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IMPORTANCE: Pediatric patients with complex medical problems benefit from pediatric sub-specialty care; however, a significant proportion of children live greater than 80 mi. away from pediatric sub-specialty care. OBJECTIVE: To identify current knowledge gaps and outline concrete next steps to make progress on issues that have persistently challenged the pediatric nephrology workforce. EVIDENCE REVIEW: Workforce Summit 2.0 employed the round table format and methodology for consensus building using adapted Delphi principles. Content domains were identified via input from the ASPN Workforce Committee, the ASPNs 2023 Strategic Plan survey, the ASPNs Pediatric Nephrology Division Directors survey, and ongoing feedback from ASPN members. Working groups met prior to the Summit to conduct an organized literature review and establish key questions to be addressed. The Summit was held in-person in November 2023. During the Summit, work groups presented their preliminary findings, and the at-large group developed the key action statements and future directions. FINDINGS: A holistic appraisal of the effort required to cover inpatient and outpatient sub-specialty care will help define faculty effort and time distribution. Most pediatric nephrologists practice in academic settings, so work beyond clinical care including education, research, advocacy, and administrative/service tasks may form a substantial amount of a faculty members time and effort. An academic relative value unit (RVU) may assist in creating a more inclusive assessment of their contributions to their academic practice. Pediatric sub-specialties, such as nephrology, contribute to the clinical mission and care of their institutions beyond their direct billable RVUs. Advocacy throughout the field of pediatrics is necessary in order for reimbursement of pediatric sub-specialist care to accurately reflect the time and effort required to address complex care needs. Flexible, individualized training pathways may improve recruitment into sub-specialty fields such as nephrology. CONCLUSIONS AND RELEVANCE: The workforce crisis facing the pediatric nephrology field is echoed throughout many pediatric sub-specialties. Efforts to improve recruitment, retention, and reimbursement are necessary to improve the care delivered to pediatric patients.

Background: We aimed to evaluate the baseline performance and improvement of ChatGPT-4 "omni" (ChatGPT-4o) and Gemini 1.5 Flash (Gemini 1.5) in answering multiple-choice questions related to pediatric nephrology after specific training. Methods: Using questions from the "Educational Review" articles published by Pediatric Nephrology between January 2014 and April 2024, the models were tested both before and after specific training with Portable Data Format (PDF) and text (TXT) file formats of the Educational Review articles removing the last page containing the correct answers using a Python script. The number of correct answers was recorded. Results: Before training, ChatGPT-4o correctly answered 75.2% of the 1395 questions, outperforming Gemini 1.5, which answered 64.9% correctly (p < 0.001). After training with PDF files, ChatGPT-4o's accuracy increased to 77.8%, while Gemini 1.5 improved significantly to 84.7% (p < 0.001). Training with TXT files showed similar results, with ChatGPT-4o maintaining 77.8% accuracy and Gemini 1.5 further improving to 87.6% (p < 0.001). Conclusions: The study highlights that while ChatGPT-4o has strong baseline performance, specific training does not significantly enhance its accuracy. Conversely, Gemini 1.5, despite its lower initial performance, shows substantial improvement with training, particularly with TXT files. These findings suggest Gemini 1.5's superior ability to store and retrieve information, making it potentially more effective in clinical applications, albeit with a dependency on additional data for optimal performance. [ABSTRACT FROM AUTHOR]

Background: Kidney transplantation is the gold standard treatment for end-stage kidney disease in children. Rare genetic systemic diseases associated with cystic kidney disease such as COL4A1-related disorder and oral facial digital syndrome type 1 could contribute to end-stage kidney disease in the pediatric population but there is scarce evidence in the literature regarding kidney transplant outcomes in these cases. Case presentation: We report a case of a 5-year-old male with COL4A1-related disorder who received a living-related donor kidney transplant from his mother. To our knowledge this is the first reported kidney transplant in a pediatric recipient with COL4A1-related disorder. We also present a case of a 16-year-old female with oral facial digital syndrome type 1 who received a deceased donor kidney transplant. Conclusions: In this case series, we discuss surgical technique, indication for kidney transplant, influence of comorbidities and intellectual disability in transplant outcomes. [ABSTRACT FROM AUTHOR]

Background: Renal diseases are frequently seen in the pediatric emergency department (ED), and usually, they are associated with morbidity and mortality if not early detected and properly managed. While acute kidney injury (AKI) can result from intrinsic kidney disease, many acute (nonrenal) conditions may be associated with AKI. In addition, children with chronic kidney disease (CKD) may also present to the ED with acute symptoms or complications. Aim: To evaluate patients presenting to the ED with renal affection, their etiology, management, and outcome. Patients and methods: This is a prospective descriptive study on 100 patients presented to Cairo University Pediatric ED with renal affection, from Jan to June 2023. Clinical data were collected including presenting manifestations, vital signs, clinical assessment, laboratory and radiological investigations, and also their plan of management or interventions needed and their outcome. Results: Seventy-three percent of the patients had underlying CKD, and they presented mostly with acute complications as urinary tract infections (20%), severe anemia (25%), disturbed conscious level (10%), convulsions (12%), and hypertensive emergency (18%). Of the AKI patients (27%), 18% had underlying renal diseases, and 9% had multisystem affection. All of our patients received conservative management, 74 patients were admitted at the hospital, 24 patients needed ICU admission, 14 patients did peritoneal dialysis, 6 did hemodialysis, and only 1 patient needed continuous renal replacement therapy. Mortality was observed in only 2% of our patients. Conclusion: Patients with renal conditions either AKI or CKD present to the ED with various acute conditions. Proper identification and provision of required services is important for a satisfactory outcome. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Pediatric chronic kidney disease (CKD) requires reliable biomarkers for early detection and monitoring. Neutrophil gelatinase-associated lipocalin (NGAL) has emerged as a potential marker due to its responsiveness to renal impairment and involvement in mineral metabolism. Objectives: To evaluate serum NGAL levels in pediatric CKD patients and explore correlations with estimated glomerular filtration rate (eGFR), ferritin, calcium-phosphorus (Ca*P) product, and total serum protein. Methods: A cross-sectional study included 54 pediatric CKD patients and 29 healthy controls. Laboratory assessments encompassed serum NGAL, creatinine, ferritin, calcium, phosphorus, and total serum protein. eGFR was calculated using the Schwartz formula. Pearson correlation and linear regression analyses determined associations between NGAL and other parameters. Results: NGAL levels were significantly higher in CKD patients compared to controls (median 453 ng/mL vs. 78 ng/mL, p < 0.001). A strong negative correlation existed between NGAL and eGFR (r = –0.81, p < 0.001). NGAL showed moderate positive correlations with ferritin (r = 0.56, p = 0.009) and CaP product (r = 0.57, p = 0.006) and a moderate negative correlation with total serum protein (r = –0.36, p = 0.225). Regression analysis confirmed NGAL as a significant predictor of eGFR, ferritin, and CaP product. Conclusions: Elevated serum NGAL levels are associated with decreased renal function and alterations in mineral metabolism in pediatric CKD patients. NGAL may serve as a valuable biomarker for assessing disease progression and guiding clinical management in this population. [ABSTRACT FROM AUTHOR]

Background: Percutaneous ultrasound-guided renal biopsy (PRB) is a key element for diagnosis and management of several renal pathologies. We aimed to lay out the experience of our pediatric nephrology unit performing PRBs. The rationale and findings of these biopsies, safety issues and considerations of the extracted data are going to be analyzed. Methods: A retrospective study was conducted from 2008 to 2023 based on the review of the medical records of pediatric patients who underwent PRBs. In total, 216 kidney biopsies in 206 patients were performed: 115 (53.2%) during the 2008–2015 period and 101 (46.8%) during the 2016–2023 period. Results: The most frequent clinical indication for PRBs was nephritic syndrome followed by nephrotic syndrome, observed in 84 (40.8%) and 72 (34.9%) patients, respectively. The predominant diagnosis was minimal change disease (MCD) (23.3%), followed by focal segmental glomerulosclerosis (FSGS) (15%) equal to lupus nephritis (LN) (15%), and immunoglobulin A nepropathy (10.2%). Minor complications, such as subcapsular hematomas were observed in approximately 15% of patients while no therapeutic intervention was needed. Conclusions: This report is the first review of pathohistological data covering a pediatric population over a 15-year period in Greece and one of the largest in southeastern Europe, especially in the Balkan region. The main indication for a PRB was nephritic syndrome; however, MCD was the main histological diagnosis. This study emphasis the fact that PRBs constitute a safe and reliable method of diagnostic approach to kidney diseases in childhood and offers important information on therapeutic approaches as well as the prognosis of these patients. [ABSTRACT FROM AUTHOR]

A 9‐month‐old male presented with vomiting and dehydration with mild hypernatremia in the context of failure to thrive. He was later diagnosed with nephrogenic diabetes insipidus (NDI) during this hospitalisation and was also found to have eosinophilic esophagitis (EoE). He has since been growing well after EoE and NDI were properly managed. Molecular genetic testing revealed an unreported deletion in AQP2 which was deemed pathogenic and of autosomal dominant inheritance when correlated with his clinical findings and family history. This case report describes the clinical course of this patient in comparison to his family members and reviews current literature on autosomal dominant NDI caused by AQP2 mutations. [ABSTRACT FROM AUTHOR]

The relatively rare proximal 17q12 microdeletion, including the deletion of the HNF1B gene, is associated with renal cysts and diabetes syndrome (RCAD). This genomic rearrangement results in a wide range of phenotypes, including renal cysts and diabetes, which are consistent with maturity-onset diabetes of the young type 5 (MODY5), Mullerian aplasia/dysgenesis, autism spectrum disorder and schizophrenia, speech delay, learning difficulties, transient neonatal hypercalcemia, and neonatal cholestasis. We describe a girl with a 17q12 microdeletion identified using CGH array analysis (about 1.4 Mb, including HNF1B and LHX1 genes). The same deletion was identified in her mother. The proband had shown cystic and hypodysplastic bilateral kidneys since birth and hypertension, while her mother had bilateral renal cysts and diabetes. Despite suggestive findings in the girl and in the mother, no clinical suspicion arose, and genetic testing was carried out only after referral to a pediatric nephrologist. In children, the identification of 17q12 microdeletion may have a significant impact on the diagnosis, prognosis, and management of renal disease and early-onset type II diabetes. This family with a 17q12 microdeletion confirms intrafamilial phenotypic variability and highlights the importance of including it early on in the analysis of the diagnostic workup of children with renal cystic diseases. [ABSTRACT FROM AUTHOR]

Background: Acute urinary tract infection (UTI) is a common disease in pediatrics, with around 8% of girls and 2% of boys experiencing a UTI by age 7y/o. UTIs can range from asymptomatic bacteriuria to acute pyelonephritis (APN) in severe cases involving renal parenchymal infection. UTI patients admitted to the pediatric ward usually have more severe clinical presentation, compared to those treated in the outpatient settings. Therefore, it will be helpful to have markers that predict the severity of the disease and the likelihood of having APN. Methods: We performed a retrospective review on all pediatric UTI/APN patients treated in the inpatient setting at a Medical Center from October 2012 to September 2022. Patients were assigned to the "hyponatremia" or "eunatremia" group according to their serum sodium concentrations. Detailed information, including renal echo, blood, and urine test results, were collected for the analysis of multivariable logistic regression model. Results: The study included 344 patients, of which 99 (28.8%) had hyponatremia, and 245 (71.2%) had normal serum sodium levels. The hyponatremia group had higher APN frequency, renal echo abnormality, and higher CRP level. In multivariable analysis, hyponatremia was independently associated with increased serum glucose (OR: 1.01, 95% CI: 1.00-1.03, p = 0.0365) and CRP levels (OR: 1.00, 95% CI: 1.00-1.01, p = 0.0417), without a significant increase in APN frequency as the final diagnosis. Conclusions: Our findings suggest that hyponatremia in pediatric UTI patients may indicate a more severe disease, such as APNs, higher CRP levels, or renal echo abnormalities. The complex mechanisms underlying hyponatremia and its predictive value for disease severity warrant further investigation. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease is associated with several complications with higher prevalence and intensity at lower glomerular filtration rate. Albuminuria is the traditional biomarker of kidney damage. Although albuminuria can contribute to renal function decline, only a few studies have been published on whether albuminuria is associated with concurrent complications of chronic kidney disease. The aim of this work was to identify the relation between albuminuria and some of concurrent complications of chronic kidney disease among children. Methods: A cross-sectional study included fifty children with chronic kidney disease stages 2–5. Measurement of blood pressure, hemoglobin, serum bicarbonate, calcium, phosphorus, albumin, and lipid profile was done. Urinary albumin/creatinine ratio was determined. The relation between albuminuria and some of the complications of chronic kidney disease among children was studied. Results: The study included 50 patients aged 3–14 years. Dyslipidemia was present in 62% of the patients, acidosis in 56%, anemia in 38%, hyperphosphatemia in 34%, hypertension in 28%, and hypocalcemia in 22%. Eighty-eight percent had albuminuria, 26% had microalbuminuria, and 62% had macroalbuminuria. A statistically significant relation was found between the degree of albuminuria and presence of anemia and hyperphosphatemia. But no significant relation was found between the degree of albuminuria and presence of hypertension, dyslipidemia, acidosis, or hypocalcemia. Conclusion: There is a significant relation between the degree of albuminuria and presence of anemia and hyperphosphatemia among children with chronic kidney disease. Key message: What is already known? Albuminuria is a biomarker of kidney damage and it can contribute to renal function decline. What this study adds? There is a relation between albuminuria and some of CKD complications among children. [ABSTRACT FROM AUTHOR]

Introduction: Children with chronic kidney disease (CKD) require a specialized diet to manage their condition and support healthy growth and development. A proper diet for children with CKD aims to control the intake of certain nutrients, such as protein, lipid, calcium, phosphorus, potassium, sodium, and vitamin D, while ensuring they receive adequate nutrition for growth and development. Aim of the study: This article discusses the epidemiology, causes and pathomechanism of CKD in children. It also presents existing clinical trials and dietary recommendations from societies such as Kidney Disease: Improving Global Outcomes (KDIGO) 2024, Kidney Disease Outcomes Quality Initiative (KDOQI) and Pediatric Renal Nutrition Taskforce (PRNT) and identifies potential sources of selected macronutrients and micronutrients in children's diets. Materials and methods: Comprehensive literature searches were performed across the main electronic databases of PubMed, Google Scholar, KDIGO2024, KDQQI and PRNT recommendations for studies published in the English language about dietary recommendations for children with CKD. Results: The approach to nutrition for children with CKD is still evolving, and specific macronutrient and micronutrient ratios should be established based on the clinical condition, the child's age, and body weight. Care should be taken to ensure that the child's diet is varied, and a healthy eating style should be promoted. [ABSTRACT FROM AUTHOR]

Background/Objectives: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing Escherichia coli -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012—2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. Methods: A total of 436 patients (301 with STEC-HUS and 135 with aHUS) were included. Results: The incidence of STEC-HUS increased during the observation period, with a mean of 3.9 cases per million age-related population (marp). The incidence of aHUS was relatively constant with a mean of 1.8/marp. The majority of patients fully recovered, although kidney sequelae were observed at 5-year follow-ups in 31% of children with STEC-HUS, 57% of aHUS subjects in the pre-eculizumab era, and 37% of aHUS subjects who had received eculizumab. The overall mortality rate was 2% for STEC-HUS and 3.7% for aHUS, with no deaths reported in children on eculizumab and mortality mainly attributed to neurological damage. A decreasing incidence of chronic kidney disease stage 5 (CKD5) due to HUS was observed. Conclusions: Despite an unchanging incidence of aHUS and an increasing incidence of STEC-HUS, the kidney outcomes of both diseases have improved significantly over the last 12 years. Mortality from HUS has dropped due to improved symptomatic treatment and the introduction of anti-C5 therapy. The development of CKD5 in childhood as a consequence of HUS has become exceptional. [ABSTRACT FROM AUTHOR]

Kidney transplantation in children with end‐stage kidney disease significantly enhances survival and quality of life but poses unique challenges related to chronic immunosuppressive therapy. In fact, despite being essential for preventing organ rejection, immunosuppressive therapy can have significant side effects specific to pediatric patients, such as adverse impacts on physiological growth, puberty, and fertility. The resulting short stature and delayed or incomplete pubertal development can profoundly affect young patients' psychological and social well‐being, impacting self‐esteem and overall quality of life, and may significantly hamper compliance and therapeutic adherence. Most studies on immunosuppression in pediatric kidney transplant recipients focus on general side effects and outcomes like long‐term graft survival or acute complications. On the other side, there is limited evidence in the current literature on the specific issues of growth, puberty, and fertility in this patient population. In this pragmatic review, we aimed to summarize the most relevant information available on these critical aspects of post‐transplant management in pediatric patients, also providing some practical indications on management strategies for minimizing these often neglected but still important complications. [ABSTRACT FROM AUTHOR]

To construct and verify an easy-to-use nomogram for predicting the risk of infectious diseases in pediatric kidney transplant recipients. Clinical data of hospitalized pediatric kidney transplant recipients were retrospectively analyzed. Meaningful variables identified from the multivariate stepwise logistic regression analysis were used to construct the nomogram. Internal validation was performed using Bootstrap resampling 1,000 times. The nomogram was evaluated using calibration, decision and receiver operating characteristic (ROC) curves. A total of 297 pediatric kidney transplant recipients were included (164 infected, 133 non-infected). Multivariate stepwise regression analysis identified white blood cell count (WBC), lymphocyte to monocyte ratio (MLR), platelet to neutrophil ratio (PNR), red cell distribution width-standard deviation (RDW-SD), and albumin (ALB) as significant predictors of postoperative infection. The nomogram, based on the five indicators, showed strong discrimination ability (AUC = 0.756; 95% CI [0.702–0.811]), with a sensitivity of 88.0% and a specificity of 54.3%. The calibration curve and decision curve further demonstrated good consistency and clinical practicality between the predicted and actual values. WBC, MLR, PNR, RDW-SD, and ALB are effective indicators for predicting postoperative infection in pediatric kidney transplant recipients. The nomogram constructed from these indicators can effectively predict and evaluate the early risk of infection in these patients. [ABSTRACT FROM AUTHOR]

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Background and Objectives: This research explores the correlation between estimated glomerular filtration rates (eGFR) obtained using the bedside Schwartz equation, and renal scintigraphy in children with congenital kidney and urinary tract abnormalities (CAKUT). The objective is to enhance understanding and management of renal health in this demographic by analyzing kidney size-function relationships. Methods: A retrospective observational analysis was performed on 94 pediatric CAKUT patients at the "Louis Turcanu" Emergency Hospital for Children, Timisoara. Kidney function data, extracted from medical records, were evaluated using the Schwartz equation, renal scintigraphy, and the gold standard iohexol clearance. Ethical approval was secured for the study, which employed descriptive and inferential statistical methods, including t-tests and correlation coefficients, to compare eGFR values. Results: Significant variances were found in eGFRs across different body surface area (BSA) percentiles. For instance, the eGFR for the right kidney in the 25th–50th BSA percentile (102.02 ± 41.52 mL/min/BSA) was notably higher than that of the left (35.60 ± 26.05 mL/min/BSA; p = 0.01). The overall sample reflected a higher eGFR in the right kidney (76.03 ± 40.91 mL/min/BSA) compared to the left (57.46 ± 35.91 mL/min/BSA; p = 0.02). Additionally, a strong positive Pearson correlation (r = 0.80, p = 0.02) was found between scintigraphy and ultrasound measures in the 50th–75th percentiles for left renal percentiles, demonstrating consistent patterns across different evaluations of kidney function. Conclusions: This comparison indicates a complex relationship between eGFR values and kidney size, suggesting potential inaccuracies in standard bedside eGFR measurements for pediatric CAKUT patients. The findings underscore the necessity for accurate diagnostic tools specifically designed for pediatric applications and advocate for the integration of multiple diagnostic techniques to improve clinical management. [ABSTRACT FROM AUTHOR]

Background: Children with chronic kidney disease (CKD) experience a lot of mental and emotional stress, which can lead to the development of depressive disorders. The prevalence of depressive disorders in CKD children is estimated to be between 7% and 35%. Objectives. The aim of our study was to analyze the prevalence and characteristics of depression and depressive symptoms in children and adolescents with CKD treated conservatively. Materials and methods: The cross-sectional, multicenter study was conducted in 73 CKD children aged 8-18 and in 92 of their parents. To assess the mental wellbeing of CKD children, Kovacs's Children's Depression Inventory 2 (CDI2) was used as CDI2: Self-Report and CDI2: Parent Form. Results: The majority of CKD children acquired medium scores in CDI2, 11% of participants reported symptoms suggesting depressive disorder, and among them 8.2% met the criteria for depression. A significant relationship was found between age and interpersonal problems, age at CKD diagnosis, and total score and ineffectiveness, CKD duration and total score/emotional problems. Depressive symptoms were associated with the stage of CKD, and they differed significantly between stages III and IV. We noticed the child-parent disagreement on reported depressive symptoms. Parents perceive their children's mental state as worse than the children themselves. Conclusions: There is a problem of depression in children with CKD treated conservatively. Variables associated with depressive symptoms in CKD children treated conservatively require further study. Key factors predisposing to the development of depression seem to be age at the time of diagnosis, disease duration, and progression of CKD from stage III to IV. Disparities between depressive symptoms self-reported by CKD children and their parents' assessment require further analysis. However, these disparaties indicate that the final diagnosis of the occurrence of depressive disorders should be based on a multidimensional assessment of the patient's situation. [ABSTRACT FROM AUTHOR]

Background: Pediatric chronic kidney disease (CKD) requires reliable biomarkers for early detection and monitoring. Neutrophil gelatinase-associated lipocalin (NGAL) has emerged as a potential marker due to its responsiveness to renal impairment and involvement in mineral metabolism. Objectives: To evaluate serum NGAL levels in pediatric CKD patients and explore correlations with estimated glomerular filtration rate (eGFR), ferritin, calcium-phosphorus (Ca*P) product, and total serum protein. Methods: A cross-sectional study included 54 pediatric CKD patients and 29 healthy controls. Laboratory assessments encompassed serum NGAL, creatinine, ferritin, calcium, phosphorus, and total serum protein. eGFR was calculated using the Schwartz formula. Pearson correlation and linear regression analyses determined associations between NGAL and other parameters. Results: NGAL levels were significantly higher in CKD patients compared to controls (median 453 ng/mL vs. 78 ng/mL, p < 0.001). A strong negative correlation existed between NGAL and eGFR (r = –0.81, p < 0.001). NGAL showed moderate positive correlations with ferritin (r = 0.56, p = 0.009) and CaP product (r = 0.57, p = 0.006) and a moderate negative correlation with total serum protein (r = –0.36, p = 0.225). Regression analysis confirmed NGAL as a significant predictor of eGFR, ferritin, and CaP product. Conclusions: Elevated serum NGAL levels are associated with decreased renal function and alterations in mineral metabolism in pediatric CKD patients. NGAL may serve as a valuable biomarker for assessing disease progression and guiding clinical management in this population.

Abstract Background Acute urinary tract infection (UTI) is a common disease in pediatrics, with around 8% of girls and 2% of boys experiencing a UTI by age 7y/o. UTIs can range from asymptomatic bacteriuria to acute pyelonephritis (APN) in severe cases involving renal parenchymal infection. UTI patients admitted to the pediatric ward usually have more severe clinical presentation, compared to those treated in the outpatient settings. Therefore, it will be helpful to have markers that predict the severity of the disease and the likelihood of having APN. Methods We performed a retrospective review on all pediatric UTI/APN patients treated in the inpatient setting at a Medical Center from October 2012 to September 2022. Patients were assigned to the “hyponatremia” or “eunatremia” group according to their serum sodium concentrations. Detailed information, including renal echo, blood, and urine test results, were collected for the analysis of multivariable logistic regression model. Results The study included 344 patients, of which 99 (28.8%) had hyponatremia, and 245 (71.2%) had normal serum sodium levels. The hyponatremia group had higher APN frequency, renal echo abnormality, and higher CRP level. In multivariable analysis, hyponatremia was independently associated with increased serum glucose (OR: 1.01, 95% CI: 1.00-1.03, p = 0.0365) and CRP levels (OR: 1.00, 95% CI: 1.00-1.01, p = 0.0417), without a significant increase in APN frequency as the final diagnosis. Conclusions Our findings suggest that hyponatremia in pediatric UTI patients may indicate a more severe disease, such as APNs, higher CRP levels, or renal echo abnormalities. The complex mechanisms underlying hyponatremia and its predictive value for disease severity warrant further investigation.

The relatively rare proximal 17q12 microdeletion, including the deletion of the HNF1B gene, is associated with renal cysts and diabetes syndrome (RCAD). This genomic rearrangement results in a wide range of phenotypes, including renal cysts and diabetes, which are consistent with maturity-onset diabetes of the young type 5 (MODY5), Mullerian aplasia/dysgenesis, autism spectrum disorder and schizophrenia, speech delay, learning difficulties, transient neonatal hypercalcemia, and neonatal cholestasis. We describe a girl with a 17q12 microdeletion identified using CGH array analysis (about 1.4 Mb, including HNF1B and LHX1 genes). The same deletion was identified in her mother. The proband had shown cystic and hypodysplastic bilateral kidneys since birth and hypertension, while her mother had bilateral renal cysts and diabetes. Despite suggestive findings in the girl and in the mother, no clinical suspicion arose, and genetic testing was carried out only after referral to a pediatric nephrologist. In children, the identification of 17q12 microdeletion may have a significant impact on the diagnosis, prognosis, and management of renal disease and early-onset type II diabetes. This family with a 17q12 microdeletion confirms intrafamilial phenotypic variability and highlights the importance of including it early on in the analysis of the diagnostic workup of children with renal cystic diseases.

Introduction: The competencies in the Medical Specialty Curriculum and Standards Development System (MSCSD) guides trainers in the preparation and execution of the training program and gives learners an idea about the state of mastery that they will reach by graduation. The aim of this study was to evaluate the knowledge of pediatric nephrologists about competencies in the field of specialization. Materials and Methods: In the cross-sectional study conducted between January 2023 and 2024, the knowledge of pediatric nephrologists about specialty competencies was investigated with the two-round Delphi technique. Digital Delphi forms were sent to all pediatric nephrologists in Türkiye via email of the specialty society. In the Delphi form, all competency titles in the MSCSD v.2.3 curriculum were transformed into propositions that could be scored with a 5-point Likert scale. The frequency of 4-5, the difference between quartiles and the median were calculated for each of the statements. For participant consensus on the propositions, the median should be 4, the frequency of 4-5 should be above 70%, and the difference between quartiles should be 2.5 or less. Results: Of the pediatric nephrologists in Türkiye, 59 responded to the first and 136 to the second round Delphi survey. In the first round, full consensus was found in the competency areas other than service delivery, while consensus was found in two of the 72 clinical competencies under the service delivery heading, namely systemic diseases and malignant diseases, and in two of the 26 interventional competencies (diversion and imaging). The analysis of the second round Delphi questionnaire based on this result showed full consensus on all competency headings. The variables of title, gender and training clinic made a difference in consensus. Conclusion: It was found that the level of knowledge of pediatric nephrologists about specialty competencies differed in terms of some variables and knowledge was more limited in young learners for whom competencies are legally binding. In conclusion, it is recommended that learners should be informed about the competencies at the beginning of specialty training and participate in updating the competency list.

Background: The growing body of research on kidney disease in children has identified a broad spectrum of genetic etiologies. Methods: We conducted a prospective study to evaluate the efficacy of an optimized genetic test and subclinical changes in a real-world context before kidney transplantation. All cases involved recipients under the age of 18 who underwent whole exome sequencing (ES) between 2013 and 2022. Results: The study population included 244 children, with a median age of 13.1 years at transplantation. ES provided a molecular genetic diagnosis in 114 (46.7%) probands with monogenic variants in 15 known disease-causing genes. ES confirmed the suspected clinical diagnosis in 74/244 (30.3%) cases and revised the pre-exome clinical diagnoses in 40/244 (16.4%) cases. ES also established a specific underlying cause for kidney failure for 19 patients who had previously had an unknown etiology. Genetic diagnosis influenced clinical management in 88 recipients (36.1%), facilitated genetic counseling for 18 families (7.4%), and enabled comprehensive assessment of living donor candidates in 35 cases (14.3%). Conclusions: Genetic diagnosis provides critical insights into the pathogenesis of kidney disease, optimizes clinical strategies concerning risk assessment of living donors, and enhances disease surveillance of recipients. [ABSTRACT FROM AUTHOR]

Background: Hematopoietic stem cell transplantation (HSCT) is the standard therapy for many disorders, however long-term complications post-HSCT in pediatrics remain a significant concern, where a pre-existent subclinical kidney damage, the use of nephrotoxic medications, or consequences of primary disease carry the hazard of acute kidney injury (AKI) and chronic kidney disease (CKD), which could be devastating complications, therefore identification of risk factors, prompt diagnosis, and treatment of CKD is vital for secure HSCT. So, we aimed to assess the kidney function to detect the development of CKD in our pediatric patient, using different formulas to measure the estimated glomerular filtration rate (eGFR). Patients and methods: A prospective cross-sectional study was conducted at the Bone Marrow Transplantation Unit, Faculty of Medicine, Ain Shams University, Cairo, Egypt, where we included all available HSCT long-term survivors, who underwent HSCT during the period between 2011 and 2018. A detailed medical history and complications were collected from patient's medical records, while clinical examination with measurement of serum creatinine, and Cystatin C (CysC) were done to determine eGFR, and hence CKD, using different methods; bedside Schwartz and Cockcroft Gault formulas (according to the age), serum CysC, and Chronic Kidney Disease in Children (CKiD) Creatinine-Cystatin C-based CKiD equation (CKiD-eGFR CysC formula). Results: We included 23 pediatric HSCT survivors, with a mean (±SD) age of 14.35 (±5.27) years. Most of our patients were diagnosed with aplastic anemia (43.5%) and beta-thalassemia major (26.1%), where HSCT, 87% was allogeneic, 4.3% cord blood; meanwhile 8.7% was autologous. The most common reported complications were AKI (56.5%), and acute Graft-versus-host disease (43.5%), meanwhile, CKD was reported in 4/23 (17.4%) according to serum creatinine bases formulas, and one (4.3%) patient according to serum cystatin C, and two (8.6%) patients based on CKiD-eGFR CysC formula. CKD was linked to the conditioning regimen by Cyclophosphamide and antithymocyte globulin, the use of vancomycin and aminoglycoside, and the history of AKI. Conclusion: CKD is not uncommon complications post-transplantation, and is strongly correlated to the previous conditioning regimen, antimicrobials, and history of AKI. The accuracy and early diagnosis of CKD necessitated the use of combined equations of eGFR calculation. CKD Controllable measures are needed to prevent renal insult in children post-transplantation. [ABSTRACT FROM AUTHOR]

Background: Cardiovascular disease (CVD) remains the primary cause of mortality for patients with early-onset chronic kidney disease (CKD), despite improvements in subject survival over the past several decades. Additionally, there is a dearth of research on CVD in children. Objectives: This research aimed to study the electrocardiographic abnormalities and Holter findings in children with predialytic CKD. Subjects and methods: A cross-sectional study that was conducted on 50 children with predialytic CKD through the period from November, 2022 to November, 2023. Data were collected from children through personal interviews with their parents or care givers and assessment of their anthropometric measures, laboratory investigations, echocardiography, ECG and holter study. Results: CKD stage 5 showed the highest percentage of abnormalities in Echocardiography and ECG results, which showed a statistically significant relationship to the stages of CKD. Left ventricular hypertrophy was the main echocardiographic abnormality while prolonged QTc duration and sinus tachycardia were the main ECG abnormalities. There was a statistically significant variation between CKD stages as regards heart rate variability parameters, with CKD stage 5 participants having the lowest heart rate variability parameters as regards PNN50, rMSSD, SDNN at 5.078 ± 8.12, 29 ± 20.89, and 34.11 ± 17.55 respectively. Also, there was statistically significant difference between CKD stages as regards Hct and phosphorus level. Conclusion: Children in the fifth stage of chronic renal disease had the highest incidence of abnormalities observed by echocardiography, electrocardiography, and holter monitoring where patients in CKD stage 5 had the lowest heart rate variability values. [ABSTRACT FROM AUTHOR]

Background: Children with chronic kidney disease (CKD) are at high risk of cardiovascular disease. Cardiovascular magnetic resonance (CMR) is the reference method for assessing cardiac remodeling. To our knowledge, no study has reported a comprehensive analysis of left ventricular(LV) cardiac remodeling using CMR in different stages of pediatric CKD. This prospective case-control study aimed to investigate cardiac remodeling in pediatric CKD, using CMR, and determine its relationship with risk factors. Method: CMR was performed in 124 children with CKD and 50 controls. The cardiac remodeling parameters included left ventricular mass index (LVMI), LV remodeling index (LVRI), and LV wall thickness. Univariable and multivariable analyses were performed to assess the cardiac remodeling risk factors. Results: Cardiac remodeling was observed in 35.5% (44/124) of children with CKD. The LVMI, LVRI, and LV wall thickness were higher in advanced stages of CKD (P < 0.05). In the CKD stage 1–2 group, a lower in the estimated glomerular filtration rate was an independent determinant of impaired LVMI (β = −0.425, P = 0.019) and LVRI (β = −0.319, P = 0.044). A higher protein to creatinine ratio(PCR) was independently associated with impaired LVRI (β = 0.429, P = 0.022). In the CKD stage 3–5 group, higher in systolic blood pressure (SBP) (β = 0.464, P = 0.005) and PCR (β = 0.852, P = 0.031) were independent determinants of impaired LVMI. Additionally, higher SBP was positively correlated with impaired LVRI(r = 0.599, P < 0.001). There was a trend toward more abnormal cardiac remodeling in the CKD stage 3–5 group with hypertension than those without. Conclusion: Cardiac remodeling is prevalent in children with CKD, from an early stage. kidney markers are independently associated with cardiac remodeling. Hypertension increases the risk of cardiac remodeling in CKD stages 3–5. Strict BP control may help reverse or prevent remodeling. [ABSTRACT FROM AUTHOR]

Patients suffering from chronic kidney disease (CKD) are particularly placed at risk of multiorgan complications. One of them is malnutrition, which adds up to a higher mortality factor among them. This study was designed to determine the usefulness of brain-derived neurotrophic factor (BDNF) and irisin assays in the assessment of CKD development. The study group included 28 children with CKD at stages 2-5 treated conservatively. The outcome of our study revealed decreased serum BDNF and irisin levels in CKD patients, whereas urine concentrations were increased for BDNF and decreased for irisin, comparing to healthy controls. There was a positive correlation between anthropometric measures and urine BDNF concentration, as well as anthropometric measures and both serum and urine irisin levels in the study group, however no dependence of the tested markers on the stage of CKD was observed. In recent years, a role of myokines was described as vital for maintaining metabolic homeostasis therefore we suspect a potential role of these multifaceted markers in detecting malnutrition in CKD children. [ABSTRACT FROM AUTHOR]

Introduction: There is a scarcity of research comparing the efficacy of cyclophosphamide and mycophenolate mofetil in childhood nephrotic syndrome. The aim was to evaluate the efficacy and safety of oral cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in children with steroid-sensitive nephrotic syndrome in terms of the proportion of children who have been off steroids for at least 6 months without proteinuria (responders). Methods: This open-label retrospective-prospective comparative study was conducted in a pediatric nephrology clinic of a referral center for children between 1 and 18 years of age with FR/SD nephrotic syndrome. Group A consisted of patients who received oral cyclophosphamide (100, 25% female) at a dose of 2–2.5 mg/kg once daily for a period of 8–12 weeks. Group B consisted of patients who received oral mycophenolate mofetil (n = 61, 18% female) (dose: 800–1200 mg/m2) for at least 12 months. Responders were defined as children who were off steroids for at least 6 months along with absence of proteinuria. Results: In the CYC group, 50% of the patients were responders, whereas 54% of the patients in the MMF group were responders (p = 0.614). The time to first relapse with CYC was 7 months (IQR 5.25–11) compared to 7 months (IQR 3.5–12) with MMF (p = 0.092). The relapse rate in the CYC group was 1.77 relapses per patient-year compared to 1.295 relapses per patient-year in the MMF group. The difference in relapse rate was significant (-0.474; 95% CI, 0.09 to 0.86 relapses/person-year) (p value = 0.009). Multivariate analysis revealed that an age of less than 5 years at the start of treatment was a significant factor for a better response to MMF (p value = 0.039, OR = 2.988, CI -1.055-8.468). Conclusions: The efficacy of MMF was similar to that of CYC in terms of response (6 months without steroids) in children with FR/SD nephrotic syndrome. MMF showed a favorable response in terms of the frequency of relapse and treatment failure. Registration of the study with Clinical Trials Registry of India: (http://ctri.nic.in;CTRI/2021/06/034421) (Dt: 28/06/2021). [ABSTRACT FROM AUTHOR]

Background: Hyperphosphatemia has been emphasized to be a significant risk factor for vascular calcification in CKD patients. This study aims to investigate the effect of magnesium supplementation on the reduction of phosphate levels and carotid intima media thickness in children as predictor on vascular calcification with CKD and hyperphosphatemia, compared to a placebo. Methods: A randomized, double-blind, placebo-controlled trial was conducted at Pediatric Ward and Outpatient Clinic of Pediatric Nephrology in our setting during October-March 2023. We compared oral magnesium supplementation (6 mg/kg body weight/day for two months) with a placebo in children with CKD and hyperphosphatemia (ages 1-18 years old). Patients who were on dialysis and had serum magnesium levels of <1.6 mg/dL and >2.4 mg/dL, and were allergic to magnesium supplementation were excluded. A paired T-test and the Wilcoxon signed-rank test were used for statistical analysis. Results: We collected 25 children in the experimental group and 25 children in the placebo group. Phosphate levels were decreased in both the magnesium supplementation and placebo groups (6.1 ± 0.79 to 6.0 ± 0.63 mg/dL; p-value = 0.852 and 6.01 ± 0.55 to 5.8 ± 0.64 mg/dL; p-value=0.365). However, when compared between groups, the reductions were not significantly different (0.1 vs 0.21; p-value=0.935). A significant improvement was found in carotid intima media thickness in both groups (0.05±0.01 to 0.05±0.01; p-value=0.000 and 0.05±0.01 to 0.05±0.01; p-value=0.000), and the reductions were significantly different (0.01 vs 0.01; p-value=0.000). Conclusion: Magnesium supplements have considerably lower phosphate levels and significantly reduced the thickness on carotid intima media in children with CKD and hyperphosphatemia. [ABSTRACT FROM AUTHOR]

Summary: Glomerular hyperfiltration and albuminuria are frequent kidney abnormalities in children with sickle cell anaemia (SCA). However, little is known about their persistence in African SCA children. This prospective study included 600 steady‐state SCA children aged 2–18 years from the Democratic Republic of Congo. Participants were genotyped for apolipoprotein L1 (APOL1) risk variants (RVs) and haem oxygenase‐1 (HMOX1) GT‐dinucleotide repeats. Kidney abnormalities were defined as albuminuria, hyperfiltration or decreased estimated creatinine‐based glomerular filtration rate (eGFRcr). At baseline, 247/600 (41.2%) participants presented with kidney abnormalities: 82/592 (13.8%) with albuminuria, 184/587 (31.3%) with hyperfiltration and 15/587 (2.6%) with decreased eGFRcr. After a median follow‐up of 5 months, repeated testing was performed in 180/247 (72.9%) available participants. Persistent hyperfiltration and persistent albuminuria (PA) were present in 29.2% (38/130) and 39.7% (23/58) respectively. eGFR normalized in all participants with a baseline decreased eGFRcr. Haemoglobinuria (p = 0.017) and male gender (p = 0.047) were significantly associated with PA and persistent hyperfiltration respectively. APOL1 RVs (G1G1/G2G2/G1G2) were borderline associated with PA (p = 0.075), while HMOX1 long repeat was not associated with any persistent kidney abnormality. This study reveals that a single screening can overestimate the rate of kidney abnormalities in children with SCA and could lead to overtreatment. [ABSTRACT FROM AUTHOR]

Glomerular kidney diseases typically begin insidiously and can progress to end stage kidney failure. Early onset of therapy can slow down disease progression. Early diagnosis is required to ensure such timely therapy. The goal of our study was to evaluate protein biomarkers (BMs) for common nephropathies that have been described for children with Alport syndrome. Nineteen candidate BMs were determined by commercial ELISA in children with congenital anomalies of the kidneys and urogenital tract, inflammatory kidney injury, or diabetes mellitus. It is particularly essential to search for kidney disease BMs in children because they are a crucial target group that likely exhibits early disease stages and in which misleading diseases unrelated to the kidney are rare. Only minor differences in blood between affected individuals and controls were found. However, in urine, several biomarker candidates alone or in combination seemed to be promising indicators of renal injury in early disease stages. The BMs of highest sensitivity and specificity were collagen type XIII, hyaluronan-binding protein 2, and complement C4-binding protein. These proteins are unrelated to inflammation markers or to risk factors for and signs of renal failure. In conclusion, our study evaluated several strong candidates for screening for early stages of kidney diseases and can help to establish early nephroprotective regimens. [ABSTRACT FROM AUTHOR]

Albuminuria has been considered the golden standard biomarker for diabetic kidney disease (DKD), but appears once significant kidney damage has already occurred. Angiopoietin-2 (Angpt-2) has been implicated in the development and progression of DKD in adults. We aimed to explore the association of serum Angpt-2 levels with DKD in children and adolescents with type 1 diabetes mellitus (T1DM) of short duration (3–5 years) and to evaluate the predictive power of serum Angpt-2 in the early detection of DKD prior to the microalbuminuric phase. The current cross-sectional study included 90 children divided into three age and sex-matched groups based on urinary albumin-to-creatinine ratio (UACR): microalbuminuric diabetic group (n = 30), non-albuminuric diabetic group (n = 30), and control group (n = 30). All participants were subjected to anthropometric measurements, serum Angpt-2 and fasting lipid profile (total cholesterol, triglycerides, LDL-C, HDL-C, and Non-HDL-C) assessment. Glomerular filtration rate was estimated based on serum creatinine (eGFR-Cr). Higher serum Angpt-2 levels were detected in both diabetic groups compared to controls and in microalbuminuric compared to non-albuminuric diabetic group. There was no detected significant difference in eGFR-Cr values across the study groups. Serum Angpt-2 was positively correlated with triglycerides, LDL, Non-HDL-C, HbA1c, and UACR, while UACR, HbA1c, and Non-HDL-C were independent predictors for serum Angpt-2. Serum Angpt-2 at level of 137.4 ng/L could discriminate between microalbuminuric and non-albuminuric diabetic groups with AUC = 0.960 and at level of 115.95 ng/L could discriminate between the non-albuminuric diabetic group and controls with AUC = 0.976. Conclusion: Serum Angpt-2 is a promising potent biomarker for the detection of early stage of DKD in childhood T1DM before albuminuria emerges. What is Known? • Urine albumin-to-creatinine ratio (UACR) and glomerular filtration rate (GFR) are the golden standard but late biomarkers for DKD. • Angiopoietin-2 has been implicated in the development and progression of DKD in adults with diabetes, but has not been explored in T1DM children with DKD. What is New? • Higher serum angiopoietin-2 was detected in diabetic groups compared to controls and in microalbuminuric compared to non-albuminuric group. • Angiopoietin-2 correlated positively with triglycerides, LDL, Non-HDL-C, HbA1c, and UACR. • Serum angiopoietin-2 is a promising early diagnostic biomarker for DKD in children with T1DM. [ABSTRACT FROM AUTHOR]

Introduction: Gingivitis is a common intraoral disease in patients with chronic kidney disease (CKD), which poses a particular interdisciplinary challenge. We aimed to determine the influence of an intensive oral prophylaxis program (OPP) compared to standard prevention measures on the tongue microbiome of young patients with CKD. Methods: Thirty patients with CKD (mean age 14.2 ± 5.2 years) and generalized gingivitis were included. The effects of the intensive OPP were compared with standard prophylaxis according to statutory health insurance (treatment as usual, TAU) as a control. Tongue swabs were taken from the patients at baseline (t1) and after 3 (t2) and 6 (t3) months. Next-generation sequencing of 16S rDNA genes was used to quantitatively characterize microbial communities. Results: There were no differences in the abundance, richness, or diversity of the observed genera and species between the two study groups at baseline or after 3 or 6 months. Furthermore, no change in predefined gingivitis and oral health bacterial clusters were found. At the phylum level, Firmicutes were decreased after intervention in the TAU group (t2TAU 42.9 ± 7.1 to t3TAU 34.8 ± 4.7 (npairs=14), p=0.003; false discovery rate 0.02). The decrease of Firmicutes was not significant in the OPP group. Conclusions: Despite the intensity of dental prophylaxis and decreasing clinical signs of inflammation and decreasing plaque amount, no clinically relevant changes in the tongue microbiome were observed. Our results confirm the conserved and stable nature of the tongue microbiome, even in children with CKD. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) is the third most common cause of death after cancer and heart disease. The continuous treatment of children with CKD was greatly challenged during the COVID-19 pandemic, which significantly impacted the CKD children's prognosis and the caregivers' psychological status. However, the influence mechanism of socioeconomic status, medical delay duration, traffic pressure, and online consultation duration on caregivers' hope and psychological resilience still lacks relevant evidence. Methods: This study investigated the general social information, hope, and psychological resilience of 247 caregivers with CKD in 13 provinces of China in March 2020. Factor analysis and an exploratory Structural Equation Model (SEM) were used to find the best-fit model, and Bootstrapping was used to calculate the 95% CI of indirect effects. Results: The factor analysis obtained four common factors, namely, socioeconomic status (annual family income, education, and career stability), medical accessibility (online consultation duration, medical delay duration, and traffic pressure), hope (positive attitude, positive action, and intimate relationship) and psychological resilience (optimism, tenacity, and strength), with the cumulative contribution rate of 65.34%. Bootstrapping obtains the socioeconomic status β = 0.30 (95% CI [0.14, 0.47], P = 0.002), medical accessibility β = 0.31 (95% CI [0.12, 0.47], P = 0.002), and hope β = 0.40 (95% CI [0.27, 0.52], P = 0.002) has a direct impact on psychological resilience of CKD children caregivers, followed by medical accessibility β = 0.20 (95% CI [0.10, 0.38], P = 0.001) and hope β = 0.23 (95% CI [0.16, 0.32], P = 0.001) plays a mediating role between socioeconomic status and psychological resilience. The indirect impact effect β = 0.35 (95% CI [0.25, 0.50], P = 0.001) is greater than the direct impact effect β = 0.30 (95% CI [0.14, 0.47], P = 0.002). Conclusions: Sufficient attention should still be given to children with immunodeficiency after the COVID-19 pandemic, such as CKD, to avoid infection of deadly. Secondly, the government should vigorously develop Primary medical institutions to ensure efficient treatment of severe patients in tertiary hospitals; Finally, the professional literacy of medical workers in remote diagnosis and treatment should be improved to enhance the country's emergency response capacity for similar major public events and the requirements for normalised epidemic prevention and control. [ABSTRACT FROM AUTHOR]

Background: Social determinants of health (SDoH) are increasingly recognized as crucial factors affecting the well-being of pediatric chronic kidney disease (CKD) patients. This study explores the impact of SDoH on the quality of life (QoL) of children with CKD in Saudi Arabia. Methods: This observational, descriptive, cross-sectional study included 50 participants with CKD stages three to five, as well as children with end-stage renal disease (ESRD) undergoing peritoneal dialysis (PD) or hemodialysis (HD) in Riyadh, Saudi Arabia. Data were collected from children and their caregivers using a validated Arabic-adapted version of the Pediatric Quality of Life Inventory (PedsQL) 3.0 and an SDoH survey. Results: The study revealed that access to education significantly affects pediatric QoL (H/Z score 2.825; P-value 0.005), and the lack of dietary recommendations was identified as a critical factor (H/Z score 1.441; P-value 0.029). ESRD patients on PD from larger families reported better QoL, whereas lower family income was associated with reduced QoL in PD patients (P-value 0.048). Compared to those in earlier stages of CKD, ESRD patients showed lower QoL (H/Z score 2.154; P-value 0.031). Conclusion: Our study provides initial insights into the relationship between SDoH and the QoL of children with CKD in Saudi Arabia. It calls for a comprehensive approach that includes these determinants in care strategies to improve patient outcomes and QoL. [ABSTRACT FROM AUTHOR]

Introduction: Children with chronic kidney disease (CKD) have parental responsibility for their care and medical management throughout their lives. The causes of distress associated with caring for a child on dialysis are rarely known to parents. The problems faced by parents require support and empowerment to be able to improve the quality of life for their children. For this reason, information is needed in the form of the challenges faced by parents or caregivers of children. Aims: This study described the experience of a parent and caregiver caring for children with chronic kidney disease. Methods: An integrative review study was conducted to explore the experience of a parent and caregiver caring for children with CKD. Electronic databases and reference lists of relevant articles were searched until March 2023. Guided by PRISMA guidelines, five electronic databases-PubMed, EBSCO, PROQUEST, Science Direct, and Google Scholar-were searched for relevant articles published in English. Results: Ten articles were identified according to the review experience of parents and caregivers in the form of lack of sleep, fatigue, stress, burden, depression, dynamics and emotional disturbances in family members, responsibility, guilt, suffocation, hopelessness, inhibition of subjectivity, lack of understanding, tensions in the family, the health care system, satisfaction, and disappointment, as well as objectification and depersonalization. Conclusion: The many problems faced by parents and caregivers in caring for children with CKD require action in the form of social support, knowledge about home care, the impact of dialysis, and the ability to be fair to siblings. [ABSTRACT FROM AUTHOR]

Introduction: The competencies in the Medical Specialty Curriculum and Standards Development System (MSCSD) guides trainers in the preparation and execution of the training program and gives learners an idea about the state of mastery that they will reach by graduation. The aim of this study was to evaluate the knowledge of pediatric nephrologists about competencies in the field of specialization. Materials and Methods: In the cross-sectional study conducted between January 2023 and 2024, the knowledge of pediatric nephrologists about specialty competencies was investigated with the two-round Delphi technique. Digital Delphi forms were sent to all pediatric nephrologists in Türkiye via email of the specialty society. In the Delphi form, all competency titles in the MSCSD v.2.3 curriculum were transformed into propositions that could be scored with a 5-point Likert scale. The frequency of 4-5, the difference between quartiles and the median were calculated for each of the statements. For participant consensus on the propositions, the median should be 4, the frequency of 4-5 should be above 70%, and the difference between quartiles should be 2.5 or less. Results: Of the pediatric nephrologists in Türkiye, 59 responded to the first and 136 to the second round Delphi survey. In the first round, full consensus was found in the competency areas other than service delivery, while consensus was found in two of the 72 clinical competencies under the service delivery heading, namely systemic diseases and malignant diseases, and in two of the 26 interventional competencies (diversion and imaging). The analysis of the second round Delphi questionnaire based on this result showed full consensus on all competency headings. The variables of title, gender and training clinic made a difference in consensus. Conclusion: It was found that the level of knowledge of pediatric nephrologists about specialty competencies differed in terms of some variables and knowledge was more limited in young learners for whom competencies are legally binding. In conclusion, it is recommended that learners should be informed about the competencies at the beginning of specialty training and participate in updating the competency list. [ABSTRACT FROM AUTHOR]

Background: Probiotics are living microorganisms that may confer health benefits to their host if administered in sufficient quantities. However, data on the use of probiotics in transplant recipients are scarce. Method: This multi‐center survey of pediatric nephrologists aimed to examine variations in practice regarding the use of probiotics in pediatric kidney transplant recipients. The survey was conducted via a 10‐item questionnaire (developed in Survey Monkey) administered to pediatric nephrologists participating in the Pediatric Nephrology Research Consortium meeting in April 2023. Results: Sixty‐four pediatric nephrologists completed the survey. Twenty‐seven (42.2%) respondents reported having prescribed probiotics to pediatric kidney transplant recipients. The primary reason for probiotic use was the treatment of antibiotic‐associated diarrhea (n = 20), with other reasons including recurrent Clostridium difficile infection (n = 15), general gut health promotion (n = 12), recurrent urinary tract infections (n = 8), and parental request (n = 1). Of those who prescribed probiotics, 48.1% held them during periods of neutropenia and 14.8% during central venous line use. Of the 64 respondents, 20 reported the lack of safety data as a concern for using probiotics in kidney transplant recipients. Conclusion: Pediatric nephrologists are increasingly prescribing probiotics to pediatric kidney transplant recipients; nevertheless, substantial practice variations exist. The paucity of safety data is a significant deterrent to probiotic use in this population. [ABSTRACT FROM AUTHOR]

To promote improved trial design in upcoming randomized clinical trials in childhood chronic kidney disease (CKD), insight in the within- and inter-patient variability of uremic toxins with its nutritional, treatment- and patient-related confounding factors is of utmost importance. In this study, the within- and inter-patient variability of a selection of uremic toxins in a longitudinal cohort of children diagnosed with CKD was assessed, using the intraclass correlation coefficient (ICC) and the within-patient coefficient of variation (CV). Subsequently, the contribution of anthropometry, estimated glomerular filtration rate (eGFR), dietary fiber and protein, and use of (prophylactic) antibiotics to uremic toxin variability was evaluated. Based on 403 observations from 62 children (median seven visits per patient; 9.4 ± 5.3 years; 68% males; eGFR 38.5 [23.1; 64.0] mL/min/1.73 m2) collected over a maximum of 2 years, we found that the within-patient variability is high for especially protein-bound uremic toxins (PBUTs) (ICC < 0.7; within-patient CV 37–67%). Moreover, eGFR was identified as a predominant contributor to the within- and inter-patient variability for the majority of solutes, while the impact of the child's anthropometry, fiber and protein intake, and antibiotics on the variability of uremic toxin concentrations was limited. Based on these findings, we would recommend future intervention studies that attempt to decrease uremic toxin levels to select a (non-dialysis) CKD study population with a narrow eGFR range. As the expected effect of the selected intervention should exceed the inter-patient variability of the selected uremic toxins, a narrow eGFR range might aid in improving the trial design. [ABSTRACT FROM AUTHOR]

Background Immunoglobulin A vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Due to a lack of evidence, treatment recommendations are based on expert opinion, resulting in variation. The aim of this study was to describe the clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy-proven IgAVN in order to identify prognostic risk factors and signals of treatment efficacy. Methods Retrospective data were collected on 1148 children with biopsy-proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analysed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow-up. Results The median follow-up was 3.7 years (interquartile range 2–6.2). At last follow-up, 29% of patients had an eGFR <90 mL/min/1.73 m2, 36% had proteinuria and 3% had chronic kidney disease stage 4–5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second-line immunosuppressive regimen being superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow-up. Conclusion In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly, there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN. [ABSTRACT FROM AUTHOR]

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder characterized by the formation of numerous fluid-filled cysts in the kidneys, leading to progressive renal failure and various extrarenal complications, including hypertension. This review explores the genetic basis of ADPKD, including emerging evidence of epigenetic mechanisms in modulating gene expression and disease progression in ADPKD. Furthermore, it proposes to examine the pathological characteristics of this condition at the nephrological, cardiovascular, nutritional, and psychological levels, emphasizing that the follow-up of patients with ADPKD should be multidisciplinary from a young pediatric age. [ABSTRACT FROM AUTHOR]

Introduction: Although estimated glomerular filtration rate (eGFR) are continuously developed for pediatric population, impact of height measurement is often neglected due to variable dynamic growth changes in children. This study aimed to compare differential impacts of eGFR values calculated by six equations that do not use height information. Materials and methods: 3503 Korean pediatric patients with creatinine/cystatin C assay-based laboratory results from 2008 to 2021 were analyzed for clinical course using a total of 8113 laboratory test results. Baseline eGFR was calculated by eight different equations including two widely used equations incorporating height parameter. Along with the agreement of CKD (chronic kidney disease) stage categorization by different equations, clinical outcome of incident CKD diagnosis in 13 years of study period were compared. Results: Among a total of 28 pairwise comparisons among eight equations, only 4 combinations of comparisons revealed optimal P15 values (≥80 % concordance), with FAS-age equation being both concordant with two equations using height parameter. Clustering of eight equations by incident CKD diagnosis in subsequent tests also highlighted FAS-age as candidate equation within the same cluster with Schwartz-bedside and FAS-height equations. When the equation values were classified into Kidney Disease Improving Global Outcomes (KDIGO) CKD stages, the distribution patterns for stage 1 and 5 were significantly different among eGFR equations. FAS-age equation revealed the highest agreement with Schwartz-bedside and FAS-height equations that incorporate the height data. Conclusions: The eGFR equation type should be considered to establish the eGFR intervals for CKD stage classification, particularly in the pediatric patient population. Cautious interpretation is required for eGFR value along with clinical context.

The article discusses the use of plasmapheresis as a potential treatment for IgA vasculitis nephritis in children, based on a case series analysis. While previous studies have shown limited evidence for other treatments, plasmapheresis has shown some benefit in patients with rapidly progressive glomerulonephritis. The study analyzed 13 children who underwent plasmapheresis and found that most patients showed improvement in kidney function, although a clear recommendation for its use is hindered by the small sample size. The authors suggest that further prospective randomized studies are needed to fully assess the efficacy of plasmapheresis in treating IgA vasculitis nephritis in children. [Extracted from the article]