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Competing Interests: Competing interests: Both authors have submitted a conflict of interests form.

Background: Human health is inextricably linked to planetary health. The desire to nurture and protect both concurrently requires the mitigation of healthcare-associated environmental harms and global initiatives that support sustainable lifestyles. Health leadership is important to bring adequate attention and action to address planetary health challenges. Health professionals are central to this endeavour, but the will and energy of a few will not be adequate to address this urgent challenge., Study: We present an appraisal of the current UK health professional standards, frameworks and curricula to identify content related to planetary health and environmental sustainability., Results: No current UK health professional standard provides statements and competencies to guide practising and trainee health professionals to focus on and advance the sustainability agenda within their clinical practice and across wider healthcare systems., Conclusion: Update of health professional standards is needed to ensure that health professionals in every specialty are supported and encouraged to lead the implementation of environmentally sustainable practices within the health sector and advocate for planetary health., Competing Interests: Competing interests: SW and EPB are associates of the Centre for Sustainable Healthcare. BW is an employee of the Centre for Sustainable Healthcare. SW is a Trustee of the Healthcare Infection Society., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

This guideline is written primarily for doctors and nurses working in dialysis units and related areas of medicine in the UK, and is an update of a previous version written in 2009. It aims to provide guidance on how to look after patients and how to run dialysis units, and provides standards which units should in general aim to achieve. We would not advise patients to interpret the guideline as a rulebook, but perhaps to answer the question: "what does good quality haemodialysis look like?"The guideline is split into sections: each begins with a few statements which are graded by strength (1 is a firm recommendation, 2 is more like a sensible suggestion), and the type of research available to back up the statement, ranging from A (good quality trials so we are pretty sure this is right) to D (more like the opinion of experts than known for sure). After the statements there is a short summary explaining why we think this, often including a discussion of some of the most helpful research. There is then a list of the most important medical articles so that you can read further if you want to - most of this is freely available online, at least in summary form.A few notes on the individual sections: 1. This section is about how much dialysis a patient should have. The effectiveness of dialysis varies between patients because of differences in body size and age etc., so different people need different amounts, and this section gives guidance on what defines "enough" dialysis and how to make sure each person is getting that. Quite a bit of this section is very technical, for example, the term "eKt/V" is often used: this is a calculation based on blood tests before and after dialysis, which measures the effectiveness of a single dialysis session in a particular patient. 2. This section deals with "non-standard" dialysis, which basically means anything other than 3 times per week. For example, a few people need 4 or more sessions per week to keep healthy, and some people are fine with only 2 sessions per week - this is usually people who are older, or those who have only just started dialysis. Special considerations for children and pregnant patients are also covered here. 3. This section deals with membranes (the type of "filter" used in the dialysis machine) and "HDF" (haemodiafiltration) which is a more complex kind of dialysis which some doctors think is better. Studies are still being done, but at the moment we think it's as good as but not better than regular dialysis. 4. This section deals with fluid removal during dialysis sessions: how to remove enough fluid without causing cramps and low blood pressure. Amongst other recommendations we advise close collaboration with patients over this. 5. This section deals with dialysate, which is the fluid used to "pull" toxins out of the blood (it is sometimes called the "bath"). The level of things like potassium in the dialysate is important, otherwise too much or too little may be removed. There is a section on dialysate buffer (bicarbonate) and also a section on phosphate, which occasionally needs to be added into the dialysate. 6. This section is about anticoagulation (blood thinning) which is needed to stop the circuit from clotting, but sometimes causes side effects. 7. This section is about certain safety aspects of dialysis, not seeking to replace well-established local protocols, but focussing on just a few where we thought some national-level guidance would be useful. 8. This section draws together a few aspects of dialysis which don't easily fit elsewhere, and which impact on how dialysis feels to patients, rather than the medical outcome, though of course these are linked. This is where home haemodialysis and exercise are covered. There is an appendix at the end which covers a few aspects in more detail, especially the mathematical ideas. Several aspects of dialysis are not included in this guideline since they are covered elsewhere, often because they are aspects which affect non-dialysis patients too. This includes: anaemia, calcium and bone health, high blood pressure, nutrition, infection control, vascular access, transplant planning, and when dialysis should be started.

Background: Steroid-sensitive nephrotic syndrome (SSNS), the most common form of nephrotic syndrome in childhood, is considered an autoimmune disease with an established classic HLA association. However, the precise etiology of the disease is unclear. In other autoimmune diseases, the identification of loci outside the classic HLA region by genome-wide association studies (GWAS) has provided critical insights into disease pathogenesis. Previously conducted GWAS of SSNS have not identified non-HLA loci achieving genome-wide significance., Methods: In an attempt to identify additional loci associated with SSNS, we conducted a GWAS of a large cohort of European ancestry comprising 422 ethnically homogeneous pediatric patients and 5642 ethnically matched controls., Results: The GWAS found three loci that achieved genome-wide significance, which explain approximately 14% of the genetic risk for SSNS. It confirmed the previously reported association with the HLA-DR/DQ region (lead single-nucleotide polymorphism [SNP] rs9273542, P =1.59×10 -43 ; odds ratio [OR], 3.39; 95% confidence interval [95% CI], 2.86 to 4.03) and identified two additional loci outside the HLA region on chromosomes 4q13.3 and 6q22.1. The latter contains the calcium homeostasis modulator family member 6 gene CALHM6 (previously called FAM26F ). CALHM6 is implicated in immune response modulation; the lead SNP (rs2637678, P =1.27×10 -17 ; OR, 0.51; 95% CI, 0.44 to 0.60) exhibits strong expression quantitative trait loci effects, the risk allele being associated with lower lymphocytic expression of CALHM6 ., Conclusions: Because CALHM6 is implicated in regulating the immune response to infection, this may provide an explanation for the typical triggering of SSNS onset by infections. Our results suggest that a genetically conferred risk of immune dysregulation may be a key component in the pathogenesis of SSNS., (Copyright © 2019 by the American Society of Nephrology.)

Background: The cardiovascular phenotype is poorly characterized in treated pediatric hypertension. Cardiovascular magnetic resonance imaging (MRI) can be used to better characterize both cardiac and vascular phenotype in children with hypertension., Objective: To use MRI to determine the cardiac and vascular phenotypes of different forms of treated hypertension and compare the results with those of healthy children., Materials and Methods: Sixty children (15 with chronic renal disease with hypertension, 15 with renovascular hypertension, 15 with essential hypertension and 15 healthy subjects) underwent MRI with noninvasive blood pressure measurements. Cardiovascular parameters measured include systemic vascular resistance, total arterial compliance, left ventricular mass and volumetric data, ejection fraction and myocardial velocity. Between-group comparisons were used to investigate differences in the hypertension types., Results: Renal hypertension was associated with elevated vascular resistance (P≤0.007) and normal arterial compliance. Conversely, children with essential hypertension had normal resistance but increased compliance (P=0.001). Renovascular hypertension was associated with both increased resistance and compliance (P≤0.03). There was no difference in ventricular volumes, mass or cardiac output between groups. Children with renal hypertension also had lower systolic and diastolic myocardial velocities., Conclusion: Cardiovascular MRI may identify distinct vascular and cardiac phenotypes in different forms of treated childhood hypertension. Future studies are needed to investigate how this may inform further optimisation of blood pressure treatment in different types of hypertension.

Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide.

We describe a quality improvement project (QIP) designed to reduce unnecessary biochemistry samples requested on a paediatric cardiology ward in Great Ormond Street Hospital. Prior to the intervention biochemistry tests were requested on a daily basis by nursing and junior doctor staff at an annual cost of around £27 000 for the ward. The lead author observed that for the majority the true indication for these biochemistry tests was for the purpose of monitoring renal function and plasma electrolytes. However, during a diagnostic analysis trying to understand the behaviours around ordering tests it appeared that a broader profile and more expensive combined test set was being requested that included unnecessary liver and bone profile analyses. A driver diagram identified three areas to target in order to rationalise blood test requests: (1) a critical understanding of the purpose of the test by those requesting the tests; (2) effective communication between professionals; and (3) improved utilisation of the computer system. An education-based QIP was initiated with the aim of reducing requests of these costly, unnecessary combined biochemical tests by half, by supporting and encouraging staff to switch to a simpler renal function assay. The project was designed to be engaging and fun and invited clinical teams to consider the cost of wasted resources in terms of the financial implications for the hospital, and in terms of the wider environmental impact of wasted resources illustrated in terms of estimated carbon dioxide use. This perhaps unusual approach of encouraging an awareness of both financial and environmental cost led to a sustained reduction in the ordering of expensive combined biochemical tests, saving an estimated £11 338 (or 13.5%) on biochemistry tests and around 17.8 tonnes of carbon dioxide across a 32-month follow-up period., Competing Interests: Competing interests: None declared.

Background: Children with chronic kidney disease (CKD) have increased cardiovascular mortality. Identifying high-risk children who may benefit from further therapeutic intervention is difficult as cardiovascular abnormalities are subtle. Although transthoracic echocardiography may be used to detect sub-clinical abnormalities, it has well-known problems with reproducibility that limit its ability to accurately detect these changes. Cardiovascular magnetic resonance (CMR) is the reference standard method for assessing blood flow, cardiac structure and function. Furthermore, recent innovations enable the assessment of radial and longitudinal myocardial velocity, such that detection of sub-clinical changes is now possible. Thus, CMR may be ideal for cardiovascular assessment in pediatric CKD. This study aims to comprehensively assess cardiovascular function in pediatric CKD using CMR and determine its relationship with CKD severity., Methods: A total of 120 children (40 mild, 40 moderate, 20 severe pre-dialysis CKD subjects and 20 healthy controls) underwent CMR with non-invasive blood pressure (BP) measurements. Cardiovascular parameters measured included systemic vascular resistance (SVR), total arterial compliance (TAC), left ventricular (LV) structure, ejection fraction (EF), cardiac timings, radial and longitudinal systolic and diastolic myocardial velocities. Between group comparisons and regression modelling were used to identify abnormalities in CKD and determine the effects of renal severity on myocardial function., Results: The elevation in mean BP in CKD was accompanied by significantly increased afterload (SVR), without evidence of arterial stiffness (TAC) or increased fluid overload. Left ventricular volumes and global function were not abnormal in CKD. However, there was evidence of LV remodelling, prolongation of isovolumic relaxation time and reduced systolic and diastolic myocardial velocities., Conclusion: Abnormal cardiovascular function is evident in pre-dialysis pediatric CKD. Novel CMR biomarkers may be useful for the detection of subtle abnormalities in this population. Further studies are needed to determine to prognostic value of these biomarkers.

Background: Relapses of nephrotic syndrome are common and are treated with a course of prednisolone (2 mg/kg/day or 60 mg/m 2 /day). This is associated with major adverse effects including diabetes, weight gain, hypertension and behavioural problems. This study is a retrospective review examining the success of treating relapses in steroid-sensitive nephrotic syndrome (SSNS) with low-dose prednisolone and the consequences on subsequent relapse rates. Furthermore, a follow-up study looked at the side-effect profile during treatment with high- versus low-dose prednisolone., Methods: Between January 2012 and July 2013, all well children with SSNS presenting with a relapse were advised to start 1 mg/kg prednisolone daily for a maximum of 7 days. In July 2015, we compared the side-effect profile of prednisolone therapy using the parent proxy PedsQL questionnaire for quality of life (QoL)., Results: Fifty patients were included in the study, with a total of 87 relapses. Sixty-one of the 87 relapses (70 %) responded within a week. Treating relapses with a reduced dose of steroids did not adversely affect the relapse rate in the 6 months preceding and following the current relapse (1.01 vs 0.86, p = 0.3). Fifteen parents completed the PedsQL questionnaire. Comparison of scores in each category showed significantly higher values in each domain during treatment with low-dose prednisolone compared with high-dose treatment (35.6 vs 18.3, p < 0.0001; 31.1 vs 15.0, p < 0.001; 38.3 vs 20.1, p < 0.0001)., Conclusion: A low-dose prednisolone regimen was successful in achieving remission in 70 % of relapses of children with SSNS, without adversely affecting the relapse rate. Parent-completed QoL questionnaires showed significantly higher scores on low-dose treatment, indicating better QoL.

To inform the development of a measure of caregiver burden for carers of children with chronic kidney disease, interviews were conducted with 16 caregivers and 10 renal healthcare professionals. A pool of 97 items generated from interviews was reduced to 60 items following review. A piloting exercise provided evidence for the usability, readability and relevance of items and informed further adaptations resulting in the 51-item Paediatric Renal Caregiver Burden Scale. Further to assessment of its psychometric properties, it is hoped that that the Paediatric Renal Caregiver Burden Scale will serve as a useful measure of caregiver burden in paediatric chronic kidney disease., (© The Author(s) 2014.)

The provision of safe care is complex and difficult to achieve. Awareness of what happens in real time is one of the ways to develop a safe system within a culture of safety. At Great Ormond Street Hospital, we developed and tested a tool specifically designed for patients and families to report harm, with the aim of raising awareness and opportunities for staff to continually improve and provide safe care. Over a 10-month period, we developed processes to report harm. We used the Model for Improvement and multiple Plan, Do, Study, Act cycles for testing. We measured changes using culture surveys as well as analysis of the reports. The tool was tested in different formats and moved from a provider centric to a person-centred tool analysed in real time. An independent person working with the families was best placed to support reporting. Immediate feedback to families was managed by senior staff, and provided the opportunity for clarification, transparency and apologies. Feedback to staff provided learning opportunities. Improvements in culture climate and staff reporting were noted in the short term. The integration of patient involvement in safety monitoring systems is essential to achieve safety. The high number of newly identified 'near-misses' and 'critical incidents' by families demonstrated an underestimation of potentially harmful events. This testing and introduction of a self-reporting, real-time bedside tool has led to active engagement with families and patients and raised situation awareness. We believe that this will lead to improved and safer care in the longer term., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background: Despite the Fistula First initiative there is still reluctance to use arteriovenous fistulas (AVF) for chronic haemodialysis (HD) in children. Our aim was to compare outcomes of AVFs and central venous lines (CVL) in children on chronic HD in a centre where AVF is the primary choice for vascular access., Patients and Methods: This was a retrospective case notes analysis of access complications, dialysis adequacy and laboratory outcomes in children who underwent dialysis for at least a year by AVF (n = 20, median age 14.2 years, range (2.9-16.5) and CVL (n = 5, median age 2.4 years, range 2.0-12.2) between January 2007 and December 2010., Results: Primary access failure rate (patient-months) was 1 per 78.8 for AVF (n = 5) and 1 per 15.5 for CVLs (n = 7, p = 0.3). Failure thereafter was 1 per 131.3 and 1 per 18.5 for AVF and CVLs respectively (n = 3 and 6 respectively; p = 0.2). The annualised hospitalisation rate for access malfunction was 0.44% and 3.1% for AVFs and CVLs respectively (p = 0.004). Patients with AVFs had a lower infection rate of 0.25 per 100 patient-months compared with CVL at 3.2 per 100 (p = 0.002). There was no difference in dialysis adequacy or laboratory values between AVF and CVL groups. Access survival rates (including both primary and secondary access failure) were significantly higher for AVF compared with CVL (p = 0.0002, hazard ratio = 0.15, 95% confidence interval 0.04-0.37)., Conclusions: Patients with AVF spend less time in hospital than those dialysed by CVLs and have a much lower access infection rate. These findings emphasise the need to use AVF as first-line access for paediatric patients on chronic HD.

Background and Objectives: Translocated endotoxin derived from intestinal bacteria has a wide range of adverse effects on cardiovascular (CV) structure and function, driving systemic inflammation, atherosclerosis and oxidative stress. This study's aim was to investigate endotoxemia across the spectrum of chronic kidney disease (CKD)., Design, Setting, Participants, & Measurements: Circulating endotoxin was measured in 249 patients comprising CKD stage 3 to 5 and a comparator cohort of hypertensive patients without significant renal impairment. Patients underwent extended CV assessment, including pulse wave velocity and vascular calcification. Hemodialysis (HD) patients also received detailed echocardiographic-based intradialytic assessments. Patients were followed up for 1 year to assess survival., Results: Circulating endotoxemia was most notable in those with the highest CV disease burden (increasing with CKD stage), and a sharp increase was observed after initiation of HD. In HD patients, predialysis endotoxin correlated with dialysis-induced hemodynamic stress (ultrafiltration volume, relative hypotension), myocardial stunning, serum cardiac troponin T, and high-sensitivity C-reactive protein. Endotoxemia was associated with risk of mortality., Conclusions: CKD patients are characteristically exposed to significant endotoxemia. In particular, HD-induced systemic circulatory stress and recurrent regional ischemia may lead to increased endotoxin translocation from the gut. Resultant endotoxemia is associated with systemic inflammation, markers of malnutrition, cardiac injury, and reduced survival. This represents a crucial missing link in understanding the pathophysiology of the grossly elevated CV disease risk in CKD patients, highlighting the potential toxicity of conventional HD and providing a novel set of potential therapeutic strategies to reduce CV mortality in CKD patients.