Pediatric Nephrology最新文献

Maternal undernutrition during pregnancy is associated with adverse effects in the offspring during adulthood and contributes to the risk of developing a number of chronic diseases. Historical events, such as famines, allow us to study the effects that food deprivation in utero has on the offspring's health. In particular, the Dutch Hunger Winter (1944-1945) and the Great Chinese Famine (1959-1961) have been extensively analysed, and it has been shown that prenatal exposure to starvation increases the risk of cardiometabolic, mental and kidney disease in adult life. More importantly, the risk can be transmitted to future generations. However, not all studies agree on the thresholds of risk of exposed subjects or on the timing of starvation during foetal life that could be held responsible for these deleterious lifelong consequences. Gender differences complicate the picture. In this narrative review, we discuss similarities and differences between the two famines and compare the available data, seeking to determine what can be learned from these tragedies.

Background: C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce.

Methods: Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6 months, 12 months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3 mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3 mg/mg with normal eGFR. Lack of remission was defined as non-response.

Results: A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8 years and of 78% at 7 years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400 ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR.

Conclusions: Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.

Background: Isotonic fluids are becoming the standard for hydration and maintenance fluid therapy, but there is no consensus on the optional choice among the different types of isotonic solution.

Methods: This study is a single-center, non-randomized controlled trial at Kobe University Hospital, Japan, between April 2021 and March 2023. The study included pediatric patients aged 1-19 years who underwent kidney biopsies. From April 2021 to March 2022, 0.9% sodium chloride (saline) was administered, and from April 2022 to March 2023, balanced crystalloids were used. The primary outcome was the occurrence of hyponatremia (< 137 mEq/L) after a kidney biopsy. Secondary outcomes included other electrolyte balances, blood gas parameters, creatinine-based estimated glomerular filtration rate (Cr-eGFR), and arginine vasopressin concentrations (UMIN Clinical Trial Registry: UMIN 000044330).

Results: Of 61 patients enrolled, 2 were excluded, leaving 34 in the saline group and 25 in the balanced crystalloid group. No hyponatremia occurred, and serum sodium concentrations were similar between both groups (138.7 vs. 138.9 mEq/L, P = 0.08). The saline group showed a greater increase in serum chloride (+ 1.7 vs. + 0.2, P < 0.01) and a greater decrease in HCO₃^- concentrations (- 0.6 vs. + 0.9, P < 0.01). There were minimal changes in pH (- 0.01 vs. - 0.01, P = 0.99) and Cr-eGFR (- 1.5 vs. + 1.1 mL/min/1.73 m², P = 0.96) in both groups.

Conclusions: During pediatric kidney biopsy, both saline and balanced crystalloids were effective in preventing hyponatremia. Although saline infusion results in higher serum chloride concentrations and lower blood HCO₃^- concentrations than balanced crystalloids infusion, the clinical significance was minimal.

Background: Acute kidney injury (AKI) is common in critically ill children and associated with adverse short-term outcomes; however, long-term outcomes are not well described.

Methods: This longitudinal prospective cohort study examined the prevalence of chronic kidney disease (CKD) and hypertension (HTN) 11 vs. 6 years after pediatric intensive care unit (PICU) admission and association with AKI. We examined children (age < 19 years) without pre-existing kidney disease 11 ± 1.5 years after PICU admission at a single center. AKI was defined using serum creatinine criteria. The primary outcome was a composite of CKD or HTN. CKD was defined as estimated glomerular filtration rate (eGFR) < 90 mL/min/1.73 m² or albuminuria. Multivariable analyses compared outcomes at 11- vs. 6-year follow-up and association with AKI during PICU admission.

Results: Of 96 children evaluated 11 years after PICU admission, 16% had evidence of CKD or HTN (vs. 28% at 6 years, p < 0.05). Multivariable analysis did not show improvement in outcomes from 6- to 11-year follow-up. eGFR decreased from 6- to 11-year follow-up (adjusted coefficient - 11.7, 95% CI - 17.6 to - 5.9) and systolic and diastolic blood pressures improved. AKI was associated with composite outcome at 6-year (adjusted odds ratio (aOR) 12.7, 95% CI 3.2-51.2, p < 0.001), but not 11-year follow-up (p = 0.31). AKI was associated with CKD (aOR 10.4, 95% CI 3.1-34.7) at 11 years.

Conclusions: This study provides novel data showing that adverse kidney and blood pressure outcomes remain highly prevalent 10 years after critical illness in childhood. The association with AKI wanes over time.

Background: Steroid-sparing immunosuppression is used in 50% of children with nephrotic syndrome, to prevent relapses and steroid-related toxicity. However, rates and predictors of prolonged remission after cyclophosphamide and tacrolimus are uncertain.

Methods: Retrospective analysis of children (1-18 years) enrolled in a longitudinal cohort. We included children diagnosed with steroid-sensitive nephrotic syndrome between 1996-2019 from Toronto, Canada. The exposure was cyclophosphamide or tacrolimus initiation. The primary outcome was prolonged remission (no further relapse or steroid-sparing immunosuppression). We evaluated predictors of prolonged remission and calcineurin inhibitor nephrotoxicity by logistic regression.

Results: Of 578 children with steroid-sensitive nephrotic syndrome, 252 received cyclophosphamide and 120 received tacrolimus. Over median 5.4-year (IQR 2.4-9.1) follow-up, prolonged remission occurred in 72 (28.6%) after cyclophosphamide and 17 (14.2%) after tacrolimus. Relapse frequency decreased after initiation of either medication. Lower prior relapse rate, more recent treatment era, and female sex were predictive of prolonged remission after cyclophosphamide treatment. Use of tacrolimus as the first steroid-sparing medication was the only factor predictive of calcineurin inhibitor nephrotoxicity.

Conclusions: Less than one-third of children achieve prolonged remission after initiating cyclophosphamide or tacrolimus, although both reduce short-term relapse rates. Few factors predict prolonged remission after cyclophosphamide or tacrolimus use, or calcineurin inhibitor nephrotoxicity.

Background: Central venous catheter (CVC)-related bloodstream infections (CRBSI) are common in children receiving hemodialysis and cause significant morbidity and healthcare costs. Unlike standard locking solutions, 4% tetrasodium EDTA (KiteLock™) has antimicrobial and antibiofilm properties. We aimed to study the safety and efficacy of 4% tetrasodium EDTA CVC locking in pediatric hemodialysis.

Methods: Single-center, before-and-after quality improvement study. We included all chronic hemodialysis patients (6 months-18 years) from 2016-2022 (before) to 2022-2024 (after). The standard CVC locking solution was changed from heparin (1000 units/mL) to 4% tetrasodium EDTA. We compared unit-level incidence of CRBSI, CVC replacement procedures (exchange or removal and reinsertion), laboratory results, alteplase use, and adverse events before and after 4% tetrasodium EDTA implementation.

Results: We included 22 pediatric chronic hemodialysis patients (median age 13.5 years, 50% female). CRBSI incidence was 0.89 infections per 1000 catheter-days (25,769 total catheter-days) before and 0.18 per 1000 catheter-days (5426 total catheter-days) after 4% tetrasodium EDTA (IRR 0.21, 95%CI 0.03-1.52). CVC replacement procedure incidence was 1.99 procedures per 1000 catheter-days (4027 total catheter-days) before and 1.29 per 1000 catheter-days (5426 total catheter-days) after 4% tetrasodium EDTA (IRR 0.65, 95%CI 0.24-1.79). There were no significant differences in hemodialysis treatment parameters, alteplase use (12% of treatments before vs. 18% after), or access complications (12% of treatments before vs. 15% after).

Conclusions: CVC locking with 4% tetrasodium EDTA was associated with sustained reductions in CRBSI and CVC replacement procedure incidence. Incorporation of 4% tetrasodium EDTA locking into standardized CVC care bundles may prolong vascular access survival.