Pediatric End-stage Renal Disease Research Articles

Family Environment, Social Support, and Health-Related Quality of Life in Adolescents Following Kidney Transplant.

Kidney transplantation is the treatment of choice for pediatric end-stage renal disease. Transplant recipients often have better neurocognitive, academic, and health-related outcomes. While there is some evidence that health-related quality of life (HRQOL) improves following kidney transplant, other studies have found adolescent transplant recipients report lower overall HRQOL than healthy peers. Current research has focused on individual-level factors affecting adjustment to organ transplant, warranting examination of HRQOL from a systems perspective. Family environment and social support contribute to a system of proximal relationships that are crucial in adolescents' development, making them important factors to study in relation to HRQOL post-transplant. The current study utilized archival data of adolescent renal transplant patients who completed surveys about their family environment, social support, and HRQOL during routine transplant clinic visits. Family cohesion, as well as social support from parents, classmates, and people in school, were positively correlated with HRQOL. Social support from people in school uniquely predicted variance in HRQOL, beyond the contribution of overall family environment after controlling for demographic factors. Relative to comparable adolescent samples, transplant recipients reported more optimal overall family environment, greater social support from teachers, and lower social support from close friends. Psychosocial interventions for adolescent kidney transplant recipients may be enhanced by collaborating with school professionals and further bolstering strengths in the family environment. Kidney transplant recipients may benefit from long-term intervention, as decreased HRQOL appears to persist years post-transplant.

#1315 Management and long-term outcome of recurrent idiopathic FSGS in pediatric renal transplant recipients

Abstract Background and Aims Focal segmental glomerulosclerosis (FSGS) is a leading cause of pediatric end-stage renal disease (ESRD). While approximately 30% of pediatric FSGS is attributed to monogenic disorders, most cases are idiopathic, carrying a high risk of post-transplantation recurrence and graft loss. Commonly used protocols for recurrent FSGS (rFSGS) include plasmapheresis or immunoadsorption, and/or immunomodulatory therapy. We retrospectively evaluated the efficacy and safety of early plasmapheresis followed by rituximab therapy for induction of remission in rFSGS. Method Between 2014-2023, 8/65 pediatric renal transplant recipients at our center were diagnosed with idiopathic FSGS. Five patients underwent preventive plasmapheresis initiated between day 7 pre-transplantation to day 1 post-transplantation, depending on donor source (living/deceased). rFSGS, was diagnosed based on nephrotic range proteinuria of no other cause, and managed with daily plasmapheresis, followed by frequency tapering. Failure to achieve complete remission while on prolonged plasmapheresis was managed with Rituximab therapy. Results 6/8 patients (75%) experienced rFSGS. All achieved partial or complete remission with plasmapheresis therapy. Nevertheless, 5/6 patients exhibited plasmapheresis-dependence, thereby necessitating rituximab therapy. Of these, 3/5 patients achieved sustained remission enabling plasmapheresis cessation, while in 2/5 showed markedly improved proteinuria, enabling reduction of plasmapheresis frequency to once weekly. Adverse effects included rituximab-induced serum sickness in one patient, requiring therapy discontinuation. While one patient suffered from graft loss due to humoral rejection, no graft was lost to rFSGS, and all other grafts remained functional over an average follow-up period of 50 months. Conclusion We suggest that early introduction of plasmapheresis and rituximab therapy effectively induces remission in most post-transplantation rFSGS, is well tolerated, and prevents graft loss. Additional, large scale studies are needed to establish our observation.

Health-related quality of life for pediatric patients with end-stage kidney disease: A systematic review and meta-analysis of the Pediatric Quality of Life Inventory (PedsQL).

Health-related quality of life (HRQoL) studies demonstrate the impact of end-stage renal disease (ESRD) on the physical and psychosocial development of children. While several instruments are used to measure HRQoL, few have standardized domains specific to pediatric ESRD. This review examines current evidence on self and proxy-reported HRQoL among pediatric patients with ESRD, based on the Pediatric Quality of Life Inventory (PedsQL) questionnaires. Following PRISMA guidelines, we conducted a systematic review and meta-analysis on HRQoL using the PedsQL 4.0 Generic Core Scale (GCS) and the PedsQL 3.0 ESRD Module among 5- to 18-year-old patients. We queried PubMed, Embase, Web of Science, CINAHL, and Cochrane databases. Retrospective, case-controlled, and cross-sectional studies using PedsQL were included. Of 435 identified studies, 14 met inclusion criteria administered in several countries. Meta-analysis demonstrated a significantly higher total HRQoL for healthy patients over those with ESRD (SMD:1.44 [95% CI: 0.78-2.09]) across all dimensional scores. In addition, kidney transplant patients reported a significantly higher HRQoL than those on dialysis (PedsQL GCS, SMD: 0.33 [95% CI: 0.14-0.53]) and (PedsQL ESRD, SMD: 0.65 [95% CI: 0.39-0.90]) concordant with parent-proxy reports. Patients with ESRD reported lower HRQoL in physical and psychosocial domains compared with healthy controls, while transplant and peritoneal dialysis patients reported better HRQoL than those on hemodialysis. This analysis demonstrates the need to identify dimensions of impaired functioning and produce congruent clinical interventions. Further research on the impact of individual comorbidities in HRQoL is necessary for developing comprehensive, integrated, and holistic treatment programs.

Predictive Factors of Renal Graft Failure in Tunisian Children and young adults: A Retrospective Study.

Pediatric end-stage renal disease is a rare but severe condition that causes numerous complications and impairs the quality of life of children. Kidney transplantation is the therapy of choice in pediatric end-stage renal disease. Our study aimed to identify the predictive factors of renal graft failure after kidney transplantation in Tunisian children and young adults. We conducted a retrospective bicentric study of children and young adults (age≤20 years) who had undergone renal transplantation between 1989 and 2019 in Tunisia. We analyzed long-term survival rates and complications after pediatric kidney transplantation and searched for predictive parameters for graft dysfunction. We used a univariate and a multivariate analysis to identify predictive factors of graft survival. A total of 112 patients underwent 115 kidney transplantations. Graft failure occurred in 30% of the cases. The overall 1-, 3-, 5- and 10-year graft survival rates were 92%, 89.1%, 85.9% and 74.5% respectively. The following parameters strongly influenced graft survival: immunosuppressive regimen including an association other than Mycophenolate mofetil- tacrolimus and corticosteroids (p=0.002), year of transplant (p<0.0001 for 1987-2000), deceased donor (p = 0.039), underlying etiology of end-stage renal disease (p=0.045), occurrence of acute or chronic rejection (p<0.001), a urine protein greater than 0.3 g/l per day (p=0.002), post-transplant urologic complications (p=0.002), five-year creatinine level>1.28 mg/dl (p<0.001). The overall 1-, 3-, 5- and 10-year patients survival rates were 97%, 95%, 90.2% and 84.4% respectively. Our study identified several predictive factors of graft failure in Tunisian children and young adults undergoing renal transplantation.

Congenital Anomalies of the Kidneys and Urinary Tract.

Congenital anomalies of the kidneys and urinary tract encompass the spectrum of disorders that include the kidneys, ureters, bladder, and urethra. These abnormalities often lead to altered renal size and location, dysplastic changes in the kidney parenchyma, and anomalies in the collecting system. Though the etiology of each of these conditions can be variable, it is known that the collection of these defects represent 40% to 50% of all pediatric end-stage renal disease worldwide. The multifaceted management of these conditions is aimed at preserving kidney function and ultimately delaying the need for transplantation. With the advancement of prenatal ultrasonographic techniques, these conditions are more likely to be diagnosed before birth, which often leads to rapid postnatal intervention and better outcomes.

Transition of care: lessons from the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) dialysis collaborative.

Acknowledging the importance of preparing the pediatric dialysis patient for successful transfer to adult providers, centers from the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Dialysis Collaborative developed transition tools and performed iterative implementation of a transition of care (TOC) program to gain real-life insight into drivers and barriers towards implementation of a transition program for patients receiving dialysis. A TOC innovation workgroup was developed in 2019 from within SCOPE Collaborative that developed nine educational modules, along with introductory letter and assessment tool to be utilized by SCOPE centers. A 4-month pilot implementation study among six centers of varying patient population (age ≥ 11years) was performed. TOC tools were further refined, and broader implementation within the collaborative was performed. Interim assessment of TOC tool utilization and implementation success was performed among 11 centers, as a foundation towards broader discussion regarding process, barriers, and success towards TOC implementation among 26 centers. Transition champion was a key driver of successful implementation, and lack of institutional support and collaboration with adult dialysis centers were important barriers towards sustainability. COVID pandemic and increased staff turnover affected longer term implementation of TOC program. Successful transition and transfer of adolescents/young adults with kidney failure on dialysis remains a challenge. This study represents the experience of the largest cohort of pediatric dialysis centers, with diversity in population size and geography, towards development and implementation of a TOC program. This adds to the resources available to assist centers towards transition and transfer, with particular focus on transitioning patients on dialysis.

INCIDENCE, RISK FACTORS, AND OUTCOME OF ACUTE KIDNEY INJURY AMONG CHILDREN IN PEDIATRIC INTENSIVE CARE UNIT IN A TERTIARY CARE HOSPITAL

Objective: The study aimed to study the incidence, risk factors, outcome, and disease pattern of acute kidney injury (AKI) among children admitted in the pediatric intensive care unit (PICU). Methods: The present study was a prospective study done to study the incidence, risk factors, and outcome of AKI among children of the age group 1 month–12 years admitted in PICU at a tertiary care hospital. Results: A total of 480 PICU admissions were recruited, of which 276 children met the inclusion criteria. Of these, AKI was diagnosed in 119 children (50.4%) using pediatric risk, injury, failure, loss, and end-stage renal disease criteria, and the remaining 117 children were classified as non-AKI. The majority of children (63%) were in the age group of 5–12 years, and the male-to-female ratio was 1.2:1. From this study, it was recorded that the pathogenesis of AKI could be attributed to acute glomerulonephritis (AGN) (85%), sepsis (74%), and others like snake bite, diarrhea, poisoning cases, scorpion sting, diabetic ketoacidosis, heart failure, and unclassified causes with observable numbers. The most common offender is shock (91.4%) with vasopressor support (85.4%). Out of 82 children on inotropic support, 46 were on adrenaline. It was observed that the majority of children (93.5%) developed AKI during the course in hospital. The mortality rates were 64.7% and 24.5% among AKI and non-AKI patients. The mean duration of hospital stays among children without AKI was 5 days. Conclusion: The presence of AKI is associated with longer PICU and hospital stay, with higher mortality imposing a significant burden to health-care system.

Association of Hypoalbuminemia with Risk of Death in Pediatric Patients with End Stage Renal Disease undergoing Hemodialysis: A Tertiary Care Experience in Pakistan

Background: One of the most important and common feature of chronic kidney disease is Hypoalbuminemia and is considered a poor prognostic factor. Low levels of Serum albumin are a predictor of growth failure and are strongly associated with increase death rate in pediatric patients undergoing hemodialysis with End Stage Renal Disease (ESRD), especially a challenge in underdeveloped countries. Aim: To evaluate the association of serum albumin level with hospitalization and rate of death in ESRD hemodialyzed pediatric patients. Study Design: Cohort study Place and Duration of Study: Department of Pediatric Nephrology, University of Child Health Sciences, Lahore from 1st January 2018 to 31st January 2022 Methodology: Ninety two children on hemodialysis and aged 5-16 years were included. Data including patient demographics, anthropometry, date of dialysis initiation, diagnosis, systolic and diastolic blood pressure, dialysis access (HD line/AV fistula), laboratory parameters, compliance to dialysis prescription, and frequency of hospitalization and combined duration of hospital stay was recorded. Results: The mean age at dialysis was 10.6±2.7 years with male to female ratio of 1.3:1. The mean duration on dialysis was 2.03±0.9 years. In our study, CAKUT (42.4%) was the most commonly occurring underlying etiology of end stage renal disease, followed by cystic renal disease (27.2%) and glomerular disease (15.2%) respectively. Regarding the systolic hypertension 22.9% were hypertensive and 35.8% were pre-hypertensive (BP 90th to 95th percentile). For diastolic hypertension, 18.5% were hypertensive and 42.4%were noted as pre-hypertensive. Majority of patients (87%) had hypoalbuminemia (serum albumin &lt; 3.5 gm/dl) at dialysis initiation. In our study the value of serum albumin has inverse relationship with \risk of mortality in ESRD hemodialyzed patients even after adjustment to other factors. There is a 70% higher risk of death with each 1gm/dl decline in serum albumin value (hazard ratio=0.300(0.122-0.740) P&lt;0.009). In addition, AVF access group has a significantly lower risk of death compared to the catheter group (hazard ratio=0.444 (0.246-0.801), P = 0.007). On generation of ROC curves, AUC obtained for serum albumin was statistically significant (AUC=0.838, 95% CI 0.758–0.918). In our study, no correlation was found between hypoalbuminemia and annual frequency of hospitalization (P&lt;0.812) and total duration of stay (P&lt;0.997).For mortality prediction, our study demonstrated a cut off value of albumin of 3g/dl with sensitivity of 80% and specificity of 68.7%. Conclusion: The independent prognostic value of serum albumin in mortality prediction at a cut off value of 3g/dl. In the final model even after adjustment of other covariates, hypoalbuminemia at dialysis initiation and type of vascular access continued to be vital indicator of risk of death in pediatric ESRD patients. However, no statistically significant association was observed between hypoalbuminemia and hospitalization in dialysis patients. Keywords: ESRD, Hypoalbuminemia, Growth failure, Haemodialysis

MP53-03 UROLOGIC RISK FACTORS FOR URINARY TRACT INFECTION AND ALLOGRAFT FAILURE IN PEDIATRIC RENAL TRANSPLANTATION

You have accessJournal of UrologyCME1 Apr 2023MP53-03 UROLOGIC RISK FACTORS FOR URINARY TRACT INFECTION AND ALLOGRAFT FAILURE IN PEDIATRIC RENAL TRANSPLANTATION Vinicius Seidel, Matthew Buell, Kai Wen Cheng, Cayde Ritchie, Adam Fisher, David Chamberlin, and Joshua Chamberlin Vinicius SeidelVinicius Seidel More articles by this author , Matthew BuellMatthew Buell More articles by this author , Kai Wen ChengKai Wen Cheng More articles by this author , Cayde RitchieCayde Ritchie More articles by this author , Adam FisherAdam Fisher More articles by this author , David ChamberlinDavid Chamberlin More articles by this author , and Joshua ChamberlinJoshua Chamberlin More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003301.03AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Urinary tract infection (UTI) causes morbidity in children with end stage renal disease (ESRD) and may lead to allograft failure. Appropriate urologic evaluation is important before and after renal transplantation. The purpose of this study is to identify risk factors for UTI and allograft failure in children with ESRD. METHODS: A retrospective review of all pediatric renal transplant patients at a single transplant center from 2013 to 2022 was performed. Inclusion criteria were patients less than 18 years of age with ESRD who underwent their first renal transplantation. Patients with less than 6 months follow-up were excluded. The primary endpoints were UTI occurrence and allograft failure. Allograft failure was defined as rejection with return to dialysis. Statistical comparisons were made using the Chi Square test and the Wilcoxon signed rank test. RESULTS: 79 children underwent renal transplantation with a median follow-up time of 56 months (6-112 months). Etiologies for renal failure were identified as renal in 60 (76%) patients and bladder in 19 (24%) patients. Renal failure in patients with congenital anomaly of kidney and urinary tract (CAKUT) was seen in 33% of renal etiologies and 100% of bladder etiologies. Overall, the UTI rate was lower pre-transplant (8%) compared to post-transplant (28%) (p=0.002). Pre-transplant bladder etiology for renal failure had a higher UTI rate when compared to the renal etiology (26% vs 2%, p=0.002). Post-transplant there was no difference in UTI rate between bladder etiology and renal etiology (37% vs 25%, p=0.32). 27% of patients had pre-transplant native kidney vesicoureteral reflux (VUR) and 11% had transplant kidney VUR. Children with native kidney VUR had a higher UTI rate pre-transplant (48% vs. 21%, p<0.05). Children with transplant kidney VUR had a higher UTI rate than those without transplant VUR (67% vs. 23%, p<0.05). Patients with poor bladder compliance and capacity on urodynamics when medically and surgically optimized, did not have increased post-transplant UTI rates. 14 (17.7%) patients developed allograft rejection. Etiology and onset time of initial renal failure, number of UTI, CAKUT, and VUR were not associated with allograft rejection. CONCLUSIONS: Pediatric ESRD patients with pre-transplant native kidney VUR and renal transplant VUR are at increased risk for UTI and should be treated. Unsafe bladders when optimized may safely undergo transplantation. Renal allograft failure was not associated with etiology, onset time of initial renal failure, number of UTI, CAKUT, and VUR. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e712 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Vinicius Seidel More articles by this author Matthew Buell More articles by this author Kai Wen Cheng More articles by this author Cayde Ritchie More articles by this author Adam Fisher More articles by this author David Chamberlin More articles by this author Joshua Chamberlin More articles by this author Expand All Advertisement PDF downloadLoading ...

MO1017PSYCHOSOCIAL REPERCUSSIONS AND QUALITY OF LIFE OF CHILDREN UNDERGOING HEMODIALYSIS IN A DEVELOPING COUNTRY

Abstract Background and Aims Despite major advances in the provision of renal replacement therapy, children and adolescents on dialysis frequently have compromised daily life activities and a worse quality of life compared with healthy peers. While these aspects have been studied extensively in affluent countries, data from developing regions are scant. The aim of our study was assessing the psychosocial repercussions and the quality of life (QoL) of children with end stage renal disease (ESRD) undergoing hemodialysis in a Moroccan tertiary care hospital and comparing them with healthy controls. Methods We performed a cross-sectional investigation, at Mohammed VI university hospital of Marrakesh, using parent/child reports of generic module for QoL assessment: PedsQLTM Inventory version 4 for both cases and controls. Disease-specific module: PedsQLTM ESRD version 3 was used for ESRD cases. Twenty-two children on maintenance hemodialysis and 50 controls were enrolled in our study. Results The mean age of the ESRD cases was 11.6 ± 4.7 years (range 4– 18 years) with female predominance (58%). Controls were age- and sex-matched. A statistically significant difference was observed between ESRD cases and controls regarding all aspects of QoL. The total QOL mean score was 51.2 ± 13.6 and 82.8 ± 11.2 among cases and controls, respectively. All individual QoL domains were significantly worse in ESRD cases. Conclusion Our findings showed that the quality of life of children undergoing hemodialysis may be dramatically affected especially in limited-resources countries. And this raises the need to introduce quality of life assessment as a part of pediatric ESRD management.

Continued reduction in peritonitis rates in pediatric dialysis centers: results of the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative.

In its first 3 years, the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative demonstrated a statistically significant increase in the likelihood of compliance with a standardized follow-up care bundle and a significant reduction in peritonitis. We sought to determine if compliance with care bundles and low peritonitis rates could be sustained in centers continuously participating for 84 months. Centers that participated from collaborative launch through the 84-month study period and provided pre-launch peritonitis rates were included. Children on maintenance peritoneal dialysis were eligible for enrollment. Changes in bundle compliance were assessed using a logistic regression model or a generalized linear mixed model (GLMM). Changes in average annualized peritonitis rates over time were modeled using GLMMs. Nineteen centers contributed 1055 patients with 1268 catheters and 17,247 follow-up encounters. The likelihood of follow-up compliance increased significantly over the study period (OR 1.05 95% confidence interval (CI) 1.03, 1.07; p < 0.001). Centers achieved ≥ 80% follow-up bundle compliance by 28 months and maintained a mean compliance of 84% between 28 and 84 months post-launch. Average monthly peritonitis rates decreased from 0.53 (95% CI 0.37, 0.70) infections per patient-year pre-launch to 0.30 (95% CI 0.23, 0.43) at 84 months post-launch, p < 0.001. Centers participating in the SCOPE Collaborative for 84 months achieved and maintained a high level of compliance with a standardized follow-up care bundle and demonstrated a significant and continued reduction in average monthly peritonitis rates.

Variability in Culture-Negative Peritonitis Rates in Pediatric Peritoneal Dialysis Programs in the United States.

International guidelines suggest a target culture-negative peritonitis rate of <15% among patients receiving long-term peritoneal dialysis. Through a pediatric multicenter dialysis collaborative, we identified variable rates of culture-negative peritonitis among participating centers. We sought to evaluate whether specific practices are associated with the variability in culture-negative rates between low- and high-culture-negative rate centers. Thirty-two pediatric dialysis centers within the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) collaborative contributed prospective peritonitis data between October 1, 2011 and March 30, 2017. Clinical practice and patient characteristics were compared between centers with a ≤20% rate of culture-negative peritonitis (low-rate centers) and centers with a rate >20% (high-rate centers). In addition, centers completed a survey focused on center-specific peritoneal dialysis effluent culture techniques. During the 5.5 years of observation, 1113 patients had 1301 catheters placed, totaling 19,025 patient months. There were 620 episodes of peritonitis in 378 patients with 411 catheters; cultures were negative in 165 (27%) peritonitis episodes from 125 (33%) patients and 128 (31%) catheters. Low-rate centers more frequently placed catheters with a downward-facing exit site and two cuffs (P<0.001), whereas high-rate centers had more patients perform dialysis themselves without the assistance of an adult care provider (P<0.001). The survey demonstrated that peritoneal dialysis effluent culture techniques were highly variable across centers. No consistent practice or technique helped to differentiate low- and high-rate centers. Culture-negative peritonitis is a frequent complication of maintenance peritoneal dialysis in children. Despite published recommendations for dialysis effluent collection and culture methods, great variability in culture techniques and procedures exists among individual dialysis programs and respective laboratory processes.

Long-term psychosocial outcomes of adults transplanted in childhood: A social work perspective.

While much research exists regarding medical outcomes for pediatric end stage renal disease (ESRD) populations and for adults receiving dialysis or kidney transplant, the understanding of long-term psychosocial outcomes for individuals diagnosed with ESRD or transplanted in childhood remains limited. A literature review was undertaken by two social work researchers to identify relationship, education, employment, and quality of life (QOL) outcomes for this population utilizing PUBMED and PsycInfo databases. Inconsistencies in the MeSH terms and keywords used across the relevant articles were noted. The impact of transplantation in childhood is significant across multiple facets of social development including but not limited to peer/intimate relationships, education and employment and QOL, and overall well-being. When compared to healthy peers, those with ESRD were delayed in all areas of social development. While improvements in psychosocial outcomes have occurred alongside improved graft and patient survival, more focused longitudinal and qualitative inquiry is needed. Partnerships are needed across disciplines and across those caring for different age cohorts in pediatric, adult nephrology, and transplant practices.

A Study of Acute Kidney Injury in a Tertiary Care Pediatric Intensive Care Unit.

The objective of this study was to calculate the incidence, severity, and risk factors for acute kidney injury (AKI) in a tertiary care pediatric intensive care unit (PICU). Also, to assess the impact of AKI and its varying severity on mortality and length of hospital and PICU stays. A prospective observational study was performed in children between 1 month and 12 years of age admitted to the PICU between July 1, 2013, and July 31, 2014 (13 months). The change in creatinine clearance was considered to diagnose and stage AKI according to pediatric risk, injury, failure, loss, and end-stage renal disease criteria. The risk factors for AKI and its impact on PICU stay, hospital stay, and mortality were evaluated. Of the total 220 patients enrolled in the study, 161 (73.2%) developed AKI, and 59 cases without AKI served as the "no AKI" (control) group. Majority (57.1%) of children with AKI had Failure grade of AKI, whereas 26.1% had Risk grade and 16.8% had Injury grade of AKI. Infancy ( p = 0.000), hypovolemia ( p = 0.005), shock ( p = 0.008), and sepsis ( p = 0.022) were found to be significant risk factors for AKI. Mortality, PICU stay, and hospital stay were comparable in children with and without AKI as well as between the various grades of renal injury (i.e., Failure, Risk, and Injury ). An exceedingly high incidence of AKI, especially of the severe Failure grade was observed in critically ill children. Infancy and frequent PICU occurrences such as sepsis, hypovolemia, and shock predisposed to AKI.

Protein Energy Wasting in a Sample of Egyptian Children on Regular Hemodialysis: Relation to Anorexigenic Hormones

BACKGROUND: Increased incidence of pediatric end-stage renal disease (ESRD) with associated serious consequences indicating a major public health problem. Malnutrition and uremic wasting are leading causes of growth impairment and increasing morbidity and mortality of pediatric ESRD patients, predominantly those on regular hemodialysis (HD). Ghrelin and obestatin, which are known appetite regulatory hormones, might have a pivotal role in uremic wasting and growth impairment in hemodialyzed children.&#x0D; AIM: The aim of the present study was to measure serum unacylated ghrelin (UAG) and obestatin and to investigate their roles in the growth impairment of Egyptian hemodialyzed children.&#x0D; SUBJECTS AND METHODS: The study included 50 hemodialyzed and 40 healthy children recruited from the Department of Nephrology, Pediatric Hospital, Ain Shams University. Full clinical examination and measurement of anthropometric indices were done. Routine labs were done as well, with an assessment of serum levels of obestatin, UAG, and insulin by enzyme linked immunosorbent assay. Furthermore, we determined fasting serum glucose and lipid profile with the calculation of homeostasis model assessment for insulin resistance (HOMA-IR).&#x0D; RESULTS: Anthropometric measurements were statistically significantly decreased in the hemodialyzed group than that of the control group (p &lt; 0.05). Weight z-score was the most affected anthropometric parameter (37 patients = 74% with underweight and 13 patients = 26% with normal weight). The hemodialyzed children showed a significant increase of UAG, obestatin, insulin, glucose, HOMA-IR, and TG, while a significant decrease of HDL-cholesterol and albumin (p &lt; 0.01). UAG had a negative correlation with Wt-z score, Ht z-score, fat mass %, albumin, and TG while obestatin was inversely correlated to Wt-z score, BMI z-score, waist circumference, and waist-height ratio (W/H).&#x0D; CONCLUSION: UAG and obestatin hormones were elevated in a group of Egyptian children on regular HD. These hormones were strongly related to the impairment of renal functions, and anthropometric parameters, dyslipidemia, hypoalbuminemia, and insulin resistance in these pediatric hemodialyzed patients.

Paediatric end-stage renal disease and renal replacement therapy in Switzerland: survival and treatment trends over four decades.

Renal replacement therapy for paediatric end-stage renal disease (ESRD) has developed steadily since its introduction five decades ago. Continuous and long-term analysis of patient outcomes is essential for quality control. The Swiss Paediatric Renal Registry, founded in 1970, includes patients diagnosed with ESRD, defined as dialysis for more than three months or renal transplantation, at age &lt;20 years. Here we describe the incidence, primary renal disease, treatment modalities and long-term outcomes over 45 years. This paper reports on 367 children and adolescents treated with chronic renal replacement therapy in Switzerland. Incidence was 5.4 per million children per year, with a tendency to increase over time. The primary renal disease was congenital anomalies of the kidney and the urinary tract in 133 (36%), monogenetic hereditary diseases in 122 (33%) and acquired diseases in 112 (31%) patients. The first renal replacement therapy was haemodialysis in 194 (53%), peritoneal dialysis in 116 (32%) and pre-emptive renal transplantation in 57 (15%) patients. Over the years, pre-emptive renal transplantation became more frequent (34% of all first renal replacement therapies in 2006&ndash;2015), reducing the duration of dialysis. Median time on dialysis until transplantation decreased from 1.60 years in 1981&ndash;90 to 0.34 years in 2010&ndash;15. Over the four decades 1970&ndash;80, 1981&ndash;90,1991&ndash;2000 and 2001&ndash;10, the one-year graft survival rate improved from 0.76 to 0.80, 0.89 and then 0.96; and the five-year graft survival rate improved from 0.44 to 0.64, 0.84 and 0.89, respectively. The five-year patient survival rates for the four decades were 0.83, 0.99, 0.93 and 0.94; and the 10-year patient survival rates were 0.75, 0.96, 0.88 and 0.94, respectively. In the four cohorts starting renal replacement therapy in the 70s, 80s, 90s and 00s, the number of children alive after five years of renal replacement therapy increased from 15 to 24, 47 and then 45 respectively. In total, 29 patients (8%) died during chronic renal replacement therapy with ESRD before the age of 20 years. Over time, a higher number of children on renal replacement therapy survived, graft survival improved, and the duration of dialysis before renal transplantation decreased.

New-onset ESRD secondary to reflux nephropathy has decreased in incidence in the United States

Vesicoureteral reflux (VUR) has been associated with the development and progression of reflux nephropathy (RN). Management of VUR has become more conservative over time as therapies have not been reliably proven to prevent renal scarring. We sought to examine the incidence of end stage renal disease (ESRD) due to RN over recent decades in the United States. The United States Renal Data System (USRDS) is a national data system that collects information about chronic kidney disease and ESRD. Since 1995, the USRDS has mandated that all dialysis centers enroll and submit data on new-onset ESRD patients. Of the over 2.5 million patients enrolled since 1996, 7314 were enrolled with a diagnosis code for RN as the primary cause of ESRD. We examined the trends seen in this patient population from 1996 to 2014 and used US census data to calculate incidence. The incidence of patients with new-onset ESRD attributed primarily to RN saw a steady decrease over time: from 1.9 per million in 1996 to 0.6 per million in the US population in 2014 (exp(B)=0.941, p<0.001). 58.8% of the patients were female, 87.4% white. The mean age at the time of ESRD onset was 47 years (IQR 30-65) and this did not significantly vary over the period (P>0.05). When comparing pediatric new-onset ESRD to adult-onset, a non-significant trend (p=0.093) was seen with a decrease in incidence of adult ESRD, but a relatively stable incidence of pediatric ESRD (mean age 12, incidence 0.2-0.6 cases per million US children/year). 44.5% of patients received renal transplantation at a mean age of 36 years, 18.9 months (IQR 5-40) after ESRD diagnosis. The mean age of mortality was 62 years old (IQR 50-76), and 5-year survival after RN ESRD diagnosis was 65.8%. As with any public database study, our findings may be limited by disease coding or reporting practices. Medical and surgical management trends practiced since the 1960s may have contributed to a decrease in the incidence of ESRD primarily attributed to RN in the United States between 1996 and 2014. Our findings may also reflect changes in diagnostic practices. ESRD attributed primarily to RN has gradually decreased in incidence in the United States between 1996 and 2014, but the age of ESRD onset has been unchanged.

P1834MONITORING ANTICOAGULATION IN PEDIATRIC PATIENTS ON REGULAR HEMODIALYSIS: CAN A DREAM COME TRUE?

Abstract Background and Aims Anticoagulation in hemodialysis aims at preventing the activation of coagulation cascade during the procedure. Appropriate anticoagulation for hemodialysis (HD) requires a precise balance between under- and over-heparinization to prevent extracorporeal circuit (ECC) clotting and bleeding, respectively. This type of balance is practiced in hemodialysis centers, on daily basis, depending on each center’s experience and tailored to every patient, rather than in accordance to a clear guideline. Our aim was to objectively evaluate the adequacy of heparin therapy used for anticoagulation in pediatric patients on regular hemodialysis. Method A follow up observational study for 12 months including 44 pediatric end stage renal disease patients on regular hemodialysis through an A-V fistula or an A-V graft. We excluded patients with a known bleeding disorder, chronic liver disease, thrombophilia, vasculitis or receiving anticoagulation for any condition. All patients were subjected to thorough history taking including dialysis history, assessment of dialysis adequacy by calculating Kt/V and blood pressure monitoring before, during and after session. Clinical evaluation of anticoagulation was done through visual inspection of extra-corporeal circuit, observing transmembrane pressure and recording the duration of compression over the puncture site at the end of the hemodialysis session. Biological monitoring was done by Bedside Activated clotting time (ACT) test. Results On initial evaluation of patients, 11 cases had either clotting or bleeding clinically (only 4 had abnormal ACT values), being on either hemodialysis (HD) or hemodiafilteration (HDF) and receiving unfractionated heparin (UFH) either as a bolus or through a pump. They were further evaluated after being put; on HDF and a heparin pump and showed neither clotting nor bleeding though 2 cases had prolonged predialysis ACT values. On HDF and heparin bolus, no bleeding or clotting were detected though 3 cases had abnormal predialysis ACT values. On HD and heparin pump no bleeding or clotting were detected, 2 cases had low kt/V, a case had abnormal ACT values (5 minutes, 1 hour and at the end ACT values). Conclusion Hemodialysis mode, rather than method of heparin delivery, had a bigger impact on correcting clinical defects of anticoagulation in our patients. ACT as a bedside test of anticoagulation adequacy during HD sessions did not offer extra benefit over clinical evaluation, which is still considered a more accurate method.

P0060COMBINED NOVEL PBX1 AND RET VARIANTS IN AN ADULT WITH SYNDROMIC RENAL HYPOPLASIA: A CASE REPORT

Abstract Background and Aims Congenital abnormalities of the kidney and urinary tract (CAKUT) represent the main cause of pediatric end-stage renal disease with vast pheno- and genotypic heterogeneity. However, its contribution to adult chronic kidney disease (CKD) is less well characterized. We here report on an adult who underwent genetic testing for previously unrecognized CAKUT. Method Phenotypic characterization of a family with mild syndromic form of kidney hypoplasia. Panel-based genetic analysis for CAKUT associated genes in DNA from peripheral blood cells, oral mucosa, and urinary cells. Results A 20-year-old male presented with bilateral hypoplastic kidneys, first diagnosed with nocturnal enuresis at the age of ten. For CKD stage 2 (eGFR 72ml/min/1.73m), hypertension, and moderate albuminuria (300mg/d) he was treated with Ramipril. Additionally, facial dysmorphism with low-set microtia, slight micrognathia, and slightly impaired intelligence was apparent. Bilateral audiograms did not reveal any hearing impairment. His father showed slightly reduced kidney size without any laboratory anomalies or extrarenal symptoms. Further family history including his mother and three half siblings presented unremarkable. Genetic analysis revealed the following two variants: First, a novel heterozygous truncating variant, absent from patient and population databases, was found in PBX1 c.992C&amp;gt;A p.Ser331* (NM_002585.3). As this variant was detected in 25% of NGS reads, we tested for potential mosaicism by direct sequencing in DNA obtained from oral mucosa and urinary epithelia. While the latter showed no PBX1 variation, mucosa cells revealed the mutated allele in 50% of reads. Segregation analysis of the father showed PBX1 wild-type. The mother was not available for genetic testing. Additionally, we identified a novel heterozygous splice site variant in RET: c.1760-5C&amp;gt;A (NM_020975.4), absent from both patient and population databases. This substitution is predicted to affect splicing at the acceptor site of exon 10. Sanger sequencing demonstrated paternal inheritance. Conclusion Pathogenic PBX1 variants were recently associated with CAKUTHED (Congenital Abnormalities of the Kidney and Urinary Tract with or without Hearing loss, abnormal Ears, or Developmental delay) syndrome (Heidet 2017), characterized by highly variable phenotypes. PBX1 encodes a homeodomain transcription factor taking part in transcriptional regulation during embryonic development. Functional studies suggest disturbed PBX1-dependent transactivation ability and altered nuclear translocation, resulting in abnormal interactions between PBX1 proteins and cofactors (Slavotinek 2017). Prevailing PBX1 expression in embryonic compared to adult kidneys support an important function in development (Le Tanno 2017). An aberrant splicing effect of the identified RET variant is supported by the fact that the father, who carries the same variant but PBX1 wild-type, has slightly reduced kidneys. Associations between abnormal kidney development and alterations of RET were first described in stillborn fetuses with renal agenesis (Skinner 2008), but also in patients with vesicoureteral reflux (Yang 2008). RET encodes for a receptor tyrosine-protein kinase, which while binding ligands such as GDNF (glial cell-line derived neurotrophic factor) is involved in signaling pathways and plays an important part during organ development. Particularly, the RET/GDNF signaling pathway plays a crucial role during development of the kidneys (Costantini 2010). Functional analyses indicate an altered phosphorylation status of RET as possible causative mechanism. To the best of our knowledge, this case is the first to report on combined PBX1 and RET gene variants associated with mild syndromic renal hypoplasia, likely mitigated by genetic mosaicism.

Low-Dose Maintenance Intravenous Iron Therapy Can Prevent Anemia in Children with End-Stage Renal Disease Undergoing Chronic Hemodialysis

Iron deficiency anemia is common in children with end-stage renal disease (ESRD) on long-term hemodialysis receiving erythropoiesis-stimulating agents. One approach to maintain the iron profile and hemoglobin levels is maintenance therapy with regular low doses of intravenous (IV) iron after initial iron repletion therapy; however, evidence for the benefits of this approach is lacking. This study evaluated the effect of IV iron maintenance therapy on anemia in children on regular hemodialysis. This retrospective cohort study included 41 pediatric ESRD patients with normal hemoglobin and iron status who underwent regular hemodialysis at the Pediatric Dialysis Unit of Cipto Mangunkusumo Hospital, Indonesia, between January 2015 and April 2019. Among these, 21 received IV iron maintenance therapy with two doses of 2 mg/kg of IV iron sucrose every 2 weeks (the treatment group) and 20 did not (the comparison group). Changes in hemoglobin and transferrin saturation were assessed after 6 weeks of observation and compared between the two groups. There was a significant reduction in the mean hemoglobin level compared with the baseline level in the comparison group (21 g/L; 95% CI, 9.3–33 g/L; p=0.001) but not in the treatment group (0.7 g/L; 95% CI, −6.6–8 g/L; p=0.84). The risk of anemia was lower in the treatment group (relative risk = 0.42; 95% CI, 0.22–0.79; p=0.003). Although majority of the patients had high baseline ferritin level, this study indicates that in our setting, ferritin may not be a reliable parameter to review the iron status, as it can be affected by chronic inflammation. Hence, the decision to start IV iron maintenance therapy in patients with hyperferritinemia should consider the patient's clinical condition and morbidity. To conclude, the coadministration of IV iron maintenance therapy is beneficial for maintaining hemoglobin levels and preventing anemia in children with ESRD who are undergoing regular hemodialysis, have achieved the target hemoglobin levels, and have normal iron status.