139 research outputs found

    Case report: Excessive daytime sleepiness as a presenting manifestation of autoimmune glial fibrillary acidic protein astrocytopathy

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    Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a recently discovered autoimmune inflammatory disease of the central nervous system. It presents with a variety of clinical symptoms, including fever, seizures, psychiatric symptoms, limber weakness, and sensory symptoms. However, the symptoms of sleep disorders have not been sufficiently addressed. Here, we report a case of GFAP-A in which the patient complained of excessive daytime sleepiness and an excessive need for sleep. Our patient was a 58-year-old male who experienced excessive daytime sleepiness for 50 days following SARS-CoV-2 infection. He was diagnosed with coronavirus disease 2019 on June 1st. On the 7th of June, he experienced excessive daytime sleepiness, nausea, reduced food intake, lower limb weakness, and dysuria. Subsequently, his sleepiness significantly deteriorated on July 21st. Five months prior, the patient underwent laparoscopic partial right nephrectomy for clear-cell renal cell carcinoma. Brain MRI revealed abnormal hyperintense lesions in the pontine brain and around the mesencephalic aqueduct on T2 and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences However, these lesions did not exhibit any pathological enhancement. Spinal cord MRI revealed lesions in the C6–C7 and T2–T3 segments on the T2 sequence. His Epworth Sleepiness Scale (ESS) score was 16 (reference range, <10), and 24-hour polysomnography supported the diagnosis of rapid-eye-movement sleep disorder and severe sleep apnea-hypopnea syndrome. Glial fibrillary acidic protein IgG antibodies were detected in the cerebrospinal fluid (1:32, cell-based assay) but not in the serum. The level of hypocretin in the cerebrospinal fluid was 29.92 pg/mL (reference range ≥110 pg/mL), suggesting narcolepsy type 1. After treatment with corticosteroids for approximately 1 month, the patient showed considerable clinical and radiological improvement, as well as an increase in hypocretin levels. Although repeated polysomnography and multiple sleep latency tests suggested narcolepsy, his ESS score decreased to 8. Our findings broaden the range of clinical manifestations associated with GFAP-A, thereby enhancing diagnostic and therapeutic strategies for this disease. Additionally, our results indicate a potential common autoimmune mechanism involving GFAP-A and orexin system dysregulation, warranting further investigation

    Bile acid profiling reveals distinct signatures in undernourished children with environmental enteric dysfunction

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    Background: Intestinal inflammation and malabsorption in environmental enteric dysfunction (EED) are associated with early childhood growth faltering in impoverished settings worldwide.Objectives: The goal of this study was to identify candidate biomarkers associated with inflammation, EED histology, and as predictors of later growth outcomes by focusing on the liver-gut axis by investigating the bile acid metabolome.Methods: Undernourished rural Pakistani infants (n = 365) with weight-for-height Z score (WHZ) \u3c -2 were followed up to the age of 24 mo and monitored for growth, infections, and EED. Well-nourished local children (n = 51) were controls, based on consistent WHZ \u3e 0 and height-for-age Z score (HAZ) \u3e -1 on 2 consecutive visits at 3 and 6 mo. Serum bile acid (sBA) profiles were measured by tandem MS at the ages of 3-6 and 9 mo and before nutritional intervention. Biopsies and duodenal aspirates were obtained following upper gastrointestinal endoscopy from a subset of children (n = 63) that responded poorly to nutritional intervention. BA composition in paired plasma and duodenal aspirates was compared based on the severity of EED histopathological scores and correlated to clinical and growth outcomes.Results: Remarkably, \u3e70% of undernourished Pakistani infants displayed elevated sBA concentrations consistent with subclinical cholestasis. Serum glycocholic acid (GCA) correlated with linear growth faltering (HAZ, r = -0.252 and -0.295 at the age of 3-6 and 9 mo, respectively, P Conclusions: Dysregulated bile acid metabolism is associated with growth faltering and EED severity in undernourished children. Restoration of intestinal BA homeostasis may offer a novel therapeutic target for undernutrition in children with EED. This trial was registered at clinicaltrials.gov as NCT03588013

    Association between ecological risks and ecosystem services in an urban agglomeration in arid China

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    Rapid urbanization leads to changes in ecosystem services and may exacerbate ecological risks. Clarifying the relationship between these two factors in a specific context is essential to explore the integrated management model and achieve sustainable regional development. However, previous studies mainly lack an integrated analysis, fail to clearly explain the mechanism of ecosystem change, and can neither support landscape ecological security construction nor spatial planning and management. This study, using the urban agglomeration on the northern slope of the Tianshan Mountains (UANSTM) as an example, applied multi-source data from 2010 to 2020, investigated the changes and relationships between ecological risks and ecosystem services, and proposes an assessment framework. The total ecosystem services (TES) of the studied agglomeration showed a decreasing trend, with an overall loss of 0.43%. Corresponding to the decrease of ecosystem services, the ecological risk was higher in the south and north of the UANSTM and lower in the northwestern, central, and eastern regions. The proportion of ecological high-risk areas was expanding. The key to the relationship between ecological risks and ecosystem services is the change in hydrological conditions. Therefore, we suggest that the UANSTM actively transforms the development and use mode of water resources and coordinates their allocation, aiming to reduce regional ecological risks and optimize the pattern of ecosystem services

    A System of Photocatalysis for NAD+ Regeneration of Product of (S)-1-Pheylethanol by Enzymic Catalysis

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    In this study, a system of photocatalysis for NAD+ regeneration of enzymatic catalysis was constructed. The optimal conditions for the coupling reaction of photocatalysis and biocatalysis were explored. Blue light was chosen for the efficient reaction and the optimal concentration of VB2 (vitamin B2, riboflavin) was determined. NAD+-dependent (R)-1-phenylethanol dehydrogenase was used in the reaction for transforming (R)-1-phenylethanol to acetophenone. The byproducts of the reaction were just H2O and O2 by means of catalase. The coupling reaction of catalysis and photocatalysis can be used for obtaining (S)-1-phenylethanol through racemization of 1-phenylethanol. Copyright © 2019 BCREC Group. All rights reserve

    Genome-wide association studies in ancestrally diverse populations: opportunities, methods, pitfalls, and recommendations

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    Genome-wide association studies (GWASs) have focused primarily on populations of European descent, but it is essential that diverse populations become better represented. Increasing diversity among study participants will advance our understanding of genetic architecture in all populations and ensure that genetic research is broadly applicable. To facilitate and promote research in multi-ancestry and admixed cohorts, we outline key methodological considerations and highlight opportunities, challenges, solutions, and areas in need of development. Despite the perception that analyzing genetic data from diverse populations is difficult, it is scientifically and ethically imperative, and there is an expanding analytical toolbox to do it well

    Windows of Opportunity in the CoPS’s Catch-Up Process: A Case Study of China’s High-Speed Train Industry

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    Over the last 20 years, the rapid development of high-speed Chinese trains has provided valuable guidelines to countries and companies eager to develop their complex product and system (CoPS) sectors. CoPS refers to the high cost and technology-intensive systems, networks, infrastructure and engineering constructs, and services. Although established studies highlight the importance of CoPS to the economy and development of a country, especially those that have yet to develop high-speed rail, scholars have not paid much attention to investigating the catch-up of CoPS industries from the windows of opportunity perspective. We aimed to fill in this research gap by analyzing the successful catch-up of Chinese high-speed trains. Based on a longitudinal case study of this industry, we analyzed and detailed the mechanisms of the catch-up process of a typical CoPS sector and its development. Our results enrich the literature of the catch-up process, CoPS, and the studies of windows of opportunity. In particular, we show that the windows of opportunity that emerge in the catch-up process of CoPS have the characteristics of endogeneity, asymmetry, and relevance. Moreover, our study further indicates that the endogenous windows of opportunity regarding policy result in the development of later windows of technology and demand. We also find that the technology’s windows of opportunity consist of the window to broaden the technology width and the window to deepen the technology depth of latecomers. In addition to theoretical contributions, our findings can help policymakers of latecomer countries better formulate CoPS industrial policies, followed by a gradual progress in successfully catch-up with the leading countries
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