Hereditary angioedema (HAE) is a rare familial disease characterized by recurrent self-limiting episodes of soft tissue swelling affecting different parts of the body. Acute HAE attacks range from benign, but disfiguring skin edema, to painful abdominal, and even life-threatening laryngeal attacks. The disease is caused by an aberrant C1 esterase inhibitor (C1-INH), which regulates complement, fibrinolytic, and contact pathways. Elevated serum level of bradykinin as a result of contact pathway activation is thought to be the major mediator of pain and edema formation in HAE. Current therapy of acute HAE attacks is limited and mainly offers symptom control. In the United States only fresh frozen plasma provides some reconstitution of C1-INH, but the efficacy and safety of this treatment is controversial. In some European countries two human derived C1-INH concentrates have been used successfully. Prophylactic therapy for patients with frequent HAE attacks is confined to attenuated androgens and in some countries anti-fibrinolytics and C1-INH are also used. To satisfy the unmet needs, investigation of one recombinant C1-INH, two drugs working on bradykinin pathway and two human derived C1-INH concentrates are underway. This review article also discusses some recent patents related to the filed.
The article discusses the role of classical and interdisciplinary methods in the study of the Soviet regions of the late Socialist era. The chronological scope of the study is limited to 1950-1980. The study uses the concept of "late socialism" in Alexei Yurchak's interpretation. The study is based on the analysis of the ideas of Viktor Mokhov on Regional Elites; of Oleg Khlevniuk and Yoram Gorlizki, on the Soviet governing regional networks; of Lorina Repina, Irina Savel'yeva, Andrey Poletayev, on historical memory. Through the vector of "region-centre"communication, the methods of research of the Soviet regional elites of the Upper Volga regions (Vladimir, Ivanovo, Kostroma, Tver, Yaroslavl regions) are considered. The study is based on interdisciplinary theoretical concepts – Kurt Lewin's theory of group dynamics and social fi eld; Morton Deutsch's and Harold B. Gerard's information model of conformity; Randall Collins' theory of reference authority; Veronika Nurkova's ideas on autobiographical memory; "categorisation" and "schematisation"concepts highlighted by Jerome Seymour Bruner.
The article raises the question of opportunities, prospects and directions in studying the regional aspect of the problem of transformation of Russian society in the 1950-1990s and the evolution of the economic system in the late USSR. The theoretical and methodological basis of the study is connected with the ideas of Mikhail Beznin and Tat’yana Dimoni on the formation of state capitalism in the late USSR; Alexei Yurchak’s, on the last Soviet generation. It is argued that the Upper Volga region (Vladimir, Ivanovo, Kostroma, Kalinin, Yaroslavl) of the late USSR as an object of historical research should be analysed simultaneously from two angles. On the one hand, it is indicated that these regions are suffi ciently typical and representative of the study of the late USSR in the political and socio-economic spheres. On the other hand, the uniqueness of the Upper Volga cluster of regions in terms of socio-cultural and mental characteristics, as well as geographical location, is emphasised. Particular attention is paid to the spatial localisation of the object of study. The role of the Upper Volga regions as large industrial and scientifi c-technical centres of Union-republican signifi cance, their importance in the militaryindustrial complex and the defence system of the USSR are examined. The formation of the infl uential layer of the political, economic and scientifi c-technical elite in the studied regions is noted. The unique characteristics of the status of the Upper Volga regions as centres of Russian statehood and national, cultural identity (the Golden Ring of Russia tourist route), their geographical proximity to Moscow are analysed.
The article examines the factors and sources of inequality and legal delimitation of the industrial societas in the USSR in the 1950-1980s. The article raises the question of the key aspects of regional and sectoral inequality of the Soviet societas. The theoretical and methodological basis of the study is associated with the paradigm of the "global intellectual history of inequality". Much attention is paid to the analysis of the concepts of "estate" and "class" in modern historiography. The article is based on the ideas of Mikhail Beznin and Tat’yana Dimoni on the legal demarcation of the production societas in the USSR and the formation of special social classes in Soviet Russia in the 1950s-1980s. An important theoretical role is played by the controversial thesis of the researcher Simon Kordonskiy on the existence of special estates – social registration groups – in the USSR. The source base of the study is represented by the official normative documents of the Soviet era, statistical data, unpublished archival documents of the Russian State Archive of Social and Political History. The article expresses a scientific hypothesis that the main criteria for inequality and legal delimitation of the production societas of the USSR included 3 indicators in the second half of the 20th century – a formally determined size of wages, social security, horizontal social mobility.
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