Pediatric Nephrology Research Articles

Fibronectin Glomerulopathy Without Typical Renal Biopsy Features in a 4-Year-Old Girl with Incidentally Discovered Proteinuria and a G417V FN1 Gene Mutation

Fibronectin glomerulopathy (FG) is caused by fibronectin 1 (FN1) gene mutations. A renal biopsy was performed on a 4-year-old girl with incidentally discovered proteinuria (150 mg/dL); her family history of renal disease was negative. Markedly enlarged glomeruli (mean glomerular diameter: 196 μm; age-matched controls: 140 μm), α-SMA-positive and Ki-67-positive mesangial cell proliferation (glomerular proliferation index 1.76), the mild expansion of mesangial areas, no immune or electron-dense deposits, normal glomerular basement membrane, and diffusely effaced foot processes were observed. Genetic testing identified a de novo heterozygous mutation (Gly417Val) in the collagen-binding site of the FN II-2 domain, prompting fibronectin immunostaining. Strong mesangial positivity was noted, hence FG was diagnosed. The follow-up period of 29 months revealed nephrotic range proteinuria, intermittent microhematuria, glomerular hyperfiltration, and preserved renal function. The biopsy features of early childhood-onset FG were compared to a case of FG with a lobular pattern diagnosed in a 44-year-old patient with undulating proteinuria, microhematuria, hypertension known for a year, and a positive family history. Early childhood-onset FG was characterized by glomerular enlargement, mesangial proliferation, and no changes that suggested fibronectin deposition disease. In summary, the novel aspects of the case were that the mutation was located at the collagen-binding site of the FN1 gene, not identified earlier, and the histologic spectrum of FG was expanded by the observed mesangial proliferative pattern and striking glomerulomegaly. Now, FG should also be considered among the monogenic causes of proteinuric kidney diseases in pediatric nephrology practice.

Engaging Students in a Dialysis Unit: A Pilot Study.

Seeking to provide early paediatric nephrology exposure to medical students in the United States, we implemented the Kids In Dialysis, Nephrology Exposure and Education (KIDNEE) club. This club served as an educational intervention in which preclinical medical students were paired with paediatric dialysis patients, as patient buddies. Students were recruited for involvement in the club through the medical school Paediatric Interest Group. For the 2022-2023 academic year, seven first-year students were paired with seven paediatric dialysis patients. Students met with their patient match weekly to play games, watch movies and to act as a friend. The evaluation aimed at assessing the feasibility and acceptability of this intervention as well as influence on student interest in paediatric chronic disease and paediatric nephrology. We developed and distributed surveys to patients, unit staff, and students after programme implementation. From October 2022 to April 2023, medical students collectively spent ~173 h in the dialysis unit. Staff and patients/families unanimously reported that they would recommend the KIDNEE club to other families. Students objectively reported an increased interest in paediatric nephrology and chronic disease and subjectively reported an increased understanding of the patient experience. The KIDNEE club pilot was both feasible and acceptable for patients/families, staff and students. It holds the potential to increase student interest in the field of paediatric nephrology. As our results are limited by small sample size given the pilot nature of the programme, future studies are needed to assess programme expansion and longitudinal influence on students' career paths.

Therapeutic Plasma Exchange in Pediatrics: An Overview from the Pediatric Nephrologists’ Perspective

Therapeutic Plasma Exchange in Pediatrics: An Overview from the Pediatric Nephrologists’ Perspective

Effects of higher dietary acid load: a narrative review with special emphasis in children.

Metabolic effects of high diet acid load (DAL) have been studied for years in adults, although only recently in children. Contemporary diets, especially those of Western societies, owe their acidogenic effect to high animal-origin protein content and low contribution of base-forming elements, such as fruits and vegetables. This imbalance, where dietary acid precursors exceed the body's buffering capacity, results in an acid-retaining state known by terms such as "eubicarbonatemic metabolic acidosis," "low-grade metabolic acidosis," "subclinical acidosis," or "acid stress". Its consequences have been linked to chronic systemic inflammation, contributing to various noncommunicable diseases traditionally considered more common in adulthood, but now have been recognized to originate at much earlier ages. In children, effects of high DAL are not limited to growth impairment caused by alterations of bone and muscle metabolism, but also represent a risk factor for conditions such as obesity, insulin resistance, diabetes, hypertension, urolithiasis, and chronic kidney disease (CKD). The possibility that high DAL may be a cause of chronic acid-retaining states in children with growth impairment should alert pediatricians and pediatric nephrologists, since its causes have been attributed traditionally to inborn errors of metabolism and renal pathologies such as CKD and renal tubular acidosis. The interplay between DAL, overall diet quality, and its cascading effects on children's health necessitates comprehensive nutritional assessments and interventions. This narrative review explores the clinical relevance of diet-induced acid retention in children and highlights the potential for prevention through dietary modifications, particularly by increasing fruit and vegetable intake alongside appropriate protein consumption.

Associations of dietary choline intake and kidney function with hyperuricemia in Chinese children and adolescents: a cross-sectional study

Limited studies have suggested an effect of dietary choline intake on uric acid levels. We aim to investigate the associations between choline intake and hyperuricemia (HUA), as well as the mediating role of kidney function in this relationship, among the Chinese population aged 6-17 years. Participants were divided into quartiles according to residual energy-adjusted dietary choline intake in our cross-sectional study. Dietary choline intake was assessed using the 24-h dietary recalls method over three consecutive days, including two weekdays and one weekend day. The primary outcome was the HUA prevalence. Based on recommendation in Clinical Paediatric Nephrology (3rd ed), HUA is defined based on fasting serum uric acid levels, with cutoffs varying by age and sex. The associations between choline intake and HUA were analysed using weighted logistic regression models, restricted cubic spline models, and linear regression models. The mediated proportions of estimated glomerular filtration rate (eGFR) in the associations were estimated with mediation effect models. The data for this study were collected from the China National Nutrition and Health Surveillance of Children and Lactating Mothers (2016-2017) conducted between October 2016 and December 2018. Eligible participants were identified through a database search conducted from October to December2023. Among the 10749 participants, 3398 (31.6%) individuals were found to have HUA. A negative dose-dependent relationship was found between dietary choline intake and HUA. Compared to participants in the lowest intake quartile of total choline, phosphatidylcholine, and betaine, those in the 4th quartile had lower odds of HUA, with odds ratio (OR) of 0.75 (95% confidence interval [95% CI], 0.63-0.90), 0.75 (95% CI, 0.64-0.89), and 0.75 (95% CI, 0.59-0.94), respectively. The eGFR mediated 10.60%-14.58% of the associations. Participants in the 4th quartile of lipid-soluble dietary choline exhibited 24.00% reduced odds of HUA compared to those in the lowest intake quartile, with an OR of 0.76 (95% CI, 0.64-0.90). Moderate to high intake of dietary choline (181.20-357.92mg/d), particularly phosphatidylcholine (120.22-207.58mg/d), and betaine (189.24-282.37mg/d), may reduce the odds of HUA by improving glomerular filtration function. Further interventional studies are needed to establish causal relationships. This work was supported by the National Natural Science Foundation of China (82003443, 42375180), the Natural Science Foundation of Guangdong Province of China (2024A1515012088), and the Construction of High-level University of Guangdong (G624330422).

IPNA clinical practice recommendations on care of pediatric patients with pre-existing kidney disease during seasonal outbreak of COVID-19.

The coronavirus disease 2019 (COVID-19) pandemic, instigated by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has profoundly impacted healthcare infrastructures around the globe. While children are usually asymptomatic or have mild symptoms, children with pre-existing kidney conditions require specialized attention. This pivotal report, championed by the International Pediatric Nephrology Association (IPNA), delivers precise and actionable recommendations tailored for pediatric patients with kidney ailments in this pandemic landscape. Central to our findings are rigorous infection control protocols. These are particularly stringent in high-risk zones, emphasizing telehealth's indispensable role, the significance of curtailing in-person consultations, and the imperative of following rigorous guidelines in regions with heightened COVID-19 prevalence. Additionally, the report delves into vaccination approaches for children with kidney issues, highlighting that the choice of vaccine is often governed by regional accessibility and policy frameworks, rather than a universal preference. A notable observation is the potential correlation between COVID-19 vaccines and specific kidney disorders. However, establishing a direct causal link remains elusive. In summary, our research accentuates the critical need for specialized pediatric kidney care during global health crises and reaffirms the continuous research imperative, especially regarding vaccination ramifications.

Sociodemographic, Clinical & Biochemical Features in Children with Nephrotic Syndrome: A Study in a Tertiary Care Hospital of Bangladesh

Background: The estimated incidence of nephrotic syndrome is between 2-7 per 100,000 children worldwide, with higher rates reported among those with African and South Asian ancestry. There is a significant gap in the literature on the role of socio-demographic modifiers of nephrotic syndrome in children in the long term. This study aimed to evaluate the sociodemographic & clinical features in children with nephrotic syndrome. Methods: This descriptive study was conducted at the Department of Pediatric Nephrology in the National Institute of Kidney Diseases & Urology (NIKDU), Sher-E-Bangla Nagar, Dhaka, from November 2019 to June 2021. Children of age group 1-12 years with primary NS enrolled in this study and a total of 108 patients were included. Informed written consent was taken from the parents. Ethical clearance was obtained from the institutional ethical committee. A purposive sampling technique was used in this study. On entry into the study, a detailed history was taken & proper physical examinations were done. Routine investigations such as complete blood count, CRP, serum albumin, serum cholesterol, serum creatinine, Mantoux test, screening tests for hepatitis-B & hepatitis-C, urine RME and culture sensitivity, X-ray chest, and serum C3 level were done. All investigation was done from NIKDU except the MT test that was done from Dhaka Shishu Hospital. Infection was screened out and appropriate measures were taken. The data were collected and preserved in a case record form (CRF). The collected data were analyzed using Statistical Package for Social Sciences (SPSS) software, version 23.0. Results: The majority of the patients (50, 46.29%) were 5 to 8 years old, followed by (40, 37.03%) 1-4 years old. Mean ±SD 5.42±2.67. Most of the patients (73, 67.6%) were male and the rest (35, 32.4%) were female, the majority (71%) belonged to rural areas. Regarding socioeconomic status, the majority of the patients (80, 74.08%) belonged to low-income families. .......

Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.

Childhood idiopathic nephrotic syndrome is an important pediatric kidney disease associated with significant morbidities and even mortality. Several guidelines have been developed to standardize the terminology and patient care among the pediatric nephrology community. Since the publication of these guidelines, there have been major breakthroughs in the disease management and the understanding of underlying pathogenesis through multi-omics investigations, including the identification of anti-nephrin autoantibodies, genetic susceptibility loci, and the pathogenic role of B cell subsets. In this educational review, we summarize the recent major advancements in idiopathic nephrotic syndrome and attempt to provide potential therapeutic approaches in both steroid-sensitive and steroid-resistant nephrotic syndrome that may shape future guideline development.

Living with a new kidney from the perspective of adolescents: A mandala-supported qualitative study.

Despite advances in transplant procedures, children and adolescents still face some challenges post-transplant and are at high risk for psychiatric, academic, and social problems. This study aims to explore the lived experiences of adolescent kidney transplant recipients through interviews and the use of mandala art therapy. This study adopted a descriptive phenomenological design and thematic analysis approach based on Husserl's philosophy. The sample included kidney transplant recipients aged 12-18 years admitted to the pediatric nephrology polyclinic of a university hospital in southern Türkiye. Data were collected through in-depth interviews conducted during a mandala art activity with 14 adolescent kidney transplant recipients. The qualitative data were analyzed using Colaizzi's seven-stage qualitative analysis method, which is suitable to the descriptive phenomenological design employed in the present study. Four themes and seven subthemes emerged from the data related to the problems and experiences of adolescents with kidney transplantation and growing up with a new kidney. (1) Education, (2) Ideas about the kidney; including the sub-themes "What the kidney transplant process gave them, Feelings about their kidneys", (3) Family; including the sub-themes "Relationship with the donor, Relationship with siblings", (4) Social environment; including the sub-themes "Experiences with hospital, Relationship with friends and Desire for friendship with a transplanted Peer". Mandala art therapy-assisted interviews are an effective communication method for adolescent kidney transplant recipients to express their feelings and thoughts and to look at life from a broader perspective. Understanding the life experiences of adolescents with kidney transplantation and the difficulties they face may enable better and more systematic planning of the care to be given to them.

Efficacy of intermittent peritoneal dialysis in renal causes of acute kidney injury in children

Background: In low-resource settings like Bangladesh, intermittent peritoneal dialysis (IPD) has been identified as the preferred modality for the management of AKI. PD is a safe, simple and inexpensive procedure and has been used in pediatric AKI patients. However, its effectiveness in treatment of renal AKI warrants further exploration. To evaluate the efficacy of IPD in treating pediatric renal AKI patients. Method: This prospective and interventional study conducted in the Department of Paediatric Nephrology at Bangladesh Shishu Hospital and Institute from January 2020 to December 2021. Children aged 1 month to 12 years of either sexes with renal AKI stages 2 or 3 who required PD were included. Each patient had IPD for 72 hours. Every day, the clinical and laboratory markers were measured; on the third day, or 72 hours later, the results were compared. Results: Majority of patients were from 1-5 years age group and predominantly male. Following 3 days of IPD, clinical parameters (tachycardia, tachypnea and edema) improved significantly. Oedema found in 23 patients before PD, however, 5 patients got a relief from it (p=0.016) after PD. The urea reduction ratio in renal AKI patients increased from 19.3 to 40.7 ml/kg/hr days, with urinary output increasing from 0.02 to 0.50 ml/kg/hr at day 3 (p<0.001). After PD, there was a significant decrease in creatinine, urea, and potassium levels (p<0.001). Besides, a noteworthy improvement of 40.7%, 36.5% and 47.7% were observed in creatinine, urea and bicarbonate levels respectively. Conclusion: IPD is well-effective in treating renal AKI in children by observing a greater improvement in clinical and laboratory parameters in the patients.

Swiss Consensus on Prenatal and Early Postnatal Urinary Tract Dilation: Practical Approach and When to Refer.

Urinary tract dilations (UTDs) are the most frequent prenatal renal anomaly. The spectrum of etiologies causing UTD ranges from mild spontaneously resolving obstruction to severe upper and lower urinary tract obstruction or reflux. The early recognition and management of these anomalies allows for improved renal endowment prenatally and ultimately better outcome for the child. The role of the general obstetrician and pediatrician is to recognize potential prenatal and postnatal cases addressed to their practice and to refer patients to specialized pediatric nephrology and urology centers with a sense of the urgency of such a referral. The aim of this paper is to offer clinical recommendations to clinicians regarding the management of neonates and children born with prenatally detected UTD, based on a consensus between Swiss pediatric nephrology centers. The aim is to give suggestions and recommendations based on the currently available literature regarding classifications and definitions of prenatal and postnatal UTD, etiologies, prenatal and postnatal renal function evaluation, investigations, antibiotic prophylaxis, and the need for referral to a pediatric nephrologist and/or urologist. The overarching goal of a systematic approach to UTD is to ultimately optimize kidney health during childhood and improve long-term renal function prognosis.

Diagnostic Value of Urinary CD80 in Typical and Atypical Nephrotic Syndrome

Background: Cluster of differentiation 80 is one of the important biomarker which is present on podocytes. Urinary CD80 concentration increases only in patients with typical nephrotic syndrome in active state and level remains normal in patients with atypical nephrotic syndrome. Objective: To evaluate urinary CD80 concentration as a diagnostic marker of typical and atypical nephrotic syndrome. Methods: This was a cross sectional study, performed in the Department of Paediatrics & Paediatric Nephrology, Chittagong Medical College Hospital, Chattogram. Thirty nine with typical and 36 with atypical nephrotic syndrome were enrolled from January 2020 to July 2021.Urinary CD80 concentration was measured and compared between typical and atypical nephrotic syndrome. Results: Mean urinary CD80 concentration in typical nephrotic syndrome (671.92 ±328.45) was found significantly higher (p<0.001) than atypical nephrotic syndrome patients (122.30 ±158.47). The area under the curve for typical nephrotic syndrome was 0.946 for urinary CD80 concentration (95% confidence interval: 0.899–0.992) (p<0.001). Urinary CD80 concentration 114.9 ng/g of creatinine was found most appropriate cut-off value with the sensitivity 92.3% and the specificity 27.8% for the differentiation of typical (active state) and atypical nephrotic syndrome. Conclusion: Urinary CD80 level found significantly higher in patients with typical nephrotic syndrome than atypical nephrotic syndrome. Along with the other diagnostic criteria, urinary CD80 may be used as a non-invasive biomarker to differentiate typical and atypical nephrotic syndrome. Bangladesh J Child Health 2023; Vol 47 (2) : 78-83

Pediatric Nephrology Workforce and Access of Children with Kidney Failure to Transplantation in the United States.

Nephrology is one of the pediatric subspecialties with the largest workforce shortage in the US. Waitlist registration is one of the first steps towards kidney transplantation and is facilitated by pediatric nephrologists. The objective of this study was to determine whether state-level density of pediatric nephrologists is associated with access to waitlisting (primary outcome) or kidney transplantation (secondary outcome) in children with kidney failure. Using Cox proportional hazards and logistic regression analyses, we studied children <18 years who developed kidney failure between 2016-2020 according to the US Renal Data System, the national kidney failure registry. The density of pediatric nephrologists (determined by the count of pediatric nephrologists per 100,000 children in each state) was estimated using workforce data from the American Board of Pediatrics and categorized into three groups: > 1, 0.5-1, and <0.5. We included 4,497 children, of whom 3,198 (71%) were waitlisted and 2,691 (60%) received transplantation. Children residing in states with pediatric nephrologist density >1 had 33% (HR 1.33; 95%CI 1.07-1.66) and 22% (HR 1.22; 95%CI 1.02-1.45) better access to waitlisting compared to those residing in states with <0.5 pediatric nephrologist density (reference group) in unadjusted and adjusted analysis, respectively. Pediatric nephrologist density was particularly important for the odds of preemptive waitlisting (adjusted OR 1.56; 95% CI 1.02-2.41). The adjusted HR was 1.25 (95% CI 1.00-1.55, p=0.046) for deceased donor transplantation and 1.24 (95% CI 0.85-1.82) for living donor transplantation for children residing in states with pediatric nephrologist density > 1 compared to the reference group. Children residing in states with higher pediatric nephrologist density had better access to waitlist registration, especially preemptively, and deceased donor transplantation.

How dialysis frequency and duration impact uremic toxin and fluid removal: a pediatric perspective.

Three-weekly 4-h hemodialysis/hemodiafiltration (HD/HDF) per week has become the "standard HD/HDF" regimen in children across the globe, although increasingly criticized, since crucial determinants such as residual kidney function and patient preferences are not considered. As a consequence, several children fail to achieve adequate dialysis while on a "standard HD/HDF." In these circumstances, an extended dialysis prescription such as short daily (2-3h/session, 5-7days a week) or nocturnal HD/HDF (6-9h/session, 3-5days a week), either at home or in a dialysis center, may be considered. The purpose of this educational review is to summarize the impact of dialysis duration and frequency on uremic toxin and fluid removal. Moreover, we aim to summarize the existing literature on HD/HDF strategies with extended dialysis duration and/or increased frequency (> 12h dialysis time per week) in pediatrics. Dialysis duration and frequency plays a crucial role in uremic toxin removal, in particular for uremic toxins with retarded transport in patients, such as phosphate, β2-microglobulin (β2m), and protein-bound uremic toxins. Also, increasing dialysis duration and/or frequency decreases the gap between plasma refilling and ultrafiltration volume), thereby decreasing the need for a high ultrafiltration rate. Observational studies in children demonstrate a beneficial effect of extended dialysis regimens (i.e., more frequent or longer duration) on blood pressure control, left ventricular hypertrophy, growth, and quality of life. PTH levels tend to decrease in the majority of studies, while hypocalcemia or suppressed PTH levels were also reported. Dietary restrictions were decreased or stopped, along with tapering of phosphate binders and potassium chelators. Extended HD/HDF regimens are beneficial in a particular group of children. Pediatric-specific international guidelines are needed to support pediatric nephrologists in determining for which children extended HD regimens are beneficial, along with increasing efforts to decrease the financial, organizational, and psychosocial barriers that are present in extended HD/HDF.

A multicenter study investigating the genetic analysis of childhood steroid-resistant nephrotic syndrome: Variants in COL4A5 may not be coincidental.

This study aimed to discuss the pathogenic hereditary factors of children with steroid-resistant nephrotic syndrome (SRNS) in Guangxi, China. We recruited 89 patients with SRNS or infantile NS from five major pediatric nephrology centers in Guangxi, and conducted a retrospective analysis of clinical data. Whole-exome sequencing analysis was also performed on all patients. The risk of progression to chronic kidney disease (CKD) was assessed using the Kaplan-Meier method and Cox proportional hazards model. The study included 69 male and 20 female participants from 86 distinct families, with the median age of disease onset being 48 months (interquartile range: 24-93). Overall, 24.7% had a family history of SRNS, whereas 13.5% exhibited extra-kidney manifestations. We identified disease-causing variants in 24.7% (22/89) of patients across eight screened genes. The most frequently detected variant was found in COL4A5, followed by NPHS2 (5.6%), NPHS1 (2.2%), PAX2 (2.2%), WT1 (1.1%), LMX1B (1.1%), NUP105 (1.1%), and COL4A6 (1.1%). Twelve of the 26 pathogenic variants were determined to be de novo. Based on gene detection results, pathogenic variants were categorized into two groups: identified and unidentified variants. The identified variant group demonstrated a significant association with positive family history, steroid resistant-style, and response to immune therapy (P<0.001). Patients with the identified genetic variant were approximately ten times more likely to develop CKD (P<0.001) than those in the unidentified group at the last follow-up. Kidney biopsy was performed on 66 patients, and minimal change disease was the most prevalent histopathological diagnosis (29 cases; 32.6%). These findings suggest that children diagnosed with SRNS exhibit a diverse range of genetic alterations. We identified the COL4A5 variant as the predominant genetic abnormality and a low frequency of NPHS1 gene involvement in these children. Gene variants may serve as an independent predictor for SRNS progression to CKD.

Diagnosis, management and treatment of the Alport syndrome - 2024 guideline on behalf of ERKNet, ERA and ESPN.

Glomerular nephropathy resulting from the genetic defects in COL4A3/4/5 genes including the classical Alport syndrome (AS) is the second commonest hereditary kidney disease characterized by persistent haematuria progressing to the need of kidney replacement therapy, frequently associated with sensorineural deafness, and occasionally with ocular anomalies. Diagnosis and management of COL4A3/4/5 glomerulopathy is a great challenge due to its phenotypic heterogeneity, multiple modes of inheritance, variable expressivity, and disease penetrance of individual variants as well as imperfect prognostic and progression factors and scarce and limited clinical trials, especially in children. As a joint initiative of the European Rare Kidney disease reference Network (ERKNet), European Renal Association (ERA Genes&Kidney) and European Society for Paediatric Nephrology (ESPN) Working Group Hereditary Kidney Disorders, a team of experts including adult and paediatric nephrologists, kidney geneticists, audiologists, ophthalmologists and a kidney pathologist were selected to perform a systematic literature review on 21 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions. The experts formulated recommendations and formally graded them at a consensus meeting with input from patient representatives and a voting panel of nephrologists representing all regions of the world. Genetic diagnostics comprising joint analysis of COL4A3/4/5 genes is the key diagnostic test already during the initial evaluation of an individual presenting with persistent haematuria, proteinuria, kidney failure of unknown origin, focal segmental sclerosis of unknown origin and possibly cystic kidney disease. Early renin-angiotensin system blockade is the standard of care therapy; sodium-glucose cotransporter-2 inhibitors may be added in adults with proteinuria and chronic kidney disease. Relatives with heterozygous COL4A3/4/5 variants should only be considered as the last possible resource for living kidney donation. This guideline provides guidance for the diagnosis and management of individuals with pathogenic variants in COL4A3/4/5 genes.

Prognosis and risk factors of IgA vasculitis nephritis in children

Objective: To investigate the prognosis and risk factors of IgA vasculitis nephritis (IgAVN) in children. Methods: A retrospective cohort study was conducted. Clinical data were collected from 264 children who were pathologically diagnosed with IgAVN at Department of Pediatric Nephrology, Tongji Hospital, affiliated with Tongji Medical College, Huazhong University of Science and Technology, between January 2011 and December 2017. All patients had a follow-up period of more than 3 years. Clinical characteristics, renal pathology, 3-year and 5-year prognosis were analyzed. The patients were grouped based on gender, age of onset (≤6 years, >6-9 years, and >9 years), pathological classification (≤Ⅲ and>Ⅲ),whether the prognosis was complete remission at 3 and 5 years. Independent sample t-tests, ANOVA or chi-squared test were used for intergroup comparisons. Spearman correlation analysis was applied for ordinal data, and multivariate Logistic regression was used to analyze factors affecting the prognosis. Receiver operating characteristic (ROC) curve was utilized to evaluate the predictive value of these factors. Results: Of the 264 children with IgAVN, 153 were male and 111 were female, the age of onset was 8.3 (6.7, 10.3) years, 118 patients (45%) with onset age >6-9 years accounted for the highest proportion. All patients presented with skin purpura and renal involvement, primarily manifesting as hematuria and/or proteinuria. Microscopic hematuria was observed in 253 patients (95.8%), while 246 patients (93.2%) showed proteinuria. In 256 patients (97.0%), hematuria or proteinuria urinalysis was detected within 6 months of skin purpura onset, and 243 patients (92.0%) underwent renal biopsy within 6 months of renal involvement. The most common clinical subtype in 264 IgAVN children was hematuria and proteinuria (204 cases, 77.3%), with grade Ⅲ being the predominant pathological classification (181 cases, 68.6%). Among children ≤6 years old, the 3-year complete remission rate was higher in males than in females (83.9% (26/31) vs. 7/16, χ²=8.12, P=0.012). Factors independently associated with poor 5-year prognosis included time from hematuria or proteinuria urinalysis to renal biopsy >6 months, elevated serum cholesterol levels, and incomplete remission 3 years post-biopsy (OR=5.41, 1.39, 6.02, 95%CI 1.40-20.86, 1.04-1.84, 2.61-13.88, all P<0.05). The serum cholesterol has a predictive value for 5-year prognosis (P=0.020, AUC=0.62, 95%CI 0.52-0.71, Youden index=0.27, cutoff=4.37). Conclusions: For children with IgAVN aged≤6 years, the 3-year prognosis is better in males than in females. Time from hematuria or proteinuria urinalysis to renal biopsy >6 months, elevated serum cholesterol levels, and incomplete remission at 3 years post-biopsy may be independent risk factors for poor 5-year prognosis in children with IgAVN.

URINARY TRACT INFECTIONS IN CHILDREN: A REVIEW OF CURRENT INTERNATIONAL GUIDELINES

Urinary tract infections (UTIs) are the most common bacterial infections in children, which can lead to renal dysfunction, especially in young children in case of complicated and recurrent course. The aim of this review was to analyze all the latest clinical guidelines on UTIs in children in the world, starting from 2018 to 2024. We analyzed current data on the incidence of UTIs in children depending on age and gender. A classification is presented, the main problems of diagnosis, the most modern approaches to treatment and prevention are discussed. Analysis of these guidelines indicates that UTIs should be diagnosed in all young children with fever over 38 ° C without a focus of infection. Empirical antibacterial therapy should be started within the first 24-48 hours, oral antibiotics have an advantage, third-generation cephalosporins are more often used today, but it is necessary to take into account the data on antibacterial resistance in your region. All modern clinical guidelines emphasize the growth of resistant pathogens and their decreasing sensitivity to protected penicillins. All guidelines recommend identifying a "high-risk recurrence group" that includes children with vesicoureteral reflux (VUR), neurogenic bladder dysfunction, constipation, and "uncircumcised" boys under 1 year of age, and timely conducting imaging diagnostics for such children. According to almost all international guidelines for the diagnosis and treatment of UTIs in children, long-term antibacterial prophylaxis should be carried out only according to strict indications, mainly in children with high VUR and low VUR, taking into account the "risk" factors. In recent years, a large number of modern international clinical guidelines on UTIs in children have appeared. This review, which includes all the latest guidelines on UTIs in children in the world over the past seven years, will allow the use of accumulated modern knowledge in the practical work of pediatricians, pediatric nephrologists, and pediatric urologists in the Russian Federation.

C3 glomerulopathy in children: a European longitudinal study evaluating outcome.

C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce. Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6months, 12months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3mg/mg with normal eGFR. Lack of remission was defined as non-response. A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8years and of 78% at 7years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR. Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.

Clinical and bacteriological profile and antibiotic sensitivity pattern in spontaneous bacterial peritonitis among children with idiopathic nephrotic syndrome

Background: Spontaneous bacterial peritonitis is a serious complication of childhood nephrotic syndrome (NS). A precise knowledge of organism causing peritonitis is important primarily to treat infection and decrease morbidity, prevent antibiotic resistance and finally to reduce mortality. Objectives were to observe the clinical and bacteriological profile and antibiotic sensitivity pattern of peritonitis in childhood NS. Methods: This cross-sectional observational study was conducted in pediatric nephrology department of Bangabandhu Sheikh Mujib medical university (BSMMU). The 44 diagnosed patient of spontaneous bacterial peritonitis among childhood idiopathic NS were enrolled as cases. The peritoneal fluid was obtained following standard procedure and examined for gross appearance, cytogical and biochemical analysis, microscopic examination with gram stain and peritoneal fluid culture along with antibiotic sensitivity. Results: All patients had complaints of abdominal pain followed by fever, vomiting, lethargy and anorexia. All cases had edema and ascites followed by abdominal tenderness, abdominal wall rigidity, rebound tenderness and cellulitis in areas other than abdominal wall. Peritoneal fluid showed neutrophilic pleocytosis and exudative fluid. We observed 27.27% gram + cocci, 6.82% gram-bacilli in gram stain examination. Here, only 6.9% cases showed positive growth including 2.3% E. coli, 2.3% Pseudomonas, 2.3% Acinetobacter and remaining 93.1% cases showed no growth. We also described the antibiotic sensitivity pattern in this study. Conclusions: In our study, patients predominantly had abdominal pain, fever, edema, ascites and abdominal tenderness. We found only 6.9% cases showed positive growth which included E. coli, Pseudomonas and Acinetobacter and demonstrated the antibiotic sensitivity and resistance pattern for these organisms.