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Background For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We examined trends in incidence and prevalence of KRT and patient survival. Methods We included all children aged Results Incidence of children commencing KRT Conclusions We found a stable incidence and increasing prevalence of European children on KRT 2007-2016. Five-year patient survival was good and was unchanged over time. These data can inform patients and healthcare providers and aid health policy makers on future resource planning of pediatric KRT in Europe., Author(s): Marjolein Bonthuis [sup.1] , Enrico Vidal [sup.2] , Anna Bjerre [sup.3] , Özlem Aydog [sup.4] , Sergey Baiko [sup.5] , Liliana Garneata [sup.6] , Isabella Guzzo [sup.7] , James [...]

Growth retardation is a major complication in children with chronic kidney disease (CKD) and on kidney replacement therapy (KRT). Conversely, better growth in childhood CKD is associated with an improvement in several hard morbidity-mortality endpoints. Data from pediatric international registries has demonstrated that improvements in the overall conservative management of CKD, the search for optimal dialysis, and advances in immunosuppression and kidney transplant techniques have led to a significant improvement of final height over time. Infancy still remains a critical period for adequate linear growth, and the loss of stature during the first years of life influences final height. Preliminary new original data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry confirm an association between the final height and the height attained at 2 years in children on KRT., Author(s): Marjolein Bonthuis [sup.1] , Jérôme Harambat [sup.2] , Kitty J. Jager [sup.1] , Enrico Vidal [sup.3] Author Affiliations: (1) ESPN/ERA-EDTA Registry, Department of Medical Informatics, Amsterdam Public Health Research [...]

Background Current guidelines advocate use of arteriovenous fistula (AVF) over central venous catheter (CVC) for children starting hemodialysis (HD). European data on current practice, determinants of access choice and switches, patient survival, and access to transplantation are limited. Methods We included incident patients from 18 European countries who started HD from 2000 to 2013 for whom vascular access type was reported to the ESPN/ERA-EDTA Registry. Data were evaluated using descriptive statistics, logistic and Cox regression models, and cumulative incidence competing risk analysis. Results Three hundred ninety-three (55.1%) of 713 children started HD with a CVC and were more often females, younger, had more often an unknown diagnosis, glomerulonephritis, or vasculitis, and lower hemoglobin and height-SDS at HD initiation. AVF patients were 91% less likely to switch to a second access, and two-year patient survival was 99.6% (CVC, 97.2%). Children who started with an AVF were less likely to receive a living donor transplant (adjusted HR, 0.30; 95% CI, 0.16-0.54) and more likely to receive a deceased donor transplant (adjusted HR, 1.50; 95% CI, 1.17-1.93), even after excluding patients who died or were transplanted in the first 6 months. Conclusions CVC remains the most frequent type of vascular access in European children commencing HD. Our results suggest that the choice for CVC is influenced by the time of referral, rapid onset of end-stage renal disease, young age, and an expected short time to transplantation. The role of vascular access type on the pattern between living and deceased donation in subsequent transplantation requires further study., Author(s): Michael Boehm [sup.1] , Marjolein Bonthuis [sup.2] , Marlies Noordzij [sup.2] , Jérôme Harambat [sup.3] , Jaap W. Groothoff [sup.4] , Ángel Alonso Melgar [sup.5] , Jadranka Buturovic [sup.6] [...]

Survival in the pediatric end-stage renal disease (ESRD) population has improved substantially over recent decades. Nonetheless, mortality remains at least 30 times higher than that of healthy peers. Patient survival is multifactorial and dependent on various patient and treatment characteristics and degree of economic welfare of the country in which a patient is treated. In this educational review, we aim to delineate current evidence regarding mortality risk in the pediatric ESRD population and provide pediatric nephrologists with up-to-date information required to counsel affected families., Author(s): Nicholas C. Chesnaye [sup.1] , Karlijn J. van Stralen [sup.2] , Marjolein Bonthuis [sup.1] , Jérôme Harambat [sup.3] , Jaap W. Groothoff [sup.4] , Kitty J. Jager [sup.1] Author [...]

Background As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data. Methods Data were available for 88 male PBS patients aged Results Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups. Conclusions This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU., Author(s): Fatos Yalcinkaya [sup.1] , Marjolein Bonthuis [sup.2] , Beyza Doganay Erdogan [sup.3] , Karlijn J. van Stralen [sup.4] , Sergey Baiko [sup.5] , Hassib Chehade [sup.6] , Heather Maxwell [...]

Background Congenital nephrotic syndrome (CNS) of the Finnish type, NPHS1, is the most severe form of CNS. Outcomes of renal replacement therapy (RRT) in NPHS1 patients in Europe were analysed using data from the ESPN/ERA-EDTA Registry. As NPHS1 is most prevalent in Finland and the therapeutic approach differs from that in many other countries, we compared outcomes in Finnish and other European patients. Methods NPHS1 mutations were confirmed in 170 children with CNS who initiated RRT (dialysis or renal transplantation) between 1991 and 2012. Finnish (n = 66) and non-Finnish NPHS1 patients (n = 104) were compared with respect to treatment policy, age at first RRT and renal transplantation (RTX), patient and graft survival, estimated glomerular filtration rate (eGFR) and growth. Age-matched patients with congenital anomalies of the kidney and urinary tract (CAKUT) served as controls. Results Finnish NPHS1 patients were significantly younger than non-Finnish patients, both at the start of RRT and at the time of RTX. We found similar overall 5-year patient survival on RRT (91 %) and graft survival (89 %) in both NPHS1 groups and CAKUT controls. At the start of RRT, height standard deviation score (SDS) was higher in Finnish patients than in non-Finnish patients (mean [95 % CI]: -1.31 [-2.13 to -0.49] and -3.0 [-4.22 to -1.91], p < 0.01 respectively), but not at 5 years of age. At 5 years of age height and body mass index (BMI) SDS were similar to those of CAKUT controls. Conclusions Overall, 5-year patient and graft survival of both Finnish and non-Finnish NPHS1 patients on RRT were excellent and comparable with CAKUT patients with equally early RRT onset and was independent of the timing of RRT initiation and RTX., Author(s): Tuula Holttä[sup.1] , Marjolein Bonthuis[sup.2] , Karlijn J. Van Stralen[sup.2] , Anna Bjerre[sup.3] , Rezan Topaloglu[sup.4] , Fatih Ozaltin[sup.5] , Christer Holmberg[sup.1] , Jerome Harambat[sup.6] , Kitty J. Jager[sup.2] [...]

End-stage renal disease (ESRD) in children is a medically challenging condition. Due to its rarity and special features, methodologically sound collaborative studies are required. In 2007, a new European registry of pediatric renal replacement therapy (RRT), the ESPN/ERA-EDTA Registry, was launched. In recent years, the Registry has provided comprehensive data on incidence, prevalence, patient characteristics, RRT modalities, and mortality in pediatric ESRD, along with relevant insights into cardiovascular risk, anemia, nutrition and growth, transplantation outcomes, and rare diseases. In this review, we describe the study design and structure underlying the ESPN/ERA-EDTA Registry, summarize the major research findings from more than 20 publications, and discuss current limitations and the future challenges to overcome., Author(s): Jerôme Harambat[sup.1] , Marjolein Bonthuis[sup.2] , Jaap W. Groothoff[sup.3] , Franz Schaefer[sup.4] , E. Jane Tizard[sup.5] , Enrico Verrina[sup.6] , Karlijn J. van Stralen[sup.2] , Kitty J. Jager[sup.2] Author [...]

Background Our aim was to determine the prevalence of sub-target hemoglobin (Hb) levels in children with a renal allograft and to identify potential determinants associated with these Hb levels. Methods Data from 3669 children with a functioning renal allograft, aged Results According to the NKF/KDOQI classification and the UK-NICE guidelines, 49.8 and 7.8 % of the patients, respectively, were anemic. Hb levels were strongly associated with graft function, with Hb levels of 12.6 g/dl in children with chronic kidney disease (CKD) stage 1, declining to 10.7 g/dl in children with CKD stage 5 (P < 0.001). Higher Hb levels were associated with the use of tacrolimus compared to ciclosporin (0.14 g/dl; 95 % confidence interval 0.02-0.27; P = 0.002). Low Hb levels were associated with an increased risk of graft failure (P = 0.01) or combined graft failure and death (P < 0.01), but not with death alone (not significant). Conclusions Anemia is present in a significant proportion of European pediatric kidney transplant recipients and is associated with renal allograft dysfunction and type of immunosuppressants used. In our patient cohort, higher Hb levels were associated with better graft and patient survival and less hypertension., Author(s): Leah A. Krischock[sup.1] , Karlijn J. van Stralen[sup.2] , Enrico Verrina[sup.3] , E. Jane Tizard[sup.4] , Marjolein Bonthuis[sup.2] , Gyorgy Reusz[sup.5] , Farida K Hussain[sup.6] , Augustina Jankauskiene[sup.7] , [...]

Background The ESPN/ERA-EDTA Registry collects data on European children with end-stage renal disease receiving renal replacement therapy (RRT) who are listed on national and regional renal registries in Europe. In this paper we report on the analysis of demographic data collected from 2009 to 2011. Methods Data on primary renal disease, incidence, prevalence, 4-year survival, transplantation rate and causes of death in paediatric patients receiving RRT were extracted from the ESPN/ERA-EDTA Registry for 37 European countries. Results The incidence of RRT in paediatric patients in Europe during the study period was 5.5 cases per million age-related population (pmarp) in patients aged 0-14 years and varied markedly between countries (interquartile range 3.4 7.0 years). The prevalence of RRT was 27.9 pmarp and increased with age, with 67% of prevalent patients living with a functioning graft. The probability of receiving a transplant within 4 years was 76.9% and was lowest in patients aged 0-4 years (68.9%). Mortality in paediatric patients treated with RRT was 55-fold higher than that of the general EU paediatric population. Overall survival at 4 years was 93.7%, with the poorest survival in patients aged 0-4 years and in patients starting on dialysis. Infections (19.9%) were the primary cause of death in European paediatric RRT patients. Conclusion Considerable variation exists in the current demographics of children treated with RRT across Europe. Keywords Paediatrics * Renal replacement therapy * Chronic kidney failure * Europe * Incidence * Prevalence * Survival, Introduction End-stage renal disease (ESRD) is a very rare condition in children [1]. Information on the number of new cases (incidence), the size of the problem (prevalence) and the outcomes [...]