Regional specialised observatories networks in technological development and innovation exemplified by the Silesia Voivodship

The Regional Specialised Observatories Network is a systemic tool to encourage interdisciplinary cooperation between the key participants of the regional innovation system in order to build the competitive advantage of the region. The network responds to the region’s requirements by creating a modern tool to monitor the effects of the pro-technological development of the region in particular areas of technology, established in the Technological Development Strategy (TDS) for the Silesian Voivodship for the years 2010-2020, which is a constituent of the Regional Innovation Strategy. The observatory network will concentrate on collecting and processing specialised knowledge in the areas of technology in accord with TDS, monitoring technological and economic trends and assessment of the endogenous technological potential of the Silesian Voivodship. The network’s operation, through the link to the regional observatory as well as to national initiatives, will stimulate many forms of cooperation and contribute to the bonding of economic circles, innovators, science and research centres, the regional government and authorities responsible for drawing up and implementing development policy. The Regional Specialised Observatories Network is an open structure geared towards collecting, processing and publicising specialised knowledge, being a trustworthy source of data and information on technological areas in the region. The article presents the Network’s impact on identifying challenges and technological trends in reference to the region’s potential.Preparation and printing funded by the National Agency for Research and Development under project “Kreator Innowacyjności – wparcie dla Przedsiębiorczości akademickiej

Life-threatening conditions in newborn - neonatal thyrotoxicosis. Case presentation

Tyreotoksykoza noworodków jest rzadkim, zagrażającym życiu (śmiertelność 12-25%) i następstwami odległymi schorzeniem. W pracy przedstawiono przypadek noworodka przyjętego na oddział intensywnej terapii w 16. dobie życia w przełomie tyreotoksycznym, u którego rozpoznanie tyreotoksykozy było utrudnione, ponieważ nie prezentował on objawów typowych dla hipertyreozy, czyli wola i objawów ocznych. Na pierwszy plan wysuwały się objawy sugerujące zakażenie wewnątrzmaciczne w postaci zielonych, mętnych wód płodowych, wcześniactwa, cech dystrofii wewnątrzmacicznej, braku przyrostu masy ciała, powiększenia wątroby i śledziony. Na zakażenie wewnątrzmaciczne wskazywały też wyniki badań pomocniczych, takie jak: wysokie stężenie białka C-reaktywnego (CRP [C-reactive protein] 51,4 mg/l, n: 0–5), leukopenia (L 2,6 × 109/l, n: 6,0-21,0) trombocytopenia (płytki krwi 57,0 × 109/l, n: 170,0-500,0), nieznaczny wzrost stężenia bilirubiny i enzymów wątrobowych (AspAt, AlAt) oraz hipomagnezemia. Kolejnym utrudnieniem rozpoznania tyreotoksykozy u noworodka było niezdiagnozowanie u matki choroby Gravesa i Basedowa, której początek datował się na trzeci trymestr ciąży.Neonatal thyrotoxicosis is a rare, life-threatening condition with mortality rates of 12–25% and late sequelae. The authors present a male newborn admitted to Intensive Care Unit with tyreotoxic crisis when 16 days old, in whom diagnosing tyreotoxicosis was hindered, since the boy did not present with typical signs, i.e. goiter and exophthalmus. His major symptoms suggested intrauterine infection (green, opaque amniotic fluid, prematurity, intrauterine dystrophy, failure to thrive, hepatomegaly and splenomegaly). Intrauterine infection was also indicated by lab results, such as high CRP values (51.4 mg/l, normal range: 0-5), leukopenia (L 2.6 × 109/l, normal range: 6.0-21.0), thrombocytopenia (platelets 57.0 × 109/l, normal range: 170.0-500.0), as well as mildly increased bilirubin and hepatic enzymes (AspAt, AlAt) and hypomagnesemia. Another hindrance of diagnosing tyreotoxicosis was failure to diagnose and treat maternal Graves’ disease with the onset in the third trimester of pregnancy

Novel mutation-deletion in the PHOX2B gene of the patient diagnosed with Neuroblastoma, Hirschsprung's Disease, and Congenital Central Hypoventilation Syndrome (NB-HSCR-CCHS) Cluster

INTRODUCTION: Neuroblastoma (NB), Hirschsprung disease (HSCR), Congenital Central Hypoventilation Syndrome (CCHS), clinically referred as the NB-HSCR-CCHS cluster, are genetic disorders linked to mutations in the PHOX2B gene on chromosome 4p12. SPECIFIC AIM: The specific aim of this project is to define the PHOX2B gene mutations as the genomic basis for the clinical manifestations of the NB-HSCR-CCHS cluster. PATIENT: A one day old male patient presented to the Jagiellonian University Medical College (JUMC), American Children Hospital, neonatal Intensive Care Unit (ICU) due to abdominal distention, vomiting, and severe apneic episodes. With the preliminary diagnosis of the NB-HSCR-CCHS, the blood and tissue samples were acquired from the child, as well as from the child’s parents. All procedures were pursued in accordance with the Declaration of Helsinki, with the patient’s Guardian Informed Consent and the approval from the Institutional Review Board. GENETIC/GENOMIC METHODS: Karyotyping was analyzed based upon Giemsa banding. The patient’s genomic DNA was extracted from peripheral blood and amplified by polymerase chain reaction. Direct microfluidic Sanger sequencing was performed on the genomic DNA amplicons. These procedures were pursued in addition to the routine clinical examinations and tests. RESULTS: G-banding showed the normal 46 XY karyotype. However, genomic sequencing revealed a novel, heterozygous deletion (8 nucleotides: c.699–706, del8) in exon 3 of the PHOX2B gene on chromosome 4. This led to the frame-shift mutation and malfunctioning gene expression product. CONCLUSION: Herein, we report a novel PHOX2B gene mutation in the patient diagnosed with the NB-HSCR-CCHS cluster. The resulting gene expression product may be a contributor to the clinical manifestations of these genetic disorders. It adds to the library of the mutations linked to this syndrome. Consequently, we suggest that screening for the PHOX2B mutations becomes an integral part of genetic counseling, genomic sequencing of fetal circulating nucleic acids and / or genomes of circulating fetal cells prenatally, while preparing supportive therapy upon delivery, as well as on neonates' genomes of intubated infants, when breathing difficulties occur upon extubation. Further, we hypothesize that PHOX2B may be considered as a potential target for gene therapy

Perception of the Łódź Industrial Architecture Route by its Inhabitants: an Example of Social Participation in Tourism Research

Łódź as a post-industrial city has great potential for post-industrial tourism. An attempt to utilise this has been the creation of the Łódź Industrial Architecture Trail, bringing together buildings related to its industrial past. According to the author, to make the trail a tourist attraction, the first people who should be aware of its value are the city’s inhabitants. The survey confirmed the very important role of social participation in creating the image of a city, and providing the basis for further work on its improvement and promotion. The article does not cover social participation as part of the process of development, but can serve as a contribution to a discussion of the role of a city’s inhabitants in shaping its tourism attractions. At the same time, the article confirms that social participation is an extremely important element of tourism research and forms an introduction to its effective use in practice

Inhibition of carotenoid biosynthesis by CRISPR/Cas9triggers cell wall remodelling in carrot

Recent data indicate that modifications to carotenoid biosynthesis pathway in plants alter the expression of genes affecting chemical composition of the cell wall. Phytoene synthase (PSY) is a rate limiting factor of carotenoid biosynthesis and it may exhibit species-specific and organ-specific roles determined by the presence of psy paralogous genes, the importance of which often remains unrevealed. Thus, the aim of this work was to elaborate the roles of two psy paralogs in a model system and to reveal biochemical changes in the cell wall of psy knockout mutants. For this purpose, Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR) and CRISPR associated (Cas9) proteins (CRISPR/Cas9) vectors were introduced to carotenoid-rich carrot (Daucus carota) callus cells in order to induce mutations in the psy1 and psy2 genes. Gene sequencing, expression analysis, and carotenoid content analysis revealed that the psy2 gene is critical for carotenoid biosynthesis in this model and its knockout blocks carotenogenesis. The psy2 knockout also decreased the expression of the psy1 paralog. Immunohistochemical staining of the psy2 mutant cells showed altered composition of arabinogalactan proteins, pectins, and extensins in the mutant cell walls. In particular, low-methylesterified pectins were abundantly present in the cell walls of carotenoidrich callus in contrast to the carotenoid-free psy2 mutant. Transmission electron microscopy revealed altered plastid transition to amyloplasts instead of chromoplasts. The results demonstrate for the first time that the inhibited biosynthesis of carotenoids triggers the cell wall remodelling

Hooligans threat to the organizations of the sports events’ arrangement.

Przedmiotem pracy jest problematyka zachowań chuliganów stadionowych i ich wpływ na zapewnienie bezpieczeństwa podczas organizacji widowisk sportowych. W pracy dokonano przeglądu środków mających na celu zapobieganie ekscesom chuligańskim oraz podjęto próbę oceny ich skuteczności. Ponadto analizie zostały poddane statystyki Komendy Głównej Policji dotyczące bezpieczeństwa na imprezach sportowych, dzięki którym można zaobserwować wpływ wprowadzanych środków na zmianę bezpieczeństwa podczas widowisk sportowych, na przestrzeni ostatnich lat.Celem niniejszego opracowania było przedstawienie zagrożeń związanych z chuligaństwem stadionowym i jego wpływ na zmiany regulacji prawnych. W pracy zastosowano metodę sondażu – badanie ankietowe.The subject of this thesis are problematic issues connected with the behaviour of hooligans and its influence on the safety procedures during the sports events' arrangement. This thesis is an overview of the means which are to help preventing hooligans' riots and a try of evaluating the efficiency of those means. Police Headquaters statistics concerning the safety during sports events were analysed. The analysis shows the influence of the newly introduced means on the change in safety during sports events over the years. The aim of this thesis was to present the threats connected with hoolyganism and its influence on the changes in law. The analysis was conducted using the method of survey

The family and its concept in the mind of young people being in conflict with the law. The image and the perspective

Rodzina jest podstawową komórką społeczną i pierwszym środowiskiem życia człowieka. Funkcjonując w sposób prawidłowy wyposaża młodego człowieka we wszelakie niezbędne umiejętności i kompetencje gwarantujące właściwe przystosowanie się do życia w społeczeństwie. Rozwój dzieci jest poważnie zagrożony w domach dysfunkcjonalnych i patologicznych, które negatywnie oddziaływają na wszystkie sfery osobowości. Jakość ekosystemu rodzinnego wpływa również na poglądy i oczekiwania młodzieży odnośnie wizji własnego życia rodzinnego. Niniejsza praca ma charakter badawczy a jej celem jest ukazanie rodziny i jej koncepcji w umyśle młodzieży będącej w konflikcie z prawem. W części teoretycznej pracy poruszono problem ewolucji zmian zachodzących w rodzinie współczesnego człowieka oraz kształtowania społecznie pożądanych postaw wobec małżeństwa i rodziny. Część metodologiczna zawiera szczegółowy opis postępowania badawczego, natomiast rozdziały analityczne dotyczą kwestii prezentacji badanych, ich środowiska rodzinnego, stosunku do małżeństwa i rodziny oraz poglądów i oczekiwań związanych z życiem rodzinnym. W celu zebrania danych empirycznych posłużono się następującymi narzędziami badawczymi: kwestionariusze ankiety: Sytuacja Rodzinna i Szkolna Badanych, Rodzina Jaką Założę, Kwestionariusz Postaw Rodzicielskich M. Plopa oraz Kwestionariusz Wywiadu z Wychowawcą. Uzyskane dane pozwoliły wysnuć wnioski, iż wewnętrzna specyfika funkcjonowania rodzin pochodzenia respondentów wpływa na posiadany subiektywnie przez nich jej obraz. Postrzeganie rodziny jako środowiska nieprzyjaznego i funkcjonującego nieprawidłowo tworzy w umyśle badanych jej negatywną wizję i na odwrót. Dodatkowo zauważono, iż młodzież wychowująca się w patologicznych i dysfunkcjonalnych domach, wyraża chęć założenia w przyszłości własnej rodziny niebędącej powieleniem negatywnych wzorów.Summary The family is the basic unit of a society and the first environment for the human life. Functioning properly it equip young people in all kinds of skills and competencies necessary to guarantee the proper adaptation to life in a society. The development of children is seriously threatened in dysfunctional and pathological homes which impact negatively on all spheres of their personality. The quality of the family`s ecosystem also affects the views and expectations of young people about their vision of family`s everyday life. This work is of a study character and its aim is to show the family and its concept in the mind of young people being in conflict with the law. In the theoretical part of my work I raise the problem of evolution of the changes taking place in the contemporary man`s family and forming the socially desirable attitudes towards marriage and family. Methodological section contains the detailed description of the research procedure. The analytical chapters are concentrated on the presentation of the respondents, their family environment, attitudes towards marriage and family, and the views and expectations of the family life.In order to collect the empirical data I used the questionnaire surveys about: The family and school situation of the respondents, family which I'll assume, M. Parental Attitudes Questionnaire by M. Plop and questionnaire of interview with the tutor. The obtained data allowed to conclusions that the internal specifics of the functioning of families of origin of respondents affects their subjectively image of it. Perception of the family as a hostile environment inproperly creates in the mind of the respondents its negative vision and vice versa. In addition it was noted that young people brought up in the pathological and dysfunctional homes are willing assumption of assuming their own family in the future which will not be a duplication of their negative patterns

Coping humor for the relationship between a sense of stress at work and job satisfaction

Background Humor is considered as a personal resource that allows individuals to effectively deal with stressful situations at work by reducing tension and perceiving the situation as less threatening. By reducing stress, humor contributes to increased job satisfaction. Material and Methods A total of 201 professionally active participants (113 women and 88 men) aged 19–60 years old were qualified for the study – the average age in the study group was 37.76 years (SD = 12.04). The study was conducted for 1 month (at the turn of February and March 2017). The following methods were used in the research: Coping Humor Scale (CHS), Satisfaction with Job Scale (SSP), Sense of Stress at Work Scale (PSS-10-P) and own survey. Results Humor coping with stress partially mediates the relationship between a sense of stress at work and job satisfaction. It is associated with less tension at work and greater job satisfaction. Conclusions Coping humor with stress increases the individual adaptation capacity in the face of stress at work, by shifting the perspective, distancing from problems at work and changing stressors appraisal as less threatening. Med Pr 2018;69(6):621–63