The Covid-19 outbreak and the subsequent lockdown have profoundly impacted families' daily life, challenging their psychological resilience. Our study aimed to investigate the immediate psychological ...consequences of the pandemic on Italian parents and children focusing on internalizing and post-traumatic symptoms. We also wanted to explore the impact of possible risk and resilience factors, e.g., lifestyle and behaviors, emotional and cognitive beliefs, on parents and children's reaction to the emergency distress. An online survey was administered during the country's nationwide lockdown to 721 Italian parents of at least one child aged between 6 and 18 years. The respondent completed the survey for himself/herself and his/her child. The survey included socio-demographic items and validated questionnaires on parents' post-traumatic stress symptoms, depression and anxiety levels, and on children's internalizing problems. Parents were asked to fill the questionnaires twice: once referring to the current emergency condition and once recalling how they and their child felt a few months before Covid-19 outbreak. Multiple regression analyses showed that specific demographic characteristics (i.e., sex and age) and psychological factors of children and parents, such as fear of contagion and the opportunity to think about possible secondary positive effects of the pandemic, had a predictive value on the presence of internalizing symptoms of both parents and children. Moreover, parents' behaviors during the lockdown period (i.e., employment status and sport practiced) were significantly related to their own internalizing symptoms; these symptoms, in turn, had a strong and positive predictive value on children's internalizing problems. Besides, analyses of variance showed that internalizing symptoms of parents and children were significantly higher during the Covid-19 pandemic than before it started. In addition to showing a direct effect of the pandemic on the psychological health of parents and children, the present results also give a series of important information on how parents perceive, and therefore influence, their children in this period of emergency. Our findings thus highlight the urgent need to provide parents with adequate support to take care of their own psychological wellbeing and to help their children coping with the direct and indirect effects of the pandemic.
Background
For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We ...examined trends in incidence and prevalence of KRT and patient survival.
Methods
We included all children aged <15 years starting KRT 2007–2016 in 22 European countries participating in the ESPN/ERA-EDTA Registry since 2007. General population statistics were derived from Eurostat. Incidence and prevalence were expressed per million age-related population (pmarp) and time trends studied with JoinPoint regression. We analyzed survival trends using Cox regression.
Results
Incidence of children commencing KRT <15 years remained stable over the study period, varying between 5.5 and 6.6 pmarp. Incidence by treatment modality was unchanged over time: 2.0 for hemodialysis (HD) and peritoneal dialysis (PD) and 1.0 for transplantation. Prevalence increased in all age categories and overall rose 2% annually from 26.4 pmarp in 2007 to 32.1 pmarp in 2016. Kidney transplantation prevalence increased 5.1% annually 2007–2009, followed by 1.5% increase/year until 2016. Prevalence of PD steadily increased 1.4% per year over the entire period, and HD prevalence started increasing 6.1% per year from 2011 onwards. Five-year unadjusted patient survival on KRT was around 94% and similar for those initiating KRT 2007–2009 or 2010–2012 (adjusted HR: 0.98, 95% CI:0.71–1.35).
Conclusions
We found a stable incidence and increasing prevalence of European children on KRT 2007–2016. Five-year patient survival was good and was unchanged over time. These data can inform patients and healthcare providers and aid health policy makers on future resource planning of pediatric KRT in Europe.
Peritoneal dialysis (PD) is the worldwide recognized preferred dialysis treatment for children affected by end-stage kidney disease (ESKD). However, due to the unphysiological composition of PD ...fluids, the peritoneal membrane (PM) of these patients may undergo structural and functional alterations, which may cause fibrosis. Several factors may accelerate this process and primary kidney disease may have a causative role. In particular, patients affected by steroid resistant primary focal segmental glomerulosclerosis, a rare glomerular disease leading to nephrotic syndrome and ESKD, seem more prone to develop peritoneal fibrosis. The mechanism causing this predisposition is still unrecognized. To better define this condition, we carried out, for the first time, a new comprehensive comparative proteomic mass spectrometry analysis of mesothelial exosomes from peritoneal dialysis effluent (PDE) of 6 pediatric patients with focal segmental glomerular sclerosis (FSGS) versus 6 patients affected by other primary renal diseases (No FSGS). Our omic study demonstrated that, despite the high overlap in the protein milieu between the two study groups, machine learning allowed to identify a core list of 40 proteins, with ANXA13 as most promising potential biomarker, to distinguish, in our patient population, peritoneal dialysis effluent exosomes of FSGS from No FSGS patients (with 100% accuracy). Additionally, the Weight Gene Co-expression Network Analysis algorithm identified 17 proteins, with PTP4A1 as the most statistically significant biomarker associated to PD vintage and decreased PM function. Altogether, our data suggest that mesothelial cells of FSGS patients are more prone to activate a pro-fibrotic machinery. The role of the proposed biomarkers in the PM pathology deserves further investigation. Our results need further investigations in a larger population to corroborate these findings and investigate a possible increased risk of PM loss of function or development of encapsulating peritoneal sclerosis in FSGS patients, thus to eventually carry out changes in PD treatment and management or implement new solutions.
Schimke immuno-osseous dysplasia (SIOD) is a rare multisystemic disorder with a variable clinical expressivity caused by biallelic variants in
. A phenotype-genotype correlation has been attempted ...and variable expressivity of biallelic
variants may be associated with environmental and genetic disturbances of gene expression. We describe two siblings born from consanguineous parents with a diagnosis of SIOD revealed by whole exome sequencing (WES). Results: A homozygous missense variant in the
gene (c.1682G>A; p.Arg561His) was identified in both patients. Despite carrying the same variant, the two patients showed substantial renal and immunological phenotypic differences. We describe features not previously associated with SIOD-both patients had congenital anomalies of the kidneys and of the urinary tract and one of them succumbed to a classical type congenital mesoblastic nephroma. We performed an extensive characterization of the immunophenotype showing combined immunodeficiency characterized by a profound lymphopenia, lack of thymic output, defective IL-7Rα expression, and disturbed B plasma cells differentiation and immunoglobulin production in addition to an altered NK-cell phenotype and function. Conclusions: Overall, our results contribute to extending the phenotypic spectrum of features associated with
mutations and to better characterizing the underlying immunologic disorder with critical implications for therapeutic and management strategies.
Peritoneal dialysis (PD) in infants represents one of the greatest challenges for pediatric nephrologists. Over recent years, positive outcome data described by several multicenter experiences and ...registry studies have increased the amount of information available to help determine whether to initiate a dialysis program in this high-risk patient population. There is no doubt that the rigorous implementation of strategies aimed at preventing infectious complications may have contributed to reducing the morbidity rate of these patients. However, the complex nature of infants with end-stage renal disease and the presence of multiple comorbidities still represent hallmarks that significantly impact on outcome. Although the rigorous application of improved scientific techniques can still contribute to enhancing PD results in infants, we have to acknowledge that the severity of illness in infants, especially at dialysis initiation, represents an undeniable and nonmodifiable factor.
The first Covid-19 pandemic affected the epidemiology of several diseases. A general reduction in the emergency department (ED) accesses was observed during this period, both in adult and pediatric ...contexts.
This retrospective study was conducted on the behalf of the Italian Society of Pediatric Nephrology (SINePe) in 17 Italian pediatric EDs in March and April 2020, comparing them with data from the same periods in 2018 and 2019. The total number of pediatric (age 0-18 years) ED visits, the number of febrile urinary tract infection (UTI) diagnoses, and clinical and laboratory parameters were retrospectively collected.
The total number of febrile UTI diagnoses was 339 (73 in 2020, 140 in 2019, and 126 in 2018). During the first Covid-19 pandemic, the total number of ED visits decreased by 75.1%, the total number of febrile UTI diagnoses by 45.1%, with an increase in the UTI diagnosis rate (+ 121.7%). The data collected revealed an increased rate of patients with two or more days of fever before admission (p = 0.02), a significant increase in hospitalization rate (+ 17.5%, p = 0.008) and also in values of C reactive protein (CRP) (p = 0.006). In 2020, intravenous antibiotics use was significantly higher than in 2018 and 2019 (+ 15%, p = 0.025). Urine cultures showed higher Pseudomonas aeruginosa and Enterococcus faecalis percentages and lower rates of Escherichia coli (p = 0.02).
The first wave of the Covid-19 pandemic had an essential impact on managing febrile UTIs in the ED, causing an absolute reduction of cases referring to the ED but with higher clinical severity. Children with febrile UTI were more severely ill than the previous two years, probably due to delayed access caused by the fear of potential hospital-acquired Sars-Cov-2 infection. The possible increase in consequent kidney scarring in this population should be considered.
Abstract
The case of a toddler with long-channel cloaca, mild chronic kidney disease (CKD) due to renal dysplasia, and early onset of ulcerative colitis (UC) is herein reported. The patient underwent ...definitive repair of cloaca, that included vaginal elongation with colon, at 5 months of age and was admitted for episodes of vaginal bleeding at 22 months of age. A vaginoscopy revealed a severe inflammation of the colonic neovagina. As rectal bleeding was also noticed, she underwent a colonscopy that showed the same macroscopic inflammatory picture. Neovaginal and colonic biopsies confirmed UC. The mother turned out to be affected by UC since adolescence. The patient is now on oral therapy with mesalazine and topical steroid and mesalazine in the neovagina. The association between cloaca and inflammatory bowel disease (IBD) is anecdotal, but the family history of IBD should be considered when planning the surgical reconstruction of patients with cloaca. In this patient, the occurrence of UC may require a new neovagina in the future and the concomitance of CKD may complicate the overall management due to the potential nephrotoxicity of drugs used for UC therapy.
Posterior urethral valves (PUVs) represent the most severe pediatric obstructive uropathy, responsible for chronic renal failure in up to 65% of cases and progression to end-stage kidney disease ...(ESKD) in about 8%-21% of patients. Unfortunately, renal outcomes have poorly improved over time. The key point is to identify patients at risk; thus, several prenatal and postnatal prognostic factors have been analyzed to improve clinical outcomes. Postnatal nadir creatinine seems to accurately predict long-term renal prognosis, but there is no definitive evidence to support this finding.
We performed a systematic review with meta-analysis to analyze the predictive value of nadir creatinine on long-term renal function in infants with PUVs.
We conducted this systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. PubMed and Cochrane Library were systematically searched for studies published from January 2008 to June 2022. All the articles were checked independently by two reviewers in two steps.
A total of 24 articles were screened, and 13 were included for data extraction. Data from 1,731 patients with PUVs were analyzed, with a mean follow-up of 5.5 years; of these, on average, 37.9% developed chronic kidney disease (CKD) and 13.6% developed ESKD. All the articles evaluated nadir creatinine as a predictor of CKD, most using a level of 1 mg/dL, with statistical significance at the 5% level. The relative risk of developing CKD in patients with creatinine values higher than the nadir cutoff considered was 7.69 (95% CI: 2.35-25.17,
= 92.20%,
< 0.001).
Nadir creatinine is the best prognostic factor for long-term renal function in patients affected by PUV. A value above the cutoff of 1 mg/dL should be considered a significant predictor for the risk of CKD and ESKD. Further studies are needed to define different nadir creatinine cutoffs for better stratification of the different CKD stages and for the development of reliable scores, which include the association of several variables.
BackgroundAcute tubulointerstitial nephritis (TIN) is a significant cause of acute renal failure in paediatric and adult patients. There are no large paediatric series focusing on the aetiology, ...treatment and courses of acute TIN.Patients, design and settingWe collected retrospective clinical data from paediatric patients with acute biopsy-proven TIN by means of an online survey. Members of four professional societies were invited to participate.ResultsThirty-nine physicians from 18 countries responded. 171 patients with acute TIN were included (54% female, median age 12 years). The most frequent causes were tubulointerstitial nephritis and uveitis syndrome in 31% and drug-induced TIN in 30% (the majority of these caused by non-steroidal anti-inflammatory drugs). In 28% of patients, no initiating noxae were identified (idiopathic TIN). Median estimated glomerular filtration rate (eGFR) rose significantly from 31 at time of renal biopsy to 86 mL/min/1.73 m2 3–6 months later (p<0.001). After 3–6 months, eGFR normalised in 41% of patients (eGFR ≥90 mL/min/1.73 m2), with only 3% having severe or end-stage impairment of renal function (<30 mL/min/1.73 m2). 80% of patients received corticosteroid therapy. Median eGFR after 3–6 months did not differ between steroid-treated and steroid-untreated patients. Other immunosuppressants were used in 18% (n=31) of patients, 21 of whom received mycophenolate mofetil.ConclusionsDespite different aetiologies, acute paediatric TIN had a favourable outcome overall with 88% of patients showing no or mild impairment of eGFR after 3–6 months. Prospective randomised controlled trials are needed to evaluate the efficacy of glucocorticoid treatment in paediatric patients with acute TIN.
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