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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: 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]

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]

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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: 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]

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]

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]

Background: The potential involvement of circular RNAs (circRNAs) and N6-methyladenosine (m6A) modification in the progression of Wilms tumor (WT) has not been fully elucidated. This study investigates the regulatory mechanisms and clinical significance of m6A-modified circMARK2 and its role in WT progression. Methods: We identified dysregulated circRNAs through deep sequencing and validated their expression by qRT-PCR in WT tissues. The biological functions of circMARK2 were assessed using clone formation, transwell migration, and orthotopic animal models. To dissect the underlying mechanisms, we employed RNA immunoprecipitation, RNA pull-down, dual-luciferase reporter assays, Western blotting, and immunofluorescence and immunohistochemical staining. Results: CircMARK2, upregulated in WT tissues, was found to be m6A-modified and promoted cytoplasmic export. It facilitated WT progression by stabilizing LIN28B mRNA through the circMARK2/IGF2BP2 interaction. In vitro and in vivo studies demonstrated that circMARK2 enhances the malignant behavior of WT cells. Clinically, higher circMARK2 levels in tumor tissues of WT patients were linked to increased tumor aggressiveness and reduced survival rates. Conclusions: Our study provides the first comprehensive evidence that m6A-modified circMARK2 contributes to WT progression by enhancing LIN28B mRNA stability, promoting cellular aggressiveness. CircMARK2 emerges as a potential biomarker for prognosis and a promising target for therapeutic intervention in WT, underscoring the clinical relevance of m6A modification in pediatric renal cancer. [ABSTRACT FROM AUTHOR]

Juvenile nephronophthisis is an inherited renal ciliopathy with cystic kidney disease, renal fibrosis, and end-stage renal failure in children and young adults. Mutations in the NPHP1 gene encoding nephrocystin-1 protein have been identified as the most frequently responsible gene and cause the formation of cysts in the renal medulla. The molecular pathogenesis of juvenile nephronophthisis remains elusive, and no effective medicines to prevent end-stage renal failure exist even today. No human cellular models have been available yet. Here, we report a first disease model of juvenile nephronophthisis using patient-derived and gene-edited human induced pluripotent stem cells (hiPSCs) and kidney organoids derived from these hiPSCs. We established NPHP1-overexpressing hiPSCs from patient-derived hiPSCs and NPHP1-deficient hiPSCs from healthy donor hiPSCs. Comparing these series of hiPSCs, we found abnormalities in primary cilia associated with NPHP1 deficiency in hiPSCs. Kidney organoids generated from the hiPSCs lacking NPHP1 formed renal cysts frequently in suspension culture with constant rotation. This cyst formation in patient-derived kidney organoids was rescued by overexpression of NPHP1. Transcriptome analysis on these kidney organoids revealed that loss of NPHP1 caused lower expression of genes related to primary cilia in epithelial cells and higher expression of genes related to the cell cycle. These findings suggested the relationship between abnormality in primary cilia induced by NPHP1 loss and abnormal proliferative characteristics in the formation of renal cysts. These findings demonstrated that hiPSC-based systematic disease modeling of juvenile nephronophthisis contributed to elucidating the molecular pathogenesis and developing new therapies. [ABSTRACT FROM AUTHOR]

Background: Proteinuria is a prevalent symptom of pediatric nephrology, while kidney biopsy remains the gold standard for kidney tissue analysis, and it is currently controversial. We report the rare case that the mutation in the AMN gene was considered to cause chronically isolated proteinuria and also suggest that renal biopsy should be chosen with caution in children with chronic isolated non-nephrotic levels of proteinuria and that genetic testing may be feasible for the early precise diagnosis. Case presentation: A 35-month-old boy presented with excessive urine foaming for more than half a month; his proteinuria was considered non-nephrotic range and urine protein electrophoresis was suggestive of mixed proteinuria; other than that, the investigations are non-specific. Given the child's chronic isolated proteinuria and good renal function, we chose to refine the genetic test rather than a renal biopsy; a compound heterozygous variant was found in the AMN gene of this child which was caused by a point mutation in the father, and a partial chromosomal deletion in the mother. Conclusions: Cubilin(encoded by CUBN), amnionless(encoded by AMN), and megalin form a multiligand receptor complex; CUBN or AMN gene variants have been implicated as a hereditary cause of megaloblastic anemia, proteinuria, and neurological impairment. In the past few decades, chronic isolated proteinuria caused by CUBN gene variants is benign, non-progressive, and has normal renal function. However, the child is the first reported case of isolated proteinuria of AMN gene mutation, indicating that the earlier diagnostic genetic sequencing in an otherwise well, not nephrotic proteinuria child may be a convenient, cost-effective, and harmless option, challenging the traditional paradigm. [ABSTRACT FROM AUTHOR]

Effective optimal pharmacotherapy requires a comprehensive understanding of the drug's pharmacokinetic properties. Chronic kidney disease (CKD) influences medication pharmacokinetics. However, whether sex differences exist in the pharmacokinetics of drugs for children with CKD is unknown. The primary aim of this article was to evaluate the effect of sex on pharmacokinetics of drugs commonly used for CKD treatment in children. Secondary outcome was to address the impact of sex in CKD disease progression. Electronic databases, PubMed, EMBASE, Google Scholar, and Web of Science were searched from inception, using Mesh terms in English for sex differences in the pharmacokinetics of drugs in children with CKD. No studies have documented sex-related differences in the pharmacokinetics of drugs for the treatment of CKD in children. As a consequence, it is difficult to predict the effect of sex on pharmacokinetics by extrapolating data from adult studies to children. Evidence to date suggests that girls generally have a higher prevalence and disease progression of CKD when compared to boys regardless of age. Understanding the pharmacokinetics and pharmacodynamics of drugs provides practical consideration for dosing optimal medication regimens. Future kinetic studies are needed evaluating the effect of sex on the pharmacokinetics and pharmacodynamics of drugs in children with CKD. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) affects adults and children globally and is a critical health concern. Cardiovascular disease (CVD) was identified over ten years ago as a leading cause of death in children with advanced CKD. Numerous studies that evaluate cardiovascular risk, disease processes, and early markers of CVD in cases with CKD have been published as a result of this observation. Children with CKD have an exceptionally high prevalence of conventional and uremia-related CVD predisposing factors, much like adults do. Objective: The aim was to study the impact of bone markers and other laboratory tests on diastolic cardiac functions in children and adolescent with CKD on regular hemodialysis. Patients and Methods: A cross-sectional observational study was conducted on 40 patients with CKD on regular hemodialysis, from May 2022 to June 2023 in the Pediatric Nephrology and Cardiology Department at Sohag University Hospital. Results: The commonest causes of CKD were congenital anomalies of the kidney and urinary tract (CAKUT) in 77.5% of patients. The mean age of patients was 14.18 ± 4.36 years. Evaluation of cardiac function showed that 57.5% of cases had diastolic dysfunction and 70% of cases had left ventricular hypertrophy (LVH). There was a statistically significant correlation between diastolic dysfunction with anemia and hyperparathyroidism (p<0.05). Hypertension was significantly accompanied by LVH (p=0.045). Conclusion: Our study revealed that the most important factors affecting all parameters of diastolic function were anemia and hyperparathyroidism. Other factors affecting the diastolic function to a lesser extent were hypertension and residual kidney function (RKF). There was a substantial correlation between LVH and hypertension. [ABSTRACT FROM AUTHOR]

Objective: To determine the prevalence of chronic kidney disease (CKD) among children of school-going age. Study Design: Cross-sectional study. Setting: Department of Pediatric Medicine, National Institute of Child Health, Karachi, Pakistan. Period: July 2023 to December 2023. Methods: A total of 130 children of both genders, aged between 5-15 years, visiting outpatient department of pediatric medicine were analyzed. Demographic and clinical information were noted. Laboratory investigations like urinalysis and serum creatinine were performed. CKD was diagnosed on the basis of "Kidney Disease Outcomes Quality Initiative (KDOQI)" guidelines. Results: In a total of 130 children, 67 (51.5%) children were male. Overall, the mean age was 8.43±2.45 years. Hypertension was diagnosed in 10 (7.7%) children. Anemia was present in 103 (79.2%) patients. The mean hemoglobin, serum creatinine, and blood urea nitrogen were 9.41±1.98 g/dl, 0.48±0.37 mg/dl, and 34.20±33.18 mg/dl, respectively. CKD was diagnosed in 10 (7.7%) children. Among these 10 children diagnosed with CKD, 7 were having CKD stage-1 whereas CKD stage-2 was noted in 3 children. Presence of hypertension (p=0.006), and albuminuria (p<0.001) were associated with CKD. Conclusion: The prevalence of CKD in school going age children was 7.7%. Presence of hypertension, and albuminuria were linked with CKD. [ABSTRACT FROM AUTHOR]

Background: Assessing bias (estimated – measured) is key to evaluating glomerular filtration rate (GFR). Stratification by subgroups can indicate where equations perform differently. However, there is a fallacy in the assessment of two instruments (e.g., eGFR and mGFR) when stratifying on the level of only one of those instruments. Here, we present statistical aspects of the problem and a solution for GFR stratification along with an empirical investigation using data from the CKiD study. Methods: Compared and contrasted biases (eGFR relative to mGFR) with 95% confidence intervals within strata of mGFR only, eGFR only, and the average of mGFR and eGFR using data from the Chronic Kidney Disease in Children (CKiD) study. Results: A total of 304 participants contributed 843 GFR studies with a mean mGFR of 48.46 (SD = 22.72) and mean eGFR of 48.67 (SD = 22.32) and correlation of 0.904. Despite strong agreement, eGFR significantly overestimated mGFR when mGFR < 30 (+ 6.2%; 95%CI + 2.9%, + 9.7%) and significantly underestimated when mGFR > 90 (–12.2%; 95%CI − 17.3%, − 7.0%). Significant biases in opposite direction were present when stratifying by eGFR only. In contrast, when stratifying by the average of eGFR and mGFR, biases were not significant (+ 1.3% and − 1.0%, respectively) congruent with strong agreement. Conclusions: Stratifying by either mGFR or eGFR only to assess eGFR biases is ubiquitous but can lead to inappropriate inference due to intrinsic statistical issues that we characterize and empirically illustrate using data from the CKiD study. Using the average of eGFR and mGFR is recommended for valid inferences in evaluations of eGFR biases. [ABSTRACT FROM AUTHOR]

Background: Acute kidney injury (AKI) is a life-threatening condition, especially in extreme age groups and when kidney replacement therapy (KRT) is necessary. Studies worldwide report mortality rates of 10–63% in pediatric patients undergoing KRT. Methods: Over 13 years, this multicenter study analyzed data from 693 patients with AKI, all receiving KRT, across 74 hospitals and medical facilities in Rio de Janeiro, Brazil. Results: The majority were male (59.5%), under one year old (55.6%), and treated in private hospitals (76.5%). Sixty-six percent had comorbidities. Pneumonia and congenital heart disease were the most common admission diagnoses (21.5% and 20.2%, respectively). The mortality rate was 65.2%, with lower rates in patients over 12 years (50%). Older age was protective (HR: 2.35, IQR [1.52–3.62] for neonates), and primary kidney disease had a three-fold lower mortality rate. ICU team experience (HR: 0.74, IQR [0.60–0.91]) correlated with lower mortality, particularly in hospitals treating 20 or more patients. Among the deceased, 40% died within 48 h of KRT initiation, suggesting possible late referral or treatment futility. Conclusions: This study confirms the high mortality in pediatric dialytic AKI in middle-income countries, underlining early mortality and offering critical insights for improving outcomes. [ABSTRACT FROM AUTHOR]

Background: Anemia is common complication in children with chronic kidney disease (CKD) mainly anemia of chronic disease (ACD), but iron deficiency anemia (IDA) is also common. Soluble transferrin receptor (sTfR) is important marker for IDA. This study aims to evaluate the utility of soluble transferrin Receptor-Ferritin Indices as markers of IDA and for differentiation ACD from IDA in childern with chronic kidney disease. Methods: cross-sectional study was conducted at Department of Pediatric Nephrology at Zagazig University Hospital including 45 anemic children (19 ACD, 13 IDA and 13 mixed). The duration of the study ranges from 6 to 12 months, Soluble serum transferrin receptor and Soluble serum transferrin receptor index were measured. Results: There was significant difference regard sTfR and sTfR index IDA group were significantly higher than other two groups then mixed and finally ACD were significantly lower. Conclusion: sTfR value was useful tool for assessment of iron status in patients with CKD, however, they are at best complementary to the existing indices of serum ferritin and TSAT. [ABSTRACT FROM AUTHOR]

Key Clinical Message: As there is no significant mutual relationship between Helicobacter pylori infection and chronic kidney disease in children, its routine study is not justified and is recommended only in symptomatic cases. Children suffering from chronic kidney disease (CKD) often complain of indigestion but, if it is accompanied by abdominal pain, it is necessary to investigate and rule out Helicobacter pylori infection to confirm functional dyspepsia. Epidemiological studies in adults have conflicting results regarding the association between Helicobacter pylori infection and CKD. In this study, we determined the prevalence of H. pylori in children with kidney failure and its relationship to their gastrointestinal symptoms. In this retrospective study, 54 children with chronic kidney failure admitted to the hemodialysis ward of the Children's Medical Center, Tehran, Iran between 2012 and 2020 were studied. The mean age of our patients was 11.89 ± 3.99 years and their sex distribution was equal. H. pylori infection was reported in only three patients with 5.6%. Based on our findings, epigastric pain in children was the most common gastrointestinal symptom (70.4%). Among all patients, three patients (5.6%) died, all of them were male (P = 0.075). The most prevalent underlying cause of kidney failure in our patients was neurogenic bladder. We did not find any significant relationship between the increased risk of chronic kidney failure and co‐infection with H. pylori. Investigating the cause of epigastric pain and looking for H. pylori is very important in CKD children under hemodialysis because if they receive a transplant the possibility of gastrointestinal complications will be increased with the use of steroid and immunosuppressive drugs. [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.

Introduction: The potential role of pathogens, particularly vector-transmitted infectious agents, as a cause of psychosis has not been intensively investigated. We have reported a potential link between Bartonella spp. bacteremia and neuropsychiatric symptoms, including pediatric acute onset neuropsychiatric syndrome and schizophrenia. The purpose of this study was to further assess whether Bartonella spp. exposure or infection are associated with psychosis. Methods: In a blinded manner, we assessed the presence of anti-Bartonella antibodies by indirect immunofluorescence assays (IFA), and infection by amplification of bacterial DNA from blood by quantitative polymerase chain reaction (qPCR), digital PCR (dPCR), and droplet digital PCR (ddPCR) in 116 participants. Participants were categorized into one of five groups: 1) controls unaffected by psychosis (n = 29); 2) prodromal participants (n = 16); 3) children or adolescents with psychosis (n = 7); 4) adults with psychosis (n = 44); and 5) relatives of a participant with psychosis (n = 20). Results: There was no significant difference in Bartonella spp. IFA seroreactivity between adults with psychosis and adult controls unaffected by psychosis. There was a higher proportion of adults with psychosis who had Bartonella spp. DNA in the bloodstream (43.2%) compared to adult controls unaffected by psychosis (14.3%, p = 0.021). The Bartonella species was determined for 18 of the 31 bacteremic participants, including infection or co-infection with Bartonella henselae (11/18), Bartonella vinsonii subsp. berkhoffii (6/18), Bartonella quintana (2/18), Bartonella alsatica (1/18), and Bartonella rochalimae (1/18). Discussion: In conjunction with other recent research, the results of this study provide justification for a large national or international multi-center study to determine if Bartonella spp. bacteremia is more prevalent in adults with psychosis compared to adults unaffected by psychosis. Expanding the investigation to include a range of vector-borne and other microbial infections with potential CNS effects would enhance knowledge on the relationship between psychosis and infection. [ABSTRACT FROM AUTHOR]

Biomarkers that accurately reflect renal function are essential in management of chronic kidney diseases (CKD). However, in children, age/physique and medication often alter established renal biomarkers. We studied whether amino acid enantiomers in body fluids correlate with renal function and whether they are influenced by physique or steroid medication during development. We conducted a prospective study of children 2 to 18 years old with and without CKD. We analyzed associations of serine/asparagine enantiomers in body fluids with major biochemical parameters as well as physique. To study consequences of kidney dysfunction and steroids on serine/asparagine enantiomers, we generated juvenile mice with uninephrectomy, ischemic reperfusion injury, or dexamethasone treatment. We obtained samples from 27 children, of which 12 had CKD due to congenital (n = 7) and perinatal (n = 5) causes. Plasma d-asparagine and the d/l-serine ratio had robust, positive linear associations with serum creatinine and cystatin C, and detected CKD with high sensitivity and specificity, uninfluenced by body size or biochemical parameters. In the animal study, kidney dysfunction increased plasma d-asparagine and the d/l-serine ratio, but dexamethasone treatment did not. Thus, plasma d-asparagine and the d/l-serine ratio can be useful markers for renal function in children. [ABSTRACT FROM AUTHOR]

Background: We aimed to examine temporal changes in the annual rate of acute kidney injury (AKI) in Danish children and associated changes in patient characteristics including potential underlying risk factors. Methods: In this population-based cohort study, we used plasma creatinine measurements from Danish laboratory databases to identify AKI episodes in children aged 0–17 years from 2007 to 2021. For each child, the first AKI episode per calendar year was included. We estimated the annual crude and sex- and age-standardized AKI rate as the number of children with an AKI episode divided by the total number of children as reported by census numbers. Using Danish medical databases, we assessed patient characteristics including potential risk factors for AKI, such as use of nephrotoxic medication, surgery, sepsis, and perinatal factors. Results: In total, 14,200 children contributed with 16,345 AKI episodes over 15 years. The mean annual AKI rate was 148 (95% CI: 141–155) per 100,000 children. From 2007 to 2021, the annual AKI rate demonstrated minor year-to-year variability without any discernible overall trend. The highest AKI rate was recorded in 2007 at 174 (95% CI: 161–187) per 100,000 children, while the lowest rate occurred in 2012 at 129 (95% CI: 118–140) per 100,000 children. In 2021, the AKI rate was 148 (95% CI: 141–155) per 100,000 children. Characteristics of children with AKI were similar throughout the study period. Conclusion: The rate of AKI among Danish children was stable from 2007 to 2021 with little variation in patient characteristics over time. [ABSTRACT FROM AUTHOR]

Background: Adult kidney transplant recipients (KTRs) fully vaccinated against COVID‐19 have substantial morbidity and mortality related to SARS‐CoV‐2 infection compared with the general population. However, little is known regarding the safety and efficacy of the COVID‐19 vaccination series in pediatric KTRs. Methods: A multicenter, retrospective observational study was performed across nine pediatric transplantation centers. Eligible KTRs fully vaccinated against COVID‐19 were enrolled and data were collected pertaining to SARS‐CoV‐2 infection incidence and severity, graft outcomes and post‐vaccination safety profile, as well as overall patient survival. Results: A total of 247 patients were included in this investigation with a median age at transplantation of 11 years (IQR 5–15). SARS‐CoV‐2 infection was observed in 30/110 (27.27%) of fully vaccinated patients, tested post‐transplant, within the defined follow‐up period. Of these patients, 6/30 (18.18%) required hospitalization and 3/30 (12.12%) required reduction in immunosuppression, with no reported deaths. De novo donor‐specific antibodies (DSAs) were found in 8/86 (9.30%) of DSA‐tested patients with two experiencing rejection and subsequent graft loss. The overall incidence of rejection and graft loss among the total cohort was 11/247 (4.45%) and 6/247 (3.64%), respectively. A 100% patient survival was observed. Conclusions: Observationally, infectious outcomes of SARS‐CoV‐2 in fully vaccinated pediatric KTRs are excellent, with a low incidence of infection requiring hospitalization and no associated deaths. Though de novo DSAs were observed, there was minimal graft rejection and graft loss reported in the total cohort. [ABSTRACT FROM AUTHOR]

Background There is interest in identifying novel filtration markers that lead to more accurate GFR estimates than current markers (creatinine and cystatin C) and are more consistent across demographic groups. We hypothesize that large-scale metabolomics can identify serum metabolites that are strongly influenced by glomerular filtration rate (GFR) and are more consistent across demographic variables than creatinine, which would be promising filtration markers for future investigation. Methods We evaluated the consistency of associations between measured GFR (mGFR) and 887 common, known metabolites quantified by an untargeted chromatography- and spectroscopy-based metabolomics platform (Metabolon) performed on frozen blood samples from 580 participants in Chronic Kidney Disease in Children (CKiD), 674 participants in Modification of Diet in Renal Disease (MDRD) Study and 962 participants in African American Study of Kidney Disease and Hypertension (AASK). We evaluated metabolite–mGFR correlation association with metabolite class, molecular weight, assay platform and measurement coefficient of variation (CV). Among metabolites with strong negative correlations with mGFR (r < −0.5), we assessed additional variation by age (height in children), sex, race and body mass index (BMI). Results A total of 561 metabolites (63%) were negatively correlated with mGFR. Correlations with mGFR were highly consistent across study, sex, race and BMI categories (correlation of metabolite–mGFR correlations between 0.88 and 0.95). Amino acids, carbohydrates and nucleotides were more often negatively correlated with mGFR compared with lipids, but there was no association with metabolite molecular weight, liquid chromatography/mass spectrometry platform and measurement CV. Among 114 metabolites with strong negative associations with mGFR (r < −0.5), 27 were consistently not associated with age (height in children), sex or race. Conclusions The majority of metabolite–mGFR correlations were negative and consistent across sex, race, BMI and study. Metabolites with consistent strong negative correlations with mGFR and non-association with demographic variables may represent candidate markers to improve estimation of GFR. [ABSTRACT FROM AUTHOR]

Congenital nephrotic syndrome (CNS) is one of the important causes of end-stage kidney disease in children. Studies on the genotype, phenotype, and clinical outcome in infants with CNS caused by genetic mutations are scarce. We analyzed the genetic background, clinical manifestations, treatment response, and prognosis of pediatric patients with CNS in Taiwan. Fifteen infants with CNS were enrolled, and 11 patients of median age 21 (interquartile range 3∼44) days caused by genetic mutations from 10 unrelated families were included in the study. Of the eleven patients, 9 had extra-renal manifestations including microcephaly, facial dysmorphism, and skeletal anomalies. More than two-thirds of the patients had disease onset before 1 month of age. Diffuse meningeal sclerosis was the most common histological characteristic. Whole exome sequencing followed by direct Sanger sequence revealed mutations in OSGEP (R247Q), WT1 (R366H and R467Q), LAMB2 (Q1209∗ and c. 5432–5451 19 bp deletion), NUP93 (D302V), and LAGE3 (c.188+1G > A). Three of the variants were novel. Corticosteroids and/or immunosuppressants were administered in 2 patients, but both were refractory to treatment. During the mean 3.5 years of follow-up, all but two died of uremia and sepsis. The two survivors reached end-stage kidney disease and required peritoneal dialysis, and one of them underwent uneventful renal transplantation. The majority of patients with CNS in Taiwan were caused by OSGEP followed by WT1 mutation. R247Q is the hotspot mutation of OSGEP in Taiwan. CNS patients in Taiwan suffer from significant morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objective: To diagnose, treat, and prevent stone recurrence, it is important to determine the metabolic risk factors that play a role in developing urinary system stone disease in children. This study assessed children with urinary system stones’ clinical, radiological, and metabolic characteristics. Material And Methods: A retrospective study was conducted on the records of pediatric patients who applied to our pediatric outpatient nephrology clinic for various reasons between February 2018 and December 2023 and were diagnosed with urinary system stones. Results: Of the 122 patients with a mean age of 4.40±4.16 years (1 month-17 years), 63 (51.6%) were boys and 59 (48.4%) were girls. In 61.4% of the children, a family history was identified. The most common presenting symptom was abdominal/flank pain or restlessness (47.5%). In 25.4% of the patients, the stones were ≤3 mm (microlithiasis), and most stones were in the upper system. One or more metabolic abnormalities have been detected during urine analysis for 58.2% of patients. The most frequent metabolic abnormalities were hypercalciuria (20.5%) and hypocitraturia (17.2%). In 74.6% of patients, the size of stones decreased or completely disappeared with medical treatment based on underlying metabolic abnormalities, and in 17.2%, they did not change at all. Only eight (6.6%) patients required interventional procedures. Conclusion: Metabolic causes should be investigated first in all children with urinary tract stones. Special medical treatments designed to alter metabolism reduce the need for invasive stone procedures. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease has a detrimental impact on the overall quality of life (QoL) in children. The regular visits to doctors, prolonged reliance on medication, frequent hospital stays, and changes in their physical appearance increase their vulnerability to developing psychiatric problems and hinder their daily activities and social interactions, resulting in isolation, poor peer relations, and low self-esteem. These factors may compel these children to depend on digital media to find connections, regulate emotions, and cope with their illnesses. The study aims to determine the prevalence of electronic addiction among minors undergoing regular hemodialysis for end-stage kidney disease (ESKD) while simultaneously assessing their parental styles and psychological adaptation. A total of 60 subjects were enrolled in the study and divided into two groups. Group A: Thirty patients undergoing regular hemodialysis for ESKD. Group B: Thirty healthy subjects of matching age and sex. All the recruited participants were subjected to semi-structured clinical interviews according to our institute's sheets, Child Behavior Checklist (CBCL), Digital Addiction Scale for Children (DASC), Parent Authority Questionnaire (PAQ), and Psychological Adaptation Scale. Results: Patients patients had a significantly higher prevalence of digital addiction compared to healthy subjects, with an average score of (87.00 ± 21.23) in dialysis patients against (53.00 ± 25.20) in normal participants, demonstrating significant differences (p< 0.001). There was a significant correlation between the duration of dialysis treatment and the prevalence of digital addiction (r = -0.415, p-value = 0.023). A significant inverse correlation was found between digital addiction and the emotional subtype of the psychological adaption rating (r= -0.375, p= 0.041). Conclusion: The findings revealed that dialysis patients have a higher incidence of digital addiction. Prolonged dialysis duration was significantly associated with increased digital addiction among patients. [ABSTRACT FROM AUTHOR]

Congenital anomalies affecting the kidneys present significant challenges in pediatric nephrology, needing precise methods for assessing renal function and guiding therapeutic intervention. Bedside Schwartz formula with the cystatin-C-based Full Age Spectrum formula and Chronic Kidney Disease in Children (CKiD) U 25 formula used in estimating glomerular filtration rate (eGFR) and also to assess if the eGFR in association with kidney length percentiles can be a monitoring parameter for the progression of chronic kidney disease in children with congenital anomalies of the kidney and urinary tract (CAKUT). A total of 64 pediatric patients (median age at diagnostic was 12 months with an interquartile range of 2 to 60) were diagnosed with congenital anomalies in the kidney and urinary tract between June 2018 and May 2023 at "Louis Turcanu" Emergency Hospital for Children in Timisoara, Romania. Baseline characteristics, CAKUT types, associated pathologies, CKD staging, and eGFR using creatinine and cystatin C were analyzed. The mean age at the moment of examination was 116.50 months; (65, 180). Chronic kidney disease staging revealed a predominance of patients in CKD stages G1 and A1. Analysis of eGFR methods revealed a small mean difference between eGFR estimated by creatinine and cystatin C, with a moderate-strong positive correlation observed between the eGFR and ultrasound parameters. Using cystatin-C-based formulas for eGFR, in conjunction with ultrasound measurements, may offer reliable insights into renal function in pediatric patients with congenital anomalies affecting the kidney and urinary tract. However, the economic aspect must be taken into consideration because cystatin C determination is approximately eight times more expensive than that of creatinine. An interdisciplinary approach is crucial for managing patients with CAKUT. [ABSTRACT FROM AUTHOR]

Antibodies to angiotensin II type 1 receptor (AT1R‐Abs) are among the most well‐studied non‐HLA antibodies in renal transplantation. These antibodies have been shown to be common in pediatric kidney transplantation and associated with antibody‐mediated rejection (AMR), vascular inflammation, development of human leukocyte donor‐specific antibodies (HLA DSA), and allograft loss. As AT1R‐Ab testing becomes more readily accessible, evidence to guide clinical practice for testing and treating AT1R‐Ab positivity in pediatric kidney transplant recipients remains limited. This review discusses the clinical complexities of evaluating AT1R‐Abs given the current available evidence. [ABSTRACT FROM AUTHOR]

Background: There are scant data on the effect of rituximab on EBV DNA levels and prevention of post‐transplant lymphoproliferative disorder (PTLD) in pediatric kidney transplant recipients with EBV DNAemia. Methods: Kidney transplant recipients with EBV DNAemia treated with rituximab to prevent PTLD between 7/1999 and 7/2019 at five pediatric centers were included. Those with confirmed PTLD at the onset of rituximab were excluded. Primary outcomes included percentage change in EBV DNAemia and occurrence of PTLD post rituximab. Results: Twenty‐six pediatric kidney transplant recipients were included. Median age at transplant was 4 years (IQR 2.1–10.3). EBV DNA load monitoring by qPCR was performed at 1–3 month intervals. EBV DNAemia onset occurred at a median of 73 days post‐transplant (IQR 52–307), followed by DNAemia peak at a median of 268 days (IQR 112–536). Rituximab was administered at a median of 9 days post peak (IQR 0–118). Rituximab regimens varied; median dose 375 mg/m2 (IQR 375–439) weekly for 1–4 doses per course. Following rituximab, EBV DNA load decreased to <10% of baseline at 120 days in 20/26 patients; however, only 30% achieved complete resolution at last follow‐up (median 2094 days post‐transplant [IQR 1538–3463]). Two (7%) developed PTLD at 915 and 1713 days post rituximab. All recipients had functioning grafts. One death occurred in a child with PTLD following remission due to unrelated reasons. Conclusions: In the largest pediatric kidney transplant recipient case series with EBV DNAemia given rituximab to prevent PTLD, rituximab achieved a short‐term reduction in DNA load; however, recurrent DNAemia is common. [ABSTRACT FROM AUTHOR]