Pediatric Nephrology Research Articles

The role of increased intestinal permeability markers in developing urinary tract infection in children of the first three years of life

Urinary tract infection (UTI) is one of the urgent problems in pediatric nephrology and pediatrics. Despite numerous works devoted to the study of pathogenetic mechanisms and development of new diagnostic measures in patients with urinary tract infection, many questions on pathogenesis have not yet been clearly elucidated. Considering that non-specific symptoms prevailing in the disease clinical picture in children of the first three years of life, it is necessary to search for reliable, early, non-invasive methods for diagnosing urinary tract infections. Purpose of the study is to determine diagnostic and pathogenetic significance of proteins that bind fatty acids as well as zonulin in diagnosis of urinary tract infection in children of the first three years of life. Materials and methods. There were examined 120 children, aged from 29 days of life to 2 years 11 months and 29 days inclusive with urinary tract infection. Control group 30 healthy children. All children were tested for serum level of proteins that bind fatty acids L-FABP and I-FABP, and the level of I-FABP, L-FABP and zonulin was measured in urine by enzyme-linked immunosorbent assay. Results. In the blood serum of children with urinary tract infection, the levels of L-FABP and I-FABP were significantly increased compared with those in children of the control group (L-FABP 753.9426.16 ng/ml vs. 148.719.6 ng/ml, I-FABP 92.971.41 ng/ml vs. 18.033.03 ng/ml, respectively, p 0.001). Fractions of urine proteins L-FABP and I- FABP in sick vs. healthy children were also determined at a higher level (6.960.19 ng/ml and 1.010.25 ng/ml vs. 0.370.01 ng/ml and 0.10.02 ng/ml, respectively, at p 0.001). The level of urine zonulin was increased (2.840.12 ng/ml) in sick vs. healthy children (0.170.04, p 0.05). Conclusion. The study allowed to identify early markers of kidney and urinary tract damage (L-FABP, I-FABP, ZO) involved in developing inflammatory bacterial process, whereas measuring urine I-FABP, L-FABP, zonulin level can be used for non-invasive UTI diagnostics in infants and young children.

Long-term kidney outcomes in children following continuous kidney replacement therapy.

Continuous kidney replacement therapy (CKRT) is a mainstay of therapy for management of severe acute kidney injury (AKI) in critically ill pediatric patients. There is limited data on the risk of chronic kidney disease (CKD) after discharge in this population. This is a single-center, retrospective cohort study of all pediatric patients ages 0-17years who received CKRT from 2013 to 2017. The study excluded patients with pre-existing CKD, those who died prior to discharge, and those who had insufficient follow-up data. Patients were followed after hospital discharge and electronic health record data was collected and analyzed to assess for incidence of and risk factors for kidney sequelae. A total of 42 patients were followed at a median time of 27months (IQR 17.2, 39.8). Of these, 26.2% had evidence of CKD and 19% were at risk for CKD. Lower eGFR at hospital discharge was associated with increased odds of kidney sequelae (aOR 0.985; 95% CI 0.972, 0.996). Ages 0- < 1 and 12-17 were not significantly different (aOR 0.235, 95% CI 0.024, 1.718) and had the highest incidence of kidney sequelae (50% and 77%, respectively). Ages 1-5 and 6-11 had a decreased odds of kidney sequelae compared to the 12-17year age group (aOR 0.098; 95% CI 0.009, 0.703 and aOR 0.035; 95% CI 0.001, 0.39, respectively). Only 54.8% of patients (n = 23) were seen in the nephrology clinic after discharge. Patients who receive CKRT for AKI have a significant risk of CKD, while follow-up with a pediatric nephrologist in these high-risk patients is sub-optimal. A higher resolution version of the Graphical abstract is available as Supplementary information.

Variation in paediatric 24-h ambulatory blood pressure monitoring interpretation by Canadian and UK physicians.

Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is widely accepted as a more accurate method for measurement of blood pressure (BP) compared to a single office-based measurement of BP. However, it is unclear how physicians interpret ABPM and make management decisions. This study's goal is to investigate variation in ABPM interpretation among paediatric nephrologists (Canada and UK) and paediatric cardiologists (Canada only) via an online survey. The survey content included baseline demographics, questions on the use and indications for ABPM, interpretation of results, and subsequent management decisions in various clinical scenarios. The survey was sent to 196 Canadian physicians, with 69 (35.2%) total responses. Thirty-five UK clinicians also completed the survey. Most respondents were >44 years old, were in practice for at least 11 years, and were university-based. There were substantial differences among clinicians in ABPM interpretation for isolated systolic, diastolic, and night-time hypertension. For example, only 53.1% of physicians would initiate or modify treatment in those with diastolic HTN in CKD. Further, even for the same abnormal ABPM parameter, the decision to start or alter treatment was influenced by the underlying medical condition. There is significant variation in clinical practice among physicians for interpretation and management of hypertension when using ABPM. Differences in guidelines among various jurisdictions, as well as knowledge gaps in the research on which guidelines are based, create ambiguity regarding ABPM interpretation and management decisions. A more protocolized approach and further insight into the reasoning behind the variation in physicians' interpretation may help to standardise practice.

Burnout in Pediatric Nephrology Fellows and Faculty: Lessons From the Sustainable Pediatric Nephrology Workforce Project (SUPERPOWER).

Physician well-being is an important contributor to both job satisfaction and patient outcomes. Rates of burnout among physicians vary by specialty, ranging from 35 to 70%. Among pediatric residents, longitudinal data demonstrates consistent rates of burnout around 50-60%, although little is known about burnout among pediatric subspecialty fellows. Specifically, the degree of burnout among pediatric nephrologists remains unknown, as does the impact faculty burnout may have on trainee burnout. We sought to evaluate prevalence and predictors of burnout among US pediatric nephrology fellows and faculty, and assess for interactions between groups. In this multi-center pilot survey of all United States pediatric nephrology training programs from February to April 2020, burnout was assessed through abbreviated Maslach Burnout Inventory and predictors were explored through survey items devoted to demographic, personal characteristics, and job and career satisfaction questions. A total of 30/34 available fellows and 86/102 faculty from 11 institutions completed the survey (overall response rate 85%). The prevalence of burnout was 13% among fellows and 16% among faculty. Demographic (age, gender, year of training, faculty rank, marital status) and program factors (fellowship size, faculty size, current block/rotation, vacation or weekend off timing) were not significantly associated with burnout. Faculty and fellows with burnout reported significantly lower quality of life (5.3 vs. 7.9, p < 0.05), higher perceived stress (2.4 vs. 1.4, p < 0.05) and lower satisfaction with career choice (66 vs. 22%) and work life balance (28 vs. 0%), compared to those without burnout (p < 0.05 for all). Other important factors positively associated with burnout included lower institutional support for wellness programs and lower satisfaction with both colleague and faculty support. Larger studies are needed to explore if burnout is truly less prevalent among pediatric nephrology fellows and faculty compared to pediatric residents and graduate physicians. A larger sample size is also necessary to determine whether any interactions exist between the faculty and trainee roles in the developments of burnout. Future studies should also explore how to promote well-being through addressing key factors such as overall learning/working environment, stress reduction, and building personal resilience.

MO1031: Burnout Syndrome Among Paediatric Nephrologists—Report on its Prevalence, Severity and Predisposing Factors

Abstract BACKGROUND AND AIMS Burnout is an occupational-related syndrome comprising emotional exhaustion, depersonalization and reduced feelings of work-related personal accomplishments. Addressing and mitigating burnout is essential for avoiding high workforce turnover, patient dissatisfaction and a lower quality of care. There are some reports on burnout among adult nephrologists and general paediatricians, but little has been known about burnout among paediatric nephrologists. The aim of our study was to assess the prevalence and severity of burnout syndrome among Polish paediatric nephrologists. METHOD A 25-items online survey consisting of abbreviated Maslach Burnout Inventory and additional self-created questions about work-related factors was completed by 97 physicians affiliated with the Polish Society of Paediatric Nephrology. 75.3% of them were women, the median time of professional experience in the study group was 15 years. RESULTS High level of emotional exhaustion, depersonalization and reduced feeling of personal accomplishments was observed in 39.2%, 38.1% and 21.6% of participants, respectively. 26.8% of participants presented at least a medium level of burnout in all three dimensions and 8.2% of them presented high three-dimensional burnout. No associations of burnout intensity and gender, job seniority and the use of holiday leave were found. 41.2% of participants stated, that they would like to take part in burnout prevention and support programme. According to the study participants, excessive bureaucracy in healthcare systems, rush and working overtime were the main job-related problems that can influence burnout intensity. CONCLUSION Burnout is an important factor in the professional landscape of paediatric nephrology. Actions aiming at reducing the risk of occupational burnout among paediatric nephrologists should be applied both at personal and institutional levels.

MO1040: Idiopathic Nephrotic Syndrome After 10 Years of Age: Is Systematic Renal Biopsy Appropriate?

Abstract BACKGROUND AND AIMS Renal biopsy (RB) is recommended at diagnosis for children presenting with isolated nephrotic syndrome (NS) after the age of 10–12 years, unlike in younger children, because of a higher frequency of other diagnoses than MCD or FSGS. Recent reports, local practices and new serological biomarkers for membranous nephropathy now question this approach. Therefore, the objective of our study was to evaluate the causes of NS and indications for RB in a population-based cohort of adolescents with NS. METHOD We conducted a retrospective multicenter study, including children aged 10–18 years diagnosed with Idiopathic-like NS between December 2007 and June 2020 in 35 paediatric and 3 paediatric nephrology (PN) departments of the Paris area (NEPHROVIR network) and patients with Idiopathic-looking NS biopsied in the three PN departments. RESULTS A total of 103 children were included (54 males). The mean age at diagnosis was 13.4 ± 2 years. A renal biopsy was performed in 76 patients: at presentation because of atypical symptoms (such as hypertension, macroscopic haematuria, organic renal failure) in 15 or for no other reason than age in 39 and after 4 weeks for steroid resistance +/– atypies in 22 patients. Histological findings were MCD (n = 49), FSGS (n = 13), mesangial proliferation (n = 3), membranous nephropathy (n = 6) and IgAN (n = 5). CONCLUSION NS was secondary in only 11% of adolescents presenting with Idiopathic-like NS, and first-line treatment with oral prednisone was appropriate in all cases. In addition, more than 40% of RB could have been avoided in steroid-sensitive NS patients. Finally, we propose that RB should only be performed in case of atypical clinical or biological history or steroid resistance after 4 weeks, as in the younger population.

FC067: Long-Term Outcome of Childhood Onset Idiopathic Nephrotic Syndrome

Abstract BACKGROUND AND AIMS Little is known about the long-term outcome during adulthood of childhood onset idiopathic nephrotic syndrome (INS). We aimed to determine which patients require long-term follow up after transition, to identify risk factors of relapse and to analyze treatment strategies. METHOD In this monocentric retrospective study, we included all patients admitted in our adult nephrology department with INS diagnosed during childhood. Patients who reached kidney failure during childhood were excluded. Data regarding the outcome at adult age were obtained through clinical database and medical charts. RESULTS Eighty-two patients (male/female = 2/1) were included, with a median age at diagnosis of 3.9 years (1.2–16.5). Sixty-eight patients had steroid-sensitive INS, including 52 with steroid-dependent nephrotic syndrome or frequently relapsing nephrotic syndrome. Fourteen patients had steroid-resistant nephrotic syndrome. A total of 89% of patients received corticosteroid sparing treatment during childhood. Median follow-up during adulthood was 6.2 (0.3–25) years. A total of 71% of patients experienced at least one relapse during adulthood. The total number of relapses during childhood and the number of relapses per year during childhood, reflecting disease activity, were significantly higher in patients who experienced relapses during adulthood than in patients who did not (Figure 1). The risk of relapse during adulthood was also significantly associated with the need for immunosuppressive regimen at the time of the transition visit (P = 0.002). To promote the successful transition of young people, we propose to organize a transition visit where the adolescent/young adult is seen jointly by pediatric and adult nephrologists, as it was done for 68% of patients in this study. The relapse rate was significantly lower (50%) in the subgroup of patients who had such a transition visit. We also found that relapse during the first two-years of adulthood follow-up was significantly associated with the risk of further relapse, highlighting the need for a close follow-up during the transition period. A total of 45% of patients received corticosteroid sparing treatment during adulthood, mainly mycophenolate mofetil (N = 23), calcineurin inhibitors (N = 21) and rituximab (N = 12). The main complications were high blood pressure (20/82, 26%) and osteopenia (19/26, 73% when bone densitometry was performed). Only one thrombo-embolic event and three severe infections were reported. At last follow-up, median eGFR was 87.1 (23.4–150.8) mL/min/1.73 m². CONCLUSION The incidence of relapses in adulthood is high in patients with active disease during childhood. A long-term follow-up is mandatory in these patients. Whereas renal function remained normal in most patients, high blood pressure and osteopenia were frequent and should be carefully monitored during adulthood. Transition visit should be carefully coordinated to prevent the risk of nonadherence.

Management of IgA Nephropathy in Pediatric Patients

The onset of IgA nephritis in childhood and adolescence often develops into chronic glomerulonephritis with declining renal function. Although these long-term consequences are known, there is still a lack of evidence-based treatment recommendations in this age group. We report data from 22 pediatric patients who were biopsied to confirm the diagnosis of IgAN at our clinical center. 14 of them were treated with corticosteroids according to the recommendations for IgA nephritis vasculitis of the German Society of Pediatric Nephrology (GPN). Improvement was achieved in the majority of all cases, with a significant reduction in proteinuria five months after initiation of therapy. Our data suggest that treatment regimens for acute IgA nephritis and IgA vasculitis nephritis may be unified and are discussed in the context of current studies.

Ambulatory blood pressure monitoring in children during the COVID-19 pandemic.

The pandemic caused by severe acute respiratory syndrome corona virus-2 (SARS-CoV-2) had profound effects on healthcare delivery in the USA and abroad. Although ambulatory blood pressure monitoring (ABPM) is the recommended method for confirming hypertension (HTN) diagnosis and management, it is unclear how the pandemic affected ABPM utilization. We surveyed 81 pediatric nephrologists from 54 pediatric nephrology centers regarding their ABPM practices during the pandemic; 56.8% of providers continued to provide ABPM to their patients, but only 21% used disposable cuffs, and only 28.4% had specific equipment cleaning protocols in place. Only a minority of 81 practitioners felt comfortable (26.2%) or very comfortable (11.2%) in following published guidelines on ABPM during the pandemic, and 22.5% felt uncomfortable or very uncomfortable (7.5%). Additionally, only about half (49.4%) of practitioners were comfortable with managing HTN via telehealth. Our findings underscore the need to supplement existing and future guidance on how to manage HTN protocols, HTN patients, and equipment during healthcare crises.

Pediatric Mineral and Bone Disorder of Chronic Kidney Disease and Cardiovascular Disease.

Chronic kidney disease is common and causes significant morbidity including shortened lifespans and decrease in quality of life for patients. The major cause of mortality in chronic kidney disease is cardiovascular disease. Cardiovascular disease within the chronic kidney disease population is closely tied with disordered calcium and phosphorus metabolism and driven in part by renal bone disease. The complex nature of renal, bone, and cardiovascular diseases was renamed as mineral and bone disorder of chronic kidney disease to encompass how bone disease drives vascular calcification and contributes to the development of long-term cardiovascular disease, and recent data suggest that managing bone disease well can augment and improve cardiovascular disease status. Pediatric nephrologists have additional obstacles in optimal mineral and bone disorder of chronic kidney disease management such as linear growth and skeletal maturation. In this article, we will discuss cardiovascular and bone diseases in chronic kidney disease and end-stage kidney disease patients with a focus on pediatric issues and concerns.

Echocardiographic Abnormalities in Children with Chronic Kidney Disease on Maintenance Hemodialysis

Objective: To identify the echocardiographic abnormalities in children with chronic kidney disease on maintenance hemodialysis.&#x0D; Study Design: Prospective longitudinal study.&#x0D; Place and Duration of Study: Hemodialysis unit of Department of Pediatric Nephrology, The Children’s Hospital and Institute of Child Health, Lahore from Jun to Nov 2020.&#x0D; Methodology: A total of 75 children of chronic kidney disease on hemodialysis were included. Blood and urine tests, chest x-ray and transthoracic echo cardio-grapy were performed to assess the cardiac morphological and functional abnormalities. &#x0D; Results: The mean age of patients was 12.14 ± 2.18 years with 54 (72%) males and 21 (28%) females. The mean duration of hemodialysis was 22.54 ± 17.01 months with mean urea reduction ratio (URR) (%) 65.58 ± 4.31 and mean Kt/v 1.3 ± 0.1. Hypertension was noted in 70 (93.3%) patients with mean systolic and diastolic blood pressure 134.07 ± 10.64 and 89.13 ± 6.78 mmHg, respectively. The echocardiography revealed the mean ejection fraction of 58.02 ± 12.40 with mild systolic and diastolic dysfunction noted in 48 (64%) patients, pericardial effusion in 23 (30.7%) cases and left ventricular hypertrophy (LVH) in 52 (69.3%) cases.&#x0D; Conclusion: As the frequency of left ventricular hypertrophy (LVH) was noted to be high in our chronic kidney disease patients on hemodialysis, there is a need to implement echocardiographic screening of these patients at regular intervals to identify and rectify cardiac complications early.

Urologic Considerations in Pediatric Chronic Kidney Disease.

Common causes of pediatric ESRD are distinct from those seen in the adult population. In the pediatric population, the most common are congenital anomalies of the kidney and urinary tract (CAKUT), affecting approximately 30% of children with CKD. These structural anomalies often require coordinated care with the pediatric urology team to address voiding issues, bladder involvement, and the potential need for surgical intervention. For pediatric nephrologists and urologists, common CAKUT that are encountered include antenatal hydronephrosis, obstructive uropathies (eg, posterior urethral valves), and vesicoureteral reflux. As more pediatric patients with CAKUT, CKD, and ESRD transition to adult care, it is important for receiving adult nephrologists to understand the clinical presentation, natural history, and prognosis for these diagnoses. This review outlines the diagnosis and potential interventions for these conditions, including strategies to address bladder dysfunction that is often seen in children with CAKUT. A discussion of these management decisions (including surgical intervention) for CAKUT, which are quite common to pediatric nephrology and urology practices, may provide unique learning opportunities for adult nephrologists who lack familiarity with these pediatric conditions.

Therapeutic trials in difficult to treat steroid sensitive nephrotic syndrome: challenges and future directions.

Steroid sensitive nephrotic syndrome is a common condition in pediatric nephrology, and most children have excellent outcomes. Yet, 50% of children will require steroid-sparing agents due to frequently relapsing disease and may suffer consequences from steroid dependence or use of steroid-sparing agents. Several steroid-sparing therapeutic agents are available with few high quality randomized controlled trials to compare efficacy leading to reliance on observational data for clinical guidance. Reported trials focus on short-term outcomes such as time to first relapse, relapse rates up to 1–2 years of follow-up, and few have studied long-term remission. Trial designs often do not consider inter-individual variability, and differing response to treatments may occur due to heterogeneity in pathogenic mechanisms, and genetic and environmental influences. Strategies are proposed to improve the quantity and quality of trials in steroid sensitive nephrotic syndrome with integration of biomarkers, novel trial designs, and standardized outcomes, especially for long-term remission. Collaborative efforts among international trial networks will help move us toward a shared goal of finding a cure for children with nephrotic syndrome.

Paediatric Dialysis at a Tertiary Hospital in South-West Nigeria: A 4-Year Report

Introduction: Dialysis is potentially lifesaving in children with acute kidney injury (AKI) or chronic kidney disease (CKD), but availability is limited in low-income countries and lower-middle-income countries (LMICs). Methods: In the present study, we perform a 4-year study of patients who received peritoneal dialysis (PD) or haemodialysis (HD) at the Paediatric Nephrology Unit of the University College Hospital Ibadan, Nigeria. Subgroup analysis was performed on patients with sepsis or malaria AKI who underwent HD or PD for predictors of in-hospital mortality. Results: A total of 167 children aged 7 days to 18 years, median 7 (interquartile range 3–12) years, (60.5% males) were studied. In total, 129 (77.2%) had AKI, while 38 had CKD. Regarding AKI, 83 children (64.3%) received HD only, 42 underwent PD only, while 4 underwent both HD and PD. Malaria AKI was treated with HD in 43 (51.8%) or PD in 8 (10.5%), while sepsis AKI was treated with HD in 20 (21.4%) or PD in 33 (78.6%). Mortality in AKI was 16.3% overall, 10.8% in children on HD only, and 26.2% in children on PD only. Patients with sepsis AKI had higher mortality compared to patients with malaria AKI (RR 7.96 [1.70–37.37]). Subgroup analysis showed that age, diagnosis, and dialysis modality were not independent risk factors for mortality. The aetiology of CKD was glomerulonephritis in 26 (68.4%): treatment was HD in 36 and PD in 2 with mortality being 26.3%. Conclusions: PD for AKI showed relatively good outcomes in a LMIC. However, funding and support for a formal dialysis program for the management of AKI and CKD are needed.

Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35months (0.4-231months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year's follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF (-) stones than in MRF ( +) stones. However, remission rate with medical treatment was higher in cases with MRF ( +) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.

Streptococcal toxic shock syndrome in an adolescent

A special form of streptococcal infection is streptococcal toxic shock syndrome (STS), characterized by rapid development of symptoms and high mortality. Patient O., 14 years old, was taken to the infectious diseases department of OGAUZ DBNo. 1 by the SMP team with complaints of shortness of breath, vomiting, loose stools in a state of moderate severity due to intoxication syndrome. Diagnosis upon admission: Acute infectious gastroenteritis of moderate severity. Acute respiratory infections rhinopharyngitis, acute bronchitis, pneumonia (?), DN1. During examination in the UAC, anemia, leukocytosis, acceleration of ESR, in the biochemical blood analysis – an increase in CRP, in the coagulogram — increased INR, APTT, RFMC, decreased PTI, in urine tests – protein, erythrocytes, on the X–ray — bilateral pleural effusion, in the tank. sputum culture — Streptoccocus oralis 10/3 KOE/ml, PCR SARS-CoV-2: negative, blood test for antistreptolysin-O (ASL-O): 800 IU/ml (norm up to 200 IU/ml), blood for sterility 19.05.20: no bacterial microflora growth was detected. After receiving laboratory data, the diagnosis was made: Acute glomerulonephritis?, Аcute intestinal infection. Double-sided hydrothorax. Internal combustion engine. Anemia of the 1st degree. The final diagnosis: Acute post-streptococcal glomerulonephritis with a debut in the form of streptococcal toxic shock syndrome, a period of extensive clinical and laboratory changes, with a decrease in the debut of kidney function in the form of acute renal failure, recovery period. Against the background of the treatment (2 courses of antibiotic therapy (cefotaxime, amoxicillin), infusion therapy, pulse therapy with metipred (5 pulses), double transfusion of freshly frozen plasma, prednisone, lasix, veroshpiron, enap, curantil, heparin, and other accompanying therapy), pronounced positive clinical and laboratory dynamics was noted. She was hospitalized for 43 days, of which 9 days were in the intensive care unit (5 days on a ventilator). On the 44th day, the child was discharged in a satisfactory condition with recommendations under the supervision of a pediatrician, a pediatric nephrologist at the place of residence

The Use of Patient-Reported Outcome Measures in Daily Clinical Practice of a Pediatric Nephrology Department.

(1) Background: Health-related quality of life (HRQoL) is lower in patients with chronic kidney disease (CKD) compared to the general population. In 2011, the KLIK PROM portal was implemented in the Emma Children’s Hospital to monitor and discuss HRQoL in daily care. This study describes and assesses the implementation and use of the KLIK PROM portal in the pediatric nephrology department. (2) Methods: CKD patients (self-report, if 8–18 years of age) and their parents (proxy-report, if 1–8 years) were invited to complete HRQoL patient-reported outcome measures (PROMs): TNO-AZL Preschool children Quality Of Life (TAPQOL) or Pediatric Quality of Life Inventory for Children (PedsQL). The PROMs were completed before and discussed during outpatient consultations. The adaptation rate—the proportion of patients/parents who were invited and completed at least one PROM—was calculated. Reported HRQoL scores of CKD patients were compared to the general population. (3) Results: In total, 142 patients (proxy- and self-report) were invited, 112 patients completed at least one PROM (adaptation rate 79%). Patients (n = 84 with informed consent for scientific use) with CKD reported lower HRQoL and HRQoL was more often impaired compared to the general Dutch population. (4) Conclusions: The implementation of KLIK was successful and its use is feasible for daily care. Using KLIK, HRQoL problems can be easily identified and monitored.

Evaluation of Etiological Causes in Children with Symptomatic Hematuria

Hematuria is an important and common symptom of urinary system pathologies in children, and careful evaluation is required for its definitive diagnosis. In this study, we aimed to determine the demographic and clinical characteristics and etiological causes of patients who applied to our pediatric nephrology clinic with hematuria. In this study, the record of 434 patients who were followed up in our clinic for macroscopic and/or microscopic hematuria were evaluated retrospectively. Demographic data, clinical findings, laboratory and imaging examinations and final diagnosis of the patients were recorded. Out of 434 patients with hematuria, 239 (55%) were male and 195 (45%) were female. Of the patients, 291 (67%) had macroscopic hematuria and 83 (19.1%) had glomerular hematuria. Non-glomerular causes were found in 80.9%, with a significantly higher rate. Most of the causes of non-glomerular hematuria were urinary tract infection and renal stones. Acute poststreptococcal glomerulonephritis (APSGN) was found to be responsible for the majority of glomerular diseases. This study shows that most of the pathologies that may cause hematuria can be detected with a detailed history, careful physical examination and simple laboratory tests.

Working Towards a Treat-to-Target Protocol in Juvenile Proliferative Lupus Nephritis - A Survey of Pediatric Rheumatologists and Nephrologists in Germany and Austria.

BackgroundTo describe treatment practices for juvenile proliferative lupus nephritis (LN) class III and IV of pediatric rheumatologists and nephrologists in Germany and Austria in preparation for a treat-to-target treatment protocol in LN.MethodsSurvey study by members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Pediatric Nephrology (GPN) on diagnostics and (concomitant) therapy of LN.ResultsFifty-eight physicians completed the survey. Overall, there was a considerable heterogeneity regarding the suggested diagnostics and management of juvenile proliferative LN. Increased urinary protein excretion, either assessed by 24 h urine collection or spot urine (protein-creatinine ratio), and reduced estimated glomerular filtration rate were specified as important parameters for indication of kidney biopsy to diagnose proliferative LN and monitoring of therapy. Corticosteroids were generally proposed for induction and maintenance therapy, most often in conjunction with either mycophenolate mofetil (MMF) or cyclophosphamide (CP) as steroid-sparing immunosuppressants. MMF was clearly preferred over CP for induction therapy of LN class III, whereas CP and MMF were equally proposed for LN class IV. MMF was most often recommended for maintenance therapy in conjunction with oral corticosteroids and continued for at least 3 years and 1 year, respectively, after remission. Hydroxychloroquine was widely accepted as a concomitant measure followed by renin-angiotensin system inhibitors in cases of arterial hypertension and/or proteinuria.ConclusionThe majority of pediatric rheumatologists and nephrologists in Germany and Austria propose the use of corticosteroids, most often in combination with either MMF or CP, for treatment of proliferative LN in children. The considerable heterogeneity of responses supports the need for a treat-to-target protocol for juvenile proliferative LN between pediatric rheumatologists and nephrologists.

Percutaneous ultrasound-guided renal biopsies in a paediatric population: comparison of coaxial and non-coaxial techniques using 18-gauge core biopsy needles.

Percutaneous ultrasound-guided biopsy is performed in paediatric patients for evaluation of diffuse renal parenchymal disease. When compared with the non-coaxial technique, the coaxial technique has the advantages of obtaining multiple tissue cores via a single capsular puncture and post-biopsy tract embolisation. To compare the coaxial and non-coaxial techniques of percutaneous ultrasound (US)-guided biopsy of native kidney parenchyma in children and adolescents with renal disease. We retrospectively identified consecutive patients who underwent percutaneous US-guided renal biopsy using an 18-gauge core biopsy needle from July 2019 to July 2021 in a single tertiary paediatric nephrology centre. Focal renal tumour biopsy and transplant kidney biopsy were excluded. The total glomerular yield, specimen adequacy, complication rate and procedural time between the coaxial and non-coaxial groups were compared. There were 34 percutaneous US-guided renal biopsies: 22 using a coaxial technique and 12 using a non-coaxial technique. The total median glomerular yield obtained was higher in the coaxial group (coaxial=37.9; non-coaxial=22.2; P=0.02). No statistically significant difference was noted between specimen adequacy (coaxial=100%; non-coaxial=91.7%; P=0.35). While no statistically significant difference was detected for overall complication rates (coaxial=13.6%; non-coaxial=41.7%; P=0.09), the coaxial group had a lower rate of haemorrhagic complications (coaxial=4.5%; non-coaxial=41.7%; P=0.01). One patient in the non-coaxial group had post-biopsy haemorrhage requiring embolisation. The procedural time was shorter in the coaxial group (coaxial=26.3 ± 7.0min; non-coaxial=51.3 ± 11.5min; P&lt;0.001). Percutaneous US-guided renal biopsy in children using the coaxial technique has significantly higher total glomerular yield, shorter procedural time and fewer haemorrhagic complications, compared to biopsies using the non-coaxial technique.