Technical requirements and devices available for long-term hemodialysis in children-mind the gap!

Children requiring long-term kidney replacement therapy are a "rare disease" cohort. While the basic technical requirements for hemodialysis (HD) are similar in children and adults, key aspects of the child's cardiovascular anatomy and hemodynamic specifications must be considered. In this article, we describe the technical requirements for long-term HD therapy for children and the devices that are currently available around the world. We highlight the characteristics and major technical shortcomings of permanent central venous catheters, dialyzers, dialysis machines, and software available to clinicians who care for children. We show that currently available HD machines are not equipped with appropriately small circuits and sensitive control mechanisms to perform safe and effective HD in the youngest patients. Manufacturers limit their liability, and health regulatory agencies permit the use of devices, only in children according to the manufacturers' pre-specified weight limitations. Although registries show that 6-23% of children starting long-term HD weigh less than 15 kg, currently, there is only one long-term HD device that is cleared for use in children weighing 10 to 15 kg and none is available and labelled for use in children weighing less than 10 kg anywhere in the world. Thus, many children are being treated "off-label" and are subject to interventions delivered by medical devices that lack pediatric safety and efficacy data. Moreover, recent improvements in dialysis technology offered to adult patients are denied to most children. We, in turn, advocate for concerted action by pediatric nephrologists, industry, and health regulatory agencies to increase the development of dedicated HD machines and equipment for children.

Patient navigator programmes for children and adolescents with chronic diseases.

BACKGROUND: Despite a substantial global improvement in infant and child mortality from communicable diseases since the early 1990s there is now a growing burden of chronic disease in children and adolescents worldwide, mimicking the trend seen in the adult population. Chronic diseases in children and adolescents can affect all aspects of their well-being and function with these burdens and their health-related consequences often carried into adulthood. Up to one third of disability-adjusted life years for children and adolescents globally are a result of chronic disease. This has profound implications for the broader family unit, communities, and health systems in which these children and young people reside. Models of chronic care delivery for children and adolescents with chronic disease have traditionally been adapted from adult models. There is a growing recognition that children and adolescents with chronic diseases have a unique set of healthcare needs. Their needs extend beyond disease education and management appropriate to the developmental stage of the child, to encompass psychological well-being for the entire family and a holistic care approach focusing on the social determinants of health. It is for this reason that patient navigators have been proposed as a potential intervention to help fulfil this critical healthcare gap. Patient navigators are trained medical or non-medical personnel (e.g. lay health workers, community health workers, nurses, or people with lived experience) who provide guidance for the patients (and their primary caregivers) as they move through complex (and often bewildering) medical and social systems. The navigator may deliver education, help to co-ordinate patient care, be an advocate for the patient (and their primary caregivers), or combinations of these. Patient navigators can assist people with a chronic illness (especially those who are vulnerable or from a marginalised population, or both) to better understand their diagnoses, treatment options, and available resources. As there is considerable variation in the purpose, design, and target population of patient navigator programmes, there is a need to systematically review and summarise the existing literature on the effectiveness of navigator programmes in children and young adults with chronic disease. OBJECTIVES: To assess the effectiveness of patient navigator programmes in children and adolescents with chronic diseases. SEARCH METHODS: We searched the Cochrane Library and Epistemonikos up to 20 January 2023 for related systematic reviews using search terms relevant to this review. We searched CENTRAL, MEDLINE, Embase, CINAHL EBSCO, conference proceedings, the International Clinical Trials Register (ICTRP) Search Portal, and ClinicalTrials.gov for primary studies. SELECTION CRITERIA: We included randomised controlled trials reporting the effect of patient navigator interventions on children and adolescents (aged 18 years or younger) with any chronic disease in hospital or community settings. Two review authors independently assessed the retrieved titles and abstracts, and where necessary, the full text to identify studies that satisfied the inclusion criteria. DATA COLLECTION AND ANALYSIS: Two review authors extracted data using a standard data extraction form. We used a random-effects model to perform a quantitative synthesis of the data. We used the I² statistic to measure heterogeneity amongst the studies in each analysis. We indicated summary estimates as mean differences (MD), where studies used the same scale, or standardised mean differences (SMD), where studies used different scales, with 95% confidence intervals (CI). We used subgroup and univariate meta-regression to assess reasons for between-study differences. We used the Cochrane RoB 1 tool to assess the methodological quality of the included studies. We used GRADE to assess the certainty of the evidence. MAIN RESULTS: We included 17 studies (2895 randomised participants). All studies compared patient navigators with standard care. Most studies were at unclear or high risk of bias. Meta-analysis was undertaken only for those studies that had the same duration of patient navigator intervention and follow-up/reporting of outcome measures. The evidence is very uncertain about the effects of patient navigator programmes compared with standard care on self-reported quality of life of children with chronic illness (SMD 0.63, 95% CI -0.20 to 1.47; I2 = 96%; 4 studies, 671 participants; very low-certainty evidence); parent proxy-reported quality of life (SMD 0.09, 95% CI -2.21 to 2.40; I2 = 99%; 2 studies, 309 participants; very low-certainty evidence); or parents' or caregivers' quality of life (SMD -1.98, 95% CI -4.13 to 0.17; I2 = 99%; 3 studies, 757 participants; very low-certainty evidence). It is uncertain whether duration of patient navigator intervention accounts for any of the variances in the changes in quality of life. The evidence is very uncertain about the effects of patient navigator programmes compared with standard care on the number of hospital admissions (MD -0.05, 95% CI -0.34 to 0.23; I2 = 99%; 2 studies, 381 participants; very low-certainty evidence) and the number of presentations to the emergency department (MD 0.06, 95% CI -0.23 to 0.34; I2 = 98%; 2 studies, 381 participants; very low-certainty evidence). Furthermore, it is unclear whether patient navigator programmes reduce the number of missed school days as data were sparse (2 studies, 301 participants). Four studies (629 participants) reported data on resource use. However, given the variation in units of analysis used, meta-analysis was not possible (very low-certainty evidence). All studies reported cost savings or quality-adjusted life year improvement (or both) in the patient navigation arm. No studies reported on adverse events (specifically, abuse of any type against the navigator, the patient, or their family members). AUTHORS' CONCLUSIONS: There is insufficient evidence at present to support the use of patient navigator programmes for children and adolescents with chronic diseases. The current evidence is based on limited data with very low-certainty evidence. Further studies are likely to significantly change these results.

Sex differences in the likelihood of pre-emptive living donor kidney transplantation, and outcomes after kidney transplantation in children and adolescents.

BACKGROUND: Data on sex-based disparities in children with kidney failure and outcomes after kidney transplantation are relatively sparse. This study examined the association between sex differences and the odds of receiving a pre-emptive living donor kidney transplantation, and post-transplant outcomes in children and adolescents. METHODS: We studied all patients (aged <20 years) who commenced kidney replacement therapy (KRT) between 2002 and 2017 using data from the ANZDATA Registry. Factors associated with graft loss and acute rejection after transplantation were assessed using multivariable Cox regression model. Differences in the odds of receiving a pre-emptive live donor transplant between sexes were assessed using adjusted logistic regression. RESULTS: Of the 757 children transplanted during the study period, 497 (65.7%) received a live donor kidney (163, 21.5% pre-emptive). In total, 168 (22.2%) patients experienced graft loss and 213 (28.1%) patients experienced a first episode of acute rejection during the median follow-up period of 6.9 years (IQR 3.5-11.5 years). There were no differences in the rates of graft loss or acute rejection by sex. Compared with boys, the adjusted hazard ratios (aHR) (95% confidence interval) for graft loss and acute rejection in girls were 0.97 (0.71-1.33) and 1.09 (0.82-1.44), respectively. Among children who received living donor kidney transplants, there were no sex differences in the odds of receiving a pre-emptive transplant (adjusted odds ratio (aOR) 0.90 (95% CI 0.56-1.45)). CONCLUSIONS: No sex differences were observed in the odds of receiving a pre-emptive living donor kidney transplant or outcomes after kidney transplantation.

An update on the global disparities in kidney disease burden and care across world countries and regions.

BACKGROUND: Since 2015, the International Society of Nephrology (ISN) Global Kidney Health Atlas (ISN-GKHA) has spearheaded multinational efforts to understand the status and capacity of countries to provide optimal kidney care, particularly in low-resource settings. In this iteration of the ISN-GKHA, we sought to extend previous findings by assessing availability, accessibility, quality, and affordability of medicines, kidney replacement therapy (KRT), and conservative kidney management (CKM). METHODS: A consistent approach was used to obtain country-level data on kidney care capacity during three phases of data collection in 2016, 2018, and 2022. The current report includes a detailed literature review of published reports, databases, and registries to obtain information on the burden of chronic kidney disease and estimate the incidence and prevalence of treated kidney failure. Findings were triangulated with data from a multinational survey of opinion leaders based on the WHO's building blocks for health systems (ie, health financing, service delivery, access to essential medicines and health technology, health information systems, workforce, and governance). Country-level data were stratified by the ISN geographical regions and World Bank income groups and reported as counts and percentages, with global, regional, and income level estimates presented as medians with interquartile ranges. FINDINGS: The literature review used information on prevalence of chronic kidney disease from 161 countries. The global median prevalence of chronic kidney disease was 9·5% (IQR 5·9-11·7) with the highest prevalence in Eastern and Central Europe (12·8%, 11·9-14·1). For the survey analysis, responses received covered 167 (87%) of 191 countries, representing 97·4% (7·700 billion of 7·903 billion) of the world population. Chronic haemodialysis was available in 162 (98%) of 165 countries, chronic peritoneal dialysis in 130 (79%), and kidney transplantation in 116 (70%). However, 121 (74%) of 164 countries were able to provide KRT to more than 50% of people with kidney failure. Children did not have access to haemodialysis in 12 (19%) of 62 countries, peritoneal dialysis in three (6%) countries, or kidney transplantation in three (6%) countries. CKM (non-dialysis management of people with kidney failure chosen through shared decision making) was available in 87 (53%) of 165 countries. The annual median costs of KRT were: US$19 380 per person for haemodialysis, $18 959 for peritoneal dialysis, and $26 903 for the first year of kidney transplantation. Overall, 74 (45%) of 166 countries allocated public funding to provide free haemodialysis at the point of delivery; use of this funding scheme increased with country income level. The median global prevalence of nephrologists was 11·8 per million population (IQR 1·8-24·8) with an 80-fold difference between low-income and high-income countries. Differing degrees of health workforce shortages were reported across regions and country income levels. A quarter of countries had a national chronic kidney disease-specific strategy (41 [25%] of 162) and chronic kidney disease was recognised as a health priority in 78 (48%) of 162 countries. INTERPRETATION: This study provides new information about the global burden of kidney disease and its treatment. Countries in low-resource settings have substantially diminished capacity for kidney care delivery. These findings have major policy implications for achieving equitable access to kidney care. FUNDING: International Society of Nephrology.

Capacity for the management of kidney failure in the International Society of Nephrology Oceania and South East Asia (OSEA) region: report from the 2023 ISN Global Kidney Health Atlas (ISN-GKHA).

The International Society of Nephrology (ISN) region of Oceania and South East Asia (OSEA) is a mix of high- and low-income countries, with diversity in population demographics and densities. Three iterations of the ISN-Global Kidney Health Atlas (GKHA) have been conducted, aiming to deliver in-depth assessments of global kidney care across the spectrum from early detection of CKD to treatment of kidney failure. This paper reports the findings of the latest ISN-GKHA in relation to kidney-care capacity in the OSEA region. Among the 30 countries and territories in OSEA, 19 (63%) participated in the ISN-GKHA, representing over 97% of the region's population. The overall prevalence of treated kidney failure in the OSEA region was 1203 per million population (pmp), 45% higher than the global median of 823 pmp. In contrast, kidney replacement therapy (KRT) in the OSEA region was less available than the global median (chronic hemodialysis, 89% OSEA region vs. 98% globally; peritoneal dialysis, 72% vs. 79%; kidney transplantation, 61% vs. 70%). Only 56% of countries could provide access to dialysis to at least half of people with incident kidney failure, lower than the global median of 74% of countries with available dialysis services. Inequalities in access to KRT were present across the OSEA region, with widespread availability and low out-of-pocket costs in high-income countries and limited availability, often coupled with large out-of-pocket costs, in middle- and low-income countries. Workforce limitations were observed across the OSEA region, especially in lower-middle-income countries. Extensive collaborative work within the OSEA region and globally will help close the noted gaps in kidney-care provision.

Epidemiology and Outcomes of Children with Kidney Failure Receiving Kidney Replacement Therapy in Australia and New Zealand.

Introduction: The incidence and outcomes of kidney replacement therapy (KRT) have been well-studied in adults, but much less so in children. This study aimed to investigate the epidemiology and outcomes of KRT in children in Australia and New Zealand from 2000 to 2020. Methods: Children aged <18 years initiating KRT in Australia and New Zealand between January 1, 2000 and December 31, 2020 and reported to the Australia and New Zealand Dialysis and Transplant Registry were included. Patient survival, technique-survival, and graft survival were analyzed by Cox regression analyses. Results: Overall, 1058 children (median [interquartile range (IQR)] age 11 [5-15] years, 41% female, 66% White) were followed-up with for a median period of 12.3 years. First KRT modalities were peritoneal dialysis (PD; 48%), hemodialysis (HD; 34%), and kidney transplantation (KT; 18%). Pre-emptive KT incidence was highest in Caucasian children (80.4%) and lowest in the Indigenous population (3.2%). There was no difference in 5-year patient survival rates between 2011 and 2020 (96.9%, 95% confidence interval [CI] 93.8-98.4) and the preceding decade, 2000-2010 (94.5%, 95% CI 90.4-96.8) (P = 0.79). There was no difference in 5-year death-censored technique survival between 2011 and 2020 (51.2%, 95% CI 39.1-62) and 2000-2010 (48.8%, 95% CI 40.5-56.6) (P = 0.27). However, 5-year derath-censored graft survival was significantly higher in 2011-2020 (88.4%, 95% CI 84.6-91.4) than in 2000-2010 (84.3%, 95% CI 80.4-87.5) (P < 0.001). Conclusions: PD is the most commonly prescribed KRT modality for children in Australia and New Zealand. Patient-survival, technique-survival, and graft survival rates are excellent and graft survival has improved over the last 2 decades.

What's new in paediatric hypertension?

Paediatric hypertension predisposes to hypertension and cardiovascular disease in adult life. Despite clear guidelines, there remains a lack of screening. Diagnosis remains challenging given the high rate of false-positive high blood pressure (BP) readings at a single visit; thus, multiple visits are required to confirm the diagnosis. Depending on the normative data sets used, hypertension in overweight and obese children can be underestimated by up to 20%. Specific BP targets are required for subgroups such as adolescents, children with chronic kidney disease (CKD) and type 1 diabetes. High dietary salt intake is a risk factor for cardiovascular disease. Given the rise in processed food consumption, children in developed nations are likely to benefit from salt restriction at a population-based level.