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Axenfeld–Rieger syndrome (ARS) is a rare, autosomal dominant disorder with genetic and morphologic variability and characterized by ocular and nonocular clinical findings. Midface hypoplasia and maxillary hypodontia are classical presenting features of this syndrome. This case report describes a dental condition, immediate treatment required and a long-term treatment approach toward a patient 5-year-of-age with ARS, who presented with significant ocular and dental anomalies.

This paper reflects one of the largest European organ companies, operating from the late 19th century to the second decade of the 21st century. The company was founded under the name Gebrüder Rieger in 1873 in the Silesian town of Krnov (then the German name Jägerndorf). In 1904 it changed its name to Rieger, which was used until the end of the Second World War. After its termination, the German owners were expelled, the factory was then taken over under national administration and subsequently nationalized. At the same time, a smaller organ workshop by Josef Kloss was also included. The factory has since used the Rieger-Kloss brand on its instruments. After 1989, the plant was privatized, but ended its activity in 2015. The work follows not only the history of the company, but also the issues of artistic and structural specifics of the Krnov instruments. On the artistic level, these organs were, in the beginning, heavily influenced by the paradigm of the so-called romantic organ (or even the stimulus of the Cecilian reform), which in Central Europe experienced the greatest development at the time of the founding of the company, and culminated at the turn of the 19th and 20th centuries, and then by the ideas of the reformist organ movement that has affected the production of the factory since about...

The thesis Political Programmes of the Czech Representation in the Second Halve of the 19th Century focuses on both programme documents and actual work of Czech political parties, whether more or less institutionalized, between the years 1848 (March Revolution) and 1918 (the downfall of Austria-Hungary). At first it summarizes the historical development in the respective period (Chapter 1), next, it analyses programmes of political parties in three broadly defined topics: 1) Organisation of the empire, question of the Czech State Right (Chapter 2); 2) Constitutionalism, civil rights and role of a citizen (Chapter 3) and 3) National matters (Chapter 4). The attitudes about each of these areas of the following political parties are defined: Bohemian nobility, National Party (till 1874) or Old Czechs (since then), Young Czechs, Social Democrats, Agrarians, Catholic parties, National Socialists, Progress parties and parties of the Radical State Right, Realists and Anarchists. For the conclusion, the author of the thesis attempts to characterise and evaluating the Czech political representation, as well as its importance for the development since 1918.

Axenfeld–Rieger syndrome (ARS) is a rare autosomal dominant inherited disorder affecting the development of the eyes, teeth and abdomen. The syndrome is characterised by complete penetrance but variable expressivity. The ocular component of the ARS phenotype has acquired most clinical attention and has been dissected into a spectrum of developmental eye disorders, of which open-angle glaucoma represents the main challenge in terms of treatment. Mutations in several chromosomal loci have been implicated in ARS, including PITX2, FOXC1 and PAX6. Full-spectrum ARS is caused primarily by mutations in the PITX2 gene. The homeobox transcription factor PITX2 is produced as at least four different transcriptional and splicing isoforms, with different biological properties. Intriguingly, PITX2 is also involved in left–right polarity determination, although asymmetry defects are not a feature of ARS. In experimental animal models and in cell culture experiments using PITX2, abundant evidence indicates that a narrow window of expression level of this gene is vital for its correct function.

The supposed dissertation entertaines about the publisher and book-seller Ignác Leopold Kober and the development of his enterprise during the period of the years 1846-1866 in the connection of the czech society of the second half of 19th century. The basic accent is focused on the I. L. Kober´s life and his influence over the contemporary czech culture and intelligence,it deals in the characteristic of his released production and significant attention is attended to the understanding to the story of the Kober´s greatest masterpiece The Slovník naučný and its importance for the czech intelligence.The story of Ignác Leopold Kober,the first czech modern publisher, hasn´t been done yet and almost forgotten and this fact shold be reformed by this dissertation. The target of this is to introduced mr. Kober´s personality as a man,who was able to consummate the piece of the old period to lay the foundation of the new age.

Riegerovo djelo Panorama della Costa e delle Isole di Dalmazia nei viaggi dei Piroscafi del Lloyd Austriaco jedinstveno je u kulturnoj povijesti istočnog Jadrana. Ni prije, a ni poslije niti jedan umjetnik neće se uhvatiti slična posla: panoramski oslikati obalu od rta Kumpar na ulazu u pulsku luku do Budve. Premda Giuseppe Rieger jest umjetnik i, premda njegove panorame nedvojbeno jesu umjetničko djelo, nije sebi dopustio ustaljenu umjetničku slobodu. Sažimanja obalnih krajolika koja su nazočna na panoramama nipošto nisu dio te slobode. Naprotiv, ona su u funkciji korektnosti prikaza, a taj je ponajprije korisniku želio podariti viziju naseljenih mjesta tog dijela istočnog Jadrana. Ako ta sažimanja ponegdje i jesu pretjerana, to je učinjeno da bi se naglasilo važnost prikazanih objekata. Toponimija koju je bilježio u stanovitom je dijelu izvorna. Poneki zemljopisni naziv teško je pronaći i na onodobnim plovidbenim kartama. To drugim riječima znači da je Rieger plovidbenu rutu vjerojatno preplovio više puta, ostajao na pojedinim mjestima duže nego što su to činili Lloydovi parobrodi, te tako upoznao ovaj akvatorij više nego što su mu to mogla omogućiti zvaničina pomoračka pomagala: karte i planovi, panorame, peljari. Time je njegovo djelo dragocjenije, jer i kroz tu prizmu vrlo pouzdano svjedoči o povijesti istočnog Jadrana polovicom 19. stoljeća. Jedan od zornijih pokazatelja toga jest notiranje toponima Tri sestre (Tre Sorelle) u Prčnju. Teško ga je naći na službenim kartama, ali svatko tko je bio u Boki kotorskoj zna za njega. Za toponim Tri sestre razinu legendarnog teško je razlučiti od povijesne istine, a to je ono što je Lloydovu klijentelu nedvojbeno moglo zanimati. Naravno, sve to znači da se Rieger okoristio svim postojećim službenim i privatnim izvorima te osobnom opservacijom u realizaciji svog obimnog posla. Panorame milanskog Vojnozemljopisnog instituta nedvojbeno su mu pritom bile od najveće koristi. Ali, svojim je djelom učinio mnogo više a ne samo korak dalje od njih. O takvim izvanrednim vrijednostima Riegerova djela ponajprije svjedoči čak pet izdanja njegovih dalmatinskih panorama tijekom 19. stoljeća. To je razlog da se pripremi i ovo. I to ne samo zato da se dočara duh nekadašnjeg pomalo bajkovitog vremena, već u prvom redu stoga što njihova umjetnička vrijednost nije umanjena ni danas. Snaga njihove likovnosti, naime, neprolazna je. Upravo iz tog razloga možda su jedan od najboljih svjedoka svih ljepota koje krase istočni Jadran, koje su u 19. stoljeću jednako kao i danas, bile onaj čimbenik koji je inicirao turiste, Europljane ponajprije, da dođu ovdje i podjele s nama, koji tu živimo, tu blagodat Mediterana.

The Rieger syndrome is an autosomal dominant disease characterized by ocular, craniofacial, and umbilical defects. Patients have mutations in PITX2, a paired-bicoid homeobox gene, also involved in left/right polarity determination. In this study we have identified a family of genes for enzymes responsible for hydroxylizing lysines in collagens as one group of likely cognate targets of PITX2 transcriptional regulation. The mouse procollagen lysyl hydroxylase (Plod)-2 gene was enriched for by chromatin precipitation using a PITX2/Pitx2-specific antibody. Plod-2, as well as the human PLOD-1 promoters, contains multiple bicoid (PITX2) binding elements. We show these elements to bind PITX2 specifically in vitro. The PLOD-1 promoter induces the expression of a luciferase reporter gene in the presence of PITX2 in cotransfection experiments. The Rieger syndrome causing PITX2 mutant T68P fails to induce PLOD-1–luciferase. Mutations and rearrangements in PLOD-1 are known to be prevalent in patients with Ehlers-Danlos syndrome, kyphoscoliosis type (type VI [EDVI]). Several of the same organ systems are involved in Rieger syndrome and EDVI.