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Fadrowski and Lesley Rees Introduction The use of chronic dialysis to sustain the lives of children with end-stage kidney disease (ESKD) has been available in developed countries since the 1970s [1, 2] Advances in technology have made long-term dialysis a viable treatment option for pediatric ESKD patients of all ages, from newborns to adolescents [2, 3] While a successful kidney transplant remains the treatment of choice for all pediatric ESKD patients, almost three-fourths of these children require chronic dialysis while awaiting transplantation for periods ranging from a few months to several years [4, 5] The pediatric dialysis population is remarkably heterogeneous in many ways, as will be described in this chapter Unlike adult dialysis populations in which the primary kidney disease diagnoses tend to cluster within a narrow range of etiologies, pediatric dialysis populations display a variety of different primary kidney disorders, many of which must still be considered in overall patient management, despite having reached end-stage levels of kidney function [6] In this chapter, we have attempted to broadly describe the pediatric dialysis patient population by examining available data on such basic demographic characteristics as age at presentation, primary kidney disease diagnosis, and dialysis modality choice Comprehensive data on the demographics of a region’s or a nation’s pediatric dialysis patient population are available from several large ESKD patient registries and a few published reviews [7–13] Our objective is not to attempt a precise accounting of these data, nor is it to systematically compare findings from one pediatric ESKD registry to another While the methodology required for such rigorous cross- registry analyses exists, it would require access to data elements beyond the summaries published in available registry reports Rather, we have attempted to use and interpret available information to provide a snapshot of pediatric chronic dialysis as it has been practiced around the world during the early decades of the twenty-first century J J Fadrowski (*) Division of Pediatric Nephrology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA e-mail: jfadrow1@jhmi.edu ources of Demographic Data S on Pediatric Dialysis Patients L Rees Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK e-mail: l.rees@ucl.ac.uk The European Dialysis and Transplant Association – European Renal Association (EDTA) The importance of differences that characterize pediatric dialysis patient © Springer Nature Switzerland AG 2021 B A Warady et al (eds.), Pediatric Dialysis, https://doi.org/10.1007/978-3-030-66861-7_3 35 36 d emographics when compared to adult patients was first understood as a result of the pioneering efforts of the EDTA, which has published an annual report containing pediatric summary data from a group of European countries for more than 20 years Many of the survey techniques and conventions piloted and refined by the EDTA were later adopted by pediatric registries in other regions During the past few decades, the work of the EDTA with regard to pediatric dialysis was supplanted by the development of national ESKD patient registries, some of which have focused on pediatric issues From its new coordinating center at the University of Amsterdam, the EDTA resumed publication of an annual report in 1998 The latest ERA-EDTA 2016 Report, available at https://www.era-edta-reg.org/index.jsp, contains summary data from 36 European countries on patients of all ages in which information on children is largely reported in aggregate for the age group 0–19 years J J Fadrowski and L Rees ernment support, which includes almost all pediatric patients Thus, while the NAPRTCS contains pediatric data compiled only in specialized pediatric kidney centers in four North American countries, the USRDS includes data on children treated in both adult and pediatric centers in the United States In addition, patients are included in USRDS pediatric reports only if they initiated dialysis prior to their 19th birthday The 2018 USRDS Annual Data Report is available on the Internet at https://www.usrds.org/adr.aspx [15] The International Pediatric Peritoneal Dialysis Network (IPPN) IPPN started as a database for peritoneal dialysis (PD) but has now expanded to include hemodialysis (HD) patients as well so that its new name is the International Pediatric Dialysis Network (IPDN) The network is a global consortium of pediatric nephrology centers dedicated to the care of children on chronic dialysis To date, 3582 patients have been enrolled in the PD registry at 126 contributing centers in The North American Pediatric Renal Trials 43 countries, and 864 patients have been enrolled and Collaborative Studies (NAPRTCS) The in the HD registry at 82 contributing centers in 36 NAPRTCS is a voluntary collaborative data- countries (http://www.pedpd.org) sharing and research effort supported by more than 140 pediatric kidney treatment centers in The International Pediatric Nephrology the United States, Canada, Mexico, and Costa Association (IPNA) IPNA is currently developRica Founded in 1987 to study kidney trans- ing a new global registry, the aim being to plantation, the NAPRTCS expanded in 1992 to improve knowledge about the incidence and outinclude children receiving dialysis in participat- comes of pediatric renal replacement therapy ing NAPRTCS transplant centers NAPRTCS (RRT) around the globe By use of a questionenrolls dialysis patients up to their 21st birthday naire to its members, so far 94 countries (repreand thus describes a slightly older cohort than senting 86.2% of the world childhood population) the other registries Information was obtained for have responded, and 84 countries report they the present review from the NAPRTCS 2011 and have the means to provide RRT to children 2014 Annual Data Reports, available at https:// Among the 84 countries providing RRT, 51 web.emmes.com/study/ped/annlrept/annlrept (60.7%) had national registries for both dialysis html [9, 14] and transplantation, (10.7%) had either a dialysis or a transplant registry, participated in interThe United States Renal Data System national registries only (7.1%), and in 18 (USRDS) The USRDS provides a different per- (21.4%), children on RRT were not followed in spective on pediatric dialysis in the United States any registry (Fig. 3.1) A systematic search of from that seen in the NAPRTCS. The USRDS the literature related to this study identified 92 pediatric data are compiled from reports submit- pediatric RRT registries, primarily national regted to the US government healthcare funding istries located in Europe, North America, and agency on all dialysis patients eligible for gov- Asia [7] 3 The Demographics of Dialysis in Children 37 Missing data National Tx and Dx registry Incomplete Tx and Dx registry Dx registry only International registry only No registry No pediatric RRT Fig 3.1 Countries with national pediatric RRT registries in place according to survey Tx Transplantation, Dx dialysis (Source: Adapted from Ploos van Amstel/Pediatric Nephrology) [7] Incidence and Prevalence Access to Dialysis Around the World Significant variations in the incidence of RRT in children exist in the world Figure 3.2 shows the incidence of RRT in children in 2008 by country [16] There are huge disparities in access to dialysis for children around the world (Fig. 3.3) [7, 17] Management of the child with ESKD is labor-intensive and costly and is restricted in countries where resources are limited Indeed, over three quarters of children on RRT live in Europe, North America, or Japan, where such treatment is available to the majority of children However, children in the rest of the world are not so advantaged: a recent metaanalysis of studies and reports from sub-Saharan African countries found that most children with ESKD remain undiagnosed and untreated and die Among those who were dialyzed, 61% received one or more dialysis sessions, and only 35% remained on dialysis for at least 3 months One-third died or were presumed to have died without transplantation The most likely explanation for the inability to commence or continue treatment was that families were unable to pay [18] The IPDN looked at the impact of economic conditions on chronic PD practices and outcomes in 33 countries around the world Compared to higher-income countries, in low-income countries, the dialysis populations had a smaller fraction of children younger than 3 years of age at dialysis initiation and were less likely to have comborbidities in addition to kidney disease Children on PD in low-income countries were also found to be approximately standard deviation shorter and had worse survival compared to those in wealthier countries [19] Differences in access to and outcomes of RRT have also been described in developed countries The incidence of pediatric RRT between 2007 and 2011 varied widely between countries in Europe, with the lowest incidence in Eastern Europe (3.6 per million children [pmc]) and the highest incidence in Northern Europe (8.1 pmc) There was not much variation in the occurrence of specific kidney diseases by region Among countries that were wealthier and spent more on healthcare and where patients pay less out of pocket for healthcare, rates of RRT were higher Thus, differences in the macroeconomics of the countries, which limit equal access to healthcare services, are speculated to be the primary driver of the varation in pediatric RRT rates in Europe [20] In the United States, Medicare, a national health insurance program, pays for dialysis in children and adults if private insurance is not J J Fadrowski and L Rees 38 20 Incidence of RRT (pmarp) 18 16 14 12 10 Fig 3.2 Incidence of RRT in children in 2008 by country The orange bars correspond to incidence in children aged 0–14 years; the sum of the orange and blue bars corresponds to the incidence in children aged 0−19 years New Zealand USA UK (Scotland) Argentina Turkey Sweden Denmark Canada Australia The Netherlands Spain* Italy* Greece Portugal Malaysia Taiwan Austria Guatemala UK France* Romania Poland Norway Uruguay Japan Switzerland Finland Russia Pmarp, Per million age-related population *France 16 out of 26 regions, Italy 13 out of 20 regions, Spain out of 18 regions in the 15- to 19-year-old age group (Source: Adapted from Harambat/Pediatric Nephrology) [16] Missing data All modalities offered HD snd PD HD only No pediatric RRT Fig 3.3 Countries where pediatric RRT (HD, PD, and/or transplant) is offered according to survey of 94 countries (Source: Adapted from Ploos van Amstel/Pediatric Nephrology) [7] available However, differences in access to therapies for ESKD exist Time to first RRT was examined among African American and non- African American children with CKD with a median age of 10 years Times to dialysis were shorter among African American children even when accounting for socioeconomic status, yet access to kidney transplant occurred later [21] Disparity in access to kidney transplant is thought to underly the increased risk of mortality observed in non-Hispanic black children compared to white children on dialysis [22] Among children with CKD in the USRDS, GFR decline was similar across income groups, but better blood pressure control and correction of height deficits were observed among the highest-income group (≥$75,000/year) compared to the lower groups [23] Efforts to improve universal access to RRT ... heterogeneous in many ways, as will be described in this chapter Unlike adult dialysis populations in which the primary kidney disease diagnoses tend to cluster within a narrow range of etiologies, pediatric... in overall patient management, despite having reached end-stage levels of kidney function [6] In this chapter, we have attempted to broadly describe the pediatric dialysis patient population by... 3.1) A systematic search of from that seen in the NAPRTCS. The USRDS the literature related to this study identified 92 pediatric data are compiled from reports submit- pediatric RRT registries,