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Background Sociocultural issues play a key role in children needing kidney replacement therapy (KRT). Methods Data of incident patients < 18 years treated with chronic dialysis or preemptive kidney transplantation (pTx) between 2007 and 2016 were retrospectively collected from the Italian Pediatric Dialysis Registry; KRT modality and outcome were compared between patients with at least one non-Italian parent ('resident foreign patients,' RFPs) and those from native parents ('domestic patients,' DPs) and between the quinquennium 2007-2011 (period 1) and 2012-2016 (period 2). Results We included 448 children (26.8% RFPs). The percentage of RFPs increased from 23 to 30.3% (p = 0.08) from periods 1 to 2. They were younger (6.7 vs. 9.4 years, p = 0.025) and less often treated with pTx (3.3 vs. 13.4%, p = 0.009) than DPs. The percentage of pTx increased from period 1 to 2 in RFPs only (8.4-18.6%, p = 0.006). Independent predictors of a lower probability of pTx were lower age, belonging to RFPs group, starting KRT in period 1 and focal segmental glomerulosclerosis or glomerulopathy as primary kidney disease. Peritoneal dialysis was the preferred dialysis modality in both groups. Age, primary kidney disease, and center size were independently associated with dialysis modality choice. Patient survival, waiting time to Tx, and dialysis modality survival were not different between the two groups. Conclusions The proportion of patients receiving KRT born from immigrant families increased in recent years in Italy. They were younger and less often treated with pTx than domestic patients. In case of dialysis, the outcome was not different between the two groups., Author(s): Fabio Paglialonga [sup.1] , Silvia Consolo [sup.1] , Enrico Vidal [sup.2] , Mattia Parolin [sup.3] , Bruno Minale [sup.4] , Mario Giordano [sup.5] , Isabella Guzzo [sup.6] , Chiara [...]

Background COVID-19 was declared a global health emergency. Since children are less than 1% of reported cases, there is limited information to develop evidence-based practice recommendations. The objective of this study was to rapidly gather expert knowledge and experience to guide the care of children with chronic kidney disease during the COVID-19 pandemic. Methods A four-round multi-center Delphi exercise was conducted among 13 centers in 11 European countries of the European Pediatric Dialysis Working Group (EPDWG) between March, 16th and 20th 2020. Results were analyzed using a mixed methods qualitative approach and descriptive statistics. Results Thirteen COVID-19 specific topics of particular need for guidance were identified. Main themes encompassed testing strategies and results (n = 4), changes in use of current therapeutics (n = 3), preventive measurements of transmission and management of COVID-19 (n = 3), and changes in standard clinical care (n = 3). Patterns of center-specific responses varied according to regulations and to availability of guidelines. Conclusions As limited quantitative evidence is available in real time during the rapid spread of the COVID-19 pandemic, qualitative expert knowledge and experience represent the best evidence available. This Delphi exercise demonstrates that use of mixed methodologies embedded in an established network of experts allowed prompt analysis of pediatric nephrologists' response to COVID-19 during this fast-emerging public health crisis. Such rapid sharing of knowledge and local practices is essential to timely and optimal guidance for medical management of specific patient groups in multi-country health care systems such as those of Europe and the US., Author(s): Fabian Eibensteiner [sup.1] , Valentin Ritschl [sup.2] , Gema Ariceta [sup.3] , Augustina Jankauskiene [sup.4] , Günter Klaus [sup.5] , Fabio Paglialonga [sup.6] , Alberto Edefonti [sup.6] , Bruno [...]

The fatty acid profiles of patients with idiopathic nephrotic syndrome (INS) are different from that of healthy controls, even during remission, revealing an increase of the pro-inflammatory omega 6 series. It is still unknown whether the concomitance of nephrotic syndrome affects the potential positive effects of the Mediterranean diet on the levels of omega 3 and 6 fatty acids. We performed a cross-sectional study to evaluate the association between the adherence to the Mediterranean diet and fatty acid profile in 54 children with INS. The dietary habits were assessed through the validated Kidmed questionnaire. Patients with higher adherence had lower levels of linoleic acid and total omega-6. Moreover, a negative correlation between proteinuria and the anti-inflammatory omega-3 series was found. In conclusion, patients with INS with proteinuria and low adherence to the Mediterranean diet have an imbalance in the omega-6/omega-3 ratio that may benefit from following the Mediterranean diet.

Background Volume overload is a known risk factor for cardiovascular complications in children on hemodialysis (HD), but a measurable index of volume overload is still lacking. Methods We propose a novel index of pre-HD volume overload based on blood volume (BV) monitoring, the first hour refill index (RI), calculated as the ratio between the ultrafiltration rate indexed for body weight during the first HD hour and the percent BV change at the first hour of the treatment. This parameter was retrospectively calculated in 121 sessions in 11 oligoanuric children and young adults on chronic HD, with median age 14.3 years (range 5.4-22.4), and its association with left-ventricular mass index, pre-HD blood pressure, and number of antihypertensive medications was evaluated. Results The median RI was 2.07 ml/kg/h/%. There was a significant correlation between RI and median LVMI (r 0.66, p = 0.028), which was 53.4 g/m.sup.2.7 (45.7-64) in patients with a median RI > 2, and 36.6 g/m.sup.2.7 (24.9-47) in those with a median RI < 2 ml/kg/h/% (p = 0.01). The number of antihypertensive drugs per patient was significantly higher in patients with a RI > 2 than in those with a RI < 2 ml/kg/h/% (three vs one per patient; p = 0.02) while blood pressure was not significantly different between the two groups. Conclusions The ratio between the ultrafiltration rate per body weight and the BV change during the first hour of a HD session could be a promising index of refill capacity and pre-HD volume overload in children and young adults on chronic HD., Author(s): Fabio Paglialonga [sup.1] , Silvia Consolo [sup.1] , Alberto Edefonti [sup.1] , Giovanni Montini [sup.1] [sup.2] Author Affiliations: (Aff1) 0000 0004 1757 8749, grid.414818.0, Pediatric Nephrology, Dialysis, and Transplant [...]

Background Chronic haemodialysis (HD) in small children has not been adequately investigated. Methods This was a retrospective investigation of the use of chronic HD in 21 children aged Results The median age of the 21 children at start of HD was 11.4 [interquartile range (IQR) 6.2-14.6] months, and HD consisted mainly of haemodiafiltration for 3-4 h in [greater than or equal to]4 sessions/week. A total of 51 central venous catheters were placed, and the median survival of tunnelled and temporary lines was 349 and 31 days, respectively (p < 0.001). Eight children (38 %) showed evidence of central vein thrombosis. Although 19 % of patients received growth hormone and 63.6 % received enteral feeding, the weight and height of these patients remained suboptimal. During the HD period the haemoglobin level increased in all patients, but not to normal levels (from 8.5 to 9.6 g/dl) despite erythropoietin administration (503-600 U/kg/week). The hospitalisation rate was 1.94/patient-year. Seventeen patients underwent renal transplantation at a median age of 3.0 years. Four patients, all affected by severe comorbidities, died during follow-up (in 2 cases due to absence of a vascular access). The 5- and 10-year cumulative survival was 82.4 and 68.7 %, respectively. Conclusions Extracorporeal dialysis is feasible in children aged, Author(s): Fabio Paglialonga[sup.1] , Silvia Consolo[sup.1] , Carmine Pecoraro[sup.2] , Enrico Vidal[sup.3] , Bruno Gianoglio[sup.4] , Flora Puteo[sup.5] , Stefano Picca[sup.6] , Maria Teresa Saravo[sup.2] , Alberto Edefonti[sup.1] , Enrico [...]

Background To date, no study has investigated the use of vitamin K antagonists (VKA) in children undergoing chronic haemodialysis (HD) with a central venous catheter (CVC). Methods Consecutive patients aged Results The VKA group consisted of nine patients (median age 10.6 years; range 1.2-15.3 years) with 11 CVC, and the standard group comprised eight patients (11.8 years; 6.1-17.3 years) with ten CVC. The 6- and 12-month CVC survival was significantly longer in the VKA group than in the standard group (100 vs. 60 % and 83.3 vs. 16.7 %, respectively; p < 0.05), with a median survival of 369 and 195 days, respectively (p < 0.05). None of the CVC in the VKA group required removal due to malfunction, as compared to four in the standard group. No major bleeding episodes occurred in either group. Conclusions Therapy with VKA would appear to be safe in children on chronic HD and may improve CVC survival in patients at increased risk of CVC thrombosis., Author(s): Fabio Paglialonga[sup.1] , Andrea Artoni[sup.2] , Simon Braham[sup.2] , Silvia Consolo[sup.1] , Alberto Giannini[sup.3] , Giovanna Chidini[sup.3] , Luisa Napolitano[sup.3] , Ida Martinelli[sup.2] , Giovanni Montini[sup.1] , Alberto Edefonti[sup.1] [...]

Background Pleural or pericardial effusions secondary to pleuro-peritoneal fistula (PPF) and pericardio-peritoneal fistula (PcPF) are rare but serious complications of peritoneal dialysis (PD). Methods We conducted a 10-year survey across all participating centres in the European Paediatric Dialysis Working Group to review the incidence, diagnostic techniques, therapeutic options and outcome of children on chronic PD with PPF and/or PcPF. Results Of 1506 children on PD there were ten cases (8 of PPF, 1 each of PcPF and PPF + PcPF), with a prevalence of 0.66 %. The median age at presentation was 1.5 [inter-quartile range (IQR) 0.4-2.4] years, and nine children were Conclusion In conclusion, PPF and PcPF are rare in children on chronic PD, but are associated with significant morbidity, requiring a change of dialysis modality in all cases. Risk factors for PPF development include age of, Author(s): Stephanie Dufek[sup.1] , Tuula Holtta[sup.2] , Michel Fischbach[sup.3] , Gema Ariceta[sup.4] , Augustina Jankauskiene[sup.5] , Rimante Cerkauskiene[sup.5] , Claus Peter Schmitt[sup.6] , Betti Schaefer[sup.6] , Christoph Aufricht[sup.7] , Elizabeth [...]

Background Little is known about the clinical impact of interdialytic weight gain (IDWG) on oligoanuric children undergoing chronic hemodialysis (HD). Methods We retrospectively assessed IDWG, left ventricular mass index (LVMI) and its changes (ΔLVMI), pre-HD systolic and diastolic blood pressure (DBP), residual urine output, Kt/V, the frequency of intradialytic symptoms, normalized protein catabolic rate, and the 3-month change in the dry weight of 16 hemodialyzed oligoanuric patients with a median age of 14.8 years (range 5.0-17.9). Results There was a significant correlation between IDWG and median LVMI (r 0.55, p = 0.026), which was 27.3 g/m.sup.2.7 (22.5-37.6) in the patients with a median IDWG of 4 % (p = 0.003). None of the four patients with an IDWG of 4 % (p = 0.003); the former also had a better median ΔLVMI (-33.5 % vs -13.0 %; p = 0.02) and a lower median DBP sds (0.24 vs 1.72, p = 0.04). Conclusions There is a significant correlation between IDWG and LVMI in pediatric oligoanuric patients on chronic HD: those with an IDWG of >4 % are at a higher risk of left ventricular hypertrophy., Author(s): Fabio Paglialonga[sup.1] , Silvia Consolo[sup.1] , Maria Albina Galli[sup.2] , Sara Testa[sup.1] , Alberto Edefonti[sup.1] Author Affiliations: (1) Pediatric Nephrology and Dialysis Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore [...]

Background Few observations on apheresis in pediatric nephrology units have been published. Methods This retrospective study involved children [less than or equal to] 18 years undergoing plasma exchange (PE), immunoadsorption (IA), or double filtration plasmapheresis (DFPP) in 12 European pediatric nephrology units during 2012. Results Sixty-seven children underwent PE, ten IA, and three DFPP, for a total of 738 PE and 349IA/DFPP sessions; 67.2 % of PE and 69.2 % of IA/DFPP patients were treated for renal diseases, in particular focal segmental glomerulosclerosis (FSGS), hemolytic-uremic syndrome (HUS), and human leukocyte antigen (HLA) desensitization prior to renal transplantation; 20.9 % of PE and 23.1 % of IA/DFPP patients had neurological diseases. Membrane filtration was the most common technique, albumin the most frequently used substitution fluid, and heparin the preferred anticoagulant. PE achieved full disease remission in 25 patients (37.3 %), partial remission in 22 (32.8 %), and had no effect in 20 (29.9 %). The response to IA/ DFPP was complete in seven patients (53.8 %), partial in five (38.5 %), and absent in one (7.7 %). Minor adverse events occurred during 6.9 % of PE and 9.7 % of IA/DFPP sessions. Conclusions PE, IA, and DFPP are safe apheresis methods in children. Efficacy is high in pediatric patients with recurrent focal segmental glomerulosclerosis (FSGS), atypical hemolytic uremic syndrome (HUS), human leukocyte antigen (HLA) sensitization, and neurological autoimmune diseases. Keywords Pediatric apheresis * Therapeutic apheresis * Plasma exchange * Immunoadsorption * Double filtration plasmapheresis, Introduction Apheresis is a widely recognized therapeutic option in pediatric patients, even though the literature on the various forms of pediatric apheresis is mostly limited to single-center, retrospective studies [1-6]. [...]

Background The optimal therapeutic regimen for managing childhood idiopathic nephrotic syndrome (INS) is still under debate. We have evaluated the choice of steroid regimen and of symptomatic treatment adopted by pediatricians and pediatric nephrologists in a large number of centers as the first step towards establishing a shared protocol Methods This was a multicenter, retrospective study. A total of 231 children (132 admitted to pediatric units) aged 6 months to Results Median steroid dosing was 55 (range 27-75) mg/[m.sup.2]/ day. The overall median cumulative dose regimen for the first episode was 3,440 (1,904-6,035) mg/[m.sup.2], and the median duration of the therapeutic regimen was 21 (9-48) weeks. The total duration and cumulative steroid dose were significantly higher in patients treated by pediatricians than in those treated by pediatric nephrologists (p=0.001 and p=0.008). Among the patient cohort, 55, 64 and 22 % received albumin infusions, diuretics and acetyl salicylic acid treatment, respectively, but the laboratory and clinical data did not differ between children treated or not treated with symptomatic drugs. Albumin and diuretic use did not vary between patients in pediatric units and those in pediatric nephrology units. Conclusions This study shows major differences in steroid and symptomatic treatment of nephrotic syndrome by pediatricians and pediatric nephrologists. As these differences can influence the efficacy of the treatments and the appearance of side-effects, shared guidelines and their implementation through widespread educational activities are necessary. Keywords Idiopathic nephrotic syndrome * Steroid regimen * Albumin infusion * Thromboembolic prophylaxis Guidelines, Introduction Idiopathic nephrotic syndrome (INS) is a rare disease (2-7 cases/year per 100,000 children aged In the early phase of the disease, symptomatic treatment is also important as many severe [...]

Background There is increasing focus on the problems involved in the transition and transfer of young adult patients from paediatric to adult renal units. This situation was addressed by the 2011 International Pediatric Nephrology Association/International Society of Nephrology (IPNA/ISN) Consensus Statement on transition. Methods We performed a survey of transition practices of 15 paediatric nephrology units across Europe 2 years after publication of the consensus statement. Results Two thirds of units were aware of the guidelines, and one third had integrated them into their transition practice. Forty-seven per cent of units transfer five or fewer patients with chronic kidney disease (CKD) stage 5 per year to a median of five adult centres, with higher numbers of CKD stages 2-4 patients. Seventy-three per cent of units were required by the hospital or government to transfer patients by a certain age. Eighty per cent of units commenced transition planning after the patient turned 15 years of age and usually within 1-2 years of the compulsory transfer age. Forty-seven per cent of units used a transition or transfer clinic. Prominent barriers to effective transition were patient and parent attachment to the paediatric unit and difficulty in allowing the young person to perform self-care. Conclusions Whereas awareness of the consensus statement is suboptimal, it has had some impact on practice. Adult nephrologists receive transferred patients infrequently, and the process of transition is introduced too late by paediatricians. Government- and hospital-driven age-based transfer policies distract focus from the achievement of competencies in self care. Variable use of transition clinics, written patient information and support groups is probably due to economic and human-resource limitations. The consensus statement provides a standard for evolving and evaluating transitionpolicies jointly agreed upon by paediatric and adult units. Keywords Adolescence * Chronic kidney disease * Guidelines * Survey * Transition * Young adult, Introduction Increasing numbers of children with chronic illness, including chronic kidney disease (CKD), are surviving into young adulthood and requiring transition to adult services [1]. Numbers transferring within localities may [...]

Protein-energy wasting (PEW) is defined as a state of decreased body protein mass and fuel reserves (body protein and fat mass) and is a common complication of chronic kidney disease (CKD). It is multifactorial: the main causative factors are hormonal imbalances and a low nutrient intake, but low residual renal function, inadequate dialysis dose, chronic inflammation and metabolic acidosis are other important contributory factors. Adult PEW has been defined, but there is no accepted definition of pediatric PEW and consequently no precise diagnostic criteria. Assessing nutritional status in children is also complicated by the absence of a gold standard, specific abnormalities in body composition, and the slowly progressive course of the disease. The evaluation of PEW should take into account all of its pathogenetic aspects, which include dietary assessment, clinical and anthropometric assessment (based on weight, height, and body mass index), a panel of biochemical parameters, and a normalized protein catabolic rate (in the case of adolescents on hemodialysis). Bioimpedance indices can be used in individual patients on a regular basis in centers with expertise. The longitudinal follow-up data relating to the above parameters are valuable for comparing patient and normative data. Given the complex nature of PEW, only a multidisciplinary approach can provide an accurate assessment of nutritional status and its derangements in children with CKD and on dialysis. Keywords Protein-energy wasting * Chronic kidney disease * Nutritional status * Malnutrition * Nutritional assessment, Introduction According to an expert panel of the International Society of Renal Nutrition and Metabolism (ISRNM), protein-energy wasting (PEW) in adults is defined as a state of decreased body protein [...]

Background Many factors may impact upon choice of renal replacement therapy (RRT) for children and adolescents, including patient and family choice, patient size and distance from the renal centre as well as logistic issues such as facilities and staffing at the unit. We report a survey of factors influencing treatment choice in 14 European paediatric nephrology units. Methods A questionnaire was developed by consensus and completed by 14 members of the European Paediatric Dialysis Working Group on facilities, staffing and family assessments impacting on choice of therapy as well as choice of therapy for 97 patients commencing initial RRT in 2011. Results All units offered all modalities of RRT, but there were limitations for pre-emptive transplantation (PET) and largely adult surgical dependence for creation of arteriovenous fistulae and transplantation. The average waiting time for a deceased donor kidney was 18.5 (range 3-36) months. Full time dietetic support was available in six of the 14 units. There was no social worker, psychology, play therapy or teaching support in three, two, seven and four units, respectively. Assessment by other members of the multidisciplinary team and home visits before choice of therapy was carried out in 50 % of units, and although all patients were discussed at team meetings, the medical opinion predominated. In terms of types of RRT, 50 % of patients were commenced on chronic peritoneal dialysis (PD), 34 % on haemodialysis (HD) and 16 % underwent pre-emptive transplantation (PET). Chronic PD predominated in patients aged 10 years. Patient and family choice and age or size of patient were predominant factors in choice of therapy with a predictable decline in renal function favouring PET and social factors HD. Conclusions Chronic peritoneal dialysis predominated as primary choice of RRT, especially in younger children. The PET rates remain low. The influence of surgeons predominanted, and national transplant rules may be significant. Most units had insufficient multiprofessional support, which may impact upon initial choice of therapy as well as sustaining families through RRT. Keywords Choice of therapy * Renal replacement therapy * Dialysis * Pre-emptive transplantation * Psychosocial * Pediatric, Introduction The choice of chronic renal replacement therapy (RRT) for each individual child and family can be a complex issue. It involves consideration of the facilities and expertise available at [...]

Background Alport syndrome (AS) is a progressive hereditary glomerular disease. Recent data indicate that aldosterone promotes fibrosis mediated by the transforming growth factor-β1(TGF-β1) pathway, which may worsen proteinuria. Spironolactone (SP) antagonizes aldosterone and this study aimed to evaluate the efficacy of SP in reducing proteinuria and urinary TGF-β1 excretion in proteinuric AS patients. Methods The study involved ten children with AS, normal renal function, and persistent proteinuria (>6 months; uPr/uCr ratio >1). SP 25 mg once a day for 6 months was added to existing ACE inhibitor treatment with or without angiotensinII receptor blockade. Urine and blood samples were examined monthly. Urinary TGF-β1 levels were measured twice before and three times during SP treatment. Plasma renin activity (PRA) and serum aldosterone levels were also measured. In eight patients, uProt/uCreat was also assessed after 9 months and 12 months of SP treatment. Results After beginning SP therapy, all patients showed significant decrease in mean uProt/uCreat ratio (1.77 ± 0.8 to 0.86 [+ or -] 0.6; p Keywords Alport syndrome * Renin-angiotensin system * Aldosterone Proteinuria Spironolactone * Urinary TGF-β1, Introduction Alport syndrome (AS) is a progressive hereditary glomerulonephritis caused by mutations in the genes encoding the α3, α4 or α5 chains of type IV collagen [1,2]. It is characterized [...]

Background To provide recommendations for the care of infants with stage 5 chronic kidney disease (CKD5). Setting European Paediatric Dialysis Working Group. Data Sources Literature on clinical studies involving infants with CKD5 (end stage renal failure) and consensus discussions within the group. Recommendations There has been an important change in attitudes towards offering RRT (renal replacement therapy) to both newborns and infants as data have accumulated on their improved survival and long-term outcomes. The management of this challenging group of patients differs in a number of ways from that of older children. The authors have summarised the basic recommendations for treating infants with CKD5 in order to support the multidisciplinary teams who endeavour on this difficult task. Keywords Infants * Neonates * Chronic kidney disease (CKD) * Haemodialysis (HD) * Peritoneal dialysis (PD), Introduction The European Paediatric Dialysis Working Group (EPDWG) was established in 1999 and comprises of paediatric nephrologists with a major interest in dialysis from 13 European countries. Recommendations produced by [...]