"Borders and Neighbourhood in Eastern and Central Europe. A Study of the Historic Development after World War II with the Case Study of the Oder-Neisse Region"

[From the Introduction]. Since the beginning of the European Union a number of scientists and politicians discuss about what the EU in the practice really means. Is it an international regime or either a federal state? In the opinion of some of them the EU is a network of states involving the pooling of sovereignty. The European Union was founded on a series of intergovernmental bargains, bargains which have more recently included the Single European Act (1986), the Maastricht Treaty (1991) and the Amsterdam Treaty (1997). More than any other kind of international organisation, the political process of the EU can be described by the term supranationality

The idea of freedom in Poland

'Polen musste im Laufe der Geschichte immer wieder um seine Freiheit kämpfen: zuerst nach den Teilungen und der nationalen Wiederbegründung 1918, dann während des Zweiten Weltkrieges und schließlich während der kommunistischen Herrschaft. Bei der Wiedererlangung seiner Unabhängigkeit 1918 wie 1989 standen die Vereinigten Staaten von Amerika im Verständnis Polens Pate. Das führte zur Entstehung eines Mythos und zur Idealisierung der USA in Polen. Der Beitrag untersucht das Freiheitsverständnis in der polnischen Bevölkerung, vergleicht die amerikanischen und polnischen Werte miteinander und behandelt abschließend die Wahrnehmung der USA in Polen.' (Autorenreferat

'Solidarnosc' - nur ein polnischer Traum von der Freiheit?

Die Verfasser gehen in ihrem Beitrag der Frage nach, welche Bedeutung diese erste, System verändernde Oppositionsbewegung heute für Polen und Europa besitzt. Der Beitrag beginnt mit einer historischen Rekapitulation der kontinuierlichen polnischen Oppositionsgeschichte seit 1956. Stärker noch als in Ungarn, gelang es den Regimes in Ost-Berlin und Prag, ihre Bevölkerung gegen die polnische Bewegung einzunehmen und negative Stereotype im Blick auf die polnische Bevölkerung zu festigen. Trotz aller Pressionen verfolgte ein harter Kern der Solidarnosc-Bewegung seine Ziele und einigte sich im Frühjahr 1989 mit der Regierung auf die Bildung eines 'Runden Tisches'. Mit dieser Strategie sollte Solidarnosc wegweisend für die anderen Oppositionsbewegungen im erodierenden Ostblock werden. In Ost wie West ordnet man Solidarnosc heute den entschiedenen Oppositions- bzw. Freiheitsbewegungen Europas zu. Sie beförderte das Streben nach Demokratisierung und veränderte so das politische, ökonomische und kulturelle Leben in Polen grundlegend. In Polen gibt es die Neigung, Solidarnosc allein als nationale Bewegung zu vereinnahmen und den sie fördernden europäischen Kontext - vor allem den KSZE-Prozess und die wirtschaftliche Hilfe aus dem Westen - eher gering einzuschätzen. Andererseits trifft es zu, dass die Konzepte der sozialliberalen wie der CDU/ FDP-Regierung, je auf ihre Weise, das bestehende System gegen Solidarnosc stabilisierten. Auch heute noch beurteilt die polnische Bevölkerung Solidarnosc überwiegend positiv und hält sie für eine wichtige europäische Bewegung. Allerdings fällt auf, dass die meisten mit Solidarnosc ein nationales Ereignis verbinden, das für ihr persönliches Leben nur eine geringe Bedeutung gespielt habe. Da die Menschen in den anderen Ostblockstaaten ebenfalls die Neigung zeigten, 'ihren' Oppositionsbewegungen eine nationale Färbung zu geben, konnte Solidarnosc bisher nicht zur Grundlage einer europäischen 'Meistererzählung' werden.' (Textauszug)''Solidarnosc' has become the primary symbol of hope as well as that of the love of freedom in Poland. Historical myths of national conservative provenance are increasingly overpowering the experiences of 1980/1981 and the political and cultural impulses that were unleashed in Poland at that time. The following questions will be addressed in the article: To what revolutionary history does Solidarnosc have a connection? What is the importance of this revolutionary movement not only for Poland but also for the Soviet Union's other satellite states? What significance did the Helsinki Accords and West Germany's 'Ostpolitik' possess in Poland's democratization?' (author's abstract)

Transapical aortic valve implantation – a rescue procedure for patients with aortic stenosis and “porcelain aorta”

Surgical aortic valve replacement (AVR) still remains the treatment of choice in symptomatic significant aortic stenosis (AS). Due to technical problems, extensive calcification of the ascending aorta (“porcelain aorta”) is an additional risk factor for surgery and transapical aortic valve implantation (TAAVI) is likely to be the only rescue procedure for this group of patients. We describe the case of an 81-year-old woman with severe AS and “porcelain aorta”, in whom the only available life-saving intervention was TAAVI

Whole-exome sequencing in patients with protein aggregate myopathies reveals causative mutations associated with novel atypical phenotypes

BACKGROUND: Myofibrillar myopathies (MFM) are a subgroup of protein aggregate myopathies (PAM) characterized by a common histological picture of myofibrillar dissolution, Z-disk disintegration, and accumulation of degradation products into inclusions. Mutations in genes encoding components of the Z-disk or Z-disk-associated proteins occur in some patients whereas in most of the cases, the causative gene defect is still unknown. We aimed to search for pathogenic mutations in genes not previously associated with MFM phenotype.METHODS: We performed whole-exome sequencing in four patients from three unrelated families who were diagnosed with PAM without aberrations in causative genes for MFM.RESULTS: In the first patient and her affected daughter, we identified a heterozygous p.(Arg89Cys) missense mutation in LMNA gene which has not been linked with PAM pathology before. In the second patient, a heterozygous p.(Asn4807Phe) mutation in RYR1 not previously described in PAM represents a novel, candidate gene with a possible causative role in the disease. Finally, in the third patient and his symptomatic daughter, we found a previously reported heterozygous p.(Cys30071Arg) mutation in TTN gene that was clinically associated with cardiac involvement.CONCLUSIONS: Our study identifies a new genetic background in PAM pathology and expands the clinical phenotype of known pathogenic mutations

Results of Polish Adult Leukemia Study Group (PALG) project assessing TP53 mutations with next-generation sequencing technology in relapsed and refractory chronic lymphocytic leukemia patients — an 18-month update

Indtroduction and methods: In chronic lymphocytic leukemia (CLL), molecular and cytogenetic diagnostics are crucial for the determination of accurate prognosis and treatment choice. Among different genetic aberrations, del(17p13) or TP53 mutations constitute high-risk factors, and early identification of such defects is a high priority for CLL patients. While cytogenetic diagnostics is well-established and accessible for the majority of CLL patients in Poland, molecular diagnostics of TP53 mutations is performed only in a few ERIC-certified centers (eight as of September 2020), and only two of these employ next-generation sequencing (NGS) for routine analysis of TP53 status in CLL patients. Here we report the interim results of a project assessing TP53 mutations with NGS technology in relapsed or refractory CLL patients with confirmed negative del(17p13) status. 249 patients from 32 clinical centers were included in the study. Results: NGS analysis revealed TP53 mutations in 42/249 (17%) patients, half of whom (21/249, 8.5%) had subclonal mutations (VAF ≤10%). These results are in line with published data in relapsed/refractory CLL patients. Conclusions: The results of the project demonstrated the feasibility and accuracy of NGS testing in CLL patients despite several initial logistical and technical obstacles. Our study also proved that, with appropriate funding, CLL patients from any hematological center in Poland can have access to state-of-the-art molecular diagnostic