Refining Kidney Survival in 383 Genetically Characterized Patients With Nephronophthisis

Introduction: Nephronophthisis (NPH) comprises a group of rare disorders accounting for up to 10% of end-stage kidney disease (ESKD) in children. Prediction of kidney prognosis poses a major challenge. We assessed differences in kidney survival, impact of variant type, and the association of clinical characteristics with declining kidney function. Methods: Data was obtained from 3 independent sources, namely the network for early onset cystic kidney diseases clinical registry (n = 105), an online survey sent out to the European Reference Network for Rare Kidney Diseases (n = 60), and a literature search (n = 218). Results: A total of 383 individuals were available for analysis: 116 NPHP1, 101 NPHP3, 81 NPHP4 and 85 NPHP11/TMEM67 patients. Kidney survival differed between the 4 cohorts with a highly variable median age at onset of ESKD as follows: NPHP3, 4.0 years (interquartile range 0.3–12.0); NPHP1, 13.5 years (interquartile range 10.5–16.5); NPHP4, 16.0 years (interquartile range 11.0–25.0); and NPHP11/TMEM67, 19.0 years (interquartile range 8.7–28.0). Kidney survival was significantly associated with the underlying variant type for NPHP1, NPHP3, and NPHP4. Multivariate analysis for the NPHP1 cohort revealed growth retardation (hazard ratio 3.5) and angiotensin-converting enzyme inhibitor (ACEI) treatment (hazard ratio 2.8) as 2 independent factors associated with an earlier onset of ESKD, whereas arterial hypertension was linked to an accelerated glomerular filtration rate (GFR) decline. Conclusion: The presented data will enable clinicians to better estimate kidney prognosis of distinct patients with NPH and thereby allow personalized counseling

Can Schools Reduce Adolescent Psychological Stress? A Multilevel Meta-Analysis of the Effectiveness of School-Based Intervention Programs

Increased levels of psychological stress during adolescence have been associated with a decline in academic performance, school dropout and increased risk of mental health problems. Intervening during this developmental period may prevent these problems. The school environment seems particularly suitable for interventions and over the past decade, various school-based stress reduction programs have been developed. The present study aims to evaluate the results of (quasi-)experimental studies on the effectiveness of school-based intervention programs targeting adolescent psychological stress and to investigate moderators of effectiveness. A three-level random effects meta-analytic model was conducted. The search resulted in the inclusion of k = 54 studies, reporting on analyses in 61 independent samples, yielding 123 effect sizes (N = 16,475 individuals). The results indicated a moderate overall effect on psychological stress. Yet, significant effects were only found in selected student samples. School-based intervention programs targeting selected adolescents have the potential to reduce psychological stress. Recommendations for practice, policy and future research are discussed

Exchange-diffusion reactions in HfSiON during annealing studied by Rutherford backscattering spectrometry, nuclear reaction analysis and narrow resonant nuclear reaction profiling

HfSiON films deposited on Si (001) by reactive sputtering were submitted to rapid thermal annealing at 1000 °C in vacuum, N2 and O2 atmospheres. The stability of the dielectric was evaluated by measuring the atomic transport and exchange of the chemical species, using Rutherford backscattering spectrometry, nuclear reaction analysis and narrow resonant nuclear reaction profiling. Annealing in O2 ambient reduced the N concentration mainly from near-surface regions where oxygen was incorporated in comparable amounts. Vacuum annealing, on the other hand, induced N loss preferentially from the Si/dielectric interface and O loss preferentially from near-surface regions. The results are explained in terms of exchange-diffusion reactions occurring in the HfSiON

Network for Early Onset Cystic Kidney Diseases—A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood

Hereditary cystic kidney diseases comprise a complex group of genetic disorders representing one of the most common causes of end-stage renal failure in childhood. The main representatives are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet–Biedl syndrome, and hepatocyte nuclear factor-1beta nephropathy. Within the last years, genetic efforts have brought tremendous progress for the molecular understanding of hereditary cystic kidney diseases identifying more than 70 genes. Yet, genetic heterogeneity, phenotypic variability, a lack of reliable genotype–phenotype correlations and the absence of disease-specific biomarkers remain major challenges for physicians treating children with cystic kidney diseases. To tackle these challenges comprehensive scientific approaches are urgently needed that match the ongoing “revolution” in genetics and molecular biology with an improved efficacy of clinical data collection. Network for early onset cystic kidney diseases (NEOCYST) is a multidisciplinary, multicenter collaborative combining a detailed collection of clinical data with translational scientific approaches addressing the genetic, molecular, and functional background of hereditary cystic kidney diseases. Consisting of seven work packages, including an international registry as well as a biobank, NEOCYST is not only dedicated to current scientific questions, but also provides a platform for longitudinal clinical surveillance and provides precious sources for high-quality research projects and future clinical trials. Funded by the German Federal Government, the NEOCYST collaborative started in February 2016. Here, we would like to introduce the rationale, design, and objectives of the network followed by a short overview on the current state of progress

Can Schools Reduce Adolescent Psychological Stress? A Multilevel Meta-Analysis of the Effectiveness of School-Based Intervention Programs

Increased levels of psychological stress during adolescence have been associated with a decline in academic performance, school dropout and increased risk of mental health problems. Intervening during this developmental period may prevent these problems. The school environment seems particularly suitable for interventions and over the past decade, various school-based stress reduction programs have been developed. The present study aims to evaluate the results of (quasi-)experimental studies on the effectiveness of school-based intervention programs targeting adolescent psychological stress and to investigate moderators of effectiveness. A three-level random effects meta-analytic model was conducted. The search resulted in the inclusion of k = 54 studies, reporting on analyses in 61 independent samples, yielding 123 effect sizes (N = 16,475 individuals). The results indicated a moderate overall effect on psychological stress. Yet, significant effects were only found in selected student samples. School-based intervention programs targeting selected adolescents have the potential to reduce psychological stress. Recommendations for practice, policy and future research are discussed