Thymomaassociated Tcell immunodeficiency following radiotherapy In a situation document

61 to 0.54). RKF was assessed in one report which demonstrated a potential reduction over 12 months with extended-hours hemodialysis. The majority of trials had high risk of bias. Extended-hours hemodialysis was not associated with improved QoL or mortality, or increased vascular access events. Adequately powered RCTs are needed to fully assess extended-hours hemodialysis. © 2020 International Society for Hemodialysis.Lateral meningocele syndrome (LMS) is due to specific pathogenic variants in the last exon of NOTCH3 gene. Besides the lateral meningoceles, this condition presents with dysmorphic features, short stature, congenital heart defects, and feeding difficulties. Here, we report a girl with neurosensorial hearing loss, severe gastroesophageal reflux disease, congenital heart defects, multiple renal cysts, kyphosis and left-convex scoliosis, dysmorphic features, and mild developmental delay. Exome sequencing detected the previously unreported de novo loss-of-function variant in exon 33 of NOTCH3 p.(Lys2137fs). Following the identification of the gene defect, MRI of the brain and spine revealed temporal encephaloceles, inner ears anomalies, multiple spinal lateral meningoceles, and intra- and extra-dural arachnoid spinal cysts. This case illustrates the power of reverse phenotyping to establish clinical diagnosis and expands the spectrum of clinical manifestations related to LMS to include inner ear abnormalities and multi-cystic kidney disease. © 2020 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc.The wide applications of Ni-rich LiNi1- x-y Cox Mny O2 cathodes are severely limited by capacity fading and voltage fading during the cycling process resulting from the pulverization of particles, interfacial side reactions, and phase transformation. The canonical surface modification approach can improve the stability to a certain extent; however, it fails to resolve the key bottlenecks. The preparation of Li(Ni0.4 Co0.2 Mn0.4 )1- x Tix O2 on the surface of LiNi0.8 Co0.1 Mn0.1 O2 particles with a coprecipitation method is reported. After sintering, Ti diffuses into the interior and mainly distributes along surface and grain boundaries. A strong surface and grain boundary strengthening are simultaneously achieved. The pristine particles are fully pulverized into first particles due to mechanical instability and high strains, which results in serious capacity fading. In contrast, the strong surface and the grain boundary strengthening can maintain the structural integrity, and therefore significantly improve the cycle stability. A general and simple strategy for the design of high-performance Ni-rich LiNi1- x - y Cox Mny O2 cathode is provided and is applicable to surface modification and grain-boundary regulation of other advanced cathodes for batteries. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.BACKGROUND Organizing centers are groups of specialized cells that secrete morphogens, thereby influencing development of their neighboring territories. Apoptosis is a form of programmed cell death reported to limit the size of organizers. Little is known about the identity of intracellular signals driving organizer cell death. Here we investigated in Xenopus the role of both the anti-apoptotic protein Myeloid-cell-leukemia 1 (Mcl1) and the cysteine proteases Caspase-3 and Caspase-7 in formation of the axial organizing center-the notochord-that derives from the Spemann organizer, and participates in the induction and patterning of the neuroepithelium. RESULTS We confirm a role for apoptosis in establishing the axial organizer in early neurula. We show that the expression pattern of mcl1 is coherent with a role for this gene in early notochord development. Using loss of function approaches, we demonstrate that Mcl1 depletion decreases neuroepithelium width and increases notochord cells apoptosis, a process that relies on Caspase-7, and not on Caspase-3, activity. Our data provide evidence that Mcl1 protein levels physiologically control notochord cells' survival and that Caspase-7 is the executioner protease in this developmental process. CONCLUSIONS Our study reveals new functions for Mcl1 and Caspase-7 in formation of the axial signalling center. learn more © 2020 Wiley Periodicals, Inc.Asymmetric organocatalysis is becoming one of the main tools for the synthesis of chiral compounds that are needed as medicines, crop protection agents, and other bioactive molecules. It can be effectively combined with various green chemistry methodologies. Intensification techniques such as ball milling, flow, high pressure, or light bring not only higher yields, faster reactions, easier product isolation but also new reactivities. More sustainable reaction media such as ionic liquids, deep eutectic solvent, green solvent alternative, and water also considerably enhance the sustainability profile of many organocatalytic reactions. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.Dormancy cycling is a key mechanism that contributes to the maintenance of long-term persistent soil seed banks but has not been recorded in long-lived woody shrub species from fire-prone environments. Such species rely on seed banks and dormancy break as important processes for post-fire recruitment and recovery. We used germination experiments with smoke treatments on fresh seeds and those buried for 1 year (retrieved in spring) and 1.5 years (retrieved the following late autumn) to investigate whether Asterolasia buxifolia, a shrub from fire-prone south eastern Australia with physiologically dormant seeds, exhibited dormancy cycling. All seeds had an obligation for winter seasonal temperatures and smoke to promote germination, even after ageing in the soil. A high proportion of germination was recorded from fresh seeds but germination after the first retrieval was significantly lower, despite high seed viability. After the second retrieval, germination returned to the initial level. This indicates a pattern of annual dormancy cycling, one of the few observations to our knowledge for a perennial species. Additionally, A. buxifolia's winter temperature and smoke requirements did not change over time, highlighting the potential for seeds to remain conditionally dormant (i.e. restricted to a narrow range of germination conditions) for long periods. For physiologically dormant species such as A. buxifolia, we conclude that dormancy cycling is an important driver of successful regeneration, allowing seed bank persistence, sometimes for decades during fire-free periods unsuitable for successful recruitment, while ensuring that a large proportion of seeds are available for recruitment when a fire occurs. This article is protected by copyright. All rights reserved.