimens is lessened. This highlights the imperative for collaborative care between oncologists and nephrologists in recognizing and treating kidney disease in patients with cancer. In response to this need, specific training programs in onconephrology as well as dedicated onconephrology clinics have appeared. This comprehensive review covers many of the critical topics in onconephrology, with a focus on acute kidney injury, chronic kidney disease, drug-induced nephrotoxicity, kidney disease in stem cell transplantation, and electrolyte disorders in patients with cancer., (© 2020 American Cancer Society.)

Background: Physician assistants (PAs) and nurse practitioners (NPs) have expanded roles in nephrology as both the patient load and acuity of care needed for this population have increased., Purpose: To evaluate workforce patterns of PAs and NPs working in nephrology over the past decade., Methods: Using the biannual survey from the National Kidney Foundation Council of Advanced Practitioners, data were collected and analyzed over the past decade., Results: Surveys of nephrology practitioners show the evolution of the dialysis-centralized practitioner to one encompassing all aspects of nephrology: hospital, intensive care unit, research, office, and all types of dialysis. Salaries and benefits have increased to compensate for the expansion of responsibilities., Implications for Practice: Physician assistants and NPs in nephrology have the opportunity to use their skills and training in caring for this high-risk population., (Copyright © 2021 American Association of Nurse Practitioners.)

Renal supportive care (RSC) is an approach integrating nephrology and palliative care to improve quality of life for people with chronic kidney disease (CKD). RSC practice varies across services; therefore, understanding clinicians' perspectives is important to the evolution and definition of RSC., Aim: To understand renal clinicians' views and experiences of RSC, palliative care and end-of-life care., Method: A cross-sectional online survey was undertaken across Australia and New Zealand between February and May 2018. Participants were asked about end-of-life care, RSC, palliative care and an ideal model of RSC., Results: Estimated response rate 13% included 382 clinicians; doctors (32%), nurses (68%); of whom 84% access specialist palliative care and 59% RSC. A lack of agreed treatment goals (86%) and late or rushed treatment decision making (85%) was associated with challenging end-of-life experiences. Variable concepts of RSC were described, with RSC being considered the same as: usual care for all CKD patients (40%), conservative (30%) or palliative care (22%). The term RSC was generally distinct from (77%) and more acceptable than palliative care (80%) with preferential RSC referral for symptoms (86% vs 69%, P < .01) and complex treatment decision making (82% vs 58%, P < .01). Aspirations for RSC included improving symptoms and quality of life (89%), with an ideal model comprising: symptom management (98%), improved nephrology and community service integration (96%) and clinician education (94%)., Conclusion: This study revealed challenges for renal clinicians in providing end-of-life care and variation of views and experiences of RSC. It represents opportunities to develop RSC aligned with clinician priorities to improve patient care., (© 2020 Asian Pacific Society of Nephrology.)

Background: Nephrology nurses and patients expressed that the clinician-patient relationship in a haemodialysis (HD) unit might be best described as being part of an extended family. However, there is a risk that professional boundaries may become blurred due to the perceived closeness of this relationship., Objective: This systematic review sought to explore the experiences of nephrology clinicians in establishing and maintaining a professional relationship with people experiencing end-stage kidney disease who are receiving HD treatment., Method: A search strategy was created using JBI systematic review guidelines for six databases (PubMed, CINAHL, EMBASE, PsycINFO, Cochrane Library and JBI library). Qualitative studies were eligible for inclusion when the participants were nephrology clinicians who provided HD treatment or management of patients in a hospital outpatient HD unit or a community HD centre., Results: Three studies met the inclusion criteria and the findings were synthesised into two themes (1) establishing a professional relationship and (2) coping mechanisms. All three studies focused on nephrology clinicians realising the importance of treating patients as individuals. Nephrology clinicians demonstrated advanced communication skills, empathy and compassion when interacting with patients, which seemed to contribute to patient satisfaction. This systematic review found some qualitative evidence to explain how nephrology clinicians establish and maintain a professional clinician-patient relationship with patients in the HD unit., Conclusion: This review highlighted a significant gap in research concerning the trajectory of the clinician-patient relationship across all members of the renal multidisciplinary health care team., (© 2020 European Dialysis and Transplant Nurses Association/European Renal Care Association.)

Objective: This study analyzed the associations of different crescents' fraction and clinical features in a Chinese lupus nephritis cohort based on the 2018 revision of ISN/RPS classification system. Methods: A total of 288 lupus nephritis patients with complete clinicopathological data and well follow-up was enrolled. The fraction of glomeruli with cellular or fibrocellular crescents based on the new system was reevaluated. The association between crescents fractions and the outcomes were further analyzed. Results: The median follow-up period was 76.5 months. Cellular or fibrocellular crescents were present in 146 (50.7%) of the total individuals. The percentage of crescents were significantly associated with severe clinical renal injury indices and other renal pathological parameter. According to the survival receiver operating characteristic (survival ROC) curve, the optimal cutoff level of cellular or fibrocellular crescents for predicting the composite events was 7.39%. By multivariable Cox hazard analysis, the presence of crescents was predictive of survival from the composite events with a hazard ratio [HR] of 2.5 (95% CI 1.190-5.431; p  = .02). Furthermore, when we used absent, present in less than 7.39% of glomeruli, and present in greater than or equal to 7.39% of glomeruli as cutoffs in all the patients, a gradation appeared, with adjusted HRs of 2.9 (95% CI 1.326-6.313; p  = .008) for crescents in greater than or equal to 7.39% of glomeruli, in reference to no crescents. Conclusion: We proposed that the crescents were not uncommon and had important clinical significance in lupus nephritis. The cutoff point of crescents as prognosticator might be nearly 7.39%.

Neonatal acute kidney injury (AKI) is common. Critically ill neonates are at risk for AKI for many reasons including the severity of their underlying illnesses, prematurity, and nephrotoxic medications. In this educational review, we highlight four clinical scenarios in which both the illness itself and the medications indicated for their treatment are risk factors for AKI: sepsis, perinatal asphyxia, patent ductus arteriosus, and necrotizing enterocolitis. We review the available evidence regarding medications commonly used in the neonatal period with known nephrotoxic potential, including gentamicin, acyclovir, indomethacin, vancomycin, piperacillin-tazobactam, and amphotericin. We aim to illustrate the complexity of decision-making involved for both neonatologists and pediatric nephrologists when managing infants with these conditions and advocate for ongoing multidisciplinary collaboration in the development of better AKI surveillance protocols and AKI mitigation strategies to improve care for these vulnerable patients.

Preeclampsia is a multisystem progressive disorder of pregnancy that can be potentially catastrophic for the mother and the fetus. It involves complex perturbations of the kidney and systemic physiology, along with long-term effects on vascular and kidney health. Thus, the nephrologist plays a key role in the peripartum and long-term management of preeclampsia. Recent translational research has improved our understanding of its pathophysiology, and there is hope for novel therapies. In this review, we discuss the evolution of diagnostic criteria and dilemmas in the diagnosis of hypertensive disorders in pregnancy. We summarize the advances in the pathogenesis and prediction of preeclampsia. We describe the management and prevention of preeclampsia focusing specially on the forthcoming strategies from the nephrologist's perspective. We address the evidence regarding long-term outcomes for the mother and the child. We end with exploring areas warranting future research., (Copyright © 2020 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

The use of 24-h ABPM has become commonplace when diagnosing and managing hypertension in the pediatric population. Multiple clinical guidelines recommend ABPM as the preferred method for identifying white-coat hypertension, masked hypertension, and determining degree of blood pressure (BP) control. Accurate, timely diagnosis and optimal management are particularly important in certain populations, such as children with chronic kidney disease (CKD), diabetes, and other conditions with increased risk for cardiovascular disease. Understanding how best to utilize ABPM to achieve these goals is important for pediatric nephrologists and other hypertension specialists. This review will provide practical information on the equipment, application, interpretation, and documentation of ABPM in the specialty clinic.

Coronavirus disease-2019 (COVID-19) has caused a pandemic that has affected millions of people worldwide. COVID-19 is caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and is spread by close contact and by respiratory droplets. It has also impacted different aspects of caring for people with kidney disease, including those with acute kidney injury (AKI), chronic kidney disease (CKD), those requiring kidney replacement therapy (KRT), and those with a kidney transplant. All of these patients are considered high risk. The lessons learned from the COVID-19 pandemic will hopefully serve to protect patients with kidney disease in a similar situation in the future., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The premature newborn faces extrauterine conditions with some systems still immature, both ana tomically and physiologically. The kidney finishes developing at the end of the third trimester of pregnancy, so it is especially exposed to alter its normal development if preterm birth occurs. This si tuation may condition, among other consequences, a lower functional renal mass and microvascular changes comprising a high risk of chronic kidney disease in the long term and arterial hypertension. This article analyzes the current evidence on these risks in premature infants and offers a nephrology follow-up scheme of these children.

Renal cell carcinoma (RCC) is a malignant tumor and the third most common urinary disease. It was estimated that RCC affected over 350,000 individuals in 2013, and there are nearly 140,000 deaths annually due to this disease. The initial masses in RCC patients are mostly confined to a single organ. However, due to the metastatic spread of cancer cells through the circulatory system, more than 30% of RCC patients relapse after surgery. The appearance of distant metastases often means that patients enter the advanced stage of cancer with low quality of life and a short expected survival time. This review aims to describe the extant research on advanced RCC, including its pathophysiology, heterogeneity, diagnosis, treatment, and prospects. We try to highlight the most suitable means of treating advanced RCC patients, focusing on comprehensive personalized treatments.

Background: Enuresis (bedwetting) affects up to 20% of five-year-olds and can have considerable social, emotional and psychological effects. Treatments include alarms (activated by urination), behavioural interventions and drugs., Objectives: To assess the effects of enuresis alarms for treating enuresis in children., Search Methods: We searched the Cochrane Incontinence Specialised Register, which contains trials identified from the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, MEDLINE In-Process, MEDLINE Epub Ahead of Print, ClinicalTrials.gov, WHO ICTRP, and handsearching of journals and conference proceedings (searched 25 June 2018), and reference lists of relevant articles., Selection Criteria: We included randomised or quasi-randomised trials of enuresis alarms or alarms combined with another intervention for treating nocturnal enuresis in children between 5 and 16 years old., Data Collection and Analysis: Two review authors independently assessed risk of bias and extracted data., Main Results: We included 74 trials (5983 children). At treatment completion, alarms may reduce the number of wet nights a week compared to control or no treatment (mean difference (MD) -2.68, 95% confidence interval (CI) -4.59 to -0.78; 4 trials, 127 children; low-quality evidence). Low-quality evidence suggests more children may achieve complete response (14 consecutive dry nights) with alarms compared to control or no treatment (RR 7.23, 95% CI 1.40 to 37.33; 18 trials, 827 children) and that more children may remain dry post-treatment (RR 9.67, 95% CI 4.74 to 19.76; 10 trials, 366 children; low-quality evidence). At treatment completion, we are uncertain whether there is any difference between alarms and placebo drugs in the number of wet nights a week (MD -0.96, 95% CI -2.32 to 0.41; 1 trial, 47 children; very low-quality evidence). Alarms may result in more children achieving complete response than with placebo drugs (RR 1.59, 95% CI 1.16 to 2.17; 2 trials, 181 children; low-quality evidence). No trials comparing alarms to placebo reported the number of children remaining dry post-treatment. Compared with control alarms, code-word alarms probably slightly increase the number of children achieving complete response at treatment completion (RR 1.11, 95% CI 0.97 to 1.27; 1 trial, 353 children; moderate-quality evidence) but there is probably little to no difference in the number of children remaining dry post-treatment (RR 0.91, 95% CI 0.79 to 1.05; moderate-quality evidence). Very low-quality evidence means we are uncertain if there are any differences in effectiveness between the other different types of alarm. At treatment completion, alarms may reduce the number of wet nights a week compared with behavioural interventions (waking, bladder training, dry-bed training, and star chart plus rewards) (MD -0.81, 95% CI -2.01 to 0.38; low-quality evidence) and may increase the number of children achieving complete response (RR 1.77, 95% CI 0.98 to 3.19; low-quality evidence) and may slightly increase the number of children remaining dry post-treatment (RR 1.39, 95% CI 0.81 to 2.41; low-quality evidence). The evidence relating to alarms compared with desmopressin in the number of wet nights a week (MD -0.64, 95% CI -1.77 to 0.49; 4 trials, 285 children) and the number of children achieving complete response at treatment completion (RR 1.12, 95% CI 0.93 to 1.36; 12 trials, 1168 children) is low-quality, spanning possible harms and possible benefits. Alarms probably slightly increase the number of children remaining dry post-treatment compared with desmopressin (RR 1.30, 95% CI 0.92 to 1.84; 5 trials, 565 children; moderate-quality evidence). At treatment completion, we are uncertain if there is any difference between alarms and tricyclics in the number of wet nights a week, the number of children achieving complete response or the number of children remaining dry post-treatment, because the quality of evidence is very low. Due to very low-quality evidence we are uncertain about any differences in effectiveness between alarms and cognitive behavioural therapy, psychotherapy, hypnotherapy and restricted diet. Alarm plus desmopressin may reduce the number of wet nights a week compared with desmopressin monotherapy (MD -0.88, 95% CI -0.38 to -1.38; 2 trials, 156 children; low-quality evidence). Alarm plus desmopressin may increase the number of children achieving complete response (RR 1.32, 95% CI 1.08 to 1.62; 5 trials, 359 children; low-quality evidence) and the number of children remaining dry post-treatment (RR 2.33, 95% CI 1.26 to 4.29; 2 trials, 161 children; low-quality evidence) compared with desmopressin alone. Alarm plus dry-bed training may increase the number of children achieving a complete response compared to dry-bed training alone (RR 3.79, 95% CI 1.85 to 7.77; 1 trial, 80 children; low-quality evidence). It is unclear if there is any difference in the number of children remaining dry post-treatment because of the wide confidence interval (RR 0.56, 95% CI 0.15 to 2.12; low-quality evidence). Due to very low-quality evidence, we are uncertain about any differences in effectiveness between alarm plus bladder training versus bladder training alone. Of the 74 included trials, 17 reported one or more adverse events, nine reported no adverse events and 48 did not mention adverse events. Adverse events attributed to alarms included failure to wake the child, ringing without urination, waking others, causing discomfort, frightening the child and being too difficult to use. Adverse events of comparator interventions included nose bleeds, headaches and abdominal pain. There is probably a slight increase in adverse events between code-word alarm and standard alarm (RR 1.34, 95% CI 0.75 to 2.38; moderate-quality evidence), although we are uncertain because of the wide confidence interval. Alarms probably reduce the number of children experiencing adverse events compared with desmopressin (RR 0.38, 95% CI 0.20 to 0.71; 5 trials, 565 children; moderate-quality evidence). Very low-quality evidence means we cannot be certain whether the adverse event rate for alarms is lower than for other treatments., Authors' Conclusions: Alarm therapy may be more effective than no treatment in reducing enuresis in children. We are uncertain if alarm therapy is more effective than desmopressin but there is probably a lower risk of adverse events with alarms than with desmopressin. Despite the large number of trials included in this review, further adequately-powered trials with robust randomisation are still needed to determine the full effect of alarm therapy., (Copyright © 2020 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

Ultrasonography is increasingly being used in the practice of nephrology, whether it is for diagnosis or management of acute or chronic kidney dysfunction, until progression to end-stage kidney disease, including preoperative assessment, access placement, and diagnosis and management of dysfunctional hemodialysis access. Point-of-care ultrasounds are also being used by nephrologists to help manage volume status, especially in patients admitted to the intensive care units, and more recently, for guiding fluid removal in the outpatient dialysis units. Fundamental knowledge of sonography has become invaluable to the nephrologist, and performance and interpretation of ultrasound has now become an essential tool for practicing nephrologists to provide patient-centered care, maximize efficiency, and minimize fragmentation of care. This review will address the growing role of ultrasonography in the management of a patient with CKD from the point of initial contact with the nephrologist throughout the spectrum of kidney disease and its consequences., (Copyright © 2020 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Alport syndrome is caused by mutations in the genes COL4A3, COL4A4 or COL4A5 and is characterised by progressive glomerular disease, sensorineural hearing loss and ocular defects. Occurring in less than 1:5000, Alport syndrome is a rare genetic disorder but still accounts for > 1% of the prevalent population receiving renal replacement therapy. There is also increasing awareness about the risk of chronic kidney disease in individuals with heterozygous mutations in Alport syndrome genes. The mainstay of current therapy is the use of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, yet potential new therapies are now entering clinical trials. The 2017 International Workshop on Alport Syndrome in Glasgow was a pre-conference workshop ahead of the 50th anniversary meeting of the European Society for Pediatric Nephrology. It focussed on updates in clinical practice, genetics and basic science and also incorporated patient perspectives. More than 80 international experts including clinicians, geneticists, researchers from academia and industry, and patient representatives took part in panel discussions and breakout groups. This report summarises the workshop proceedings and the relevant contemporary literature. It highlights the unique clinician, patient and researcher collaborations achieved by regular engagement between the groups.