Jaap Groothoff - Academia.edu (original) (raw)

Papers by Jaap Groothoff

Research paper thumbnail of Renal replacement therapy for children throughout the world: the need for a global registry

Pediatric nephrology (Berlin, Germany), 2018

To describe the factors affecting the incidence of renal replacement therapy (RRT) among children... more To describe the factors affecting the incidence of renal replacement therapy (RRT) among children, information from RRT registries is required. We aimed to give an overview of existing pediatric RRT registries worldwide, identify regions with a need to commence or increase data collection on pediatric RRT, and provide a rationale for developing a global RRT registry. A survey assessing pediatric RRT registry status was sent to International Pediatric Nephrology Associateion (IPNA) members in 127 countries in January 2016. The survey was complemented by a systematic literature search for active pediatric RRT registries. Complete survey responses were retrieved from 94 countries (representing 86.2% of the world childhood population), with 84 (81.2%) having the means to provide RRT to children, given that there are no other limitations such as financial, social, or religious restraints. Fifty-one (35.3%) countries had national registries for both dialysis and transplantation, nine (30....

Research paper thumbnail of Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry

Pediatric Nephrology, 2017

Background As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease ... more 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 <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). 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 pa-On behalf of the ESPN WG CAKUT/UTI/ bladder dysfunction * Marjolein Bonthuis

Research paper thumbnail of The association of donor and recipient age with graft survival in paediatric renal transplant recipients in a European Society for Paediatric Nephrology/European Renal Association–European Dialysis and Transplantation Association Registry study

Nephrology Dialysis Transplantation, 2017

Background. The impact of donor age in paediatric kidney transplantation is unclear. We therefore... more Background. The impact of donor age in paediatric kidney transplantation is unclear. We therefore examined the association of donor-recipient age combinations with graft survival in children. Methods. Data for 4686 first kidney transplantations performed in 13 countries in 1990-2013 were extracted from the ESPN/ ERA-EDTA Registry. The effect of donor and recipient age combinations on 5-year graft-failure risk, stratified by donor source, was estimated using Kaplan-Meier survival curves and Cox regression, while adjusting for sex, primary renal diseases with a high risk of recurrence, pre-emptive transplantation, year of transplantation and country. Results. The risk of graft failure in older living donors (50-75 years old) was similar to that of younger living donors {adjusted hazard ratio [aHR] 0.74 [95% confidence interval (CI) 0.38-1.47]}. Deceased donor (DD) age was non-linearly associated with graft survival, with the highest risk of graft failure found in the youngest donor age group [0-5 years; compared with donor ages 12-19 years; aHR 1.69 (95% CI 1.26-2.26)], especially among the youngest recipients (0-11 years). DD age had little effect on graft failure in recipients' ages 12-19 years. Conclusions. Our results suggest that donations from older living donors provide excellent graft outcomes in all paediatric recipients. For young recipients, the allocation of DDs over the age of 5 years should be prioritized.

Research paper thumbnail of Racial variation in cardiovascular disease risk factors among European children on renal replacement therapy-results from the European Society of Paediatric Nephrology/European Renal Association - European Dialysis and Transplant Association Registry

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2017

Racial differences in overall mortality rates have been found in children on renal replacement th... more Racial differences in overall mortality rates have been found in children on renal replacement therapy (RRT). We used data from the European Society of Paediatric Nephrology/European Renal Association - European Dialysis and Transplant Association Registry to study racial variation in the prevalence of cardiovascular disease (CVD) risk factors among European children on RRT. We included patients aged <20 years between 2006-13 who (i) initiated dialysis treatment or (ii) had a renal transplant vintage of ≥1 year. Racial groups were defined as white, black, Asian and other. The CVD risk factors assessed included uncontrolled hypertension, obesity, hyperphosphataemia and anaemia. Differences between racial groups in CVD risk factors were examined using generalized estimating equation (GEE) models while adjusting for potential confounders. In this study, 1161 patients on dialysis and 1663 patients with a transplant were included. The majority of patients in both groups were white (73...

Research paper thumbnail of Mortality risk disparities in children receiving chronic renal replacement therapy for the treatment of end-stage renal disease across Europe: an ESPN-ERA/EDTA registry analysis

Lancet (London, England), Jan 20, 2017

We explored the variation in country mortality rates in the paediatric population receiving renal... more We explored the variation in country mortality rates in the paediatric population receiving renal replacement therapy across Europe, and estimated how much of this variation could be explained by patient-level and country-level factors. In this registry analysis, we extracted patient data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry for 32 European countries. We included incident patients younger than 19 years receiving renal replacement therapy. Adjusted hazard ratios (aHR) and the explained variation were modelled for patient-level and country-level factors with multilevel Cox regression. The primary outcome studied was all-cause mortality while on renal replacement therapy. Between Jan 1, 2000, and Dec 31, 2013, the overall 5 year renal replacement therapy mortality rate was 15·8 deaths per 1000 patient-years (IQR 6·4-16·4). France had a mortality rate (9·2) of more than 3 SDs ...

Research paper thumbnail of Infants Requiring Maintenance Dialysis: Outcomes of Hemodialysis and Peritoneal Dialysis

American journal of kidney diseases : the official journal of the National Kidney Foundation, Jan 9, 2016

The impact of different dialysis modalities on clinical outcomes has not been explored in young i... more The impact of different dialysis modalities on clinical outcomes has not been explored in young infants with chronic kidney failure. Cohort study. Data were extracted from the ESPN/ERA-EDTA Registry. This analysis included 1,063 infants 12 months or younger who initiated dialysis therapy in 1991 to 2013. Type of dialysis modality. Differences between infants treated with peritoneal dialysis (PD) or hemodialysis (HD) in patient survival, technique survival, and access to kidney transplantation were examined using Cox regression analysis while adjusting for age at dialysis therapy initiation, sex, underlying kidney disease, and country of residence. 917 infants initiated dialysis therapy on PD, and 146, on HD. Median age at dialysis therapy initiation was 4.5 (IQR, 0.7-7.9) months, and median body weight was 5.7 (IQR, 3.7-7.5) kg. Although the groups were homogeneous regarding age and sex, infants treated with PD more often had congenital anomalies of the kidney and urinary tract (CAK...

Research paper thumbnail of Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry

Pediatric Nephrology, 2016

Research paper thumbnail of Kidney Versus Combined Kidney and Liver Transplantation in Young People With Autosomal Recessive Polycystic Kidney Disease: Data From the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant (ESPN/ERA-EDTA) Registry

American journal of kidney diseases : the official journal of the National Kidney Foundation, Jan 20, 2016

The choice for either kidney or combined liver-kidney transplantation in young people with kidney... more The choice for either kidney or combined liver-kidney transplantation in young people with kidney failure and liver fibrosis due to autosomal recessive polycystic kidney disease (ARPKD) can be challenging. We aimed to analyze the characteristics and outcomes of transplantation type in these children, adolescents, and young adults. Cohort study. We derived data for children, adolescents, and young adults with ARPKD with either kidney or combined liver-kidney transplants for 1995 to 2012 from the ESPN/ERA-EDTA Registry, a European pediatric renal registry collecting data from 36 European countries. Liver transplantation. Transplantation and patient survival. 202 patients with ARPKD aged 19 years or younger underwent transplantation after a median of 0.4 (IQR, 0.0-1.4) years on dialysis therapy at a median age of 9.0 (IQR, 4.1-13.7) years. 32 (15.8%) underwent combined liver-kidney transplantation, 163 (80.7%) underwent kidney transplantation, and 7 (3.5%) were excluded because transpl...

Research paper thumbnail of Mortality risk in European children with end-stage renal disease on dialysis

Kidney International, 2016

We aimed to describe survival in European pediatric dialysis patients and compare the differentia... more We aimed to describe survival in European pediatric dialysis patients and compare the differential mortality risk between patients starting on hemodialysis (HD) and peritoneal dialysis (PD). Data for 6473 patients under 19 years of age or younger were extracted from the European Society of Pediatric Nephrology, the European Renal Association, and European Dialysis and Transplant Association Registry for 36 countries for the years 2000 through 2013. Hazard ratios (HRs) were adjusted for age at start of dialysis, sex, primary renal disease, and country. A secondary analysis was performed on a propensity score-matched (PSM) cohort. The overall 5-year survival rate in European children starting on dialysis was 89.5% (95% confidence interval [CI] 87.7%-91.0%). The mortality rate was 28.0 deaths per 1000 patient years overall. This was highest (36.0/1000) during the first year of dialysis and in the 0- to 5-year age group (49.4/1000). Cardiovascular events (18.3%) and infections (17.0%) were the main causes of death. Children selected to start on HD had an increased mortality risk compared with those on PD (adjusted HR 1.39, 95% CI 1.06-1.82, PSM HR 1.46, 95% CI 1.06-2.00), especially during the first year of dialysis (HD/PD adjusted HR 1.70, 95% CI 1.22-2.38, PSM HR 1.79, 95% CI 1.20-2.66), when starting at older than 5 years of age (HD/PD: adjusted HR 1.58, 95% CI 1.03-2.43, PSM HR 1.87, 95% CI 1.17-2.98) and when children have been seen by a nephrologist for only a short time before starting dialysis (HD/PD adjusted HR 6.55, 95% CI 2.35-18.28, PSM HR 2.93, 95% CI 1.04-8.23). Because unmeasured case-mix differences and selection bias may explain the higher mortality risk in the HD population, these results should be interpreted with caution.

Research paper thumbnail of Racial Disparities in Access to and Outcomes of Kidney Transplantation in Children, Adolescents, and Young Adults: Results From the ESPN/ERA-EDTA (European Society of Pediatric Nephrology/European Renal Association−European Dialysis and Transplant Association) Registry

American Journal of Kidney Diseases, 2016

Background: Racial disparities in kidney transplantation in children have been found in the Unite... more Background: Racial disparities in kidney transplantation in children have been found in the United States, but have not been studied before in Europe. Study Design: Cohort study. Setting & Participants: Data were derived from the ESPN/ERA-EDTA Registry, an international pediatric renal registry collecting data from 36 European countries. This analysis included 1,134 young patients (aged #19 years) from 8 medium-to high-income countries who initiated renal replacement therapy (RRT) in 2006 to 2012. Factor: Racial background. Outcomes & Measurements: Differences between racial groups in access to kidney transplantation, transplant survival, and overall survival on RRT were examined using Cox regression analysis while adjusting for age at RRT initiation, sex, and country of residence. Results: 868 (76.5%) patients were white; 59 (5.2%), black; 116 (10.2%), Asian; and 91 (8.0%), from other racial groups. After a median follow-up of 2.8 (range, 0.1-3.0) years, we found that black (HR, 0.49; 95% CI, 0.34-0.72) and Asian (HR, 0.54; 95% CI, 0.41-0.71) patients were less likely to receive a kidney transplant than white patients. These disparities persisted after adjustment for primary renal disease. Transplant survival rates were similar across racial groups. Asian patients had higher overall mortality risk on RRT compared with white patients (HR, 2.50; 95% CI, 1.14-5.49). Adjustment for primary kidney disease reduced the effect of Asian background, suggesting that part of the association may be explained by differences in the underlying kidney disease between racial groups. Limitations: No data for socioeconomic status, blood group, and HLA profile. Conclusions: We believe this is the first study examining racial differences in access to and outcomes of kidney transplantation in a large European population. We found important differences with less favorable outcomes for black and Asian patients. Further research is required to address the barriers to optimal treatment among racial minority groups.

Research paper thumbnail of Prevalence and Predictors of Anemia Following Renal Transplantation in European Children

Research paper thumbnail of Dialysate cancer antigen 125 levels in children treated with peritoneal dialysis

Advances in peritoneal dialysis. Conference on Peritoneal Dialysis, 2000

Peritoneal mesothelial cells are important for local host defense and membrane integrity. Dialysa... more Peritoneal mesothelial cells are important for local host defense and membrane integrity. Dialysate cancer antigen 125 (dCA125) has been shown to be a good marker for the mesothelial cell mass in adult peritoneal dialysis (PD) patients. In children on PD, no information is available yet. We measured dCA125 in 65 dialysate samples from 24 PD children with a median age of 9.2 years (range: 2-18 years) and a median treatment time of 2.6 years (range: 0.1-9.3 years) on PD. The median dCA125 concentration was 8 U/mL (range: 2.3-30.7 U/mL), and the CA125 appearance rate (CA125AR) was 66.5 U/min/1.73 m2 (range: 18-282 U/min/1.73 m2). On cross-sectional analysis, a negative correlation was found between dCA125 and duration of PD treatment (r = -0.3, p = 0.04). No relation was found between age and dCA125 or CA125AR when the first measurement from each child was considered. No correlation was found between dCA125 and the mass transfer area coefficient of creatinine (MTACcreat). Longitudinal ...

Research paper thumbnail of Demographics of paediatric renal replacement therapy in Europe: 2007 annual report of the ESPN/ERA-EDTA registry

Pediatric Nephrology, 2010

Few data are available regarding the epidemiology of endstage renal disease (ESRD) in children. T... more Few data are available regarding the epidemiology of endstage renal disease (ESRD) in children. The European Society of Paediatric Nephrology (ESPN), in collaboration with the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA), has recently established a meta-registry of European paediatric patient registries [1]. Thirty European countries reported individual patient data with information on date of birth, gender, start date renal replacement therapy (RRT), treatment modality at the start, changes in treatment, and important events such as The ESPN/ERA-EDTA registry study group consists of:

Research paper thumbnail of Clear peritoneal effluent in a child on CCPD with a phlegmonous appendicitis

Pediatric Nephrology, 2005

Peritonitis as a result of a perorated appendicitis is a rare but life-threatening situation in a... more Peritonitis as a result of a perorated appendicitis is a rare but life-threatening situation in a patient on peritoneal dialysis (PD). As far as we are aware, the combination of clear dialysate effluent and phlegmonous appendicitis in a patient on PD has not previously been described. We report a 16-year-old girl with acute onset of abdominal pain and vomiting who turned out to have phlegmonous appendicitis, despite having a clear dialysate effluent with normal cell count, and who subsequently developed E coli peritonitis after surgery.

Research paper thumbnail of Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation

Pediatric Nephrology, 2010

Research paper thumbnail of Likelihood of children with end-stage kidney disease in Europe to live with a functioning kidney transplant is mainly explained by nonmedical factors

Pediatric Nephrology, 2013

Registry data can be used to assess associations between medical and health-policy factors and th... more Registry data can be used to assess associations between medical and health-policy factors and the likelihood of children on renal replacement therapy (RRT) to live with a functioning kidney transplant in Europe. A survey questionnaire was distributed among renal registry representatives in 38 European countries, and additional data was obtained from the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) registry. Thirty-two countries with a pediatric RRT program responded. The median percentage of children by country on RRT with a functioning transplant was 62 % (interquartile range 39-77). One per million population increase in donation rate from deceased donors was associated with a 5 % increase in the percentage of functioning transplants; the existence of an intermediate and high pediatric priority policy doubled and tripled this percentage, respectively, compared with no priority, whereas an increase in living donor pediatric kidney transplant rate of one per million children was associated with a 14 % higher percentage of functioning transplants. The percentage of functioning transplants was also strongly associated with the gross domestic product (GDP). Considerable variations exist in the percentages of prevalent pediatric RRT populations with functioning renal transplants across Europe. A macroeconomic indicator such as GDP is the most important determinant of these international differences. Efforts should be made for living donation programs and pediatric allocation priority to increase access to kidney transplantation for children.

Research paper thumbnail of Simultaneous reversal of risk factors for cardiac death and intensified therapy in long-term survivors of paediatric end-stage renal disease over the last 10 years

Nephrology Dialysis Transplantation, 2013

Background. In an extended long-term follow-up of patients on chronic renal replacement therapy (... more Background. In an extended long-term follow-up of patients on chronic renal replacement therapy (RRT) since childhood (LERIC study), we observed a substantial reduction in cardiovascular (CV) death over the last decade. In this study, we investigated the contemporaneous changes in risk factors for CV death and cardioprotective therapy. Methods. The cohort consisted of 140 Dutch patients, who were born before 1979 and started RRT before 15 years of age between 1972 and 1992. We compared the prevalence of various factors in 2000 and 2010 by calculating matched odds ratios (OR matched). Results. Median age of patients was 38.5 years (range 23.2-50.8) in 2010, after a median time on RRT of 28 years. The prevalence of CV risk factors decreased from 41.3% in 2000 to

Research paper thumbnail of Quality of life in adults with end-stage renal disease since childhood is only partially impaired

Nephrology Dialysis Transplantation, 2003

Background. Little is known about the quality of life of adults with end-stage renal disease (ESR... more Background. Little is known about the quality of life of adults with end-stage renal disease (ESRD) since childhood. In a long-term follow-up study, we assessed quality of life in these patients and compared their outcomes with those in the general population and in dialysis patients with adult-onset of ESRD. Methods. All Dutch adult patients with onset of ESRD at age 0-14 years between 1972 and 1992 were asked to complete the RAND-36 questionnaire. We obtained clinical information by reviewing all available medical charts, and by current history. Patient scores were compared with those of age-matched healthy controls and with those of patients with adult onset of dialysis, of whom data were derived from a national study on the adequacy of dialysis (NECOSAD-2). Results. We obtained a complete RAND-36 score in 131 of 187 surviving patients. Clinical characteristics of participants and non-participants were similar. Compared with the general population, we found a higher prevalence of impaired quality of life in dialysis patients in the following domains: physical functioning, role limitations due to physical health, social functioning (SF), general health perception (GH) and physical component summary. In other domains, impaired quality of life was equally or less prevalent (mental component summary). In transplanted patients, only impaired GH and SF were more prevalent. In dialysis patients with adult ESRD onset, impaired quality of life was more prevalent than in the general population in all domains, except in emotional role limitations. Conclusions. Dialysis patients with paediatric ESRD have an impaired physical quality of life, but in contrast to dialysis patients with adult ESRD onset, they have a normal mental quality of life.

Research paper thumbnail of Dyslipidaemia in children on renal replacement therapy

Nephrology Dialysis Transplantation, 2013

Background. Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originate... more Background. Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originates from adult patients and small paediatric studies. We describe the prevalence of dyslipidaemia, and potential determinants associated with lipid measures in a large cohort of paediatric ESRD patients. Methods. In the ESPN/ERA-EDTA registry, lipid measurements were available for 976 patients aged 2-17 years from 19 different countries from the year 2000 onwards. Dyslipidaemia was defined as triglycerides >100 mg/dL (2-9 years) or >130 mg/dL (9-17 years), high-density lipoprotein (HDL) cholesterol <40 mg/dL or non-HDL cholesterol >145 mg/dL. Missing data were supplemented using multiple imputation. Results. The prevalence of dyslipidaemia was 85.1% in peritoneal dialysis (PD) patients, 76.1% in haemodialysis (HD) patients and 55.5% among renal allograft recipients. Both low and high body mass index (BMI) were associated with a less favourable lipid profile. Younger age was associated with a worse lipid profile among PD patients. HDL levels significantly improved after transplantation, whereas no significant improvements were found for triglyceride and non-HDL levels. In transplant recipients, use of cyclosporin was associated with significantly higher non-HDL and HDL levels than tacrolimus usage (P < 0.01). In transplant patients with eGFR < 29 mL/min/1.73 m 2 , the mean triglyceride level was 137 mg/dL (99% confidence interval (CI): 119-159) compared with 102 mg/dL among those with eGFR > 90 mL/min/1.73 m 2 (P < 0.0001). Conclusions. Dyslipidaemia is common among paediatric ESRD patients in Europe. Young age and PD treatment are associated with worse lipid profiles. Although lipid levels generally improve after transplantation, dyslipidaemia may persist due to decreased graft function, high BMI or to the use of certain immunosuppressants.

Research paper thumbnail of Important differences in management policies for children with end-stage renal disease in the Netherlands and Belgium--report from the RICH-Q study

Nephrology Dialysis Transplantation, 2011

Background. The low prevalence of childhood end-stage renal disease and the small centre sizes ha... more Background. The low prevalence of childhood end-stage renal disease and the small centre sizes have been a barrier for clinical studies and the development of evidence-based guidelines for chronic renal replacement therapy (cRRT) in children. Few data exist on the quality of care for these patients and the applicability of existing guidelines. The aim of this study is to quantify variation in treatment policies and actually delivered care in nine centres that deliver cRRT for children. Methods. We surveyed treatment policies in all nine centres in the Netherlands and Belgium and compared them with the actually provided therapies and with recommendations from available guidelines. Data on treatment policies were gathered by questionnaires; actually provided care and outcomes were registered prospectively from 2007 to 2010. Results. Data on policies and actual patient care were obtained from all nine centres. We found relevant differences between centres in treatment policies on various topics, e.g. estimated glomerular filtration rate threshold as an indication for initiation of cRRT, preferred initial mode of cRRT, peritoneal dialysis catheter care, haemodialysis frequency and vascular access. Discrepancies were seen between stated treatment policies and actual performed therapies. For the majority of policies, no evidence-based guidelines are available. Conclusions. Health care disparities exist due to large and unwanted variation in treatment policies between hospitals providing cRRT for children. Delivered care does not live up to stated policies, for which clear and internationally accepted guidelines are lacking.

Research paper thumbnail of Renal replacement therapy for children throughout the world: the need for a global registry

Pediatric nephrology (Berlin, Germany), 2018

To describe the factors affecting the incidence of renal replacement therapy (RRT) among children... more To describe the factors affecting the incidence of renal replacement therapy (RRT) among children, information from RRT registries is required. We aimed to give an overview of existing pediatric RRT registries worldwide, identify regions with a need to commence or increase data collection on pediatric RRT, and provide a rationale for developing a global RRT registry. A survey assessing pediatric RRT registry status was sent to International Pediatric Nephrology Associateion (IPNA) members in 127 countries in January 2016. The survey was complemented by a systematic literature search for active pediatric RRT registries. Complete survey responses were retrieved from 94 countries (representing 86.2% of the world childhood population), with 84 (81.2%) having the means to provide RRT to children, given that there are no other limitations such as financial, social, or religious restraints. Fifty-one (35.3%) countries had national registries for both dialysis and transplantation, nine (30....

Research paper thumbnail of Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry

Pediatric Nephrology, 2017

Background As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease ... more 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 <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). 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 pa-On behalf of the ESPN WG CAKUT/UTI/ bladder dysfunction * Marjolein Bonthuis

Research paper thumbnail of The association of donor and recipient age with graft survival in paediatric renal transplant recipients in a European Society for Paediatric Nephrology/European Renal Association–European Dialysis and Transplantation Association Registry study

Nephrology Dialysis Transplantation, 2017

Background. The impact of donor age in paediatric kidney transplantation is unclear. We therefore... more Background. The impact of donor age in paediatric kidney transplantation is unclear. We therefore examined the association of donor-recipient age combinations with graft survival in children. Methods. Data for 4686 first kidney transplantations performed in 13 countries in 1990-2013 were extracted from the ESPN/ ERA-EDTA Registry. The effect of donor and recipient age combinations on 5-year graft-failure risk, stratified by donor source, was estimated using Kaplan-Meier survival curves and Cox regression, while adjusting for sex, primary renal diseases with a high risk of recurrence, pre-emptive transplantation, year of transplantation and country. Results. The risk of graft failure in older living donors (50-75 years old) was similar to that of younger living donors {adjusted hazard ratio [aHR] 0.74 [95% confidence interval (CI) 0.38-1.47]}. Deceased donor (DD) age was non-linearly associated with graft survival, with the highest risk of graft failure found in the youngest donor age group [0-5 years; compared with donor ages 12-19 years; aHR 1.69 (95% CI 1.26-2.26)], especially among the youngest recipients (0-11 years). DD age had little effect on graft failure in recipients' ages 12-19 years. Conclusions. Our results suggest that donations from older living donors provide excellent graft outcomes in all paediatric recipients. For young recipients, the allocation of DDs over the age of 5 years should be prioritized.

Research paper thumbnail of Racial variation in cardiovascular disease risk factors among European children on renal replacement therapy-results from the European Society of Paediatric Nephrology/European Renal Association - European Dialysis and Transplant Association Registry

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2017

Racial differences in overall mortality rates have been found in children on renal replacement th... more Racial differences in overall mortality rates have been found in children on renal replacement therapy (RRT). We used data from the European Society of Paediatric Nephrology/European Renal Association - European Dialysis and Transplant Association Registry to study racial variation in the prevalence of cardiovascular disease (CVD) risk factors among European children on RRT. We included patients aged <20 years between 2006-13 who (i) initiated dialysis treatment or (ii) had a renal transplant vintage of ≥1 year. Racial groups were defined as white, black, Asian and other. The CVD risk factors assessed included uncontrolled hypertension, obesity, hyperphosphataemia and anaemia. Differences between racial groups in CVD risk factors were examined using generalized estimating equation (GEE) models while adjusting for potential confounders. In this study, 1161 patients on dialysis and 1663 patients with a transplant were included. The majority of patients in both groups were white (73...

Research paper thumbnail of Mortality risk disparities in children receiving chronic renal replacement therapy for the treatment of end-stage renal disease across Europe: an ESPN-ERA/EDTA registry analysis

Lancet (London, England), Jan 20, 2017

We explored the variation in country mortality rates in the paediatric population receiving renal... more We explored the variation in country mortality rates in the paediatric population receiving renal replacement therapy across Europe, and estimated how much of this variation could be explained by patient-level and country-level factors. In this registry analysis, we extracted patient data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry for 32 European countries. We included incident patients younger than 19 years receiving renal replacement therapy. Adjusted hazard ratios (aHR) and the explained variation were modelled for patient-level and country-level factors with multilevel Cox regression. The primary outcome studied was all-cause mortality while on renal replacement therapy. Between Jan 1, 2000, and Dec 31, 2013, the overall 5 year renal replacement therapy mortality rate was 15·8 deaths per 1000 patient-years (IQR 6·4-16·4). France had a mortality rate (9·2) of more than 3 SDs ...

Research paper thumbnail of Infants Requiring Maintenance Dialysis: Outcomes of Hemodialysis and Peritoneal Dialysis

American journal of kidney diseases : the official journal of the National Kidney Foundation, Jan 9, 2016

The impact of different dialysis modalities on clinical outcomes has not been explored in young i... more The impact of different dialysis modalities on clinical outcomes has not been explored in young infants with chronic kidney failure. Cohort study. Data were extracted from the ESPN/ERA-EDTA Registry. This analysis included 1,063 infants 12 months or younger who initiated dialysis therapy in 1991 to 2013. Type of dialysis modality. Differences between infants treated with peritoneal dialysis (PD) or hemodialysis (HD) in patient survival, technique survival, and access to kidney transplantation were examined using Cox regression analysis while adjusting for age at dialysis therapy initiation, sex, underlying kidney disease, and country of residence. 917 infants initiated dialysis therapy on PD, and 146, on HD. Median age at dialysis therapy initiation was 4.5 (IQR, 0.7-7.9) months, and median body weight was 5.7 (IQR, 3.7-7.5) kg. Although the groups were homogeneous regarding age and sex, infants treated with PD more often had congenital anomalies of the kidney and urinary tract (CAK...

Research paper thumbnail of Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry

Pediatric Nephrology, 2016

Research paper thumbnail of Kidney Versus Combined Kidney and Liver Transplantation in Young People With Autosomal Recessive Polycystic Kidney Disease: Data From the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant (ESPN/ERA-EDTA) Registry

American journal of kidney diseases : the official journal of the National Kidney Foundation, Jan 20, 2016

The choice for either kidney or combined liver-kidney transplantation in young people with kidney... more The choice for either kidney or combined liver-kidney transplantation in young people with kidney failure and liver fibrosis due to autosomal recessive polycystic kidney disease (ARPKD) can be challenging. We aimed to analyze the characteristics and outcomes of transplantation type in these children, adolescents, and young adults. Cohort study. We derived data for children, adolescents, and young adults with ARPKD with either kidney or combined liver-kidney transplants for 1995 to 2012 from the ESPN/ERA-EDTA Registry, a European pediatric renal registry collecting data from 36 European countries. Liver transplantation. Transplantation and patient survival. 202 patients with ARPKD aged 19 years or younger underwent transplantation after a median of 0.4 (IQR, 0.0-1.4) years on dialysis therapy at a median age of 9.0 (IQR, 4.1-13.7) years. 32 (15.8%) underwent combined liver-kidney transplantation, 163 (80.7%) underwent kidney transplantation, and 7 (3.5%) were excluded because transpl...

Research paper thumbnail of Mortality risk in European children with end-stage renal disease on dialysis

Kidney International, 2016

We aimed to describe survival in European pediatric dialysis patients and compare the differentia... more We aimed to describe survival in European pediatric dialysis patients and compare the differential mortality risk between patients starting on hemodialysis (HD) and peritoneal dialysis (PD). Data for 6473 patients under 19 years of age or younger were extracted from the European Society of Pediatric Nephrology, the European Renal Association, and European Dialysis and Transplant Association Registry for 36 countries for the years 2000 through 2013. Hazard ratios (HRs) were adjusted for age at start of dialysis, sex, primary renal disease, and country. A secondary analysis was performed on a propensity score-matched (PSM) cohort. The overall 5-year survival rate in European children starting on dialysis was 89.5% (95% confidence interval [CI] 87.7%-91.0%). The mortality rate was 28.0 deaths per 1000 patient years overall. This was highest (36.0/1000) during the first year of dialysis and in the 0- to 5-year age group (49.4/1000). Cardiovascular events (18.3%) and infections (17.0%) were the main causes of death. Children selected to start on HD had an increased mortality risk compared with those on PD (adjusted HR 1.39, 95% CI 1.06-1.82, PSM HR 1.46, 95% CI 1.06-2.00), especially during the first year of dialysis (HD/PD adjusted HR 1.70, 95% CI 1.22-2.38, PSM HR 1.79, 95% CI 1.20-2.66), when starting at older than 5 years of age (HD/PD: adjusted HR 1.58, 95% CI 1.03-2.43, PSM HR 1.87, 95% CI 1.17-2.98) and when children have been seen by a nephrologist for only a short time before starting dialysis (HD/PD adjusted HR 6.55, 95% CI 2.35-18.28, PSM HR 2.93, 95% CI 1.04-8.23). Because unmeasured case-mix differences and selection bias may explain the higher mortality risk in the HD population, these results should be interpreted with caution.

Research paper thumbnail of Racial Disparities in Access to and Outcomes of Kidney Transplantation in Children, Adolescents, and Young Adults: Results From the ESPN/ERA-EDTA (European Society of Pediatric Nephrology/European Renal Association−European Dialysis and Transplant Association) Registry

American Journal of Kidney Diseases, 2016

Background: Racial disparities in kidney transplantation in children have been found in the Unite... more Background: Racial disparities in kidney transplantation in children have been found in the United States, but have not been studied before in Europe. Study Design: Cohort study. Setting & Participants: Data were derived from the ESPN/ERA-EDTA Registry, an international pediatric renal registry collecting data from 36 European countries. This analysis included 1,134 young patients (aged #19 years) from 8 medium-to high-income countries who initiated renal replacement therapy (RRT) in 2006 to 2012. Factor: Racial background. Outcomes & Measurements: Differences between racial groups in access to kidney transplantation, transplant survival, and overall survival on RRT were examined using Cox regression analysis while adjusting for age at RRT initiation, sex, and country of residence. Results: 868 (76.5%) patients were white; 59 (5.2%), black; 116 (10.2%), Asian; and 91 (8.0%), from other racial groups. After a median follow-up of 2.8 (range, 0.1-3.0) years, we found that black (HR, 0.49; 95% CI, 0.34-0.72) and Asian (HR, 0.54; 95% CI, 0.41-0.71) patients were less likely to receive a kidney transplant than white patients. These disparities persisted after adjustment for primary renal disease. Transplant survival rates were similar across racial groups. Asian patients had higher overall mortality risk on RRT compared with white patients (HR, 2.50; 95% CI, 1.14-5.49). Adjustment for primary kidney disease reduced the effect of Asian background, suggesting that part of the association may be explained by differences in the underlying kidney disease between racial groups. Limitations: No data for socioeconomic status, blood group, and HLA profile. Conclusions: We believe this is the first study examining racial differences in access to and outcomes of kidney transplantation in a large European population. We found important differences with less favorable outcomes for black and Asian patients. Further research is required to address the barriers to optimal treatment among racial minority groups.

Research paper thumbnail of Prevalence and Predictors of Anemia Following Renal Transplantation in European Children

Research paper thumbnail of Dialysate cancer antigen 125 levels in children treated with peritoneal dialysis

Advances in peritoneal dialysis. Conference on Peritoneal Dialysis, 2000

Peritoneal mesothelial cells are important for local host defense and membrane integrity. Dialysa... more Peritoneal mesothelial cells are important for local host defense and membrane integrity. Dialysate cancer antigen 125 (dCA125) has been shown to be a good marker for the mesothelial cell mass in adult peritoneal dialysis (PD) patients. In children on PD, no information is available yet. We measured dCA125 in 65 dialysate samples from 24 PD children with a median age of 9.2 years (range: 2-18 years) and a median treatment time of 2.6 years (range: 0.1-9.3 years) on PD. The median dCA125 concentration was 8 U/mL (range: 2.3-30.7 U/mL), and the CA125 appearance rate (CA125AR) was 66.5 U/min/1.73 m2 (range: 18-282 U/min/1.73 m2). On cross-sectional analysis, a negative correlation was found between dCA125 and duration of PD treatment (r = -0.3, p = 0.04). No relation was found between age and dCA125 or CA125AR when the first measurement from each child was considered. No correlation was found between dCA125 and the mass transfer area coefficient of creatinine (MTACcreat). Longitudinal ...

Research paper thumbnail of Demographics of paediatric renal replacement therapy in Europe: 2007 annual report of the ESPN/ERA-EDTA registry

Pediatric Nephrology, 2010

Few data are available regarding the epidemiology of endstage renal disease (ESRD) in children. T... more Few data are available regarding the epidemiology of endstage renal disease (ESRD) in children. The European Society of Paediatric Nephrology (ESPN), in collaboration with the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA), has recently established a meta-registry of European paediatric patient registries [1]. Thirty European countries reported individual patient data with information on date of birth, gender, start date renal replacement therapy (RRT), treatment modality at the start, changes in treatment, and important events such as The ESPN/ERA-EDTA registry study group consists of:

Research paper thumbnail of Clear peritoneal effluent in a child on CCPD with a phlegmonous appendicitis

Pediatric Nephrology, 2005

Peritonitis as a result of a perorated appendicitis is a rare but life-threatening situation in a... more Peritonitis as a result of a perorated appendicitis is a rare but life-threatening situation in a patient on peritoneal dialysis (PD). As far as we are aware, the combination of clear dialysate effluent and phlegmonous appendicitis in a patient on PD has not previously been described. We report a 16-year-old girl with acute onset of abdominal pain and vomiting who turned out to have phlegmonous appendicitis, despite having a clear dialysate effluent with normal cell count, and who subsequently developed E coli peritonitis after surgery.

Research paper thumbnail of Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation

Pediatric Nephrology, 2010

Research paper thumbnail of Likelihood of children with end-stage kidney disease in Europe to live with a functioning kidney transplant is mainly explained by nonmedical factors

Pediatric Nephrology, 2013

Registry data can be used to assess associations between medical and health-policy factors and th... more Registry data can be used to assess associations between medical and health-policy factors and the likelihood of children on renal replacement therapy (RRT) to live with a functioning kidney transplant in Europe. A survey questionnaire was distributed among renal registry representatives in 38 European countries, and additional data was obtained from the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) registry. Thirty-two countries with a pediatric RRT program responded. The median percentage of children by country on RRT with a functioning transplant was 62 % (interquartile range 39-77). One per million population increase in donation rate from deceased donors was associated with a 5 % increase in the percentage of functioning transplants; the existence of an intermediate and high pediatric priority policy doubled and tripled this percentage, respectively, compared with no priority, whereas an increase in living donor pediatric kidney transplant rate of one per million children was associated with a 14 % higher percentage of functioning transplants. The percentage of functioning transplants was also strongly associated with the gross domestic product (GDP). Considerable variations exist in the percentages of prevalent pediatric RRT populations with functioning renal transplants across Europe. A macroeconomic indicator such as GDP is the most important determinant of these international differences. Efforts should be made for living donation programs and pediatric allocation priority to increase access to kidney transplantation for children.

Research paper thumbnail of Simultaneous reversal of risk factors for cardiac death and intensified therapy in long-term survivors of paediatric end-stage renal disease over the last 10 years

Nephrology Dialysis Transplantation, 2013

Background. In an extended long-term follow-up of patients on chronic renal replacement therapy (... more Background. In an extended long-term follow-up of patients on chronic renal replacement therapy (RRT) since childhood (LERIC study), we observed a substantial reduction in cardiovascular (CV) death over the last decade. In this study, we investigated the contemporaneous changes in risk factors for CV death and cardioprotective therapy. Methods. The cohort consisted of 140 Dutch patients, who were born before 1979 and started RRT before 15 years of age between 1972 and 1992. We compared the prevalence of various factors in 2000 and 2010 by calculating matched odds ratios (OR matched). Results. Median age of patients was 38.5 years (range 23.2-50.8) in 2010, after a median time on RRT of 28 years. The prevalence of CV risk factors decreased from 41.3% in 2000 to

Research paper thumbnail of Quality of life in adults with end-stage renal disease since childhood is only partially impaired

Nephrology Dialysis Transplantation, 2003

Background. Little is known about the quality of life of adults with end-stage renal disease (ESR... more Background. Little is known about the quality of life of adults with end-stage renal disease (ESRD) since childhood. In a long-term follow-up study, we assessed quality of life in these patients and compared their outcomes with those in the general population and in dialysis patients with adult-onset of ESRD. Methods. All Dutch adult patients with onset of ESRD at age 0-14 years between 1972 and 1992 were asked to complete the RAND-36 questionnaire. We obtained clinical information by reviewing all available medical charts, and by current history. Patient scores were compared with those of age-matched healthy controls and with those of patients with adult onset of dialysis, of whom data were derived from a national study on the adequacy of dialysis (NECOSAD-2). Results. We obtained a complete RAND-36 score in 131 of 187 surviving patients. Clinical characteristics of participants and non-participants were similar. Compared with the general population, we found a higher prevalence of impaired quality of life in dialysis patients in the following domains: physical functioning, role limitations due to physical health, social functioning (SF), general health perception (GH) and physical component summary. In other domains, impaired quality of life was equally or less prevalent (mental component summary). In transplanted patients, only impaired GH and SF were more prevalent. In dialysis patients with adult ESRD onset, impaired quality of life was more prevalent than in the general population in all domains, except in emotional role limitations. Conclusions. Dialysis patients with paediatric ESRD have an impaired physical quality of life, but in contrast to dialysis patients with adult ESRD onset, they have a normal mental quality of life.

Research paper thumbnail of Dyslipidaemia in children on renal replacement therapy

Nephrology Dialysis Transplantation, 2013

Background. Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originate... more Background. Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originates from adult patients and small paediatric studies. We describe the prevalence of dyslipidaemia, and potential determinants associated with lipid measures in a large cohort of paediatric ESRD patients. Methods. In the ESPN/ERA-EDTA registry, lipid measurements were available for 976 patients aged 2-17 years from 19 different countries from the year 2000 onwards. Dyslipidaemia was defined as triglycerides >100 mg/dL (2-9 years) or >130 mg/dL (9-17 years), high-density lipoprotein (HDL) cholesterol <40 mg/dL or non-HDL cholesterol >145 mg/dL. Missing data were supplemented using multiple imputation. Results. The prevalence of dyslipidaemia was 85.1% in peritoneal dialysis (PD) patients, 76.1% in haemodialysis (HD) patients and 55.5% among renal allograft recipients. Both low and high body mass index (BMI) were associated with a less favourable lipid profile. Younger age was associated with a worse lipid profile among PD patients. HDL levels significantly improved after transplantation, whereas no significant improvements were found for triglyceride and non-HDL levels. In transplant recipients, use of cyclosporin was associated with significantly higher non-HDL and HDL levels than tacrolimus usage (P < 0.01). In transplant patients with eGFR < 29 mL/min/1.73 m 2 , the mean triglyceride level was 137 mg/dL (99% confidence interval (CI): 119-159) compared with 102 mg/dL among those with eGFR > 90 mL/min/1.73 m 2 (P < 0.0001). Conclusions. Dyslipidaemia is common among paediatric ESRD patients in Europe. Young age and PD treatment are associated with worse lipid profiles. Although lipid levels generally improve after transplantation, dyslipidaemia may persist due to decreased graft function, high BMI or to the use of certain immunosuppressants.

Research paper thumbnail of Important differences in management policies for children with end-stage renal disease in the Netherlands and Belgium--report from the RICH-Q study

Nephrology Dialysis Transplantation, 2011

Background. The low prevalence of childhood end-stage renal disease and the small centre sizes ha... more Background. The low prevalence of childhood end-stage renal disease and the small centre sizes have been a barrier for clinical studies and the development of evidence-based guidelines for chronic renal replacement therapy (cRRT) in children. Few data exist on the quality of care for these patients and the applicability of existing guidelines. The aim of this study is to quantify variation in treatment policies and actually delivered care in nine centres that deliver cRRT for children. Methods. We surveyed treatment policies in all nine centres in the Netherlands and Belgium and compared them with the actually provided therapies and with recommendations from available guidelines. Data on treatment policies were gathered by questionnaires; actually provided care and outcomes were registered prospectively from 2007 to 2010. Results. Data on policies and actual patient care were obtained from all nine centres. We found relevant differences between centres in treatment policies on various topics, e.g. estimated glomerular filtration rate threshold as an indication for initiation of cRRT, preferred initial mode of cRRT, peritoneal dialysis catheter care, haemodialysis frequency and vascular access. Discrepancies were seen between stated treatment policies and actual performed therapies. For the majority of policies, no evidence-based guidelines are available. Conclusions. Health care disparities exist due to large and unwanted variation in treatment policies between hospitals providing cRRT for children. Delivered care does not live up to stated policies, for which clear and internationally accepted guidelines are lacking.