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                <text>BOALA POMPE: CRITERII DE DIAGNOSTIC ŞI TRATAMENT</text>
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                <text>Georgeta Diaconu, Ioana Grigore, M. Burlea</text>
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                <text>Boala Pompe este o afecţiune neuromusculară rară, cu transmitere autosomal recesivă, datorată unui defi citenzimatic lizozomal de acid alfa glucozidază (GAA) care determină acumularea de glicogen în ţesuturi.Forma cu debut precoce, în perioada de sugar, este cea mai severă şi se manifestă prin hipotonie şicardiomiopatie cu deces în primii ani de viaţă. Diagnosticul poate fi confi rmat printr-o evaluare cantitativă a activităţii enzimei GAA la nivelul fi broblaştilor, prin biopsie musculară sau cutanată. Acestă metodă se realizează prin proceduri invazive, iar rezultatele se obţin după câteva săptămâni, ceea ce întârzie diagnosticul. În ultimii ani s-a dezvoltat o nouă tehnică de diagnostic care cercetează activitatea enzimatică pe mostre de sânge uscat, care nu este invazivă şi care permite diagnosticul precoce al bolii Pompe.Tratamentul constă în terapie de substituţie enzimatică cu alglucozidază alfa, care modifi că evoluţia naturalăa bolii, la pacienţii cu debut infantil prelungind supravieţuirea, iar la cei cu debut tardiv îmbunătăţind distanţa parcursă în mers şi stabilizând funcţia pulmonară.</text>
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                <text>2012</text>
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                <text>boala pompe, copil, terapie enzimatică</text>
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                <text>DOI: 10.37897/RJP.2012.3.1</text>
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                <text>Revista Romana de Pediatrie</text>
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                <text>Ergin Çiftçi, Fevziye ÇOKSÜER</text>
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                <text>Coronaviruses (CoV) are enveloped, single-chain positive RNA viruses constituting a fairly large family. 229E, OC43, NL63 and HKU1 species are common in humans, often causing mild to moderate respiratory infections. On the other hand, new coronaviruses have emerged, causing severe respiratory infection such as “severe acute respiratory syndrome” (SARS) in 2002 and “Middle East respiratory syndrome” (MERS) in 2012. The most recent coronavirus, SARS-CoV-2, causing COVID-19 disease was identified in 2019. While people of all ages are susceptible to COVID-19 infection, those with underlying chronic diseases and elderly age have increased susceptibility. There is no specific antiviral drug for COVID-19 infection. Therefore, treatment strategy is mostly aimed at supportive treatment and prevention of complications. The main protection measure is to avoid contact with the person infected with COVID-19. Following the identification of COVID-19 disease, it exceeded the region where it was located, the number of cases increased gradually, and intercontinental reports were made. COVID-19 is a highly pathogenic respiratory virus threatening global public health today. In order to eradicate the virus, it is necessary to clarify the source of the virus, to develop specific, effective treatments and a safe vaccine. Effective surveillance and appropriate infection control measures are critical to prevent the spread of the virüs.</text>
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                <text>DOI: 10.5578/flora.202002</text>
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                <text>Flora Infeksiyon Hastalıkları ve Klinik Mikrobiyoloji Dergisi</text>
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                <text>Bilimsel Tip Yayinevi</text>
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                <text>Muhammad Mahboob Ali</text>
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                <text>Besides human death economic catastrophe, which has already been started around the world due to COVID19, a question arises how to tackle the devastating nature of this pandemic. The study is basically done based on secondary sources. Time period of the study was up to April 6, 2020. The study tried to get a conceptual view for time demanding collaborative effort among the global arena so that this pandemic cannot make long-lasting devastating impact on global economy. The study also did a small opinion poll survey by email/cell phone, land phone, and personally asking by maintaining social distance among 108 respondents considering three segments of the income strata of the people in Bangladesh. Based on the respondents replies, Figure 2 was drawn, which is shown in Appendix.</text>
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                <text>aggregate demand, aggregate supply shock, COVID-19, Informal Economy, joint analysis of fiscal and monetary policy, Unemployment</text>
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                <text>DOI: 10.21511/gg.03(1).2020.03</text>
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                <text>International relations</text>
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                <text>The COVID-19 outbreak continues to evolve with the number of cases increasing in Malaysia, placing a significant burden on general practitioners (GPs) to assess and manage suspected cases. GPs must be well equipped with knowledge to set up their clinics, use Personal Protective Equipment (PPE) appropriately, adopt standard protocols on triaging and referrals, as well aseducate patients about PPE. The correct use of PPE will help GPs balance between personal safety and appropriate levels of public concern.</text>
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                <text>Li Sun, Yaling Xing, Xiao-Juan Chen, Yang Zheng, Yudong Yang, Daniel B Nichols, Mark A. Clementz, Bridget S Banach, Kui Li, Susan C. Baker, Zhongbin Chen</text>
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                <text>Viruses have evolved elaborate mechanisms to evade or inactivate the complex system of sensors and signaling molecules that make up the host innate immune response. Here we show that human coronavirus (HCoV) NL63 and severe acute respiratory syndrome (SARS) CoV papain-like proteases (PLP) antagonize innate immune signaling mediated by STING (stimulator of interferon genes, also known as MITA/ERIS/MYPS). STING resides in the endoplasmic reticulum and upon activation, forms dimers which assemble with MAVS, TBK-1 and IKKε, leading to IRF-3 activation and subsequent induction of interferon (IFN). We found that expression of the membrane anchored PLP domain from human HCoV-NL63 (PLP2-TM) or SARS-CoV (PLpro-TM) inhibits STING-mediated activation of IRF-3 nuclear translocation and induction of IRF-3 dependent promoters. Both catalytically active and inactive forms of CoV PLPs co-immunoprecipitated with STING, and viral replicase proteins co-localize with STING in HCoV-NL63-infected cells. Ectopic expression of catalytically active PLP2-TM blocks STING dimer formation and negatively regulates assembly of STING-MAVS-TBK1/IKKε complexes required for activation of IRF-3. STING dimerization was also substantially reduced in cells infected with SARS-CoV. Furthermore, the level of ubiquitinated forms of STING, RIG-I, TBK1 and IRF-3 are reduced in cells expressing wild type or catalytic mutants of PLP2-TM, likely contributing to disruption of signaling required for IFN induction. These results describe a new mechanism used by CoVs in which CoV PLPs negatively regulate antiviral defenses by disrupting the STING-mediated IFN induction.</text>
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                <text>DOI: 10.1371/journal.pone.0030802</text>
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                <text>Viral respiratory tract infections are one of the leading causes of morbidity and mortality in pediatric pathology.The authors provide the latest information on the structure, pathogenesis, clinical features, epidemiology andresponse to treatment of newly discovered viruses affecting the respiratory tract in children: bocavirus, metapneumovirus, coronaviruses, rhinovirus C.</text>
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                <text>bocavirus, Metapneumovirus, coronaviruses, rhinovirus C, Pneumonia, Bronchiolitis</text>
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                  <text>Dominio científico: Coronavirus</text>
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                <text>Editoriale/Editorial</text>
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                <text>Augusto Balloni</text>
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            <name>Description</name>
            <description>An account of the resource</description>
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                <text>Le numerose vittime del coronavirus impongono anche riflessioni filosofiche ed etiche poiché rimandano all’indagine sull’uomo, ai suoi valori, alle sue esigenze in ogni ambito del vivere, con particolare riguardo alla salute.</text>
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            <name>Date</name>
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                <text>2020</text>
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            <name>Identifier</name>
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                <text>DOI: </text>
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            <name>Source</name>
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                <text>Rivista di Criminologia, Vittimologia e Sicurezza</text>
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            <name>Publisher</name>
            <description>An entity responsible for making the resource available</description>
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                <text>Società Italiana di Vittimologia</text>
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                <text>Social pathology. Social and public welfare. Criminology</text>
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            <name>Language</name>
            <description>A language of the resource</description>
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