Pediatric Nephrology Research Articles

NPHS1 and NPHS2 Variants Associated with Early-Onset Frequently Relapsing or Steroid-Dependent Nephrotic Syndrome

Nephrotic syndrome (NS) is the most common diagnosis in pediatric nephrology and is evolving as a leading cause of uremia [1]. Affected individuals have massive proteinuria in utero, and NS develops soon after birth (especially in premature infants)

Variants in genes coding for collagen type IV α-chains are frequent causes of persistent, isolated hematuria during childhood.

Children with persistent, isolated microscopic hematuria typically undergo a limited diagnostic workup and are monitored for signs of kidney disease in long-term longitudinal follow-up, which can delay diagnosis and allow disease progression in some cases. To determine the clinical utility of genetic screening in this population, we performed targeted genetic testing using a custom, 32-gene next-generation sequencing panel for progressive kidney disease on children referred to the Texas Children's Hospital Pediatric Nephrology clinic for persistent, microscopic hematuria (n = 30; cohort 1). Patients with microscopic hematuria identified by urinalysis on at least two separate occasions were eligible for enrollment, but those with other evidence of kidney disease were excluded. Results were analyzed for sequence variants using the American College of Medical Genetics and Genomics (ACMG) guideline for data interpretation and were validated using a secondary analysis of a dataset of children with hematuria and normal kidney function who had undergone genetic testing as part of an industry-sponsored program (cohort 2; n = 67). In cohort 1 33% of subjects (10/30) had pathogenic or likely pathogenic (P/LP) variants in the type IV collagen genes (COL4A3/A4/A5), and 10% (3/30) had variants of uncertain significance in these genes. The high diagnostic rate in type IV collagen genes was confirmed in cohort 2, where 27% (18/67) of subjects had P/LP variants in COL4A3/A4/A5 genes. Children with persistent, isolated microscopic hematuria have a high likelihood of having pathogenic variants in type IV collagen genes and genetic screening should be considered. A higher resolution version of the Graphical abstract is available as Supplementary information.

Can Steroid Response in Idiopathic Childhood Nephrotic Syndrome be Predicted? A Single Center Quasi-Experimental Study

Objective: To predict the role of clinical risk factors and urinary β2-microglobulin levels as a biomarker for steroid-resistant nephrotic syndrome.
 Study Design: Quasi-experimental study.
 Place and Duration of Study: Paediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi Pakistan, from Jun 2019 to Nov 2020.
 Methodology: All children (3 months to 12 years) with either first episode or relapse of the nephrotic syndrome were included. A stored urine sample was used on 100 patients with steroid-sensitive (group-1) and 35 patients with steroid-resistant nephrotic syndrome (group-2). In addition, histopathology of all patients with steroid-resistant nephrotic syndrome was recorded.
 Results: Both groups and those who had focal segmental glomerulosclerosis were compared and analysed to evaluate the predictability of steroid response. There was a significant association in both groups for microscopic haematuria, hypertension, heavy proteinuria (urine spot protein to creatinine ratio >10 g/g) and increased β2-microglobulin levels (> 3× normal) as individual risk factors (p<0.01). The sensitivity of β2-microglobulin levels was 78% and a positive predictive value of 80%. Multivariate regression analysis on steroid-resistant nephrotic syndrome as a group did not confer a higher risk; however, for children with focal segmental glomerulosclerosis, the likelihood of steroid unresponsiveness was significantly higher for the same parameters.
 Conclusion: The addition of biomarker measurement and known clinical risk factors helped predict steroid-resistant focal segmental glomerulosclerosis. However, further studies are warranted before these results can be generalized.

Advanced chronic kidney disease among UK children

The UK Renal Registry currently collects information on UK children with kidney failure requiring long-term kidney replacement therapy (KRT), which supports disease surveillance and auditing of care and outcomes; however,...

Development and pilot implementation of Iranian Hemolytic Uremic Syndrome Registry

BackgroundPatients with Hemolytic Uremic Syndrome (HUS) face late diagnosis and lack of appropriate treatment because of a lack of knowledge and experience in this field. A prerequisite for such knowledge is the development of research infrastructures such as a registry system. Therefore, this study aimed to develop and describe the HUS registry in accordance with the Iranian health system and implement its software system.MethodsWe first interviewed 10 pediatric nephrologists and after analyzing the interviews, we identified the features and requirements and the data related to HUS. Then, during two rounds of the Delphi technique (the first round with 23 participants and the second round with 18 participants), the model of this registry was finalized based on the agreement of at least 75% of specialists. At the next step, based on the agreed requirements, IRI.HUS.Reg (Iranian Hemolytic Uremic Syndrome Registry) software was developed and implemented in a pediatric hospital.ResultsWe classified 369 meaning units of interviews in 41 codes and 7 final themes including purposes of the registry (10 codes), inclusion criteria (7 codes), data collection method (4 codes), data quality control (6 codes), data sources (4 codes), data analysis (3 codes) and software features (7 codes). These 7 feature groups (67 subgroups) and 12 data classes (138 data elements) include demographic data, referrals, examinations, clinical signs, causes, laboratory tests, medical histories, paraclinical measures, treatments, outcomes, patient’s status at discharge, and follow-up data were reviewed by the Delphi panelists, and finally, 64 features and 131 data elements were accepted by at least 78% agreement. Then, we developed and implemented a registry software system in a hospital.ConclusionWe implemented IRI.HUS.Reg based on related features, 12 data classes agreed by specialists, literature review, and comparison with other existing registries. Therefore, the data collected in this registry can be compared with other data from existing registries in other countries.

Successful ABO and HLA incompatible kidney transplantation in children in the UK

BackgroundThere is increasing evidence of good short-term and medium-term outcomes of ABO incompatible (ABOi) and HLA incompatible (HLAi) kidney transplantation with pre-transplant positive crossmatches in paediatric practice. However, there remain concerns regarding the higher risks of infective complications and antibody-mediated rejections. The aim of our study is to show longer-term follow-up on all ABOi and HLAi paediatric kidney transplant recipients (pKTR) in the UK.MethodsQuestionnaires specifying kidney transplant type, desensitisation requirement and kidney allograft function were sent to 13 paediatric nephrology centres that performed kidney transplantation in children and young people under 18 years of age who received an ABOi and/or HLAi transplant between 1 January 2006 and 31 December 2016. Patient and kidney allograft survival were compared between ABOi, HLAi and ABO/HLA compatible (ABOc/HLAc) groups.ResultsAmong 711 living donor kidney transplants performed in the UK, 23 were ABOi and 6 were HLAi. Patient survival was 87%, 100% and 96% in ABOi, HLAi and ABOc/HLAc groups, respectively, at median follow-up of 6.8 (3.6–14.0) years post-transplant. Death-censored kidney allograft survival was 100% in all 3 groups at last follow-up. There were no cases of primary non-function in ABOi or HLAi groups, but 2% in the ABOc/HLAc group. There was one reported case of Epstein-Barr viral-induced post-transplant lymphoproliferative disorder.ConclusionLonger term follow-up has shown that ABOi and HLAi kidney transplantation are feasible for pKTR where no compatible donors are available, and that minimising desensitisation should be achieved where possible.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information.

Severe Fetal CAKUT (Congenital Anomalies of the Kidneys and Urinary Tract), Prenatal Consultations, and Initiation of Neonatal Dialysis.

Pediatric nephrology prenatal consultations for congenital anomalies of the kidney and urinary tract (CAKUT) and criteria for kidney replacement therapy initiation in neonatal end-stage kidney disease (ESKD) are not well described. We evaluated pediatric nephrology approaches to prenatal CAKUT counseling and neonatal dialysis initiation. A 35-question Qualtrics survey was distributed via the North American Pediatric Renal Trials and Collaborative Studies email list between January and March 2021. Thirty-nine pediatric nephrology centers completed the survey. All but one responding center (n = 38) provide prenatal CAKUT consultations and neonatal dialysis, with wide variability in reported multispecialty involvement. Nearly half (47%) of centers utilize written/unwritten criteria for offering neonatal dialysis. The most common contraindications to neonatal dialysis were parental refusal (61%), contraindication to access placement by surgeons (55%), and birth weight (BW) contraindication (55%, with < 1,500 g being the most common BW contraindication). Overall, 79% of centers reported caring for < 5 neonates with ESKD in the past year, 61% use hemodialysis therapies prior to peritoneal dialysis in neonates requiring dialysis, and 100% transition to peritoneal dialysis by hospital discharge. Many pediatric nephrology programs provide prenatal CAKUT consultations and neonatal dialysis, but with variability in practice approach. Further multicenter research regarding prenatal consultations and neonatal dialysis outcomes is necessary to further improve care delivery to this population.

The Clinical and Genetic Features in Chinese Children With Steroid-Resistant or Early-Onset Nephrotic Syndrome: A Multicenter Cohort Study.

Steroid-resistant nephrotic syndrome (SRNS) is one of the major causes of end-stage kidney disease (ESKD) in children and young adults. For approximately 30% of children with SRNS results from a genetic cause. In this study, genotype-phenotype correlations in a cohort of 283 pediatric patients with SRNS or early-onset NS (nephrotic syndrome presenting within the first year of life) from 23 major pediatric nephrology centers in China were analyzed. All patients were performed with next-generation sequencing and Sanger sequencing. The overall mutation detection rate was 37.5% (106 of 283 patients). WT1 was the most frequently detected mutation, followed by NPHS1, NPHS2, and ADCK4, and these four major causative genes (WT1, NPHS1, NPHS2, and ADCK4) account for 73.6% of patients with monogenic SRNS. Thirteen of 106 individuals (12.3%) carried mutations in ADCK4 that function within the coenzyme Q10 biosynthesis pathway. In the higher frequently ADCK4-related SRNS, two mutations, c.737G>A (p.S246N) and c.748G>C (p.D250H), were the most prevalent. Our study provides not only definitive diagnosis but also facilitate available targeted treatment for SRNS, and prediction of prognosis and renal outcome. Our indications for genetic testing are patients with FSGS, initial SRNS, cases of positive family history or those with extra-renal manifestations.

CURRENT PRACTICES OF BP MEASUREMENT IN CHILDREN AND ADOLESCENTS ACROSS EUROPEAN COUNTRIES: A SURVEY BY THE HYPERCHILDNET COST ACTION WG ON DEVICES

Objective: Objective: The main objective was to carry out a survey on current practices of BP measurement used among health care providers involved in the care of children and adolescents. Design and method: Design and methods: A 22 questions survey was released online from September 2, 2021 to September 27, 2021. The survey's link was sent to primary care physicians and pediatricians from the 22 European COST country members and it was also posted on the HyperChildNET website. Results: Results: The survey was assessed by 476 individuals and fully completed by 293. It was answered by general practioneers and pediatricians as well as pediatric nephrologists, pediatric cardiologists and pediatric endocrinologists, half of them working in primary care and the other half in hospital settings. Office BP measurement was used for clinical decision-making by 49% of the survey respondents while 29.7% used ABPM (Ambulatory BP Monitoring) and 16% home BP measurements. Only 11% routinely screen for high BP in normotensive children (Figure). Auscultatory and oscillometric devices were used in 57% and 41% of the settings, respectively. The main reason for selecting a BP device was availability in the institution in 60%, ease to use in 25.5% and validation in the relevant age group in 11.5%. Cuff size was selected according to individual child or adolescent mid arm circumference by 55% of the responders. A usual pitfall was combining cuffs from different devices and manufactures reported by 47.7% of the responders. Moreover, 42% performed only one BP measurement at each office BP visit. There was heterogeneity on the reference values used to evaluate the BP status with 27.4% using country specific BP reference tables and the rest different guidelines’ staging schemes. Eighty-three % used published reference values and 15.4% internet-based application for BP staging in children and adolescents. Conclusions: Conclusions: Variation in practices among health care providers show the emerging need of defining criteria to benchmark the different BP measurement devices, as well as to promote awareness for the evaluation of hypertensive children across Europe.

Lumasiran in the Management of Patients with Primary Hyperoxaluria Type 1: From Bench to Bedside.

Primary hyperoxaluria (PH) is a rare genetic disease caused by excessive hepatic production and elevated urinary excretion of oxalate that leads to recurrent nephrolithiasis, nephrocalcinosis and, eventually, kidney failure. As glomerular filtration rate declines, oxalate accumulates leading to systemic oxalosis, a debilitating condition with high morbidity and mortality. Although PH is usually diagnosed during infancy, it can present at any age with different phenotypes, ranging from mild symptoms to extremely debilitating manifestations. PH is an autosomal recessive disorder and, to date, three types have been identified: PH1, PH2 and PH3. PH1 is the most common and most aggressive type, accounting for almost 80% of primary hyperoxaluria diagnoses. Until 2020, general treatment for PH1 consisted mainly in high fluid intake, urine alkalization, surgical management of recurrent nephrolithiasis and eventually, if and when kidney failure occurred, intensive dialysis regimens and transplantation strategies (simultaneous or sequential liver-kidney transplant or isolated liver/kidney transplant in carefully selected patients). Specific treatment did and still consists in administration of pyridoxine hydrochloride, although it is only effective in a subset of PH1 patients. Lumasiran, a novel biological drug based on mRNA interference that has been recently approved in the US and European Union, showed promising results and is set to be a turning point in the management of PH1. This literature review aims to summarize the available evidence on PH1 treatment with lumasiran, in order to provide both pediatric and adult nephrologists and clinicians with the knowledge for the identification and management of PH1 patients suitable for treatment.

Long-Term Efficacy and Safety of Repeated Rituximab to Maintain Remission in Idiopathic Childhood Nephrotic Syndrome: An International Study.

Long-term outcomes after multiple courses of rituximab among children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) are unknown. A retrospective cohort study at 16 pediatric nephrology centers from ten countries in Asia, Europe, and North America included children with FRSDNS who received two or more courses of rituximab. Primary outcomes were relapse-free survival and adverse events. A total of 346 children (age, 9.8 years; IQR, 6.6-13.5 years; 73% boys) received 1149 courses of rituximab. A total of 145, 83, 50, 28, 22, and 18 children received two, three, four, five, six, and seven or more courses, respectively. Median (IQR) follow-up was 5.9 (4.3-7.7) years. Relapse-free survival differed by treatment courses (clustered log-rank test P<0.001). Compared with the first course (10.0 months; 95% CI, 9.0 to 10.7 months), relapse-free period and relapse risk progressively improved after subsequent courses (12.0-16.0 months; HRadj, 0.03-0.13; 95% CI, 0.01 to 0.18; P<0.001). The duration of B-cell depletion remained similar with repeated treatments (6.1 months; 95% CI, 6.0 to 6.3 months). Adverse events were mostly mild; the most common adverse events were hypogammaglobulinemia (50.9%), infection (4.5%), and neutropenia (3.7%). Side effects did not increase with more treatment courses nor a higher cumulative dose. Only 78 of the 353 episodes of hypogammaglobulinemia were clinically significant. Younger age at presentation (2.8 versus 3.3 years; P=0.05), age at first rituximab treatment (8.0 versus 10.0 years; P=0.01), and history of steroid resistance (28% versus 18%; P=0.01) were associated with significant hypogammaglobulinemia. All 53 infective episodes resolved, except for one patient with hepatitis B infection and another with EBV infection. There were 42 episodes of neutropenia, associated with history of steroid resistance (30% versus 20%; P=0.04). Upon last follow-up, 332 children (96%) had normal kidney function. Children receiving repeated courses of rituximab for FRSDNS experience an improving clinical response. Side effects appear acceptable, but significant complications can occur. These findings support repeated rituximab use in FRSDNS.

Treatment-Associated Side Effects in Patients with Steroid-Dependent Nephrotic Syndrome.

Introduction:Nephrotic syndrome is one of the most extensively studied pediatric diseases for a nephrologist. Treatment for patients suffering from it has greatly improved the prognosis and reduced the mortality rate to 3% or less. Steroid medication is the first line, but non-steroid immunosuppressive drugs are useful in limiting steroid side effects and maintaining long-term remission. Objective: The aim of the study is to choose the best treatment for each patient in order to minimize long-term effects of corticosteroid therapy, taking into account the child's age, gender and other factors to optimize the quality of life. Materials and methods:Materials and methods: This is a retrospective study of pediatric patients older than one year and younger than 18 years of age diagnosed with idiopathic corticosteroid nephrotic syndrome. Study participants were treated in the Department of Pediatric Nephrology of "M. S. Curie" Emergency Clinical Hospital for Children, Bucharest, Romania, between January 2013 and December 2020. Thirty-seven subjects with steroid-dependent nephrotic syndrome from a total of 125 patients diagnosed with nephrotic syndrome were included in the present study. All study participants underwent clinical examination and laboratory tests and were regularly monitored. Results and discussion: Long-term corticosteroid therapy can lead to unwanted complications such as hypertension, short stature, behavior disturbances, or osteopenia. To minimize certain side effects, a second line steroid-sparing agents can be used due to their ability to induce complete remission and maintain it in patients with steroid-dependent nephrotic syndrome. Conclusion: Our study describes the most frequent side effects encountered in the Department of Nephrology in patients with steroid-dependent nephrotic syndrome. The purpose was to emphasize that pediatric patients' quality of life depends on limiting long-term corticosteroid treatment and preventing adverse reactions or complications.

Compensatory epidemic of RSV infections during the COVID-19 pandemic. An analysis of infections in children hospitalised in the Department of Paediatrics, Paediatric Nephrology and Allergology of the Military Medical Institute in Warsaw in 2020–2021

Seasonal outbreaks of respiratory syncytial virus are common among young children and represent an important clinical problem. The infection can affect all floors of the respiratory system. Respiratory syncytial virus is highly infectious and is the most common aetiological agent of bronchiolitis. It is estimated that 90% of children develop respiratory syncytial virus infection by the age of 2 years. In Poland and other countries of the northern hemisphere, these infections usually occur between October and May, with a peak in January–February. During the global COVID-19 pandemic, a significant change was observed in the epidemiology of respiratory syncytial virus infections in Europe and worldwide. The massive use of nonpharmacological interventions, such as closing nurseries, kindergartens, limited interpersonal contact, social distancing, strict hygiene rules and the use of protective masks, resulted in the absence or episodic occurrence of many seasonal infections, including those of respiratory syncytial virus aetiology, in 2020. In 2021, a so-called compensatory epidemic of syncytial virus aetiology was observed, i.e. a significant increase in the incidence associated with the extinction of collective immunity and an increasing proportion of susceptible individuals. The aim of the present study was to retrospectively analyse the incidence of respiratory syncytial virus infections in children hospitalised in the Department of Paediatrics, Paediatric Nephrology and Allergology of the Military Institute of Medicine in Warsaw in the years 2020–2021. We also discussed the prevention and treatment of infections, as well as the association of bronchiolitis with the development of asthma in children.

Appropriateness of Antibiotic Prescribing in Hospitalized Children: A Focus on the Real-World Scenario of the Different Paediatric Subspecialties.

Background: Antibiotics are prescribed for children both in hospital and community settings, particularly at preschool age. Italy shows a high rate of inappropriate antibiotic prescriptions which may represent a serious problem in the hospital scenario. Thus, the aim of this study was to investigate appropriateness of antibiotic prescribing in the context of different paediatric subspecialties in a hospital setting. Methods: Antibiotics prescribing was retrospectively analysed in paediatric patients (0–18 years) admitted in the emergency paediatrics, general paediatrics, paediatric nephrology and rheumatology units between January and December 2019. Patients were stratified by age in neonates, infants, toddlers, children and adolescents. Assessments were conducted by trained local assessors and appropriateness was classified as appropriate, inappropriate and not assessable. Results: Empirical antibiotics were mainly prescribed following a diagnosis of respiratory, gastrointestinal and/or urinary infection. A total of 825 antibiotic prescriptions were recorded in the three subspecialties; 462 antibiotic prescriptions (56%) out of 825 were assessed as inappropriate and 55 prescriptions (6.7%) were not assessable. Inappropriateness considerably varied within subspecialties: the risk of inappropriate antibiotic prescribing was higher in emergency paediatrics and general paediatric than in children, according to age. Ceftriaxone and clarithromycin were the most inappropriate prescribed antibiotics in the emergency paediatrics whereas amoxicillin/clavulanic acid represented the most inappropriate antibiotic prescribed in general paediatrics. Conclusion: The present data may be useful in order to reduce inappropriate antibiotic prescribing in the paediatric setting; antibiotic stewardship and clinical improvement programs in hospital paediatric care are strongly recommended.

Paediatric kidney transplantation: Towards a framework for pretransplant urological evaluation.

The role of the urologist in paediatric kidney transplantation has evolved alongside advances in management for the various causes of end-stage kidney disease. Improvements in antenatal intervention and postnatal care have seen children with increasingly complex urological anomalies survive until transplant. Once solely responsible for the oversight of a child's surgical care, the paediatric urologist now works within a multidisciplinary transplant team, alongside transplant surgeons, paediatric nephrologists, transplant coordinators, psychologists, social workers, and transitional care specialists. We sought to identify available pretransplant evaluation frameworks to guide urological preparation and decision-making. Drawing from available evidence and reflecting on multi-institutional experience, we propose a streamlined approach to urologic assessment, which recognises that optimal transplant outcomes in this heterogenous cohort require lower urinary tract dysfunction to be carefully defined preoperatively.

Hematuria and Proteinuria in a Patient With Recurrent Pulmonary Illnesses: A Quiz

A 19-year-old man presented to an adult nephrology practice to establish care. He had a history of recurrent hematuria and proteinuria in the setting of upper respiratory tract illnesses. This was first noted at age 14 when he had gross hematuria associated with episodes of fever and pharyngitis 2 months apart. Pharyngeal swab was negative for Streptococcus both times. He felt generally well otherwise. His pediatrician suspected IgA nephropathy (IgAN) and referred him to a pediatric nephrologist, who reported the same impression and recommended a conservative management strategy.

Primary hyperoxaluria: the pediatric nephrologist's point of view.

ABSTRACTThe clinical presentation of primary hyperoxaluria in children ranges from mildly symptomatic nephrocalcinosis to very early onset end-stage kidney failure with systemic oxalosis, a devastating complication. We review the various manifestations of pediatric hyperoxaluria, treatment options for children with preserved kidney function and appropriate dialysis regimens. Liver or combined liver/kidney transplantation is currently the only definitive treatment for primary hyperoxaluria type 1, but novel RNA interference treatments offer hope for the future. Finally, we address the medical and ethical dilemmas facing pediatricians treating children with hyperoxaluria.

Comparing Pediatric Nephrologists’ and Palliative Care Physicians’ Perspectives on Palliative Care for Children with End-Stage Kidney Disease: A National Survey Study (GP727)

Comparing Pediatric Nephrologists’ and Palliative Care Physicians’ Perspectives on Palliative Care for Children with End-Stage Kidney Disease: A National Survey Study (GP727)

Targeted Training for Subspecialist Care in Children With Medical Complexity.

BackgroundChildren with medical complexity (CMC) are prone to medical errors and longer hospital stays, while residents do not feel prepared to provide adequate medical care for this vulnerable population. No educational guidance for the training of future pediatric tertiary care specialists outside their field of expertise involving the multidisciplinary care of CMC exists. We investigated pediatric residents past educational needs and challenges to identify key learning content for future training involving care for CMC.MethodsThis was a prospective mixed-methods study at a single pediatric tertiary care center. Qualitative semi-structured interviews with residents were conducted, submitted to thematic content analysis, linked to the American Board of Pediatrics (ABP) general pediatrics content outline, and analyzed with importance performance analysis (IPA). Quantitative validation was focused on key themes of pediatric nephrology within the scope of an online survey among pediatric residents and specialists.ResultsA total of 16 interviews, median duration 69 min [interquartile range IQR 35], were conducted. The 280 listed themes of the ABP general pediatrics content outline were reduced to 165 themes, with 86% (theoretical) knowledge, 12% practical skills, and 2% soft skills. IPA identified 23 knowledge themes to be of high importance where improvement is necessary and deemed fruitful. Quantitative validation among 84 residents and specialists (response rate 55%) of key themes in nephrology yielded high agreement among specialists in pediatric nephrology but low interrater agreement among trainees and “trained” non-nephrologists. The occurrence of themes in the qualitative interviews and their calculated importance in the quantitative survey were highly correlated (tau = 0.57, p = 0.001). Two clusters of high importance for other pediatric specialties emerged together with a contextual cluster of frequent encounters in both in- and outpatient care.ConclusionRegarding patient safety, this study revealed the heterogeneous aspects and the importance of training future pediatric tertiary care specialists outside their field of expertise involving the multidisciplinary care of CMC. Our results may lay the groundwork for future detailed analysis and development of training boot camps that might be able to aid the improvement of patient safety by decreasing preventable harm by medical errors, especially for vulnerable patient groups, such as CMC in tertiary care pediatrics.

Evaluation of the Claria sharesource system from the perspectives of patient/caregiver, physician, and nurse in children undergoing automated peritoneal dialysis.

BackgroundAutomated peritoneal dialysis (APD) is increasingly preferred worldwide. By using a software application (Homechoice with Claria sharesource system (CSS)) with a mod-M added to the APD device, details of the home dialysis treatment become visible for PD nurses and physicians, allowing for close supervision. We aimed to evaluate the perceptions of patients/caregivers, PD nurses, and physicians about the advantages and disadvantages of CSS.MethodsThree different web-based questionnaires for patients/caregivers, nurses, and physicians were sent to 15 pediatric nephrology centers with more than 1 year of experience with CSS.ResultsRespective questionnaires were answered by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses. Most of the nurses and physicians (87% and 73%) reported that CSS improved patient monitoring. A total of 73% of nurses suggested that CCS is not well known by physicians, while half of them reported reviewing CSS data for all patients every morning. Sixty-eight percent of physicians thought that CSS helps save time for both patients/caregivers and healthcare providers by reducing visits. However, only 20% of patients/caregivers reported reduced hospital visits. A total of 90% of patients/caregivers reported that being under constant monitoring made them feel safe, and 83% stated that the patient’s sleep quality improved.ConclusionsA remote monitoring APD system, CSS, can be successfully applied with children for increased adherence to dialysis prescription by giving shared responsibility and may help increase the patient’s quality of life. This platform is more commonly used by nurses than physicians. Its potential benefits should be evaluated in further well-designed clinical studies with larger patient groups.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information. Supplementary InformationThe online version contains a graphical abstract available at 10.1007/s00467-022-05563-9.