Pediatric Nephrology最新文献

Background: The native kidney biopsy is an important diagnostic procedure in pediatric nephrology. Recent meta-analyses did not find the size of the needle as a risk factor for bleeding complications, but they were predominantly based on adult studies. There are few papers comparing the safety and core adequacy in pediatric native kidney biopsy.

Methods: We present a large single-center retrospective study performed in a tertiary pediatric nephrology center. Data of children who received a real-time ultrasound-guided native kidney biopsy with a 16- or an 18-gauge needle from 2018 to 2024 were analyzed.

Results: Overall, 1040 children (644 boys) were included, with a median age of 10.25 (6.6; 14.23) years. One hundred three (9.9%) patients experienced bleeding complications. Perinephric hematoma was reported in 86 (8.3%) cases, gross hematuria in 18 (1.7%), and 3 (0.3%) children required transfusion. Multivariate regression analysis revealed the needle size (OR for 16-gauge 2.06, 95% CI 1.22-3.47, p = 0.007) as a risk factor for complications in the overall cohort and in children under 12 years old. The needle size did not affect complication rates in children aged 12-18 years. Inadequate kidney cores were reported in 37 (4.5%) cases; OR for 18-gauge needles (OR 5.08, 95% CI 1.07-24.21, p = 0.041) was found.

Conclusions: Use of a 16-gauge needle reduces the risk of obtaining an inadequate core in comparison with an 18-gauge. An 18G needle has a safety advantage over a 16G needle in children younger than 12 years. A 16G needle is as safe as an 18G needle and should be used for native kidney biopsy in children older than 12 years.

Background: Accurate glomerular filtration rate estimation (eGFR) is essential for managing pediatric kidney transplant recipients. Given the physiology of pediatric patients receiving adult-donor kidneys, identifying the most appropriate plasma creatinine (PCr)-based formula-pediatric or adult-specific-is crucial.

Methods: This cross-sectional study included pediatric kidney transplant recipients (age 1-18 years) who received adult-donor kidneys. We compared agreement thresholds of various pediatric and adult PCr-based GFR equations with CKiD 2012 combined PCr‒cystatin C (PCr-CystC) equation via intraclass correlation coefficients (ICCs), concordance correlation coefficients (CCCs), total deviation index (TDI), P30 performance metric (P30), Bland-Altman plots, and receiver-operating characteristic (ROC) analysis. Correlation between CKiD under 25 (U25) PCr-CystC and reference CKiD 2012 equation was also evaluated.

Results: One hundred twenty samples were collected from 23 recipients (mean age = 14.2 ± 3.4 years) and donors (mean age = 31.7 ± 10.0 years). Schwartz-Lyon equation demonstrated the highest performance with the reference (ICC = 0.913, CCC = 0.911, TDI = 14.0 mL/min/1.73 m², P30 = 99.2%). U25 (ICC = 0.922, CCC = 0.882, P30 = 93.3%), full age spectrum (FAS)-height (ICC = 0.897, CCC = 0.877, P30 = 96.7%), and Bedside Schwartz equations (ICC = 0.850, CCC = 0.819, P30 = 89.2%) showed comparable performance. Bland-Altman plots revealed proportional bias (p < 0.05), leading to ROC analysis, which identified eGFR < 70 mL/min/1.73 m² for Schwartz-Lyon, U25, and FAS-height, and < 60 mL/min/1.73 m² for Bedside Schwartz as optimal agreement thresholds, beyond which each equation showed increased bias. Subgroup analyses also showed better performance in patients aged 10-18 years. Additionally, U25 PCr-CystC equation showed excellent agreement with the reference (ICC = 0.993, CCC = 0.990, P30 = 100%).

Conclusions: Schwartz-Lyon equation demonstrated the highest performance among PCr-based equations with the reference in pediatric kidney transplant recipients, particularly when eGFR was < 70 mL/min/1.73 m² and in patients aged 10-18 years. U25 PCr-CystC equation showed best overall agreement with the reference and should be preferred where CystC measurement is feasible.

Background: Measures of early postnatal fluid balance may be associated with severe intraventricular hemorrhage (sIVH) and/or death in extremely preterm infants in the first postnatal week.

Methods: A single-center, retrospective cohort study including actively treated inborn infants weighing ≥ 400 g and 22-27 weeks' gestation from 2014-2021. Longitudinal mixed effect models compared daily fluid balance covariates including serum sodium, percent weight change, total fluid intake, urine output, and fluid balance (daily weight - birth weight /birth weight × 100) among infants with and without sIVH or death, during the first seven postnatal days. Multiple regression and machine learning models were developed to predict sIVH and/or death. Variables that were incorporated into the models included measures of fluid balance, gestational age, birth weight, antenatal corticosteroids, multiples, and sex.

Results: We included 932 infants with mean ± SD gestational age of 25w2d ± 11d and birth weight of 746 ± 212 g of whom 195 (20.9%) had sIVH and/or death. Lower percentage weight change (p < 0.001), higher total fluid intake (p = 0.007), higher sodium (p = 0.007), and positive early fluid balance (p < 0.001) were associated with sIVH and/or death even after adjustment for baseline characteristics. The area under the receiver-operating curve (AUC) for regression models predicting sIVH and/or death incorporating baseline characteristics improved after adding fluid balance measures from 0.75 to 0.80, while the AUC for machine learning models improved from 0.72 to 0.84.

Conclusions: In extremely preterm infants, early fluid status measures were associated with risk of sIVH and/or death. The addition of fluid status measures improves the performance of models predicting sIVH and/or death.

Virtually every protein in our bodies binds protons, resulting in a specific electrical charge. If the surrounding proton concentration changes, such as in acidosis or alkalosis, the charge of the protein can change, as well, which in turn can affect protein folding and thus function. Therefore, disturbances of acid-base homeostasis can have serious clinical consequences, and for this reason, the blood pH is usually strictly controlled with the kidneys playing a major part in the long-term maintenance of acid-base homeostasis. Here we review alterations in blood pH that can occur as a side effect of medical treatments, with this first part discussing those resulting in acidosis.

Background: The renin-angiotensin-aldosterone system is essential for blood pressure regulation, and its absence in children with bilateral kidney loss predisposes them to hypotension unresponsive to standard therapies. However, the longer-term hemodynamic consequences and neurologic risks in this setting remain poorly understood.

Methods: We report three pediatric patients with bilateral congenital or acquired kidney absence that developed prolonged hypotension unresponsive to multiple antihypotensive therapy. A systematic literature review identified seven additional cases. The clinical features, interventions, and outcomes are descriptively summarized for these 10 patients.

Results: All three patients exhibited hypotension lasting 6-28 days despite preserved cardiac function without hypovolemia. In the combined literature review of 10 patients, the median age at hypotension onset was 7 months (range, 1-48 months). The etiology of kidney loss was congenital in 2 patients and bilateral nephrectomy in 8 patients. The median interval between kidney loss and hypotension onset was 14 days (range, 0 days-3 years). Among the 10 patients, blood pressure normalized in 8, of whom 4 survived without neurologic complications. Four patients developed hypoxic-ischemic encephalopathy. Five patients died, including three with neurologic complications. Management strategies included fluid overload, vasopressors, and midodrine. Angiotensin II was associated with favorable outcomes in two cases.

Conclusions: Children with bilateral kidney absence can recover from prolonged hypotension; however, this condition is associated with an increased risk of neurologic complications. Careful monitoring and supportive strategies, including approaches reported to be associated with recovery such as Angiotensin II or fluid overload, may help minimize risk.

Background: Recent investigations have indicated that telitacicept significantly diminishes proteinuria levels in adult patients diagnosed with IgA nephropathy (IgAN). The therapy has been correlated with a substantial reduction in galactose-deficient IgA1 (Gd-IgA1) levels. The present study endeavors to clarify the efficacy and safety profile of telitacicept in the treatment of IgAN among pediatric patients.

Methods: This study is a retrospective, observational, single-center research on 12 pediatric patients with IgAN treated with telitacicept as adjunctive therapy, mainly via weekly subcutaneous injections at 2.5 mg/kg, up to a maximum of 160 mg. During telitacicept treatment, ACEI use was maintained, and immunosuppressive doses were gradually reduced.

Results: A total of 12 patients with a mean age of 11.75 years were enrolled. The median follow-up duration post-telitacicept initiation was 9 months. The median 24 h urinary proteinuria decreased from 0.98 (0.5, 9.8) g/1.73 m² at baseline to 0.36 (0.08, 2.49) g/1.73 m² at month 12. At the final follow-up, 7 patients (58.3%) achieved complete remission. During the follow-up period, routine urinalysis demonstrated a significant improvement in urinary red blood cell counts for all patients. No serious adverse events (AEs) were reported in any patient during treatment with telitacicept.

Conclusions: Telitacicept has exhibited both effectiveness and safety in the treatment of children diagnosed with IgAN.

Chronic non-communicable diseases pose a significant global health challenge, with the human gut microbiota emerging as a key player in several (patho) physiological functions, including immunity, metabolic homeostasis, and inflammation. While dysbiosis, or imbalance in taxonomy and function of gut microbiota, has been implicated in chronic kidney disease (CKD), whether it is a cause or consequence of the disease remains controversial. Understanding the gut microbiota's role in CKD pathogenesis is essential for developing novel therapeutic interventions. CKD in children presents unique opportunities for studying disease-specific mechanisms due to the absence of comorbidities typically seen in adults, such as diabetes, obesity, and hypertension, although few studies exist. On the other hand, unlike the relatively stable gut microbiota of healthy adults, the infant's microbiome undergoes significant development and maturation during the early years of life. Integrating knowledge from both pediatric and adult populations may provide a comprehensive understanding of gut microbiota dysbiosis in CKD. This review aims to provide an overview of the gut microbiota's development in healthy individuals and CKD patients and discusses how these findings can inform personalized treatment approaches to CKD.

Background: Nephrolithiasis (NL) and nephrocalcinosis (NC) are common, recurrent conditions globally. While monogenic causes are increasingly recognized, data on their prevalence and spectrum remain limited in Saudi pediatric populations.

Methods: This retrospective cross-sectional study was conducted in our tertiary care center from January 2008 to April 2023. Pediatric patients (0-18 years) with radiologically confirmed NL/NC who underwent genetic testing were included. Clinical, biochemical, radiological, and genetic data were analyzed. Genetic variants were classified using ACMG criteria, and segregation analysis was performed when available.

Results: Of 186 pediatric patients diagnosed with NL/NC, 54 (29.03%) underwent genetic testing. Median age at diagnosis was 3 months [IQR: 3-60], with median follow-up 56 months [IQR: 24-108]. Genetic mutations related to NL/NC were identified in 35/54 patients (64.81%), most commonly in CLDN16 (28.57%), SLC2A2 (17.14%), AGXT (11.43%), and SLC12A1 (8.57%). Thirteen novel variants were identified, with eleven linked to NL/NC phenotypes. Eight patients (14.81%) developed kidney failure requiring kidney replacement therapy; CLDN16 was significantly associated with kidney failure and transplant (P = 0.003), and AGXT with liver transplant (P < 0.001). Notably, the MOCS1 gene was found in a patient with early-onset neurological symptoms, hypouricemia, and later confirmed NL/NC.

Conclusion: Monogenic causes were identified in 35 of 54 (64.81%) Saudi pediatric patients with NL/NC who underwent genetic testing, a prevalence higher than reported internationally, likely due to the high consanguinity rate. Our findings underscore the importance of genetic testing in early-onset NL/NC. We recommend adding MOCS1 to the list of genes associated with monogenic NL/NC.