Our understanding of the acute respiratory distress syndrome in children is limited, and literature is dominated by investigations in adult patients. Recent preclinical studies suggest that the susceptibility to and severity of acute respiratory distress syndrome in children could differ from that in adults. We assessed the incidence and mortality of acute respiratory distress syndrome reported in children in studies published in the last two decades. Medline, Embase, and CINAHL databases were searched up to August 2014. Articles reporting study data on population- or PICU-based incidence and mortality of acute respiratory distress syndrome in children (> 36 wk gestation and < 18 yr old) were selected. Two authors independently collected data and assessed methodological quality and risk of bias of selected studies. Pooled estimates of incidence and mortality were calculated using random-effects models. To explore heterogeneity, influence of study characteristics, including median year of conduct, study location, inclusion and exclusion criteria, and study design and quality, was assessed by meta-regression analysis. Twenty-nine studies reported on incidence and 32 on mortality. Pooled weighted estimate of the population-based and PICU-based incidence of pediatric acute respiratory distress syndrome was 3.5 (95% CI, 2.2-5.7) cases per 100,000 person years and 2.3% (95% CI, 1.9-2.9), respectively. Pooled weighted mortality was 33.7% (95% CI, 28.6-39.7). There were no trends over time, but mortality was significantly associated with study location. This systematic review and meta-analysis shows a low incidence but a high mortality. Its results also indicate that both incidence and mortality of pediatric acute respiratory distress syndrome have not changed over the last two decades and that mortality depends on the geographic location of studies.

Results from preclinical studies suggest that age-dependent differences in host defense and the pulmonary renin–angiotensin system (RAS) are responsible for observed differences in epidemiology of acute respiratory distress syndrome (ARDS) between children and adults. The present study compares biomarkers of host defense and RAS in bronchoalveolar lavage (BAL) fluid from neonates, children, adults, and older adults with ARDS. In this prospective observational study, we enrolled mechanical ventilated ARDS patients categorized into four age groups: 20 neonates (< 28 days corrected postnatal age), 29 children (28 days–18 years), 26 adults (18–65 years), and 17 older adults (> 65 years of age). All patients underwent a nondirected BAL within 72 h after intubation. Activities of the two main enzymes of RAS, angiotensin converting enzyme (ACE) and ACE2, and levels of biomarkers of inflammation, endothelial activation, and epithelial damage were determined in BAL fluid. Levels of myeloperoxidase, interleukin (IL)-6, IL-10, and p-selectin were higher with increasing age, whereas intercellular adhesion molecule-1 was higher in neonates. No differences in activity of ACE and ACE2 were seen between the four age groups. Age-dependent differences in the levels of biomarkers in lungs of ARDS patients are present. Especially, higher levels of markers involved in the neutrophil response were found with increasing age. In contrast to preclinical studies, age is not associated with changes in the pulmonary RAS.

CONTEXT Idiopathic nephrotic syndrome (INS) in children is a disease with considerable morbidity, yet the incidence and risk for relapse have not been systematically reviewed. OBJECTIVE To estimate the overall pooled weighted incidence and risk for relapse of INS in children. DATA SOURCES Medline and Embase (until December 2020). STUDY SELECTION All studies reporting incidence (per 100 000 children per year) and/or risk for relapse (the proportion of patients who experience ≥1 relapse) of INS in children (age: <18 years) were eligible. DATA EXTRACTION After quality assessment, data were extracted: study (design, localization, and sample size) and patient (age, sex, steroid response, and ethnicity) characteristics, incidence, and risk for relapse. RESULTS After screening, 73 studies were included for analysis (27 incidence, 54 relapse). The overall pooled weighted estimate and corresponding prediction interval (PI) of the incidence was 2.92 (95% PI: 0.00–6.51) per 100 000 children per year. Higher incidences were found in non-Western countries (P < .001). Incidence tended to be lower in white children, but this was not significant. The overall pooled weighted estimate of the risk for relapse was 71.9% (95% PI: 38.8–95.5). Between 1945 and 2011, incidence did not change (P = .39), yet the risk for relapse decreased significantly (P = .024), from 87.4% to 66.2%. LIMITATIONS There was no full-text availability (n = 33), considerable heterogeneity, and limited studies from Africa, Latin America, and Asia. CONCLUSIONS INS has a low incidence with ethnic variation but high risk for relapse. Although corticosteroids have significantly reduced the risk for relapse, it remains unacceptably high, underscoring the need for alternative treatment strategies.

Introduction: Primary FSGS is an important cause of ESRD in children. FSGS recurrence after kidney transplantation is associated with early graft loss. No guidelines for treatment of FSGS recurrence exist. We conducted a survey to gain insight into variation of treatment between centers.Methods: A survey was sent to all members of the ESPN on behalf of the “Renal Transplantation” and “Idiopathic Nephrotic Syndrome” working groups.Results: Fifty‐nine nephrologists from 31 countries responded, reporting 807 FSGS patients, with 241 (30%) FSGS recurrences after transplantation. Recurrence varied from 0% to 100% between respondents. Native nephrectomy before or during transplantation was performed, respectively, always (37%), never (39%), or on clinical indication (17%). Half of the respondents started preventive treatment before transplantation, using PF (n = 10); R (n = 4); PF or IA, plus R (n = 9); cyclosporine (n = 2); or unknown (n = 4). Immunosuppressive therapy for patients without known mutations consisted of a combination of steroids, tacrolimus/cyclosporine, and MMF, with or without IL‐2R‐blockade in, respectively, 61% and 86% of the respondents. Sixty‐three percent applied a similar regimen to patients with known mutations. FSGS recurrence was treated with PF or IA, plus R by 66% of respondents; 54% observed no response. Complete remission in >50% of patients was reported by 41% of the respondents.Discussion: FSGS recurrence after transplantation is common, but varies greatly between centers. We found great variability in preventive and therapeutic treatment regimens. Future research should focus on predisposing factors, including biopsy findings and genetic mutations, and standardized treatment.

(1) Background: Health-related quality of life (HRQoL) is lower in patients with chronic kidney disease (CKD) compared to the general population. In 2011, the KLIK PROM portal was implemented in the Emma Children’s Hospital to monitor and discuss HRQoL in daily care. This study describes and assesses the implementation and use of the KLIK PROM portal in the pediatric nephrology department. (2) Methods: CKD patients (self-report, if 8–18 years of age) and their parents (proxy-report, if 1–8 years) were invited to complete HRQoL patient-reported outcome measures (PROMs): TNO-AZL Preschool children Quality Of Life (TAPQOL) or Pediatric Quality of Life Inventory for Children (PedsQL). The PROMs were completed before and discussed during outpatient consultations. The adaptation rate—the proportion of patients/parents who were invited and completed at least one PROM—was calculated. Reported HRQoL scores of CKD patients were compared to the general population. (3) Results: In total, 142 patients (proxy- and self-report) were invited, 112 patients completed at least one PROM (adaptation rate 79%). Patients (n = 84 with informed consent for scientific use) with CKD reported lower HRQoL and HRQoL was more often impaired compared to the general Dutch population. (4) Conclusions: The implementation of KLIK was successful and its use is feasible for daily care. Using KLIK, HRQoL problems can be easily identified and monitored.

Background: The aetiology of idiopathic nephrotic syndrome (INS) remains partially unknown. Viral infections have been associated with INS onset. Since we observed fewer first onset INS cases during the Covid-19 pandemic, we hypothesised that lower INS incidence was the result of lockdown measures. Therefore, the aim of this study was to evaluate the incidence of childhood INS before and during the COVID-19 pandemic in two independent European INS cohorts. Methods: Children with new INS in the Netherlands (2018–2021) and Paris area (2018–2021) were included. We estimated incidences using census data for each region. Incidences were compared using two proportion Z-tests. Results: A total of 128 and 324 cases of first onset INS were reported in the Netherlands and Paris area, respectively, corresponding to an annual incidence of 1.21 and 2.58 per 100,000 children/year. Boys and young children (< 7 years) were more frequently affected. Incidence before and during the pandemic did not differ. When schools were closed, incidence was lower in both regions: 0.53 vs. 1.31 (p = 0.017) in the Netherlands and 0.94 vs. 2.63 (p = 0.049) in the Paris area. During peaks of hospital admissions for Covid-19, no cases were reported in the Netherlands or Paris area. Conclusions: Incidence of INS before and during the Covid-19 pandemic was not different, but when schools were closed during lockdown, incidence was significantly lower. Interestingly, incidences of other respiratory viral infections were also reduced as was air pollution. Together, these results argue for a link between INS onset and viral infections and/or environmental factors. Graphical