Primary immunodeficiency diseases, first recognized 60 years ago, are inherited disorders that affect human adaptive and innate immunity. In most cases, affected individuals experience recurrent infections, but they may also suffer from autoimmune diseases and malignancies. This third edition of Primary Immunodeficiency Diseases provides readers with the historic and scientific background, clinical presentations, immunologic characteristics, and the molecular/genetic underpinnings of this rapidly enlarging class of diseases. With up-to-date diagnostic tools and therapeutic options - from prophylactic anti-infective measures to hematopoietic stem cell transplantation and gene therapy - this volume will remain an authoritative resource on this increasingly important area.

Here come the library babies, ready to give you a tour of their favourite local library! There's no place more magical than a library, where adventures can be had both inside the pages of a book and out. Here you can find hidden nooks for reading, encounter new worlds during story time, and take the big step of signing up for your very first library card. From puppet shows to getting to take out towering stacks of books to enjoy at home, this joyful board book teaches every child that through books and libraries, the possibilities are endless. And the fun doesn't end there: Library Babies includes oodles of activities and educational reading tips to help parents enjoy the book with their children over and over again!

New York babies don't just say "Mama" - they say "MoMA"! And they know all of the important shapes, like pizza and bagels and pretzels. In New York Baby, diverse babies act as the guides, showcasing where they live and play in the big city while providing an authentic tour of all things local in this colorful board book. From famed attractions to iconic sights, this educational and entertaining read captures the essence of what it means to live in and visit New York City. The last spread offers tips for activities to enjoy on every page of the book, from finding hidden objects to pointing to shapes and letters, allowing the book to be read over and over again!

*Winner of the Opera Prima Bologna Ragazzi Prize 2020* A stylish and funny search-and-find adventure with a twist, packed with twin-sister mischief. The store is a riot of dots and stripes, patterns and checks, so it's not easy to find one little girl in a spotty dress. Children will love finding Harriet on every page as the madcap chase moves from the fashion floor to home furnishings and into the toy department. More and more people join in until at last naughty Harriet is reunited with her family. Jam-packed with action and details to find and spot, Where Is Your Sister? is a strikingly confident debut from Puck Koper. Her blue, red and black artwork is printed in eye-catching pantone inks, and her remarkable eye for composition and funny characters marks her out as a talent to watch.

The relationship between politics and law in the early People's Republic of China was highly contentious. Periods of intentionally excessive campaign justice intersected with attempts to carve out professional standards of adjudication and to offer retroactive justice for those deemed to have been unjustly persecuted. How were victims and perpetrators defined and dealt with during different stages of the Maoist era and beyond? How was law practiced, understood, and contested in local contexts? This volume adopts a case study approach to shed light on these complex questions. By way of a close reading of original case files from the grassroots level, the contributors detail procedures and question long-held assumptions, not least about the Cultural Revolution as a period of "lawlessness."

Kaum ein Unternehmen ist nicht von internationalem Wettbewerb betroffen. Exporte, internationale Direktinvestitionen und multinationale Unternehmen sind nicht nur Schlagworte die sich taglich in den Medien finden, die Auswirkungen internationaler Unternehmensaktivitat beeinflussen auch erheblich die betriebswirtschaftliche Forschung. Dieser Sammelband bietet einen Uberblick uber aktuelle Ansatze, Theorien und empirische Studien zu diesen Themen. Geschrieben von renommierten Wissenschaftlern und erfahrenen Praktikern macht das Buch den Leser in kompakter und kompetenter Weise mit den aktuellen Trends auf diesen Gebieten vertraut.

Offering unique, comprehensive coverage of both basic science and clinical scenarios, Clinical Immunology: Principles and Practice, 6th Edition, brings you up to date with every aspect of this fast-changing field. It examines the molecular, cellular, and immunologic bases of immunologic diseases and their broader systemic implications; it also includes complete coverage of common and uncommon immunologic disorders. Updated with all the latest immunologic research and clinical implications, including breakthrough immunotherapies and molecular-based treatment protocols, this fully revised edition provides authoritative guidance from some of the most respected global leaders in immunology in one complete, well-illustrated volume. Includes extensive revisions that reflect rapidly expanding research and clinical advances, including breakthrough drug and immunotherapies such as immune checkpoint inhibitors, immunotherapies for cancer, precision medicine, and transfusion medicine. Contains new chapters on COVID-19, immune responses, and the role of the immune system; immunoregulatory deficiencies; immune checkpoints; CAR T cells, including new cellular-based immunotherapy; gene therapy, including CRISPR and gene selection; and a clinically focused chapter on asthma. Provides new genetics content focused on data applications. Addresses notable advances in key areas such as the importance of the microbiota to normal immune system development and to the pathogenesis of immunologic and inflammatory diseases; relationships between the innate and adaptive immune systems; progress in rapid and cost-effective genomics; cell signaling pathways and the structure of cell-surface molecules; and many more. Covers hot topics such as the role of genetics and genomics in immune response and immunologic disease, atherosclerosis, recurrent fever syndromes, aging and deficiencies of innate immunity, the role of microbiota in normal immune system development and in the pathogenesis of immunologic and inflammatory diseases, and novel therapeutics. Features a user-friendly format with color-coded boxes highlighting critical information on Key Concepts, Clinical Pearls, Clinical Relevance, and Therapeutic Principles. Summarizes promising research and development anticipated over the next 5-10 years with "On the Horizon" boxes and discussions of translational research. An eBook version is included with purchase. The eBook allows you to access all of the text, figures and references, with the ability to search, customize your content, make notes and highlights, and have content read aloud.

This research and teaching volume has been composed in honour of Rosalie Tung, a distinguished institution builder, thought leader and educator in the field of international business (IB). The volume addresses Rosalie Tung's main research focus in a career that has already spanned several decades, namely the analysis of distance facing multinational enterprises (MNEs), with a focus on state-of-the-art conceptual and fact-based empirical developments in the realm of cultural and institutional distance elements. The impact of distance on international business transactions and operations remains ill-understood. How should distance be conceptualized? Which dimensions of distance should be considered? Is distance always a cost, or can it sometimes confer value? This twelfth volume in the Progress in International Business Research series presents extensive accounts of the contemporary scientific debate on how to assess the impacts of distance, both negative and positive ones, on the conduct of international business. This volume covers five dimensions related to the concept, cost and value of distance, in International business: * The concept of distance * The cost of cultural and psychic distance * The cost of institutional distance * The value of distance * Alternative lenses for IB research

The relationship between politics and law in the early People's Republic of China was highly contentious. Periods of intentionally excessive campaign justice intersected with attempts to carve out professional standards of adjudication and to offer retroactive justice for those deemed to have been unjustly persecuted. How were victims and perpetrators defined and dealt with during different stages of the Maoist era and beyond? How was law practiced, understood, and contested in local contexts? This volume adopts a case study approach to shed light on these complex questions. By way of a close reading of original case files from the grassroots level, the contributors detail procedures and question long-held assumptions, not least about the Cultural Revolution as a period of "lawlessness."

A board book that celebrates two of our favourite things: bookstores and babies!

The journey starts in a sunny day in New York City and ends on a beautiful San Francisco night with stops in Mexico City, Rio de Janeiro, London, Paris, Cape Town, Cairo, Beijing, and Tokyo. These friendly babies welcome us to their cities with delightful greetings in their original language (with English translations) in a simple narration that will appeal to any global mini citizen.

The brutality and racial hatred exhibited by Japan's military during the Pacific War piqued outrage in the West and fanned resentments throughout Asia. Public understanding of Japan's wartime atrocities, however, often fails to differentiate the racial agendas of its military and government elites from the racial values held by the Japanese people. While not denying brutalities committed by the Japanese military, Honored and Dishonored Guests overturns these standard narratives and demonstrates rather that Japan's racial attitudes during wartime are more accurately discerned in the treatment of Western civilians living in Japan than the experiences of enemy POWs. The book chronicles Western communities in wartime Japan, using this body of experiences to reconsider allegations of Japanese racism and racial hatred. Its bold thesis is borne out by a broad mosaic of stories from dozens of foreign families and individuals who variously endured police harassment, suspicion, relocation, starvation, denaturalization, internment, and torture, as well as extraordinary acts of charity. The book's account of stranded Westerners-from Tokyo, Yokohama, and Kobe to the mountain resorts of Karuizawa and Hakone-yields a unique interpretation of race relations and wartime life in Japan.

This wide-ranging collection seeks to reassess conventional understanding of Japan’s Asia-Pacific War by defamiliarizing and expanding the rhetorical narrative. Its nine chapters, diverse in theme and method, are united in their goal to recover a measured historicity about the conflict by either introducing new areas of knowledge or reinterpreting existing ones. Collectively, they cast doubt on the war as familiar and recognizable, compelling readers to view it with fresh eyes. Following an introduction that problematizes timeworn narratives about a "unified Japan" and its "illegal war" or "race war," early chapters on the destruction of Japan’s diplomatic records and government interest in an egalitarian health care policy before, during, and after the war oblige us to question selective histories and moral judgments about wartime Japan. The discussion then turns to artistic/cultural production and self-determination, specifically to Osaka rakugo performers who used comedy to contend with state oppression and to the role of women in creating care packages for soldiers abroad. Other chapters cast doubt on well-trod stereotypes (Japan’s lack of pragmatism in its diplomatic relations with neutral nations and its irrational and fatalistic military leadership) and examine resistance to the war by a prominent Japanese Christian intellectual. The volume concludes with two nuanced responses to race in wartime Japan, one maintaining the importance of racial categories while recognizing the "performance of Japaneseness," the other observing that communities often reflected official government policies through nationality rather than race. Contrasting findings like these underscore the need to ask new questions and fill old gaps in our understanding of a historical event that, after more than seventy years, remains as provocative and divisive as ever. Defamiliarizing Japan’s Asia-Pacific War will find a ready audience among World War II historians as well as specialists in war and society, social history, and the growing fields of material culture and civic history.

Es vergeht kaum ein Tag, an dem die Medien nicht uber die dynamische Wirtschaftsentwicklung in China berichten. Ein Engagement in China muss jedoch sorgfaltig gepruft werden, da schleppende Genehmigungsverfahren, Patent- und Markenrechtsverletzungen, Probleme mit lokalen Zulieferern oder interkulturelle Managementkonflikte an der Tagesordnung sind. In diesem Buch werden die Bedingungen fur auslandische Unternehmen in China anhand zahlreicher Praxisbeispiele dargestellt und konkrete Entscheidungshilfen fur die Planung und Durchfuhrung eines betrieblichen Engagements gegeben. Im Mittelpunkt stehen unterschiedliche Strategien des Markteintritts und der Marktbearbeitung, die Gestaltung von Organisation, Personalmanagement und Controlling sowie die Beziehungen zur staatlichen Administration. Das Buch, das auf der langjahrigen Beratung und Untersuchung deutscher Firmen basiert, richtet sich sowohl an Unternehmen, die gegenwartig ein Engagement in China prufen, als auch an solche, die bereits vor Ort prasent sind. Dirk Holtbugge ist Professor fur Internationales Management an der Universitat Erlangen-Nurnberg, Jonas Puck ist dort wissenschaftlicher Mitarbeiter. Beide haben langjahrige Erfahrungen in der Beratung und Evaluierung von deutschen Unternehmen, die in China investieren.

Making basic numbers fun to learn, this board book has the young reader count from 1 to 10 using some of New York's most famous symbols. The Statue of Liberty, the Brooklyn Bridge, the Empire State Building, apples, subway cars, yellow taxis, and more are all depicted here using beautiful illustrations, vivid colors, and detailed design. The book also includes a complete bilingual location list in both English and Spanish.

The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders - disorders featuring an increased susceptibility to infections and, in, certain conditions, an increased rate of malignancies and autoimmune disorders. Since the publication of the first edition, a flurry of new disease entities has been defined and new treatment regimens have been introduced, the most spectacular being successful treatment by gene therapy for two genotypes of combined immunodeficiency. The first edition marked a historic turning point in the field of immunodeficiencies, demonstrating that many of the disorders of the immune system could be understood at a molecular level. This new edition can proudly document the tremendous pace of progress in dissecting the complex immunologic networks responsible for protecting individuals from these disorders.

I. Kapitel Einleitung und Problemstellung . . . . . . . 11 1. Simulation und Unternehmungsspiel 11 2. Ziele der Untersuchung . . . . . . 15 11. Kapitel Zur Konstruktion von Absatzmarktsimulatoren . . . . . 18 1. Begriff und Arten von Absatzmarktsimulatoren 18 1. 1. Eigangsgrossen. . . . . . . . . . . . . . . 19 1. 2. Ausgangsgrossen . . . . . . . . . . . . . . 20 2. Absatzmarktsimulatoren in Unternehmungsspielen 20 2. 1. Der Ansatz von Selten . . . . . . . . . . 21 2. 2. Der Absatzmarktsimulator von Vance . . . . . . 22 2. 3. Die Simulation des Absatzmarktes bei Shubik 24 2. 4. Absatzmarktsimulatoren und die Nachfrage im Oligopol 27 3. Anforderungen an die Eigenschaften von Absatzmar- simulatoren in Unternehmungsspielen . . . . . . . . . 30 4. Vorschlage zur Verbesserung von Absatzmarktsimulatoren 32 4. 1. Systeme linearer Nachfragefunktionen . . . . . . . . . 35 4. 2. Ein Redistributionsalgorithmus fur Absatzmarktsimu- toren . . . . . . . . . . . . 37 4. 2. 1 Zwei Spezialfalle: Angebotsmonopol und -duopol 38 4. 2. 1. 1 Das Angebotsmonopol 38 4. 2. 1. 2 Das Angebotsduopol . . . . . 40 4. 2. 2 Defmition des Verfahrens . . . 52 4. 2. 3 . Einige wichtige Eigenschaften des Redistributionsverf- rens . . . . . . . . . . . . . 56 4. 3. Erweiterung des Verfahrens . . . . . 60 4. 3. 1 Mehrproduktuntemehmungen . . . . 60 4. 3. 2 Behandlung der Falle x1(t) = 0 . . . 61 Weitere nachfrage wirksame Parameter 4. 3. 3 62 4. 3. 4 Ansatze zur Dynamisierung des Absatzmarktsimulators 64 4. 3. 4. 1 Befriedigung des Nachfrageuberhangs . . . . . . . . 64 4. 3. 4. 2 Dynamisierung von Aktionsparameterwirkungen 66 4. 4. Zusammenfassung der Rechenschritte zu einem Flussd- gramm . . . . . . . . . . . . . . . . . . . . . . . . 69 5 III. Kapitel 73 Ein spezielles Unternehmungsspielmodell 73 1. Defmition des Algorithmus 73 1. 1. Das Modell der Unternehmungen Der Absatzmarktsimulator 75 1. 2. Die Verknupfung der Unternehmungsmodelle mit dem Ab 1. 3."

The brutality and racial hatred exhibited by Japan's military during the Pacific War piqued outrage in the West and fanned resentments throughout Asia. Public understanding of Japan's wartime atrocities, however, often fails to differentiate the racial agendas of its military and government elites from the racial values held by the Japanese people. While not denying brutalities committed by the Japanese military, Honored and Dishonored Guests overturns these standard narratives and demonstrates rather that Japan's racial attitudes during wartime are more accurately discerned in the treatment of Western civilians living in Japan than the experiences of enemy POWs. The book chronicles Western communities in wartime Japan, using this body of experiences to reconsider allegations of Japanese racism and racial hatred. Its bold thesis is borne out by a broad mosaic of stories from dozens of foreign families and individuals who variously endured police harassment, suspicion, relocation, starvation, denaturalization, internment, and torture, as well as extraordinary acts of charity. The book's account of stranded Westerners-from Tokyo, Yokohama, and Kobe to the mountain resorts of Karuizawa and Hakone-yields a unique interpretation of race relations and wartime life in Japan.

Marcel Broodthaers filled his twelve-year artistic career with more ideas and works than most artists manage in a lifetime. This career began in 1964, following a period of more than two decades labouring in some obscurity as a poet in the Belgian Surrealist circle of Rene Magritte and Paul Nouge. He also wrote articles on art during these years, including early critiques of Pop art. Traversing media freely - from installation and sculpture to artist's books, prints, film and writings - Broodthaers embodied the `post- media artist' for whom any form could be recruited in the service of a larger conception. Those conceptions included institutional critique (of which he is a pioneer), art-historical critique, pastiche and philosophical-linguistic puzzles. Edited by Broodthaers' daughter Marie-Puck, and with a range of both classic and never-before-seen works, a biography, exhibition chronology and a selected bibliography, this volume is the largest and most authoritative Broodthaers monograph ever published.

Angeregt durch die in Firmendruckschriften und wissenschaftlichen Arbeiten enthaltenen Hinweise auf die gunstigen Festigkeits-Gewichts-Verhaltnisse glas faserverstarkter Kunststoffe, haben sich schon haufig Leichtbaukonstrukteure die Frage vorgelegt, ob nicht viele der bisher aus Metall oder Holz gefertigten Bauteile vorteilhaft aus Glasfaserkunststoff hergestellt werden konnten, zumal die technologischen Eigenarten der Glasfaserkunststoffe Ersparnisse an Betriebs mitteln und Lohnkosten in Aussicht stellen. Ein Grund dafur, dass trotzdem die Anwendung von Glasfaserkunststoff als tragender Werkstoff in hochbeanspruchten Konstruktionsteilen, beispielsweise im Fahrzeug- und Flugzeugbau, bisher auf Einzelfalle beschrankt blieb, liegt anscheinend darin, dass dem Konstrukteur keine garantierten Festigkeitswerte oder zulassigen Spannungen angegeben werden konnen, wie er es bei der Ver wendung von Halbzeugen aus metallischem Werkstoff gewohnt ist. Die Festig keit von Holzern kann zwar auch erheblichen Schwankungen unterliegen, jedoch konnen durch eine Eingangskontrolle im Festigkeitslabor des Betriebes die Eigenschaften einer Holzlieferung eingegrenzt werden und dem Konstrukteur somit zumindest ungefahre Festigkeitswerte des zur Verarbeitung gelangenden Werkstoffes an die Hand gegeben werden. Im Gegensatz dazu entsteht die Festigkeit des Glasfaserkunststoffes erst bei der Fertigung des Bauteiles. Die Festigkeit des Glasfaser-Kunstharz-Verbundstoffes wird beeinflusst durch die Art des Fasermaterials und des Kunstharzbindemittels, die anteilmassigen Mengen beider Stoffe, die Orientierung der Fasern, die Variablen des Hartungsprozesses und noch manche andere Verarbeitungsbedingungen. Eine Kontrolle der mecha nischen Eigenschaften ist demzufolge nur noch an Probeabschnitten moglich, die dem fertigen Bauteil entnommen werden, oder an solchen Proben, die unter sorgfaltiger Beachtung aller Einflussgrossen unter gleichen Bedingungen wie das Bauteil selbst hergestellt werden."