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Book missense mutation with the TP63 gene within a newborn using Hay-Wells/Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndrome: specialized medical record as well as follow-up.
BACKGROUND AND PURPOSE Cognitive impairment occurs frequently in multiple sclerosis (MS). However, the prevalence and clinical characteristics of cognitive MS phenotype are not well established. The aim of the study was to characterize the clinical course and neurocognitive impairment of patients with MS meeting an Expanded Disability Status Scale (EDSS)-defined cognitive phenotype. METHODS A total of 2302 patients from the Comprehensive Longitudinal Investigation of Multiple Sclerosis at Brigham and Women's Hospital (CLIMB) study were studied. Predominant cognitive MS phenotype was defined as EDSS Cerebral Functional System (FS) subscore ≥3 and remaining EDSS FS subscores ≤2 on at least one clinical visit. Demographic/clinical characteristics, phenotype stability and neurocognitive domain impairment of these subjects were assessed. RESULTS A total of 60 of 2302 (2.6%) patients (age 52.8 ± 10.8 years, 68% female, 82% relapsing MS) met criteria for phenotype designation. A total of 29 of 60 (48%) were designatgnitive status. © 2020 European Academy of Neurology.Asthma affects 300 million people across all age-groups and ethnicities and is the most common chronic condition affecting children. In the UK, the health care costs associated with asthma are estimated at £1.1 billion, however this amount typically excludes some societal costs (e.g. absence from work to care for children). The total number of days missed from school in England in the 2017/18 academic year was 59.1 million of which 54.7% were due to illness although the reasons for those illness-related absences are unknown. This article is protected by copyright. All rights reserved.Integration of various photodetectors with different light-sensitive materials and detection capacity is an inevitable way to achieve entire color/spectrum detection. However, the uneven capacity of each photodetector would drag the overall performance behind, especially the response speed. A response time down to nanosecond level has not previously been reported for a filter-free color/spectrum-sensitive photodetector, as far as is known. Here, a self-powered filterless color-sensitive photodetection array based on an in situ formed gradient perovskite absorber film with continuously tunable bandgap is demonstrated. Ultrahigh-speed response at nanosecond level is achieved in all the ingredient photodetectors. The junction capacitance being influenced by carrier concentration in the absorber is identified to be responsible for the detection speed. Without any optic or mechanical supporting system, the designed color detector exhibits an external quantum efficiency (EQE) up to 94% and a high spectral resolution of around 80 nm for the whole visible spectrum. This work offers a guidance to achieve fast response of perovskite-based photodetectors from the point of view of carrier-donor control and demonstrates a new avenue to establish color-sensitive photodetectors/spectrometers. © 2020 WILEY-VCH Verlag GmbH & Co. #link# KGaA, Weinheim.Ferroelectric field-effect transistors (FeFETs) are one of the most interesting ferroelectric devices; however, they, usually suffer from low interface quality. The recently discovered 2D layered ferroelectric materials, combining with the advantages of van der Waals heterostructures (vdWHs), may be promising to fabricate high-quality FeFETs with atomically thin thickness. Here, dual-gated 2D ferroelectric vdWHs are constructed using MoS2 , hexagonal boron nitride (h-BN), and CuInP2 S6 (CIPS), which act as a high-performance nonvolatile memory and programmable rectifier. It is first noted that the insertion of h-BN and dual-gated coupling device configuration can significantly stabilize and effectively polarize ferroelectric CIPS. Through this design, the device shows a record-high performance with a large memory window, large on/off ratio (107 ), ultralow programming state current (10-13 A), and long-time endurance (104 s) as nonvolatile memory. As for programmable rectifier, a wide range of gate-tunable rectification behavior is observed. Moreover, the device exhibits a large rectification ratio (3 × 105 ) with stable retention under the programming state. This demonstrates the promising potential of ferroelectric vdWHs for new multifunctional ferroelectric devices. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.Organic color-centers (OCCs) have emerged as promising single-photon emitters for solid-state quantum technologies, chemically specific sensing, and near-infrared bioimaging. However, these quantum light sources are currently synthesized in bulk solution, lacking the spatial control required for on-chip integration. The ability to pattern OCCs on solid substrates with high spatial precision and molecularly defined structure is essential to interface electronics and advance their quantum applications. Herein, a lithographic generation of OCCs on solid-state semiconducting single-walled carbon nanotube films at spatially defined locations is presented. By using light-driven diazoether chemistry, it is possible to directly pattern p-nitroaryl OCCs, which demonstrate chemically specific spectral signatures at programmed positions as confirmed by Raman mapping and hyperspectral photoluminescence imaging. This light-driven technique enables the fabrication of OCC arrays on solid films that fluoresce in the shortwave infrared and presents an important step toward the direct writing of quantum emitters and other functionalities at the molecular level. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.Invasive alien species are a significant threat to both economic and ecological systems. Identifying the processes that give rise to invasive populations is essential for implementing effective control strategies. We conducted an ancestry analysis of invasive feral swine (Sus scrofa, Linnaeus, 1758), a highly destructive ungulate that is widely distributed throughout the contiguous United States, to describe introduction pathways, sources of newly emergent populations and processes contributing to an ongoing invasion. Comparisons of high-density single nucleotide polymorphism genotypes for 6,566 invasive feral swine to a comprehensive reference set of S. scrofa revealed that the vast majority of feral swine were of mixed ancestry, with dominant genetic associations to Western heritage breeds of domestic pig and European populations of wild boar. Further, read more of invasive feral swine over the past 30 years was attributable to secondary introductions from established populations of admixed ancestry as opposed to direct introductions of domestic breeds or wild boar.
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