Clinical Heterogeneity in Patients With FOXP3 Mutations Presenting With Permanent Neonatal Diabetes

OBJECTIVE—Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is caused by FOXP3 mutations. We aimed to determine the prevalence, genetics, and clinical phenotype of FOXP3 mutations in a large cohort with permanent neonatal diabetes (PNDM)

The IUCN Global Standard for Nature-based Solutions™ as a tool for enhancing the sustainable development of marine aquaculture

This paper applies the IUCN Global Standard for Nature based Solutions™ self-assessment tool (published in 2020) to two aquaculture case studies. Data from the case studies were compiled by the authors. In Zanzibar, secondary data were obtained through a previous project, which included a stakeholder workshop in Zanzibar (in 2019) and one deliverable published by the IUCN on Zanzibar of their catalogue “Aquaculture and Marine Conservation”. In Indonesia, the original data were provided by the Blue Natural Capital Funding Facility (BNCFF) and the associated local teams. The analysis of the data, the information provided, and the scoring itself were done by the authors, in association with local teams in both areas. The results of the two assessments, discussed in the paper and presented in detail in the Supplementary materials, can be considered original research, never previously published in a scientific journal. The concept of Nature-based Solutions (NbS) was proposed by the International Union for Conservation of Nature (IUCN) to protect, restore, and sustainably manage natural and modified ecosystems for achieving a variety of societal benefits. The IUCN released the IUCN Global Standard for NbS™ to help design, assess, strengthen, and upscale NbS interventions. In the current context of growing uncertainties for the future of coastlines and oceans, aquaculture has been recognized as a positive activity for achieving sustainable development in coastal communities; supporting food security, poverty alleviation, and economic resilience; and contributing to the conservation of marine ecosystems in some cases. However, the sustainability of aquaculture systems has often been criticized. Aquaculture initiatives in coastal areas can achieve both nature conservation and sustainable development objectives, but reflection on the conditions under which this would happen is needed. This article examines aquaculture systems through the lens of the NbS concept and the IUCN Global Standard for NbS™, along with other sustainability concepts and instruments currently used in the context of aquaculture. The application of the IUCN Global Standard for NbS™’s to two case studies is explored: seaweed farming in Zanzibar in marine conservation areas and shrimp farming coupled with mangrove restoration in Indonesia. The results show that the NbS concept underpinning the IUCN Global Standard for NbS™ could help in the overall assessment of aquaculture systems and improve their sustainability by highlighting both their positive outcomes and issues requiring further examination in relation to marine biodiversity benefits, socio-economic development, and/or governance. The IUCN Global Standard for NbS™ could provide an operational framework to implement existing concepts, such as the Ecosystem Approach to Aquaculture, contribute to clarifying critical issues in aquaculture development, and provide guidance for the development of a new type of aquaculture project, specifically designed as NbS. This finding advocates the context-dependent exploration and promotion of aquaculture projects as NbS

Estimating Global ‘‘Blue Carbon’’ Emissions from Conversion and Degradation of Vegetated Coastal Ecosystems

Recent attention has focused on the high rates of annual carbon sequestration in vegetated coastal ecosystems—marshes, mangroves, and seagrasses—that may be lost with habitat destruction (‘conversion’). Relatively unappreciated, however, is that conversion of these coastal ecosystems also impacts very large pools of previously-sequestered carbon. Residing mostly in sediments, this ‘blue carbon’ can be released to the atmosphere when these ecosystems are converted or degraded. Here we provide the first global estimates of this impact and evaluate its economic implications. Combining the best available data on global area, land-use conversion rates, and near-surface carbon stocks in each of the three ecosystems, using an uncertainty-propagation approach, we estimate that 0.15–1.02 Pg (billion tons) of carbon dioxide are being released annually, several times higher than previous estimates that account only for lost sequestration. These emissions are equivalent to 3–19% of those from deforestation globally, and result in economic damages of $US 6–42 billion annually. The largest sources of uncertainty in these estimates stems from limited certitude in global area and rates of land-use conversion, but research is also needed on the fates of ecosystem carbon upon conversion. Currently, carbon emissions from the conversion of vegetated coastal ecosystems are not included in emissions accounting or carbon market protocols, but this analysis suggests they may be disproportionally important to both. Although the relevant science supporting these initial estimates will need to be refined in coming years, it is clear that policies encouraging the sustainable management of coastal ecosystems could significantly reduce carbon emissions from the land-use sector, in addition to sustaining the well-recognized ecosystem services of coastal habitats

Search for dark matter produced in association with bottom or top quarks in √s = 13 TeV pp collisions with the ATLAS detector

A search for weakly interacting massive particle dark matter produced in association with bottom or top quarks is presented. Final states containing third-generation quarks and miss- ing transverse momentum are considered. The analysis uses 36.1 fb−1 of proton–proton collision data recorded by the ATLAS experiment at √s = 13 TeV in 2015 and 2016. No significant excess of events above the estimated backgrounds is observed. The results are in- terpreted in the framework of simplified models of spin-0 dark-matter mediators. For colour- neutral spin-0 mediators produced in association with top quarks and decaying into a pair of dark-matter particles, mediator masses below 50 GeV are excluded assuming a dark-matter candidate mass of 1 GeV and unitary couplings. For scalar and pseudoscalar mediators produced in association with bottom quarks, the search sets limits on the production cross- section of 300 times the predicted rate for mediators with masses between 10 and 50 GeV and assuming a dark-matter mass of 1 GeV and unitary coupling. Constraints on colour- charged scalar simplified models are also presented. Assuming a dark-matter particle mass of 35 GeV, mediator particles with mass below 1.1 TeV are excluded for couplings yielding a dark-matter relic density consistent with measurements

Nonlinear Dirac Equations

Herr S, Bejenaru I, Candy T. Nonlinear Dirac Equations. In: Frey D, Guo Z, Hassell A, Portal P, Rozendaal J, Yung P-L, eds. MATRIX-MFO Tandem Workshop/Small Collaboration: Rough Wave Equations: report 45/2021. Oberwolfach Reports. Vol 18. Berlin: European Mathematical Society - EMS - Publishing House GmbH; 2021: 2469-2470

Early indication of noise-induced hearing loss from PMP use in adolescents: A cross-sectional analysis

Context: Distortion product otoacoustic emissions (DPOAEs) may indicate preclinical noise-induced hearing loss (NIHL) in adolescents from unsafe personal music player (PMP) use. Aims: The objective, therefore, was to observe preclinical signs of NIHL in 9 th grade adolescents with clinically normal hearing by comparing DPOAE signals between different levels of A-weighted equivalent PMP exposure. Settings and Design: Subjects were recruited from all secondary-level schools located in the city of Regensburg, Germany during two academic years 2009/2010 and 2010/2011. Subjects and Methods: A-weighted equivalent sound pressure levels (SPLs) for a 40-hour work week (LAeq. 40h) were estimated from questionnaire responses on output and duration of PMP use of the previous week. Subjects were then categorized into four levels of exposure: 85 to = 90 A-weighted Decibel [dB(A)]. DPOAE signals were collected by trained audiological staff, applying a standard optimized protocol, at the Department of Otorhinolaryngology of the University Hospital Regensburg. Statistical Analysis Used: Mean DPOAE signals were compared between levels by unpaired t test. Novel linear regression models adjusting for other leisure noise exposures and with outcome variables DPoutcome and 4 kilo Hertz (kHz) DPOAEs estimated effects between levels. Results: A total of 1468 subjects (56% female, mostly aged 15 or 16 years) were available for analysis. Comparison of DPOAE means by PMP exposure typically showed no greater than 1 dB difference between groups. In fact, comparisons between >= 90 dB(A) and < 80 dB(A) presented the least differences in magnitude. Both DPoutcome and 4kHz linear regression models presented a weak association with the 4-level PMP exposure variable. An expected dose-response to PMP exposure was not observed in any analyses. Conclusions: DPOAE signal strength alone cannot indicate preclinical NIHL in adolescents

Longitudinal analysis of leisure noise exposure among adolescents with special focus on portable listening devices: the OHRKAN cohort study

The aim was to investigate leisure noise exposure and sociodemographic determinants of risky leisure noise exposure over five years in 2148 students visiting grade 9 of any school type in a German city from 2009-2011. Within the OHRKAN cohort study, leisure noise exposure was calculated from literature-retrieved sound pressure levels (SPLs) and self-reported duration of 18 leisure activities at baseline and two follow-ups. Risky exposure was defined as exceeding 85 dB(A) averaged over a 40-h-week. Determinants of risky total leisure noise (TLN) exposure and risky exposure to portable listening devices (PLDs) were investigated using generalised estimating equations (GEEs). Up to 73% of students exceeded noise levels of 85 dB(A) at some timepoint. The noise exposure and importance of different leisure activities changed with increasing age. Risky exposure to TLN and PLDs was associated with lower education, single parent households and being male. Risky PLD exposure was additionally associated with a migrant background. Current prevention measures for leisure noise exposure must be extended to at-risk groups. Besides enhancing campaigns in lower education schools, acoustical insulation in sports halls, noise warnings on tools or in videogames could address especially men. Migrants need education about healthy PLD use in their native language if necessary

Audiometric notch and extended high-frequency hearing threshold shift in relation to total leisure noise exposure: An exploratory analysis

Background: Studies investigating leisure noise effect on extended high frequency hearing are insufficient and they have inconsistent results. The aim of this study was to investigate if extended high-frequency hearing threshold shift is related to audiometric notch, and if total leisure noise exposure is associated with extended high-frequency hearing threshold shift. Materials and Methods: A questionnaire of the Ohrkan cohort study was used to collect information on demographics and leisure time activities. Conventional and extended high-frequency audiometry was performed. We did logistic regression between extended high-frequency hearing threshold shift and audiometric notch as well as between total leisure noise exposure and extended high-frequency hearing threshold shift. Potential confounders (sex, school type, and firecrackers) were included. Results: Data from 278 participants (aged 18–23 years, 53.2% female) were analyzed. Associations between hearing threshold shift at 10, 11.2, 12.5, and 14 kHz with audiometric notch were observed with a higher prevalence of threshold shift at the four frequencies, compared to the notch. However, we found no associations between total leisure noise exposure and hearing threshold shift at any extended high frequency. Conclusion: This exploratory analysis suggests that while extended high-frequency hearing threshold shifts are not related to total leisure noise exposure, they are strongly associated with audiometric notch. This leads us to further explore the hypothesis that extended high-frequency threshold shift might be indicative of the appearance of audiometric notch at a later time point, which can be investigated in the future follow-ups of the Ohrkan cohort

Estimating global "blue carbon" emissions from conversion and degradation of vegetated coastal ecosystems.

Recent attention has focused on the high rates of annual carbon sequestration in vegetated coastal ecosystems--marshes, mangroves, and seagrasses--that may be lost with habitat destruction ('conversion'). Relatively unappreciated, however, is that conversion of these coastal ecosystems also impacts very large pools of previously-sequestered carbon. Residing mostly in sediments, this 'blue carbon' can be released to the atmosphere when these ecosystems are converted or degraded. Here we provide the first global estimates of this impact and evaluate its economic implications. Combining the best available data on global area, land-use conversion rates, and near-surface carbon stocks in each of the three ecosystems, using an uncertainty-propagation approach, we estimate that 0.15-1.02 Pg (billion tons) of carbon dioxide are being released annually, several times higher than previous estimates that account only for lost sequestration. These emissions are equivalent to 3-19% of those from deforestation globally, and result in economic damages of $US 6-42 billion annually. The largest sources of uncertainty in these estimates stems from limited certitude in global area and rates of land-use conversion, but research is also needed on the fates of ecosystem carbon upon conversion. Currently, carbon emissions from the conversion of vegetated coastal ecosystems are not included in emissions accounting or carbon market protocols, but this analysis suggests they may be disproportionally important to both. Although the relevant science supporting these initial estimates will need to be refined in coming years, it is clear that policies encouraging the sustainable management of coastal ecosystems could significantly reduce carbon emissions from the land-use sector, in addition to sustaining the well-recognized ecosystem services of coastal habitats