7,529 research outputs found

    Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID)

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    Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA allows accumulation of toxic metabolites causing defects of both cell mediated and humoral immunity leading to ADA severe combined immune deficiency (SCID), a condition that can be fatal in early infancy if left untreated. Hematopoietic stem cell transplant is curative but is dependent on a good donor match. Other therapeutic options include enzyme replacement therapy (ERT) with pegademase bovine (PEG-ADA) and more recently gene therapy. PEG-ADA has been used in over 150 patients worldwide and has allowed stabilization of patients awaiting more definitive treatment with hematopoietic stem cell transplant. It affords both metabolic detoxification and protective immune function with patients remaining clinically well, but immune reconstitution is often suboptimal and may not be long lived. We discuss the pharmacokinetics, immune reconstitution, effects on systemic disease and side effects of treatment with PEG-ADA. We also review the long-term outcome of patients receiving ERT and discuss the role of PEG-ADA in the management of infants and children with ADA-SCID, alongside other therapeutic options

    ADA newsletter

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    This archived document is maintained by the State Library of Oregon as part of the Oregon Documents Depository Program. It is for informational purposes and may not be suitable for legal purposes.Mode of access: Internet from the Oregon Government Publications Collection.Text in English

    The very short story in the time of revolution. al-Mihmāz (the Spur) and the Syrian author Zakariyā Tāmir

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    The mass protests swept through the Middle East in early 2011 underlined the role of modern information-communication technologies (ICT). From a literary point of view, the “Arab Spring” inevitably marked the birth of a new model of writing, characterised by a more participatory, global and immediate manner of expression that could be defined as Humanism 2.0. In this context, we may insert the experimental writing by the famous Syrian author Zakariyā Tāmir: on the al-Mihmāz (The Spur) Facebook page the writer begins a literary journey publishing daily posts and explicitly supporting the Syrian revolution. This contribution intends to analyse a few of Tāmir’s most significant posts published on Facebook. The time span is 2012, just one year after the Syrian revolution: thanks to aphorisms, posts and short stories, a new literary pact with potential readers is inaugurated, within a phenomenon that we can call al-adab al-raqmī (digital literature

    Introduction a "Iraq After 2003: When Trauma Becomes Art. Myth, History, and Literature"

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    The article aims at introducing the volume "Iraq After 2003: When Trauma Becomes Art. Myth, History, and Literature", edited By Ada Barbaro. The volume aims to provide lenses that examine, from various angles, a country, Iraq, in the aftermath of the quintessential trauma of its contemporary history, namely, 2003.The volume is thus a collection of "narratives", of narrative acts that render the relationship between reality and its discourse almost oxymoronic. The present work, born in the wake of the conference «Iraq After 2003: When Trauma Becomes Art. Myth, History, and Literature», is therefore published as an outcome of the scientific project "Forms, Languages, and [Con]texts of Tàrìkh: Writing and Rewriting History in Iraq", of which the author of these pages is the Principal Investigator

    19 Patung Kayu 'Penjaga' Ditemukan di Situs Kuno Chan Chan Peru

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    Para arkeolog menemukan sebuah koridor yang berisi 19 patung kayu di situs arkeologi Chan Chan dekat Trujillo, Peru Utara, pada Senin (22/10/2018). Menurut para arkeolog, patung tersebut berusia sekitar 800 tahun dan merupakan 'penjaga' di Chimú, Chan Chan. Menurut Henry Gayoso Rullier, seorang arkeolog, patung tersebut sudah ada sejak 1.100 dan 1.300 Masehi dan menjadi bagian dari Chan chan. Chan Chan sendiri merupakan kota terbesar di Amerika sebelum kedatangan Columbus dan terdaftar sebagai situs Warisan Dunia UNESCO pada tahun 1986. Ia memiliki lebih dari 10.000 bangunan

    Kritik Sastra Humanis dalam Novel Totto-Chan To Totto-Chan Tachi Karya Tetsuko Kuroyanagi

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    Penelitian ini bertujuan untuk mendeskripsikan unsur-unsur humanisme dalam novel Totto-Chan To Totto-Chan Tachi dengan menggunakan teori humanisme Blackham. Data yang digunakan berupa penggalan narasi dan kalimat dalam novel Totto-Chan To Totto-Chan Tachi. Metode yang digunakan metode deskriptif kualitatif dengan menganalisis unsur humanisme yang ada dalam novel. Teknik pengumpulan data yang digunakan adalah studi kepustakaan. Analisis dilakukan terhadap 22 (dua puluh dua) data berupa penggalan narasi berbentuk kalimat. Berdasarkan hasil penelitian ditemukan 4 (empat) unsur humanisme antara lain (1) otonom (2) realistis (3) tanggung jawab pada diri sendiri dan (4) tanggung jawab pada kemanusiaan pada umumnya. Empat unsur humanisme yang ada mengambarkan perilaku manusia yang menjadikan humanisme sebagai jalan tengah dalam kehidupan sehari-hari

    Il culto di san Michele in Campania. Antonino e Catello

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    Il volume, diviso in quattro sezioni, approfondisce l'origine e la diffusione del culto di San Michele in Campania, nelle sue varie espressioni. Nella prima parte vengono indagati gli insediamenti micaelici, con particolare riferimento alle numerose grotte che, sull'esempio di quella garganica, furono dedicate a San Michele soprattutto lungo l'asse Salerno-Avellino- Benevento. Il volume analizza numerosi testi agiografici campani che attestano la presenza dell'Angelo talvolta con generiche funzioni di angelo custode, talaltra con gli attributi tipici di San Michele. Una cospicua sezione del volume è dedicata al dossier agiografico di Antonino e Catello, rispettivamente monaco e vescovo di Sorrento e Stabia, un'area da sempre roccaforte bizantina. Sulla base di elementi storici e di elementi rivenienti dal dossier agiografico viene posticipata all'XI secolo - e quindi di oltre un secolo rispetto alle posizioni della critica- l'epoca di composizione della Vita Antonini (XI secolo). In quest'epoca Sorrento vive un particolare momento di vivacità e di rinnovamento a livello politico, sociale e religioso. Nei testi agiografici Antonino e Catello sono co-fondatori del Santuario del Monte Faito, considerato il santuario nazionale dei Bizantini, anche per ragioni di antagonismo e rivalità con il Santuario di San Michele del Gargano, santuario nazionale dei LongobardiThe book, divided into four parts, is a thorough analysis of the cult of St. Michael in Campania, in its various expressions. In the first part the author presents micaelici settlements with particular reference to the numerous caves that, based on the example of the Gargano, were dedicated to the Angel, especially along the road Salerno-Avellino-Benevento. The book contains an examination of the hagiographic texts from Campania attesting the presence of Angel, sometimes in its generic function of custos, sometimes with attributes typical of Michael. The author analyzes the hagiographic dossier of Antoninus and Catello, respectively monaco and bishop of Sorrento and Stabiae. The author basing on historical data and data emerging from the same dossier, delaies more than a century the composition of the Vita Antonini (11th century). In this century Sorrento lived a revival on political-institutional-religious level. The anonymous author of Life - probably a monaco /bishop - approaches Antonino (IX century) and Catello (sixth century) making the abbot of Sorrento co-starred in the foundation of the sanctuary of St. Michael on Mount Fait, considered a national shrine of the Byzantines in Naples, also for reasons of competition with the sanctuary of Mount St. Michael Gargano, traditionally linked to the Lombards

    The construction of Karen Karnak: The multi-author-function

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    This thesis is situated within the comparatively recent developments of Web 2.0 and the emergence of interactive WikiMedia, and explores the mode of authorship within a Read/Write culture compared to that of a Read/Only tradition. The hypothesis of this study is that the role of the audience has become merged with the author, and as such, represents new functions and attributes, distinct from a more conventional concept of authorship, in which the roles of audience and author are more separate. Read/Write and participatory culture, as defined by this study, is focused on collaboration, and includes the influences of D.I.Y. culture, Open-Source practices and the production of text by multiple authors. Multi-authorship presents a re-thinking of several concepts which support the notion of the individual author, since the focus of multi-authorship is not on attribution and ownership of a finished text, but on the continued malleability of a text. Modes of multi-authorship, demonstrated in the use of the pseudonyms Alan Smithee and Karen Eliot, represent declarative authors whose names signify multiple origins, whilst concurrently indicating a distinct body of work. The function of these names form an important context to this study, since primary research involves the construction of an experimental mode of multi-authorship utilising WikiMedia technology and the interaction of thirty nine participants, who are invited to create a body of work under the collective pseudonym Karen Karnak. The data generated by this experiment is analysed using aspects of Michel Foucault's author-function to identify and determine power structures inherent in the WikiMedia context. The interplay of power structures, including concepts such as identity, ownership and the body of work, affect the resulting mode of authorship and contribute to the construction of Karen Karnak, suggesting further areas of research into the emerging multi-author

    ... ADA Program report

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    Began with 2020.This archived document is maintained by the State Library of Oregon as part of the Oregon Documents Depository Program. It is for informational purposes and may not be suitable for legal purposes.Mode of access: Internet from the Oregon Government Publications Collection.Text in English

    Iraq after 2003: when trauma becomes art. Myth, history, and literature

    No full text
    The volume aims to provide lenses that examine, from various angles, a country, Iraq, in the aftermath of the quintessential trauma of its contemporary history, namely, 2003.The volume is thus a collection of "narratives", of narrative acts that render the relationship between reality and its discourse almost oxymoronic. The present work, born in the wake of the conference «Iraq After 2003: When Trauma Becomes Art. Myth, History, and Literature», is therefore published as an outcome of the scientific project "Forms, Languages, and [Con]texts of Tàrìkh: Writing and Rewriting History in Iraq", of which the author of these pages is the Principal Investigator
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