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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 [...]

Background The aim of this study was to analyze psychosocial aspects of chronic kidney disease (CKD) in children treated with automated peritoneal dialysis (APD). Methods The study assessed 41 children > 2 (range 2.1-18) years of age and their parents. Data concerning the illness and sociodemographic parameters were collected. Patients completed the Paediatric Quality of Life Inventory (PedsQL) and their parents the PedsQL-proxy version, General Health Questionnaire (GHQ-12), Berlin Social Support Scales (BSSS), and Caregiver's Burden Scale (CBS). Results Parents rated their children's overall health-related quality of life (QoL) as well as their physical and emotional functioning lower than the patients themselves. The majority of primary caregivers had a medium level of the total burden index in the CBS and higher values in the scales need for support and perceived available support than in the received support (BSSS). In the GHQ-12, 51.2% of primary caregivers had scores >2 points, which indicated the possible occurrence of abnormal mental functioning. Conclusions Financial support for patients' families is necessary. Parents who provide primary care to children on PD require, above all, emotional support and assistance in self-fulfilment. More than half of them may have impaired mental function. There is the strong need to provide continuous psychological care for caregivers. Differences in perception of the children's activity in varied areas by the patients themselves and their caregivers may contribute to further problems within families. Keywords Children * Peritoneal dialysis * Health-related quality of life * Parents' perceived burdens * Primary caregivers, Introduction Home peritoneal dialysis (PD) is the preferred chronic dialysis modality for children with end-stage renal disease (ESRD) [1]. Thanks to technological advances, inter alia, various PD regimens and solutions, [...]

Background/aim: Children on dialysis are under increased risk of influenza and invasive pneumococcal disease. Although vaccination against these microorganisms are recommended in dialysis patients and despite the fact that these vaccines can reduce disease burden and rates of hospitalization due to infection, vaccination rates are below expected and desired. We aimed to evaluate influenza and pneumococcal vaccination and infection rates in European pediatric dialysis centers. Materials and methods: In 16 centers from 11 countries, 357 pediatric dialysis patients were evaluated retrospectively during 1 year of observation period between 01.01.2014 and 01.01.2015. Results: In all centers, vaccination policy included immunization of dialysis patients with inactive influenza vaccine and pneumococcal conjugate vaccine (PCV). Fifty percent of the centers recommended pneumococcal polysaccharide vaccine following routine PCV series. A significantly higher pneumococcal vaccination rate (43.9%) was seen in peritoneal dialysis (PD) patients compared to those on hemodialysis (HD) (32.9%) (p = 0.035), while the rates for influenza were similar (42.4% and 46.1% respectively, p = 0.496). Among all dialysis patients, 2.2% (n = 8) developed pneumonia and 6.4% (n = 23) was infected by Influenza. Pneumococcic pneumonia rate was 5% for 140 patients who received antipneumococcal vaccine, while only one pneumonia episode was recorded out of 217 unvaccinated patients (p = 0.007). The influenza virus infection rates were similar for patients vaccinated and nonvaccinated (7 % and 6 %, respectively). Conclusions: Although influenza and pneumococcal vaccines are highly recommended in pediatric dialysis patients, vaccination rates were lower than expected. Pneumococcal vaccination rates were higher in PD compared to the patients on HD. The rate of children with influenza infection was higher than pneumonia. The efficacy of influenza and pneumococcal vaccines was highlighted by the low infection rates. Higher pneumonia rates in patients vaccinated against pneumococcus compared to unvaccinated ones might be due to coexisting risk factors. [ABSTRACT FROM AUTHOR]

Dear Editors, We read with great interest the article by Sato et al., recently published in Pediatric Nephrology, who presented a patient with atypical Pneumocystis jiroveci pneumonia (PCP) with multiple [...]

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450-1098) ml/m in Null/null, 403 (260-538) ml/m in Null/mis, 230 (169-357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150-267) ml/m in CKD stage 1, 472 (266-880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies., (© 2021. The Author(s).)

To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (≤ 3 months; VEBNE) and early (4-15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ≤ 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort.

Patients with bladder dysfunction comprise over 30% of pediatric patients on renal replacement therapy. We report on a successful cadaveric pre-emptive renal transplantation performed in a boy born with posterior urethral valve. Following bilateral ureterocutaneostomies, left nephrectomy and valve resection, at 6 years of age a continent ileocolonocystoplasty was performed. The boy started intermittent daytime catheterization, passing urine both via urethra and fistula. At the age of 18 he received a renal transplant. Continuing the previous regime, at 1.5 years follow up his graft is well functioning (GFR >75 ml/min/1.73 m2) with sporadic episodes of urinary tract infection.

Introduction: The mode of PD treatment is dependent on the individual transport properties of the peritoneal membrane. Two multicentre trials performed in the U.S. (PPDSC) and Europe (MEPPS) have established reference curves for solute equilibration in children performed with the use of 1100 ml/m2 fill volume in the former and 1000 ml/m2 in the latter study., Aim of the Study: Assessment of basal peritoneal membrane equilibration based on PET tests in polish children and adolescents treated with chronic peritoneal dialysis., Material and Methods: 58 PET tests from patients treated at 8 Polish PD centres were analysed. The mean time of performing PET test was 6,5 months after the start of PD therapy. All of the patients had been peritonitis free from onset. The mean fill volume was 1021 (906-1170) ml/m2., Results: Based on the results of creatinine and glucose equilibration we established basal peritoneal solute transport curves for polish PD children using an average fill volume of 1020 ml/m2. The following values were obtained at 4hrs of dwell time for 2.27% glucose solution: D/P for creatinine = 0.68 +/- 0.15 and D/Do for glucose = 0.39 +/- 0.12., Conclusions: The DIP creatinine equilibration curves were similar to the previously published reference curves for children, whereas those for glucose was significantly lower. Using a fill volume scaled to body surface area of 1020 ml/m2 equilibration curves for glucose and creatinine are similar in children over 1 year of age and adults.

We retrospectively analysed peritoneal dialysis treatment in 29 infants dialysed in 9 paediatric centres in Poland in the years 1993-2004. The mean age at the start of dialysis was 4.9 +/- 3.5 months (range 2 days to 11 months), mean body mass 5.6 +/- 2.5 kg (range 2.5 to 11 kg). The mean duration of PD was 6.8 +/- 3.9 in the first year of life and total duration of the therapy 34 +/- 27 months. Of the 29 infants 4 died (2 in infancy), 11 underwent renal transplantation, in 2 children PD was stopped (they received a conventional treatment) and 12 were still dialysed at the date of data collection. The peritonitis rate was 1/9.5 patient-month and exit site infection rate 1/16 patient-month up to 1 year of life. 9 children (31%) required hernia repairs and in 9 catheters were replaced. Chronic peritoneal dialysis in infants is associated with high risk of infections and surgical complications and remains a challenge for paediatric nephrologists.

Malnutrition is a major problem in patients with chronic renal failure, especially in children in the first three years of life. Meeting high energy needs in these patients may be difficult because of anorexia and vomiting, which are common in predialysis renal failure. Nutritional support for children with chronic renal failure often involves the use of nasogastric tubes or gastrostomy feeding. We describe an infant with pre-dialysis renal failure and severe malnutrition in whom only insertion of percutaneus endoscopic gastrostomy provided adequate caloric intake and satisfactory weight gain.

Unlabelled: One of the objectives of Polish Registry of Renal Replacement Therapy in Children established on 31st Dec. 2000 was to collect complete data on etiology of end stage renal disease (ESRD) in polish children., Material and Methods: Data on 469 patients (251 boys, 218 girls) aged 0-22 years treated with renal replacement therapy (RRT) at 13 pediatric dialysis units in Poland from 2000 to 2004 were analyzed. The mean age at start of dialysis was 10 years and 3 months. Renal diseases were defined according to EDTA coding system. Data is presented for the whole group, in 5-year age groups and separately for both sexes., Results: Congenital and genetic renal diseases were the cause of ESRF in 56% of the polish population of children and adolescents on RRT. 39% of causes were acquired diseases, 5% remained unidentified. Congenital and genetic causes dominated in children < 5 years of age (71%). They accounted for 49%, 61% and 45% of causes in the consecutive 5-year age groups. The most numerous group of congenital diseases leading to ESRF were uropathies 37% and 25% of causes in the consecutive age groups. In boys the most frequent uropathy was obstructive uropathy (25%), the majority caused by posterior urethral valves. In girls the most frequent uropathies were reflux nephropathy (10%) and nephropathy secondary to neurogenic bladder (9%). Uropathies were followed by renal hypo-dysplasia without urinary tract anomalies (11%) and cystic diseases (10%)., Conclusions: Congenital kidney anomalies and genetic diseases are the leading cause of end-stage renal disease in children up to 15 years of age.