Use of national and international growth charts for studying height in european children: Development of up-to-date european height-for-age charts

Background: Growth charts based on data collected in different populations and time periods are key tools to assess children's linear growth. We analyzed the impact of geographic factors and the secular trend on height-for-age charts currently used in European populations, developed up-to-date European growth charts, and studied the effect of u

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

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. 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. 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. 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.Peer reviewe

Dyslipidaemia in children on renal replacement therapy

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 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 90 mL/min/1.73 m2 (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 immunosuppressant

Growth in children on kidney replacement therapy: a review of data from patient registries

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

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

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. 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). 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. 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.Peer reviewe

The ERA Registry Annual Report 2022: Epidemiology of Kidney Replacement Therapy in Europe, with a focus on sex comparisons

ESKD; Dialysis; Kidney transplantation; Patient survivalEnfermedad renal terminal; Diálisis; Trasplante de riñón; Supervivencia del pacienteMalaltia Renal Crònica Terminal (MRCT); Diàlisi; Trasplantament de ronyó; Supervivència del pacientThe European Renal Association (ERA) Registry collects data on kidney replacement therapy (KRT) in patients with end-stage kidney disease (ESKD). This paper summarizes the ERA Registry Annual Report 2022, with a special focus on comparisons by sex. The supplement of this paper contains the complete ERA Registry Annual Report 2022. Data was collected from 53 national and regional KRT registries from 35 countries. Using this data, incidence, and prevalence of KRT, kidney transplantation rates, survival probabilities, and expected remaining lifetimes were calculated. In 2022, 530 million people of the European general population were covered by the ERA Registry. The incidence of KRT was 152 per million population (pmp). In incident patients, 54% were 65 years or older, 64% were male, and the most common primary renal disease (PRD) was diabetes mellitus (22%). At KRT initiation, 83% of patients received haemodialysis, 12% received peritoneal dialysis, and 5% underwent pre-emptive kidney transplantation. On 31 December 2022, the prevalence of KRT was 1074 pmp. In prevalent patients, 48% were 65 years or older, 62% were male, the most common PRD was of miscellaneous origin (18%), 56% of patients received haemodialysis, 5% received peritoneal dialysis, and 39% were living with a functioning graft. In 2022, the kidney transplantation rate was 40 pmp, with most kidneys coming from deceased donors (66%). For patients starting KRT between 2013 to 2017, 5-year survival probability was 52%. Compared with the general population, the expected remaining lifetime was 66% and 68% shorter for males and females, respectively, receiving dialysis, and 46% and 49% shorter for males and females, respectively, living with a functioning graft.The ERA Registry is funded by the European Renal Association (ERA). This article was written by R. Boenink et al. on behalf of the ERA Registry, which is an official body of the ERA. N.M. reports being board member for the NICE guidelines and Cyprus Renal Association. M.M.V. reports receiving grants from Instituto de Salud Carlos; consulting fees, payment for lectures, support for attending meetings and/or travel from Novo-Nordisk, Astra Zeneca, Boherinheim, Ingelheim-Lilly, Bayer, Menarini, Vifor; and is president of the Nephrology Madrilenian Society. A.M.D.S. ´ reports being board member for the Voivodeship Consultant for Nephrology. M.F.S.R. reports receiving consulting fees from Fresenius, Baxter, Nipro; payment for lectures from Baxter, Fresenius, Physidia; and support for attending meetings and/or travel from Vifor, Fresenius, NovoNordisk; and is board member of Fresenius European Home Dialysis Advisory Board and the Direction Committee of the Spanish Society of Nephrology. M.O.V. reports receiving consulting fees of SANOFI and support for attending meetings and/or travel by Fressenius and Sandoz. A.Å. reports receiving grants from Oripharm and AstraZeneca; payments from Oripharm and Glenmark; and receipt of study drug from AstraZeneca. I.R. reports being president of the Czech Society of Nephrology and Secretary-Treasurer of ERA 2017- 2024. M.O.R. reports payment for lectures from AstraZeneca; and is board member of AstraZeneca, ISN Eastern & Central Europe Regional Board, Ministry of Social Affairs of Estonia, and Nordic Peritoneal Dialysis Council. A.O. has received grants from Sanofi; is director of the Catedra Mundipharma-UAM of diabetic kidney disease and the Catedra AstraZeneca-UAM of chronic kidney disease and electrolytes; has received consultancy or speaker fees or travel support from Advicciene, Astellas, AstraZeneca, Amicus, Amgen, Fresenius Medical Care, GSK, BoehringerIngelheim, Bayer, Sanofi-Genzyme, Menarini, Kyowa Kirin, Alexion, Idorsia, Chiesi, Otsuka, Sysmex, Novo-Nordisk, and Vifor Fresenius Medical Care Renal Pharma; and is board member of ERA council and SOMANE. K.J.J. reports receiving funds from European Renal Association and European Society for Paediatric Nephrology, and is board member of SHARE RR working group. V.S.S. reports having support for the present manuscript from European Renal Association. All other co-authors declare that they have no relevant financial interests

Adult Height in Patients with Advanced CKD Requiring Renal Replacement Therapy during Childhood.

BACKGROUND AND OBJECTIVES: Growth and final height are of major concern in children with ESRD. This study sought to describe the distribution of adult height of patients who started renal replacement therapy (RRT) during childhood and to identify determinants of final height in a large cohort of RRT children. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A total of 1612 patients from 20 European countries who started RRT before 19 years of age and reached final height between 1990 and 2011 were included. Linear regression analyses were performed to calculate adjusted mean final height SD score (SDS) and to investigate its potential determinants. RESULTS: The median final height SDS was -1.65 (median of 168 cm in boys and 155 cm in girls). Fifty-five percent of patients attained an adult height within the normal range. Adjusted for age at start of RRT and primary renal diseases, final height increased significantly over time from -2.06 SDS in children who reached adulthood in 1990-1995 to -1.33 SDS among those reaching adulthood in 2006-2011. Older age at start of RRT, more recent period of start of RRT, cumulative percentage time on a functioning graft, and greater height SDS at initiation of RRT were independently associated with a higher final height SDS. Patients with congenital anomalies of the kidney and urinary tract and metabolic disorders had a lower final height than those with other primary renal diseases. CONCLUSIONS: Although final height remains suboptimal in children with ESRD, it has consistently improved over time

The ERA Registry Annual Report 2021: a summary

[EN] Background The European Renal Association (ERA) Registry collects data on kidney replacement therapy (KRT) in patients with end-stage kidney disease (ESKD). This paper is a summary of the ERA Registry Annual Report 2021, including a comparison across treatment modalities.Methods Data was collected from 54 national and regional registries from 36 countries, of which 35 registries from 18 countries contributed individual patient data and 19 registries from 19 countries contributed aggregated data. Using this data, incidence and prevalence of KRT, kidney transplantation rates, survival probabilities and expected remaining lifetimes were calculated.Result In 2021, 533.2 million people in the general population were covered by the ERA Registry. The incidence of KRT was 145 per million population (pmp). In incident patients, 55% were 65 years or older, 64% were male, and the most common primary renal disease (PRD) was diabetes (22%). The prevalence of KRT was 1040 pmp. In prevalent patients, 47% were 65 years or older, 62% were male, and the most common PRDs were diabetes and glomerulonephritis/sclerosis (both 16%). On 31 December 2021, 56% of patients received haemodialysis, 5% received peritoneal dialysis, and 39% were living with a functioning graft. The kidney transplantation rate in 2021 was 37 pmp, a majority coming from deceased donors (66%). For patients initiating KRT between 2012-2016, 5-year survival probability was 52%. Compared to the general population, life expectancy was 65% and 68% shorter for males and females receiving dialysis, and 40% and 43% shorter for males and females living with a functioning graft.The ERA Registry is funded by the European Renal Association (ERA). This article was written by B.A. Boerstra et al. on behalf of the ERA Registry, which is an official body of the ERA. P.B. reports payments from AstraZeneca and Takeda. S.B. reports consulting fees from GSK, Bayer, and AstraZeneca. A.C.A. reports payments from Diaverum Spain. F.J. reports payments from AstraZeneca, Boehringer Ingelheim, Servier, and Merck; and support for attending meetings and/or travel from Servier, AstraZeneca, Pfizer, and Fresenius. J.M. reports receiving support for attending meetings and/or travel from CSL Vifor. M.F.S.-R. reports receiving consulting fees from Baxter, Fresenius, and Nipro; payments from Baxter and Fresenius; and support for attending meetings and/or travel from Vifor, Fresenius, and Palex. I.Z. reports consulting fees from Astellas, Pharma, and Bayer; and payments from AstraZeneca, Bayer, Behringer Ingelheim, Norameda, and Swixx Biopharma. A.O. reports receiving grants from Sanofi; and consultancy or speaker fees or travel support from Advicciene, Astellas Pharma, AstraZeneca, Amicus, Amgen, Boehringer Ingelheim, Fresenius Medical Care, GSK, Bayer, Sanofi-Genzyme, Menarini, Mundipharma, Kyowa Kirin, Lilly, Alexion, Freeline, Idorsia, Chiesi, Otsuka, Novo-Nordisk, Sysmex, and Vifor Fresenius Medical Care Renal Pharma. K.J.J. reports receiving funds from ERA during the conduct of the study and grants from ESPN. V.S.S. reports receiving funds from ERA.Boerstra, BA.; Boenink, R.; Astley, ME.; Bonthuis, M.; Elhafeez, SA.; Arribas Monzón, F.; Asberg, A.... (2024). The ERA Registry Annual Report 2021: a summary. Clinical Kidney Journal. 17(2). https://doi.org/10.1093/ckj/sfad28117

Ten-year trends in epidemiology and outcomes of pediatric kidney replacement therapy in Europe : data from the ESPN/ERA-EDTA Registry

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 agedPeer reviewe