Pediatric Nephrology最新文献

Background: Hyperkalemia following nephrectomies in children is a poorly studied, potentially life-threatening complication. We describe the frequency and risk factors for hyperkalemia in pediatric patients who underwent nephrectomies resulting in an anephric state.

Methods: This was a single center retrospective cohort study of patients < 21y who underwent nephrectomies resulting in an anephric state. Possible predictors included age, sex, preoperative potassium value, preoperative dialysis modality, preoperative urine output, procedure type, surgical approach, intraoperative lactated Ringer's (LR), and intraoperative blood. The primary outcome was intraoperative and postoperative day 0 and day 1 hyperkalemia. Hyperkalemia was defined as potassium > 5.1 mEq/L (severe > 5.9) in children ≥ 1y and > 5.5 (severe > 6.1) in children < 1y. Treatments for hyperkalemia were recorded. We examined associations with hyperkalemia using Wilcoxon rank-sum test, Pearson's Chi-squared test, or Fisher's exact test. We used Youden's Index to determine the preoperative potassium cutoff for predicting hyperkalemia.

Results: Among 46 patients who underwent nephrectomies resulting in an anephric state, 23 (50%) experienced hyperkalemia that was severe in 18 (39%), treated in 21 (46%), and precipitated transfer to the intensive care unit in 5 (11%). In unadjusted analysis, higher preoperative potassium (p < 0.001) and intraoperative LR (p = 0.018) were associated with a higher risk of postoperative hyperkalemia. A preoperative potassium of >3.85 mEq/L was associated with the development of hyperkalemia (p < 0.001).

Conclusions: Clinically important hyperkalemia is a common complication following nephrectomy that results in an anephric state. Targeting a preoperative potassium < 3.85 mEq/L may decrease intraoperative and postoperative hyperkalemia.

Background: Limited data exist on the use of novel iron therapies in children with chronic kidney disease (CKD). We conducted a cross-over study to compare iron polymaltose complex (IPC) and liposomal iron in pediatric patients with CKD and iron deficiency anemia (IDA).

Methods: Cross-over study of 33 children with CKD and IDA was conducted. They were randomized into 2 groups (group A: 17 patients, group B: 16 patients) to receive either liposomal iron or IPC for 3 months. After an 8-week washout period, they were switched to the other therapy. Red cell and iron indices, as well as bone minerals and 25(OH)D₃, were measured at baseline and after each 3-month period. A follow-up visit was conducted at 4 weeks during the treatment period to report any possible adverse events.

Results: Hb levels increased by at least 1 g/dL in 48% following liposomal iron therapy and 51.5% following IPC therapy. There was no statistically significant difference in ΔHb, ΔFe, ΔsTR (transferrin receptor), or ΔTSAT (transferrin saturation) levels between the groups (p > 0.05). By mixed model analysis, IPC showed a higher Hb and TSAT and lower TRresponse compared with liposomal iron. IPC, but not liposomal iron, led to a significant reduction in serum phosphorus in both groups. Thirty-six percent of IPC recipients experienced adverse effects, compared to 3% of liposomal iron recipients.

Conclusions: Both IPC and liposomal iron effectively improved iron status in children with CKD and IDA. However, IPC indicated a superior response, whereas liposomal iron was associated with a more favorable tolerability profile.

Background: APOL1 high-risk variants predispose to chronic kidney disease (CKD) in individuals of African genetic ancestry due to podocyte toxicity. As congenital anomalies of the kidney and urinary tract (CAKUT) have variable outcomes potentially driven by podocyte injury, we hypothesize that the outcome of children with CAKUT is influenced by APOL1 risk genotypes.

Methods: APOL1 risk status was determined in children and adults with CAKUT from African genetic ancestry using DNA microarrays or exome sequencing. Phenotypic information and CKD outcomes at last follow-up were collected. We computed odds ratios (OR) and hazard ratios between APOL1-high risk (HR) vs. low risk (LR) carriers under different models using logistic regression and Cox proportional hazards.

Results: Clinical data were available for 195 patients (median age 15.35 years, [IQR 11.01-19.87]), including 20 (10.3%) carrying APOL1-HR. Under a recessive model, APOL1-HR vs. LR showed no differences in proteinuria or hypertension (OR 0.24, 95% CI 0.04-1.30, p = 0.10 and OR 0.99, 95% CI 0.38-2.54, p = 0.98, respectively), nor in CKD stage ≥ G2 risk (OR 0.71, 95% CI 0.29-1.78, p = 0.47). Median time until kidney failure for APOL1 risk groups was 11.59 years [IQR 7.15-21.47] and 14.35 years ([IQR 9.16-17.20]; p = 0.73) for HR and LR, respectively. Additional exploratory analyses showed comparable outcomes between HR and LR variant carriers.

Conclusions: This pilot study identified no association between APOL1 risk genotypes and kidney outcomes in patients with CAKUT across genetic models. With APOL1-targeted therapies emerging, large-scale prospective studies are needed to identify individuals with CAKUT who may benefit from these treatment strategies.

Background: Accurate volume assessment is essential in the management of children on dialysis as excess volume is a key cause of arterial hypertension and is associated with the development of hypertension-mediated organ injury. Our objective was to describe NT-proBNP concentrations in a cohort of children receiving in-centre chronic intermittent haemodialysis with preserved systolic cardiac function and to evaluate relevant associations.

Methods: This was a retrospective study including prevalent children aged < 18 years who were established on IHD and had preserved systolic cardiac function.

Results: There were 24 children, mean age 10.3 ± 5.8 years, of whom 8 had urine output < 150 mL/day, median dialysis vintage 130.3 ± 364.8 days. There were 161 post-IHD NT-proBNP measurements performed, of which 130 measurements (80.7%) were abnormal when defined as a concentration above 598 ng/L; median (IQR) 2046 (794, 5275) ng/L [Log 3.34 ± 0.58 ng/L]. Children with urine output > 150 mL/day had a mean ± SD, log NT-proBNP of 3.25 ± 0.51 ng/L and those with < 150 mL/day 3.43 ± 0.65 ng/L (P = 0.047). Following multivariable regression analysis, log NT-proBNP positively associated with systolic blood pressure z-score (SBP_z-POST) (β = 0.15, P < 0.01) and non-White ethnicity (β = 0.10, P = 0.02) and negatively with UO > 150 mL/day (β = -0.28, P < 0.01) and haemoglobin concentrations (β = -0.01, P = 0.01). There was no significant association between log NT-proBNP with primary kidney disease, sex or interdialytic weight gain.

Conclusions: We observed most post-haemodialysis NT-proBNP measurements to be abnormal despite preserved systolic cardiac function in children established on IHD. The utility of a single measurement remains limited and serial measurements and improved understanding of reasons for variation in intra-patient measurements may be more useful when used alongside standard clinical parameters in this population.

Acute kidney injury (AKI) is a frequent and severe condition in hospitalized children, leading to significant morbidity, mortality, and long-term risk of chronic kidney disease. This review explores the gut-kidney axis, a concept describing the bidirectional relationship between the gut microbiome and kidney function, as a critical driver of pediatric AKI. In critically ill children, interventions such as broad-spectrum antibiotics and necessary nutritional support strategies (e.g., parenteral nutrition or fasting) can cause profound gut microbial imbalance (dysbiosis). This dysbiosis initiates a deleterious feedback loop, exacerbating kidney injury. Key mechanisms include the disruption of the intestinal barrier (leaky gut), which allows bacterial endotoxins to enter the bloodstream, triggering renal inflammation via Toll-like receptor 4 signaling. Concurrently, the dysbiotic gut increases production of directly nephrotoxic gut-derived uremic toxins, such as indoxyl sulfate and p-cresyl sulfate, while failing to produce protective anti-inflammatory metabolites like short-chain fatty acids. While therapies targeting the microbiome, such as probiotics, prebiotics, and fecal microbiota transplantation, are theoretically promising, their clinical use in pediatric AKI is unsupported by evidence and carries substantial risks, particularly iatrogenic infection. A significant knowledge gap exists due to a relative lack of pediatric-specific clinical research. The conclusion emphasizes an urgent need for longitudinal, multi-omics studies in children to understand this axis, identify functional biomarkers, and develop safe, targeted therapies to improve outcomes.

Background: Recent technologic advances in neonatal dialysis have changed current dialysis practices. The goal of this study was to define demographics, diagnoses, initial dialysis modality, modality changes, and outcomes of neonates receiving dialysis.

Methods: Retrospective, multicenter cohort of neonates (≤ 30 days of age) from 26 U.S. centers, who received dialysis between 6/2017 and 5/2022. Measures of central tendency were calculated to describe the cohort stratified by the primary dialysis-related diagnosis including acute kidney injury requiring dialysis (AKI-D), stage 5 chronic kidney disease (CKD 5), hyperammonemia, and other causes. The primary outcome was death during the initial hospitalization.

Results: For the 405 neonates in this cohort, AKI-D (57%) was the most common dialysis-related diagnosis, followed by CKD 5 (29%) and hyperammonemia (11%). Most neonates (58%) had a birth weight between 2500 and 3500 g. The most common initial neonatal dialysis modality was continuous kidney replacement therapy (CKRT) with (35%) or without extracorporeal membrane oxygenation (ECMO) (36%). Only 26% of neonates received peritoneal dialysis (PD) as their initial dialysis modality. Thirty percent of neonates received more than one dialysis modality. The overall mortality was 50% (59% in AKI-D and 41% in CKD 5). Withdrawal of care was reported as the most common reason for death. The odds of death were lower for neonates with AKI-D receiving PD as the initial modality when compared to CKRT (aOR 0.28, 95% CI 0.09-0.84) or CKRT with ECMO (aOR 0.24, 95% CI 0.10-0.58), but this association was not seen in neonates with CKD 5.

Conclusions: This multicenter, contemporary neonatal dialysis cohort shows increased use of CKRT as the initial dialysis modality but also shows that the mortality of neonates on dialysis remains high. Additional studies on modern neonatal dialysis are needed.

Pediatric nephrology patients have high rates of treatment nonadherence, thus increasing their risk for poor health outcomes. Key modifiers of adherence among pediatric nephrology patients include local environment (place of residence, school setting, clinic setting), socioeconomic status, and health literacy. Where patients live affects access to health care, community, and school resources that support adherence and disease management. Socioeconomic challenges, such as lower income, food insecurity, and housing instability, may lead patients and families to prioritize immediate survival needs over adherence. Lower health literacy is common among pediatric nephrology patients and their caregivers and may contribute to difficulties communicating with care teams, understanding health information, and implementing medical recommendations. This educational review aims to (1) conceptualize local environment, socioeconomic status, and health literacy, as well as their interconnections, within an established socio-ecological framework of the individual, family, community, and health care system factors impacting pediatric adherence, (2) synthesize recent literature on the influence of these domains on adherence in pediatric nephrology, and (3) highlight strategies to mitigate barriers within each domain and promote adherence among pediatric nephrology patients and their families.

Background: Ravulizumab drug monitoring has not been explored for maintenance therapy in patients with complement-mediated (atypical) hemolytic uremic syndrome (aHUS). Phase III trials suggest the standard dosing regimen provides troughs about threefold higher than needed to suppress complement activity. We describe the use of ravulizumab in pediatric patients with aHUS in remission, exploring potential modified dosing strategies based on serum drug levels and complement markers.

Methods: This single-center, retrospective cohort study included pediatric patients with aHUS in remission receiving outpatient ravulizumab infusions between June 30, 2023, and March 31, 2024, with at least one ravulizumab trough. Patients received a standard (SR) or a modified (MR) regimen, determined by the nephrologist. The primary outcome was to describe troughs and corresponding AH50 for patients on at least two equal doses. Secondary outcomes included comparison of troughs by regimen, intra-patient variability, possible adverse drug events (pADE), and drug costs.

Results: Nine patients were included. The mean ravulizumab trough level was 399.1 (± 107.3) mcg/mL. All patients exceeded the goal of 175 mcg/mL and achieved AH50 < 10%. Four patients (44%) received ravulizumab according to a MR. No difference was observed in ravulizumab trough levels between SR and MR groups (P = 0.67). Patients with multiple troughs showed low intra-patient variability (CV < 25%). pADE rates were similar across regimens, and MR was associated with lower drug costs.

Conclusions: Individualized maintenance regimens of ravulizumab based on trough and complement monitoring appear safe and effective while reducing drug costs. Further study is needed to define the optimal ravulizumab maintenance dosing strategy.