Pediatric Nephrology最新文献

Background: This retrospective, descriptive study, conducted in a single-center PICU from June 2014 to May 2023, aimed to analyze the efficacy of adjunctive regional citrate anticoagulation for continuous kidney replacement therapy (CKRT) circuits during extracorporeal membrane oxygenation (ECMO).

Methods: Patients were divided into two groups based on their CKRT anticoagulation strategy: those receiving regional citrate anticoagulation in addition to systemic heparin (UFH + RCA group) and those receiving only systemic heparin (UFH group). CKRT circuits were also classified as either UFH + RCA or UFH to analyze outcomes specific to each anticoagulation strategy. CKRT circuit lifespan estimation was calculated by dividing the total CKRT duration by the number of circuits used.

Results: During the study period, 110 pediatric patients were treated with ECMO at our PICU. During ECMO, 64 (58.2%) of these patients required CKRT. Fluid overload and acute kidney injury were the primary indications for CKRT. While not statistically significant, the median estimate CKRT circuit lifespan was longer in the citrate group [84 (38.4-112.0)] than the heparin group [52 (12.0-408.0)]. Circuit changes due to clotting were significantly higher in the heparin group compared to the citrate group (58.1% vs. 31.7%, p = 0.00). Kaplan-Meier analysis revealed a statistically significant difference in the timing of clotting-related circuit changes, favoring UFH + RCA (p = 0.02).

Conclusions: To the best of our knowledge, our study represents the first comparison of UFH + RCA and UFH alone for CKRT in pediatric ECMO patients. Our findings suggest that using UFH + RCA might help the circuit last longer by decreasing changes caused by clotting. Prospective studies on this topic are needed.

Low molecular weight heparin (LMWH) is widely used for the treatment and prevention of many venous thromboembolic disorders. LMWH acts through its ability to potentiate inhibition of factor Xa. LMWH undergoes primary renal excretion and therefore its elimination can be severely impacted by kidney disease. We report on the case of an infant with stage 5 chronic kidney disease (CKD) who received a prolonged accidental overdose of dalteparin. The infant was symptomatic with a falling haemoglobin, perinephric hematoma, and oozing venipuncture sites. Given the inability to predict drug clearance in this infant and her significant risk of bleeding, she underwent a successful trial of continuous veno-venous hemofiltration (CVVH) for the treatment of LMWH overdose. Whilst on CVVH, a steady downward trend in anti-Xa levels over 24 h was observed.

Background: Kidney involvement in pediatric sarcoidosis is rare and often underrecognized, leading to diagnostic delays and treatment challenges. We report six patients with renal sarcoidosis to highlight their diverse presentations and outcomes and challenges in management.

Methods: Medical records of patients diagnosed with renal sarcoidosis during 2020-24 were reviewed. Sarcoidosis was diagnosed based on clinical and histological features and exclusion of alternative causes and managed according to unit protocols. Information on clinical features, laboratory and radiologic findings, histopathology, treatment, and follow-up were compiled.

Results: We present six patients with sarcoidosis, presenting with kidney involvement at the age of 1.5-14 years, and followed up for 7-138 months. All patients had acute kidney injury (AKI) of whom two required hemodialysis. Proteinuria was present in all patients, while four patients had microscopic hematuria or leukocyturia. Hypercalcemia with hypercalciuria, distal renal tubular acidosis, and nephrocalcinosis were seen in five, two, and one case, respectively. Granulomatous interstitial nephritis was confirmed histologically in all cases. While initial therapy with corticosteroids led to clinical remission in all cases, five patients had nine relapses, necessitating second-line immunosuppression with mycophenolate mofetil, azathioprine, or methotrexate; one patient received antitumor necrosis factor therapy. Median eGFR at last follow up was 59.7 (range 12.7-132) ml/min/1.73 m²; three progressed to chronic kidney disease (CKD) stages G3-G5.

Conclusions: Kidney involvement in pediatric sarcoidosis manifests in diverse forms, ranging from isolated biochemical abnormalities to severe AKI. While prompt immunosuppression might preserve kidney function, patients require close monitoring for relapses, and progression to CKD.

Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) and lupus nephritis (LN) are two major, life-threatening complications in childhood-onset SLE (cSLE). Data regarding the epidemiology and prognosis of children with concurrent NPSLE and LN remain scarce. This study aimed to investigate the clinical characteristics, associated factors, and outcomes of NPSLE in Chinese children with LN.

Methods: A retrospective cohort study was conducted at the Paediatric Nephrology Centre of Hong Kong Children's Hospital, including 95 Chinese children with biopsy-proven cLN. Comparisons were made between children with and without NPSLE.

Results: Of 95 Chinese children with cLN, 11 (12%) developed NPSLE, and 31 NPSLE events were reported. Estimated glomerular filtration rate < 30 mL/min/1.73 m² at diagnosis of LN (OR_adj 6.7, 95% CI 1.29-35.1) and higher maximal proteinuria during the observation period (OR_adj 1.07, 95% CI 1-1.13) were predictive of NPSLE upon multivariable analysis. Compared to children with LN who did not develop NPSLE, significantly more children who developed subsequent NPSLE flare following initial kidney involvement had a history of medication non-adherence (100% vs. 25%, p < 0.001), higher degree of proteinuria at the diagnosis of LN (urine protein/creatinine ratio, 5.7 vs. 2.4 mg/mg, p = 0.04) and during the entire observation period (urine protein/creatinine ratio, 13.2 vs. 3.3 mg/mg, p = 0.004). Patients with NPSLE had significantly lower complete remission rates for LN at 6- and 12-month post-induction (27.3% vs. 70.2%, p = 0.014; 45.5% vs. 83.3%, p = 0.01, respectively). Kaplan-Meier analysis showed that patients with NPSLE had worse kidney and patient survivals (log-rank test, p < 0.001, 0.0014, respectively) than those without NPSLE.

Conclusions: Worse kidney and patient survivals are observed in cLN patients with NPSLE. Severe LN manifestation and medication non-adherence are associated with the development of NPSLE.

Background: Nephrotic syndrome (NS) is a common pediatric kidney disorder characterized by proteinuria, hypoalbuminemia, and edema. Leukotrienes (LTs), as inflammatory mediators, may contribute to NS pathogenesis and influence treatment response. This study aimed to assess urinary leukotriene E4 (LTE4) levels in children with an initial onset of NS and evaluate their potential as biomarkers for steroid responsiveness.

Methods: In this observational cohort study, 41 children with a first episode of NS and 41 age- and sex-matched healthy controls were enrolled. Patients were classified into steroid-sensitive NS (SSNS; n = 29) and steroid-resistant NS (SRNS; n = 12) groups following initial steroid therapy. Urinary LTE4 levels were measured prior to treatment, using enzyme-linked immunosorbent assay (ELISA).

Results: Urinary LTE4 levels were significantly elevated in children with NS compared to controls (p = 0.001). Although urinary LTE4 to urinary creatinine (U cr) ratios were also higher in patients, the difference did not reach statistical significance (p = 0.09). No significant correlations were observed between urinary LTE4 levels and urinary protein excretion or serum albumin. Furthermore, urinary LTE4 levels did not significantly differ between SSNS and SRNS groups. A receiver operating characteristic (ROC) curve analysis showed poor predictive value of urinary LTE4 for steroid responsiveness, with area-under-the-curve (AUC) values near 0.5.

Conclusions: While urinary LTE4 levels are elevated in children with NS, they failed to reliably differentiate between SSNS and SRNS. These findings suggest a limited role for urinary LTE4 as a predictive biomarker of steroid responsiveness in pediatric NS. However, future large-scale studies incorporating both plasma and urinary leukotriene profiles are warranted to validate its role in disease pathogenesis and treatment response.

Background: Parental stress in pediatric chronic illness is well known; however, there is a dearth of literature describing parental stress in pediatric chronic kidney disease (CKD). This pilot study evaluated parenting stress in mild to moderate pediatric CKD relative to caregivers of healthy, typically developing children.

Methods: The study included 38 children, ages 6 to 12 years, and their parents (CKD Group = 10, Typical Group = 28). Pediatric CKD participants had mild to moderate kidney dysfunction for at least 3 months. Parents completed the Parenting Stress Index (PSI-3) as a measure of their current stress.

Results: Serial linear regressions revealed no significant group differences on the Child, Parent, Total Stress, or Life Stress domains; however, exploratory analyses revealed significant parental stress on the subscales of Mood and Acceptability, as well as concerns about their own Competence and Health. Compared to the control group, parents of patients with CKD also reported significantly high levels of stress on Adaptability (50% versus 21.4%), Mood (60% versus 25%), and Acceptability (50% versus 10.7%).

Conclusions: While overall levels of parenting stress were not unduly elevated in group comparisons, increased stress levels with respect to their child's mood, acceptability, and their own personal health were noted. The proportion of CKD parent ratings reaching significantly high stress levels also was uniformly high, particularly in Adaptability, Mood, Acceptability, (parental) Competence, and Total Stress. These pilot results should guide future studies exploring parent/family factors and potential interventions for reducing parenting stress and related burdens in the clinical care of children with CKD.

Background: Pediatric hypertension is a growing health concern, with prolonged exposure to high blood pressure potentially causing electrical instability and increasing arrhythmia risk. The index of cardiac electrophysiological balance (iCEB), calculated as QT interval divided by QRS duration, is a potential non-invasive marker for arrhythmogenesis. This study aimed to evaluate iCEB and corrected iCEB (iCEBc) in hypertensive children and investigate their relationship with arrhythmic risk.

Methods: This cross-sectional study included 81 children with primary hypertension and 36 age- and sex-matched healthy controls. Office blood pressure, 24-h ambulatory blood pressure monitoring (ABPM), standard echocardiography, and 12-lead electrocardiograms (ECGs) were obtained. QT, QTc, Tp-e, Tp-e/QT, Tp-e/QTc, iCEB, and iCEBc were calculated. Echocardiographic measurements and laboratory parameters were also evaluated.

Results: The mean age of the hypertensive group was 13.8 ± 3 years, with 60.5% males. Most (64.2%) demonstrated a non-dipping BP pattern. Echocardiography showed preserved ejection fraction (72.7 ± 5.4%) and shortening fraction (42 ± 5.1%), with left ventricular hypertrophy (LVH) observed in 8.6% of cases. ECG analysis revealed significantly prolonged QTc interval (416.8 ± 30.2 ms vs. 401.8 ± 23.4 ms; p = 0.008), iCEB (3.92 vs. 3.44; p = 0.02), and iCEBc (4.58 vs. 4.09; p = 0.001) values in hypertensive patients compared to controls. No significant differences were observed in Tp-e, Tp-e/QT, or Tp-e/QTc.

Conclusion: Children with hypertension exhibit subclinical alterations in cardiac electrophysiology, including significantly elevated iCEB and iCEBc values, which may indicate electrical instability and a higher arrhythmia risk. These indices may serve as practical, non-invasive tools for early detection of subclinical electrophysiological changes in pediatric hypertension.

Rare monogenic diseases are increasingly identified in children with chronic kidney disease. We describe a consanguineous preterm male infant with a clinical picture of advanced kidney dysfunction and severe renal salt-wasting, highly suggestive of prenatal onset Bartter syndrome. Patient's follow-up was characterized by severe polyuria; episodes of hyponatremia, hypokalemia, and hypochloremia; and metabolic alkalosis and hyperuricemia. We found a homozygous pathogenic variant in the TFCP2L1 gene, a transcription factor required for normal kidney development, that regulates acid-base and salt-water homeostasis. To our knowledge, there is only one published case of a child with TFCP2L1 gene pathogenic variants with a similar phenotype. This report adds evidence to TFCP2L1 as a cause of monogenic kidney disorders. Rare kidney dysplasias may manifest as phenocopies of primary tubulopathies. Genetic diagnosis plays a major role and should be carefully considered in patients with refractory course to standard treatment to facilitate management and family counselling.