Pediatric Renal Disease Research Articles

Genetic and radiological aspects of pediatric renal cystic disease: A case series

Renal cystic diseases are common conditions whose etiology can be highly heterogeneous. They require a correct approach for adequate diagnosis and management. We aimed to illustrate part of the spectrum of renal cystic diseases through some clinical cases managed in our service. We describe 11 clinical cases including clinical entities such as renal multicystic dysplasia, and autosomal dominant and autosomal recessive polycystic renal disease, among other pathologies. Renal cystic diseases are heterogeneous in their clinical presentation, natural history, radiological findings, and genetic and pathophysiological basis. An integral clinical approach is needed to get a clear etiological diagnosis and offer adequate individualized care and follow-up for patients.

Introducing Exome Sequencing as Part of the Diagnostic Algorithm for Pediatric Nephrology Patients in Bulgaria: A Single-Center Experience

Introduction: In pediatric kidney patients, where clinical presentation is often not fully developed, and renal biopsy is too risky or inconclusive, it may be difficult to establish the underlying pathology. In cases such as these, genetic diagnosis may be used to guide treatment, prognosis, and counseling. Given the large number of genes involved in kidney disease, introducing next-generation sequencing with extended gene panels as part of the diagnostic algorithm presents a viable solution. Methods: A cohort of 87 consecutive independent cases (83 children and 4 terminated pregnancies) with renal disease was recruited. Exome sequencing with MiSeq or NovaSeq 6000 (Illumina) platforms and analysis of extended gene panels were used for genetic testing. Results: Depending on the presenting pathology, the cases were grouped as patients with glomerular disease, ciliopathies, congenital anomalies, renal electrolyte imbalances, and chronic/acute kidney disease. The overall diagnostic yield was approximately 42% (37 out of 87), with most disease-causing mutations found in COL4A3, COL4A4, COL4A5, and PKHD1 genes. A change or clarification of preliminary diagnosis or adjustment of initial treatment plan based on the results of the genetic testing was made for approximately one-third of the children with meaningful genetic findings (11 out of 37). Discussion: Our results prove the value of targeted exome sequencing as a non-invasive, versatile, and reliable diagnostic tool for pediatric renal disease patients. Providing genetic diagnosis will help for a better understanding of disease etiology and will give the basis for optimal clinical management and insightful genetic counseling.

Advances in metabolomics profiling of pediatric kidney diseases: A review.

Pediatric renal diseases encompass a diverse array of pathological conditions, often engendering enduring ramifications. Metabolomics, an emergent branch of omics sciences, endeavors to holistically delineate alterations in metabolite compositions through the amalgamation of sophisticated analytical chemistry techniques and robust statistical methodologies. Recent advancements in metabolomics research within the realm of pediatric nephrology have been substantial, offering promising avenues for the identification of robust biomarkers, the elaboration of novel therapeutic targets, and the intricate elucidation of molecular mechanisms. The present discourse aims to critically review the progress in metabolomics profiling pertinent to pediatric renal disorders over the previous 12 years.

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.

The Role of Genetic Testing in Pediatric Renal Diseases: Diagnostic, Prognostic, and Social Implications.

Pediatric renal diseases vary widely and are linked to high morbidity and mortality; hence, early diagnosis is vital. Presently, genetic testing is being incorporated into the standard of care for children and their families with kidney disease, primarily as a diagnostic tool. In the present review, we aim to collect all potential evidence from relevant studies that reported the role of genetic testing in pediatric renal disease diagnostic, prognostic, and social implications. We have conducted both electronic and manual searches within PubMed, the Cochrane Library, Web of Science, and Scopus to find relevant studies. Studies from the years 2013-2023 were included. Case reports with limited sample sizes and no descriptive statistics, along with review papers and meta-analyses, were excluded from this review. Quality assessment for all included studies was performed. The pooled diagnostic yields were calculated using the common effect and random effect models utilizing the R program (R Foundation for Statistical Computing, Vienna, Austria). The pooled result for the diagnostic yield as per the common effect model is a pooled proportion of 0.42 (42%) 95% confidence interval (CI): [0.39,0.44], while with the random effects model the pooled proportion is 0.43 (43%) 95% CI: [0.31,0.57]. The diagnostic yield for the included studies ranged from 78.10% to 16.8%. The spectrum of kidney diseases included nephrolithiasis/nephrocalcinosis, glomerular diseases, cystic kidney disease, ciliopathies, tubulopathies, chronic kidney disease, and congenital anomalies of the kidneys and urinary tracts (CAKUT), while hematuria and proteinuria were reported by two studies and autosomal recessive and autosomal dominant idiopathic kidney disease was reported by only one study. Genetic testing validates clinical diagnosis and aids in tailoring management strategies; hence, a more precise treatment plan is developed and unnecessary investigations are avoided, which is crucial in the case of children during routine nephrology clinic visits. Genetic counselling is of the utmost importance, so all ethical and social concerns related to genetic testing are addressed in addition to patient satisfaction.

Role of the Gut Microbiota in Children with Kidney Disease.

Disruption of the composition and structure of the gut microbiota, namely dysbiosis, dictates the pathophysiology of kidney diseases. The bidirectional kidney-gut axis is of interest in chronic kidney disease (CKD); the uremic milieu leads to intestinal dysbiosis and gut microbial metabolites and toxins implicated in the loss of kidney function and increased comorbidity burden. Considering that kidney diseases can originate in childhood or even earlier in fetal life, identification of the pathogenetic connection between gut microbiota dysbiosis and the development of pediatric renal diseases deserves more attention. This review concentrates on the pathogenic link between dysbiotic gut microbiota and pediatric renal diseases, covering CKD, kidney transplantation, hemodialysis and peritoneal dialysis, and idiopathic nephrotic syndrome. Gut microbiota-targeted therapies including dietary intervention, probiotics, prebiotics, postbiotics and fecal microbial transplantation are discussed for their potential for the treatment of pediatric renal diseases. A deeper understanding of gut microbiota in pediatric renal diseases will aid in developing innovative gut microbiota-targeted interventions for preventing or attenuating the global burden of kidney diseases.

Outcome of Miniperc Technique in Paediatric Age Group: A Prospective Interventional Study

Introduction: Reducing the Percutaneous Nephrolithotomy (PCNL) tract size in paediatric patients with renal stones reduces the morbidity associated with the procedure. Miniperc (Mini PCNL) is a modification of standard PCNL using small size instruments. Aim: To evaluate the postoperative outcome of Miniperc technique in the treatment of renal stones in paediatric age group. Materials and Methods: This was a prospective interventional study conducted at the Department of Urology, Govt. Mohan Kumaramangalam Medical College & Hospital, Salem, Tamil Nadu, India from January 2020 to January 2022. There were 25 patients of renal stone disease belonging to paediatric age group (<8 years), operated by a single surgeon with the Miniperc technique. For all the cases 14F-16F sheaths, 12 Fr Nephroscope and 8-9.5 Fr semirigid ureteroscope, pneumatic lithotripsy and 30-Watt Holmium laser as energy sources were used. Stone-Free Rate (SFR), operative time, hospital stay, and complication rates were assessed. Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) version 26.0. Results: The mean age of the children was 5.2±3.2 years and the average stone size was 1.9 cm. Mean operative time was 74 minutes. The mean hospital stay was 1.5 days. The overall SFR was 89.7%, (N=22) which increased after secondary procedures to 94.12% (N=23). Intraoperative bleeding was seen in 3 (12%) patients and postoperative fever in 4 (16%) patients. Conclusion: Miniperc is a promising and safe technique for paediatric renal stone disease management.

Feasibility of contrast-enhanced ultrasonography (CEUS) in evaluating renal microvascular perfusion in pediatric patients

BackgroundChanges in renal microvascular perfusion are involved in several kidney diseases. Contrast-enhanced ultrasonography (CEUS) quantitative analysis can enable the estimation of renal microvascular perfusion non-invasively. However, to date, few pediatric patients with renal disease have been subjected to CEUS quantitative analysis. This study aimed to explore the feasibility of CEUS in evaluating renal microvascular perfusion in pediatric patients and paving its way to clinical practice.MethodsSeventeen pediatric patients with chronic kidney disease (CKD) and five children without kidney disease were consecutively examined using CEUS. Quantitative analysis of CEUS images based on time-intensity curve (TIC) fittings was performed using specialized software. Quantitative parameters of wash-in microvascular blood flow, including A, k, B, and TtoPk, were generated from three regions of interest (ROIs) each in the cortex and medulla of each kidney.ResultsCEUS was performed in all children successfully and safely without the use of sedatives. All parameters (A, B, k, and TtoPk) demonstrated no statistical differences among the three sampling ROIs in the renal cortex and medulla. All parameters (A, B, k, and TtoPk) showed no statistical differences between the left and right sides of kidneys both in cortices and medullas. Comparing with patients with CKD stage 3–5, both control group and patients with CKD stage 1–2 had significantly higher values of parameter A in the renal cortex (p = 0.025 and p = 0.031, respectively). In control group and patients stage 1–2, the values of parameters k in the renal cortices were significantly higher than that in the renal medullas, while in patients with CKD stage 3–5, parameter k showed no statistically significant differences between the renal cortex and medulla (p = 0.173).ConclusionCEUS is safe and practicable in pediatric patients with chronic kidney disease. Renal microvascular perfusion estimated by CEUS could be a robust approach in the evaluation of pediatric renal diseases. Parameters A and k derived from CEUS quantitative analysis can provide great potential in non-invasive assessment of renal microvascular perfusion impairment in pediatric CKD.

The clinical relevance of native vitamin D in pediatric kidney disease.

Hypovitaminosis D has been reported to be common in chronic kidney disease (CKD) as well as in proteinuric disorders. We reviewed available evidence to assess clinically relevant effects of low vitamin D status and native vitamin D (NVD) therapy, in pediatric renal diseases. Online medical databases were searched for articles related to vitamin D status, associations of hypovitaminosis D and effects of NVD therapy in kidney disease.Hypovitaminosis D was associated with worse skeletal, cardiovascular, inflammatory, and renal survival outcomes in CKD. Low serum 25 hydroxy-vitamin D (25[OH]D) levels correlated positively with glomerular filtration rate and negatively with serum parathyroid (PTH) levels. However, to date, evidence of benefit of NVD supplementation is restricted mainly to improvements in serum PTH, and biochemical 25[OH]D targets form the basis of clinical practice recommendations for NVD therapy.In nephrotic syndrome (NS) relapse, studies indicate loss of 25[OH]D along with vitamin D binding protein in urine, and serum total 25[OH]D levels are low. Preliminary evidence indicates that free 25[OH]D may be a better guide to the biologically active fraction. NVD therapy in NS does not show consistent results in improving skeletal outcomes and hypercalciuria has been reported when total 25[OH]D levels were considered as indication for therapy.NVD formulations should be regularised, and therapy monitored adequately to avoid adverse effects.

MO036: Detection of copy number variations by single-nucleotide-polymorphism array in patients with end-stage renal disease of unknown etiology

Abstract BACKGROUND AND AIMS Establishing the genetic basis of renal disease is pivotal to improve diagnostic and treatment strategies. Pathogenic copy number variations (CNVs) caused by chromosomal imbalances contribute significantly to the pediatric renal disease burden, especially in children with congenital anomalies of the kidney and urinary tract (CAKUT). The role of CNVs in chronic kidney disease of unknown etiology (uCKD) is scarcely investigated, although this group comprises about 20% of adults with end-stage renal disease (ESRD). We screened for causal CNVs in a preliminary sample of patients with ESRD from unknown etiology. METHOD We recruited 94 patients registered with ESRD ≤age 50 from uCKD or hypertension from the Danish Nephrology Registry. Medical records and previous kidney biopsies were initially reviewed, and patients were excluded if a specific cause of kidney disease other than hypertension was identified (n = 35). We performed genome-wide single-nucleotide-polymorphism (SNP) array in 59 patients to identify copy number gains and losses. All CNVs were systematically evaluated as possible causes of kidney disease and broadly classified into three main categories: ‘Normal’, ‘Pathogenic’ or ‘Variant of uncertain significance.’ RESULTS The SNP array results were normal in 57 patients (96.6%). In the remaining two patients (3.4%), we detected pathogenic CNVs with incomplete penetrance in hot spot loci associated with CAKUT: One male patient with ESRD at age 18 was a heterozygous carrier of a 545 kb microdeletion on chromosome 16p11.2 (29.63 Mb to 30.18 Mb). He had left-side renal agenesis and a hypoplastic right kidney at presentation, but no history of developmental delay or dysmorphic features. The microdeletion was considered likely causal, because it included the CAKUT driver gene TBX6 similar to other heterozygous carriers of 16p11.2 microdeletions with isolated renal agenesis or dysplasia. A second patient with ESRD at age 49 had 3.66 Mb duplication on chromosome 1q21.1 (145.43 Mb to 149.09 Mb) in heterozygous form. The patient had no reported CAKUT or other features associated with CNVs in the 1q21.1 loci, and the duplication was therefore deemed unrelated to his renal disease. CONCLUSION Disease-causing CNVs are not frequent in this limited sample of patients with ESRD from unknown etiology. CNV analysis by SNP-array may be most effective in patients with uCKD combined with CAKUT features.

New Insights from Metabolomics in Pediatric Renal Diseases.

Renal diseases in childhood form a spectrum of different conditions with potential long-term consequences. Given that, a great effort has been made by researchers to identify candidate biomarkers that are able to influence diagnosis and prognosis, in particular by using omics techniques (e.g., metabolomics, lipidomics, genomics, and transcriptomics). Over the past decades, metabolomics has added a promising number of ‘new’ biomarkers to the ‘old’ group through better physiopathological knowledge, paving the way for insightful perspectives on the management of different renal diseases. We aimed to summarize the most recent omics evidence in the main renal pediatric diseases (including acute renal injury, kidney transplantation, chronic kidney disease, renal dysplasia, vesicoureteral reflux, and lithiasis) in this narrative review.

Ultrasonography in pediatric renal and urinary tract disease

Ultrasonography in pediatric renal and urinary tract disease

Changing pattern of renal disease in children at pediatric nephrology clinic of a tertiary teaching hospital, Nepal: 10-year review

Introduction: Children present with different renal diseases with variations according to different geographic locations. This study aims to determine the trend of pediatric renal disease presenting. Method: This retrospective study from pediatric nephrology clinic register during 2008 to May 2018 at Patan Hospital, Patan Academy of Health Sciences, Nepal to analyze the changing pattern of renal disease. The prevalence and characteristic of renal diseases were compared between the 1st and 2nd 5-y period, the age at presentation and gender was analyzed. Mean age at presentation was analyzed by independent t-test, a p-value <0.05 was considered significant. The study was approved by the ethical committee. Result: A total of 352 children visited the nephrology clinic, male:female ratio 1.42:1, mean age 6.25±4.5 y, the majority in 0-5 y. Congenital anomalies of the kidney and urinary tracts (CAKUT) were 39%, mostly vesicoureteral reflux. Acute glomerulonephritis and the nephrotic syndrome were seen in 32.7% and 23.6%. Children <5 y of age presented with CAKUT while ≥5 y with acute glomerulonephritis. The mean age at presentation for CAKUT was 2.9±3 y and acquired diseases 8.46±3.91 y, p-value<0.05; and for glomerulonephritis 9.8±2.91 y and nephrotic syndrome 6.91±4.08 y, p-value<0.05. Conclusion: There was a decrease in the number of children visiting the nephrology clinic. Children <5 y presented with CAKUT while ≥5 y had acquired conditions. Children with acute glomerulonephritis were of a higher age than children with nephrotic syndrome.

Role of Renal Biopsy in Managing Paediatric Renal Disease in a Tertiary Care Setting

Introduction: The contribution of renal biopsy is of major importance in many paediatrics renal diseases. This prospective study analysed the clinic-pathological spectrum of the biopsied cases, complications related to renal biopsy and its effect on management. Methods: USG guided percutaneous renal biopsy was performed in indicated children. Laboratory findings, complications of the procedure, histological diagnosis and its effect on management were obtained from all patients who underwent renal biopsy from April 2019 to March 2020. The data were finally analysed using descriptive statistics. Results: Total 50 cases were enrolled during the study period. There were 24 males and 26 females with age range from two to 16 years. The common indications for biopsy were acute nephritic syndrome (25 cases), including both primary glomerulonephritis (11 cases) and secondary glomerulonephritis (14 cases), of which lupus nephritis contributed (11/25) cases, followed by atypical/ steroid resistant or dependent nephrotic syndrome (18 cases). Among primary glomerulonephritis, diffuse proliferative glomerulonephritis was most common pathological finding (11/25). One had crescentric glomerulonephritis. Lupus nephritis predominantly had Class IV/V pathology (6/11). Of 18 nephrotic syndrome cases, nine had minimal-change-disease and five had focal-segmental-glomerulosclerosis. The complications associated with procedure were self-resolving gross hematuria in eight cases, perirenal hematoma requiring plasma transfusion in one case. Adequate renal tissue sample was obtained in 47 of the renal biopsies. Three cases underwent repeat biopsy. Immunosuppressive therapy was altered as per the biopsy report among 18 cases. Conclusions: Our study showed that renal biopsy is a safe, reliable and effective technique in children. It resolutes many diagnostic dilemma and helps in effective management.

Asymmetric Dimethylarginine (ADMA) in Pediatric Renal Diseases: From Pathophysiological Phenomenon to Clinical Biomarker and Beyond.

Asymmetric dimethylarginine (ADMA), an endogenous nitric oxide (NO) synthase inhibitor, inhibits NO synthesis and contributes to the pathogenesis of many human diseases. In adults, ADMA has been identified as a biomarker for chronic kidney disease (CKD) progression and cardiovascular risk. However, little attention is given to translating the adult experience into the pediatric clinical setting. In the current review, we summarize circulating and urinary ADMA reported thus far in clinical studies relating to kidney disease in children and adolescents, as well as systematize the knowledge on pathophysiological role of ADMA in the kidneys. The aim of this review is also to show the various analytical methods for measuring ADMA and the issues tht need to be addressed before transforming to clinical practice in pediatric medicine. The last task is to suggest that ADMA may not only be suitable as a diagnostic or prognostic biomarker, but also a promising therapeutic strategy to treat pediatric kidney disease in the future.

Different approaches among physicians to treat pediatric stone disease: a survey-based study.

Pediatricians, surgeons and subspecialties as pediatric urology and nephrology are involved in the diagnosis and treatment of pediatric renal stone disease (RSD). The aim of this study was to determine diagnostic and treatment approaches, of different disciplines, and to assess differences in their routine diagnostic and treatment protocols. A questionnaire was designed and administered to the participants of the RSD sessions in national congresses of all disciplines in 2017 to evaluate the diagnostic and treatment routines of specialties (surgeons and pediatricians) and subspecialties (pediatric nephrologists and pediatric urologists) for RSD. A total, of 324 questionnaires were analyzed, from 88 pediatricians (27 %), 121 urologists (37 %), 23 pediatric surgeons (7 %), 54 pediatric nephrologists (17 %), and 38 pediatric urologists (12 %). Both groups agreed on the necessity of metabolic evaluation. For distal ureter stones that were ≥ 6 mm; surgeons preferred ureteroscopy (URS), pediatricians preferred shock wave lithotripsy (SWL) (p < 0.001) and subspecialties preferred URS for the treatment (p = 0.636). For lower calix stones less than 1 cm surgeons and subspecialists preferred SWL, while pediatricians preferred hydration (p < 0.001, p = 0.371). For the stone between 1.1 and 2 cm, surgeons preferred intrarenal surgery (RIRS) and SWL, pediatricians preferred SWL (p = 0.001). For larger stones, surgeons and subspecialists preferred percutaneous nephrolithotomy (PCNL), and pediatricians preferred SWL (p = 0.458 p = 0.001). Pediatric urologist chose low-dose computerized tomography as a diagnostic radiologic evaluation (p = 0.029). There are differences between the disciplines who take an active role in diagnosis and treatment of RSD.

POS-446 SPECIFIC FEATURES AND OUTCOME OF NEPHROCALCINOSIS IN CHILDREN: ABOUT A SERIES OF 30 CASES

Nephrocalcinosis is a rare pediatric renal disease resulting in calcifications of the kidney tissue. It comes from various causes, especially genetic mutations. Management is based on symptomatic measures and treatment of the underlying condition. The aim of this study (from January 2010 to June 2020) is to underly the specific features of nephrocalcinosis in our pediatric population. For this, we gathered retrospective data of 30 children diagnosed with nephrocalcinosis. The mean age at diagnosis was of 3,5 years. One-third of our patients had first-degree consanguineous parents, while two-thirds of them were males. Similar cases in siblings and family members were absent. The majority of children presented with polyuria, polydipsia, and dehydration at diagnosis. Also, half of them had simultaneous urologic calculus. In five cases, nephrocalcinosis was discovered on routine abdominal ultrasound while the children were asymptomatic. The sonographic classification was in favor of stage III nephrocalcinosis in 23 cases. The underlying disease was represented mostly by tubulopathies in 40% of cases. Distal tubular acidosis was predominant. Nephrocalcinosis secondary to recurrent urinary tract infections was seen in 10% of our children. While, prematurity, primary hyperoxaluria, and familial hypercalciuria were rare (both seen in 10% of children). One child developed chronic kidney disease stage II after a follow up of seven years. Meanwhile, management was successful in stopping the evolution of the disease in all the remaining cases. It consisted on hyperhydration in all cases, alkalinization of urine in the case of acidosis, along with the specific treatment of the underlying disease. Early management of nephrocalcinosis in children can prevent the evolution of chronic kidney disease. Systematic screening should be implemented in high-risk children, especially those with tubulopathies. The high frequency of consanguineous marriages in our population suggests a genetic predisposition that needs to be proven by further studies.

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.

Epidemiology of Pediatric Renal Diseases and its Histopathological Spectrum - A Single-Center Experience from India.

Pediatric renal biopsy is an uncommon event, and the spectrum of the disease is evaluated and managed mostly on the clinical grounds. Compared to adults, the indications for renal biopsy in pediatric population are very few. We reviewed the pediatric renal biopsies received at our tertiary center in Mumbai, India, over a period of six years to study the incidence of various medical renal diseases, their spectrum on histology and its correlation with electron microscopy (EM). A total of 65 pediatric renal biopsies in the age group of 0-12 years were evaluated over a period of six years. The mean age of our patients was 7.9 years, with a median of 8.8 years with a male-to-female ratio of 1.3:1. The overall most common indication for biopsy was nephrotic syndrome (NS) including steroid-resistant NS, followed by proteinuria and nephritic syndrome. Majority of the lesions included in the study were primary glomerular disease (71%) while secondary glomerular disease amounted to 18%. The spectrum of disease includes minimal-change disease (MCD) (27.7%), followed by membranoproliferative glomerulonephritis (MPGN) (15.38%), focal segmental glomerulosclerosis (FSGS) (9.23%), lupus nephritis (7.7%), hemolytic uremic syndrome (7.7%), MPGN (6.15%), advanced renal disease (6.15%), membranous glomerulonephritis (3.07%), and crescentic glomerulonephritis (3.07%). This study is an important contribution to the epidemiology of pediatric renal disease spectrum in the Indian population. We conclude that MCD is the most common pathology seen in pediatric age group, with NS as the most common indication for biopsy. There is a steady increase in the incidence of FSGS in the pediatric population with frequent relapses and an increase in the incidence of steroid resistance. However, with the use of immunofluorescence and EM, an accurate diagnosis is possible, so an early renal biopsy should be planned in nonresponding cases and at times even before starting the treatment for appropriate treatment.