Pediatric Nephrology最新文献

Climate change represents a major threat to the health and wellbeing of children and families worldwide. Changes to the environment are already having and increasingly will have an impact on kidney health, particularly in pediatrics, where children are more vulnerable to heat injuries, environmental toxins, and issues related to access to care. At the same time, the environmental cost of healthcare is high, contributing substantially to greenhouse gas emissions, waste generation, and resource depletion. Within healthcare, the environmental impact of kidney care-particularly dialysis therapies-is disproportionately high, and children, who are initiated on dialysis therapies younger and will require treatments for longer periods, have an amplified contribution. Integrating sustainability into pediatric kidney care is not only a health imperative but it also reflects the values of the next generation. In this review, we discuss the effects of climate change on pediatric kidney health, the impact of dialysis therapies on the deteriorating environment, and strategies for incorporating sustainable health practices in the pediatric setting.

Background: Breakthrough urinary tract infection (BT-UTI) among children with vesicoureteral reflux (VUR) receiving continuous antibiotic prophylaxis (CAP) remains a clinical challenge with heterogeneous risk profiles and no validated prediction tools. We aimed to identify robust risk factors and develop an evidence-based predictive score for BT-UTI.

Methods: We conducted a systematic review and meta-analysis following PRISMA and Cochrane guidance, searching PubMed, Web of Science, and Embase for cohort studies. Pooled effect estimates were calculated using appropriate fixed- or random-effects models; a scoring system was derived by natural logarithm transformation of pooled estimates. External validation used a cohort of 158 children with VUR receiving CAP at Children's Hospital of Chongqing Medical University (2021-2024).

Results: Twenty-four cohort studies (n = 3,264) were included; pooled BT-UTI incidence was 30.5%. From 26 candidate factors, eight stable predictors were retained and incorporated into the score: age < 1 year (OR 2.04), bilateral reflux (OR 1.81), high-grade reflux IV-V (OR 2.65), symptomatic UTI (OR 2.74), history of recurrent UTI (OR 2.17), bladder and bowel dysfunction (BBD; OR 1.81), increased ureteral diameter ratio (UDR; MD 0.139), and DMSA renal scar formation (OR 4.40). External validation showed AUC 0.88 (95% CI 0.82-0.93); at a cutoff of 17 points accuracy was 0.84, and sensitivity 0.93. Calibration and decision-curve analyses indicated good agreement and a positive net clinical benefit.

Conclusions: We developed and externally validated an eight-item BT-UTI risk score for children with VUR on CAP that demonstrates high discrimination and clinical utility for individualized risk stratification and prevention planning and implementation guidance available.

Background: Cystinuria is a rare genetic disorder that causes recurrent cystine stones and significant morbidity, particularly in children. Tiopronin, the main pharmacologic therapy when conservative measures fail, reduces cystine precipitation and excretion; however, real-world safety data remain scarce.

Methods: We evaluated tiopronin's adverse-event profile using the FDA Adverse Event Reporting System (FAERS) by reviewing reports from Q1 2014 to Q1 2025. After deduplication per FDA guidance, only cases in which tiopronin was recorded as the primary suspect drug were included. Adverse events were coded using MedDRA version 28.0 at the Preferred Term and System Organ Class levels, and disproportionality analysis was performed with Reporting Odds Ratio, Proportional Reporting Ratio, Bayesian Confidence Propagation Neural Network, and Multi-item Gamma Poisson Shrinker algorithms.

Results: A total of 1,838 unique cases were identified, with 69.6% involving pediatric patients. Most reports originated from the United States (99.4%) and were submitted by physicians (86.5%). Significant safety signals emerged in seven System Organ Classes and 67 Preferred Terms. In addition to expected cystinuria-related events, we detected unlabelled signals such as hyposmia, skin atrophy, breath odour, tongue discoloration, and incorrect dosage administered, the latter being particularly relevant in children.

Conclusion: This first FAERS-based pharmacovigilance study of tiopronin identified both known and novel safety signals, underscoring challenges in pediatric dosing and the need for enhanced pharmacovigilance, accurate treatment oversight, and improved education for families and clinicians. Limitations include underreporting, incomplete data, and potential confounding by indication.

Background: The link between obesity, urine metabolic disturbances, and the development of kidney stones in children and adolescents remains controversial.

Objectives: The objective of this work is to identify the association between BMI, urine metabolic disturbance, and pediatric nephrolithiasis.

Data sources: PubMed, Embase, Web of Science, Cochrane Library, and Scopus databases were searched from inception to June 30, 2023.

Study eligibility criteria: Case-control, cohort, and cross-sectional studies with nephrolithiasis in patients aged 21 years or younger, with the topic of BMI and urine metabolic disruption were included.

Participants and inventions: Children and adolescents with nephrolithiasis are the participants.

Study appraisal and synthesis methods: We assessed the quality of the included studies using the AHRQ and NOS assessment, but the heterogeneity of the studies did not allow for data synthesis and meta-analyses.

Result: Hypercalciuria, hypocitraturia, and hyperoxaluria are the most prevalent in pediatric nephrolithiasis. Obese pediatric patients would have different urinary mineral profiles depending on the region, dietary habits, and heritability. Also, we found that children of different ages may have varying risk factors for stones. Increasing fluid intake is a simple and affordable strategy for preventing kidney stones, as recommended for children. Improving poor nutritional habits would aid in the decrease of metabolic variables involved in stone formation.

Conclusions and implications of key findings: Obesity alone may not contribute to stone formation in pediatrics, but a combination of metabolic factors, different urinary mineral profiles, and abnormal metabolic status may predispose to stones in children, owing to children tending to undergo rapid changes in growth in a relatively short period, and partly due to different risk factors for stones in different regions. In addition to BMI, other indicators such as %BF (percent body fat) and BRI (body roundness index) may be used to comprehensively assess nutritional status and metabolism in children. Evaluating individual metabolic profiles and promoting increased fluid intake are simple, cost-effective strategies for preventing kidney stones in this population.

Systematic review registration number: CRD4202456817.

Background: Kidney failure (KF) in children and adolescents leads to reduced lifespan and compromised health. Alport syndrome (AS) is a leading hereditary cause of KF in children. Angiotensin-converting enzyme inhibitors (ACEi) have demonstrated efficacy in delaying KF in young people living with AS, but non-adherence can compromise their therapeutic benefits. To investigate the adherence to ACEi in children and adolescents with AS, a liquid chromatography-mass spectrometry (LCMS)-based method was developed for objective verification of recent medication intake at two different time points in this cohort study.

Methods: Urine samples from 58 children enrolled in the EARLY PRO-TECT Alport trial were analyzed. An LCMS-based method was established and validated to simultaneously screen and quantify both ramipril and ramiprilat in urine samples. Participants were not informed in advance of the medication intake measurements.

Results: A total of 106 urine samples from 58 patients with early stages of chronic kidney disease (mean estimated glomerular filtration rate, 130 ± 32 mL/min/1.73 m²) were analyzed at two different time points. All 13 negative control samples (100%; 95% confidence intervals [CI] 75.7% to 100%) were identified correctly. Adherence to ACEi at the time of sampling was consistently high, with 96% (47/49; 95% CI 86% to 99.5%) and 95% (42/44; 95% CI 84.5% to 99.4%) of children showing confirmed drug intake at initial and second adherence measurements.

Conclusion: This study demonstrated that children with chronic kidney disease, when treated with ACEi within a clinical trial, show high adherence to the prescribed medication.

Background: The objective was to compare the efficacy of single-dose versus double-dose rituximab (RTX) regimens in children with frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS).

Methods: This retrospective study analyzed 53 pediatric FRNS/SDNS patients treated at Guangzhou First People's Hospital from January 2020 to November 2024. Patients received either single-dose (n = 36, one RTX infusion at month 0) or double-dose (n = 17, two infusions at 0 and 3 months) RTX (375 mg/m², maximum 500 mg/dose). The RTX dosing regimen was determined based on disease severity, socioeconomic factors, and physicians' medication preferences, rather than randomization. Some patients were receiving baseline therapy with prednisone, mycophenolate mofetil, and tacrolimus. Both groups underwent steroid tapering and were followed up for at least 6 months, with 45 patients completing 12-month follow-up. Primary endpoints were relapse frequency and cumulative prednisone usage.

Results: At the 6-month and 12-month follow-ups, there were no differences in relapse rates between the single-dose and double-dose groups (31% (11/36) vs. 47% (8/17) at 6 months; 36% (13/36) vs. 53% (9/17) at 12 months). Furthermore, at the 12-month follow-up, no differences were observed between the single-dose and double-dose groups in the annual relapse frequency (0.7 ± 1.1 vs. 1.0 ± 1.2 times/year) or the cumulative prednisone usage (0.21 ± 0.12 vs. 0.22 ± 0.14 mg/(kg·d)). Kaplan-Meier analysis revealed no difference in relapse-free survival between groups (log-rank test, χ² = 2.561, P = 0.110). At 6 months, 19% (7/36) of single-dose and 24% (4/17) of double-dose patients were CD19 +B cell depleted (< 1%). No major adverse events were seen in either group.

Conclusion: Single-dose and double-dose RTX regimens demonstrate comparable efficacy in maintaining remission in pediatric FRNS/SDNS patients over 12 months. The extended-interval double-dose regimen provides no significant clinical advantage, suggesting that a single-dose strategy may be sufficient while reducing treatment costs.

Background: Acute kidney injury (AKI) is common in very-low-birthweight (VLBW) infants. Both fluid overload and dehydration can lead to AKI. Our aim was to examine the associations between early fluid and sodium intake and AKI.

Methods: This retrospective cohort study comprised 421 VLBW infants born at < 32 weeks. Detailed data on fluid management during the first 24 h of life, diuresis and weight changes during the first postnatal week and plasma creatinine measurements during the first 2 postnatal weeks were acquired from an electronic patient information system. AKI was defined according to the KDIGO definition modified for neonates.

Results: The incidence of AKI was 8.6%, and on average, it was diagnosed on the fifth postnatal day. Higher total fluid intake (mL/kg/24 h) during the first day of life was associated with an increased risk of AKI (OR, 1.015; 95% CI, 1.005-1.025; p < 0.01), analysis adjusted for gestational age and being small-for-gestational age. The highest fluid intake quartile had a 5.6-fold risk of developing AKI when compared with the lowest quartile (p = 0.01). A higher total sodium intake (mmol/kg/24 h) was associated with an increased risk of AKI (OR, 1.12; 95% CI, 1.03-1.21; p < 0.01). Among infants with AKI, a substantial proportion of early fluid and sodium intake (median 27% and 59% of total intake, respectively) originated from volume expanders. Infants diagnosed with AKI exhibited an average weight gain of + 2.8% by the second day of life.

Conclusions: As part of efforts to minimize the risk of AKI, avoiding excessive fluid and sodium administration during the first postnatal hours may be beneficial.

Background: Kidney replacement therapy (KRT) is commonly used to treat critically ill children for a variety of reasons, including hyperammonemia. KRT management in children with hyperammonemia not due to inborn errors of metabolism is challenging.

Case presentation: We report a complex case of hyperammonemia in a 17-year-old critically ill female patient, emphasizing the challenges of management in a pediatric intensive care setting. Despite the initiation of kidney replacement therapy (KRT) and progressive increases in the prescribed dialytic dose, the patient's ammonia levels continued to escalate. This prompted a reevaluation of her metabolic needs, with a focus on optimizing glucose delivery to facilitate ammonia metabolism and dialytic clearance. Adjustments to increase the delivered glucose, along with careful monitoring of glucose removal during KRT, ultimately led to the stabilization of her ammonia levels.

Conclusion: This case underscores the intricate interplay between metabolic support and dialytic strategies in the management of hyperammonemia. The use of a glucose delivery calculator is proposed. This case highlights the need for individualized, dynamic approaches in critically ill pediatric patients.