Manifestations In People Research Articles

Effects and mechanisms of supramaximal high-intensity interval training on extrapulmonary manifestations in people with and without chronic obstructive pulmonary disease (COPD-HIIT): study protocol for a multi-centre, randomized controlled trial

BackgroundBeyond being a pulmonary disease, chronic obstructive pulmonary disease (COPD) presents with extrapulmonary manifestations including reduced cognitive, cardiovascular, and muscle function. While exercise training is the cornerstone in the non-pharmacological treatment of COPD, there is a need for new exercise training methods due to suboptimal adaptations when following traditional exercise guidelines, often applying moderate-intensity continuous training (MICT). In people with COPD, short-duration high-intensity interval training (HIIT) holds the potential to induce a more optimal stimulus for training adaptations while circumventing the ventilatory burden often associated with MICT in people with COPD. We aim to determine the effects of supramaximal HIIT and MICT on extrapulmonary manifestations in people with COPD compared to matched healthy controls.MethodsCOPD-HIIT is a prospective, multi-centre, randomized, controlled trial with blinded assessors and data analysts, employing a parallel-group designed trial. In phase 1, we will investigate the effects and mechanisms of a 12-week intervention of supramaximal HIIT compared to MICT in people with COPD (n = 92) and matched healthy controls (n = 70). Participants will perform watt-based cycling two to three times weekly. In phase 2, we will determine how exercise training and inflammation impact the trajectories of neurodegeneration, in people with COPD, over 24 months. In addition to the 92 participants with COPD performing HIIT or MICT, a usual care group (n = 46) is included in phase 2. In both phases, the primary outcomes are a change from baseline in cognitive function, cardiorespiratory fitness, and muscle power. Key secondary outcomes include change from baseline exercise tolerance, brain structure, and function measured by MRI, neuroinflammation measured by PET/CT, systemic inflammation, and intramuscular adaptations. Feasibility of the interventions will be comprehensively investigated.DiscussionThe COPD-HIIT trial will determine the effects of supramaximal HIIT compared to MICT in people with COPD and healthy controls. We will provide evidence for a novel exercise modality that might overcome the barriers associated with MICT in people with COPD. We will also shed light on the impact of exercise at different intensities to reduce neurodegeneration. The goal of the COPD-HIIT trial is to improve the treatment of extrapulmonary manifestations of the disease.Trial registrationClinicaltrials.gov: NCT06068322. Prospectively registered on 2023-09-28.

Peri-ictal psychiatric manifestations in people with epilepsy: An umbrella review.

We aimed to conduct an umbrella review to summarize the existing evidence regarding the prevalence of peri-ictal psychiatric manifestations (PM) in people with epilepsy (PWE) including pre-ictal, ictal, and postictal stages. Databases were searched up to June 2023 for systematic reviews (SR) of observational studies that included patients with epilepsy peri-ictal PM. Data selection, data extraction, and risk of bias assessment (with the AMSTAR-2 instrument) were performed by two independent reviewers. We performed a narrative synthesis using previous guidelines. We used a self-developed decision table according to the GRADE system adapted for narrative outcomes if the certainty of outcomes was not determined by systematic review authors. Four SRs were included comprising 66 primary studies (n = 10 217). Three SRs evaluated one period (pre-ictal, ictal, and postictal), and one did not determine it. During the pre-ictal period, the more prevalent symptom was confusion, although with a low certainty (due to the heterogeneity and serious risk of bias). One systematic review that only included case reports evaluated the ictal period, finding mood/anxiety disorders, psychosis, and personality changes. The postictal period included the most PM (anxiety: 45.0% and depressive symptoms: 43.0%), with very low certainty, due to risk of bias, potential publication bias, heterogeneity, and failure to report the confidence intervals. With very low certainty, epileptic periods are characterized by a wide spectrum of PM, being postictal symptoms the most prevalent, predominantly anxiety, and depressive symptoms. Further understanding of these PM of epilepsy could improve the attention of the people with epilepsy. In this review of reviews, we summarize the frequency in which psychiatric manifestations occur in relation to an epileptic seizure. A total of 10 217 patients were reported in the reviews. The most common manifestations included symptoms of anxiety and depression, as well as changes in the normal behavior of the patient. These manifestations occurred most frequently right after the seizure finished.

Clinicopathological concordance of mucocutaneous manifestations in people living with HIV: A cross-sectional study.

To describe the frequency and clinicopathological concordance of mucocutaneous manifestations in people living with HIV (PLWH) and its correlation with CD4+ T lymphocyte count and HIV viral load. Cross-sectional study of patients diagnosed with HIV infection who underwent skin biopsy for histopathological study from 1992 to 2022. Skin diseases were categorized as opportunistic and sexually transmitted infections, inflammatory dermatoses, benign cutaneous neoplasms, and premalignant and malignant cutaneous neoplasms. Clinicopathological concordance was classified as complete, partial or discordant. Frequency of skin diseases are presented by category and according to lymphocyte CD4+ count and HIV viral load. A total of 659 patients were included of whom 88.5% (n = 583) were male. The most frequent diagnostic category was opportunistic or sexually transmitted infections in 34% (n = 224) and the most frequently found condition was Kaposi sarcoma in 17% (n = 112). Clinicopathological concordance was complete in 53.7% (n = 354) of cases, partial in 26.7% (n = 176) and discordant in 19.6% (n = 129). Among the 282 patients with available serological data, 58.9% (n = 166), 23.8% (n = 67) and 17.4% (n = 49) had CD4+ counts below 200, between 200 and 499, and above 500 cells/μl, respectively. Although there is a high variability in skin conditions which people with HIV may present, there was a high rate of clinicopathological concordance (80.4%). We emphasize the importance of diagnostic skin biopsies due to their diverse morphological presentation. The frequency of skin diseases in PLWH depending on different clinical settings should aid the clinician in reaching an adequate diagnosis in this population.

Design of geometric flower pattern for clothing based on deep learning and interactive genetic algorithm

Abstract Geometric and floral models are an important part of clothing and have been used for thousands of years. Although the styles of geometric models and flower models have undergone changes over the centuries, they are still one of the important factors of clothing patterns, the important carrier of aesthetics, and the manifestation of people’s spiritual views and cultural needs. The development and application of digital printing technology have freed people from excessive dependence on sewing and embroidery processes. Therefore, while deeply studying the design of clothing patterns, this work sorted and analyzed the geometric flower models of clothing through interactive genetic algorithms, and optimized programming to enrich the models of clothing with geometric textures. The results showed that the deviation value of geometric flower pattern design was constantly decreasing, while the optimal strategy value was constantly increasing. The mean deviation value was 0.82, which was a decrease of 0.21 on the seventh day compared with the first day; the mean value of the optimal strategy value was 0.84, which was an increase of 0.19 on the seventh day compared with the first day. The visual effect and creativity of the clothing flower pattern design under the interactive genetic algorithm are better than the traditional flower pattern design, and the visual effect and creativity under the interactive genetic algorithm are 9% higher than the traditional one.

A280 HEPATOBILIARY DISEASE IN INDIVIDUALS LIVING WITH FRAGILE X SYNDROME: A CASE SERIES

Abstract Background Fragile X syndrome is a genetic disorder known to cause intellectual disability through decreased or absent FMR protein (FMRP). Basic science studies have identified potential associations with hepatobiliary inflammation. Clinical reports of liver disease in Fragile X syndrome are however infrequent. Aims We describe 3 individuals living with Fragile X syndrome and chronic liver disease. Methods A case series and literature review were performed. Results The 1st person is a woman with concurrent Fragile X and autosomal recessive polycystic kidney disease, initially seen by Hepatology at 18 y.o. with evidence of hepatic fibrosis on abdominal ultrasound with normal liver enzymes (Table 1). She subsequently developed liver cirrhosis and portal hypertension, as well as biliary duct dilatation seen on MRI. Her overall presentation was thought to be in keeping with Caroli syndrome and congenital hepatic fibrosis, and she is currently pending genetic screening for hepatorenal ciliopathy. The 2nd person is a woman with Fragile X syndrome and ulcerative colitis, who initially presented to Hepatology at 24 y.o. with mixed liver enzyme abnormalities. Lab tests over time revealed immunology suggestive of autoimmune hepatitis (AIH) (Table 1). Subsequent investigations demonstrated primary sclerosing cholangitis (PSC) with cirrhosis. Her disease is currently managed as AIH/PSC overlap syndrome with ursodeoxycholic acid (UDCA), tacrolimus, mycophenolate, and budesonide. The 3rd person is a man with Fragile X syndrome who initially presented to Hepatology at 17 y.o. with acute hepatitis with AIH immunology (Table 1). Liver biopsy at the time demonstrated findings suggestive of early PSC as well as possible AIH. He continued to have progressive cirrhosis as well as overt PSC on subsequent abdominal imaging and is currently managed as AIH/PSC overlap syndrome with UDCA, azathioprine, and budesonide. A review of literature revealed that asides from known intellectual, behavioral, and physical effects of Fragile X syndrome, there are minimal reports of other associated medical complications including liver problems or autoimmune conditions including IBD. There are however reports of autoimmunity in those with altered FMR1 (Fragile X Mental Retardation 1) gene mutations. Conclusions These 3 cases of liver disease in Fragile X highlight young age of presentation for chronic liver disease with both biliary and immune-mediated characteristics, along with progression to cirrhosis. Additional review for liver manifestations in people living with Fragile X would help understand if this association is incidental or causal. Investigations of individuals with Fragile X syndrome and liver disease Funding Agencies None

ANALYSIS OF MUSABAQAH TILAWATIL QUR'AN ACTIVITIES IN INCREASING MOTIVATION FOR MEMORIZING THE QUR'AN IN MADRASAH ALIYAH TAHFIZHIL QUR'AN MEDAN

In memorizing the Qur'an, motivation is needed, both motivation to add new memorization, motivation to maintain memorization, and discipline motivation to divide time so that the quality and quantity of memorization are maintained properly. However, in reality, many memorizers of the Qur'an still have low motivation, so they have difficulty memorizing the Qur'an. Many want to memorize the Al-Qur'an but are worried that they will not be able to memorize it in the future. Musabaqah Tilawatil Qur'an (MTQ) is an effort to provide motivation to the younger generation to read, memorize and understand the Qur'an. With the existence of Musabaqah Tilawatil Qur'an (MTQ) it becomes a forum to increase motivation to memorize the Al-Qur'an and as a means for memorizers of the Al-Qur'an to test the quality of their memorization. The rapid development of MTQ in Indonesia is a manifestation of people's love for the Qur'an. MHQ in this case is considered to be able to motivate memorizing the Al-Qur'an, so that it is hoped that the students at Madrasah Aliyah Tahfizhil Qur'an Medan have good memorization skills and motivation to memorize. From this research it can be concluded that it can be concluded that the students who memorize the Al-Qur'an at Madrasah Aliyah Tahfizhil Qur'an Medan have their own way of memorizing the Al-Qur'an. This can be seen from their ability to apply self-motivation, self-management and preparation for MTQ.

Periodization of General Elections: Ideas and Refinements in Indonesia

General election is one part of the practice of democracy in Indonesia. In this case, the general election is a manifestation of people's sovereignty. In general, the principles of elections include the principles of direct, public, free, confidential, honest and fair. This research seeks to explore legal constructions related to the periodic principle of general elections which must be held periodically every five years, which is a separate principle in general elections. This research is normative legal research with a statutory and conceptual approach. The results of the study confirm that to achieve and ensure that general elections are held a justice process and proportionally, the orientation of legal construction on the periodic principle has actually been explicitly stated in the 1945 Constitution of the Republic of Indonesia and is based on a systematic interpretation. In this case, in terms of periodic aspect orientation, it is part of the general election principle that is obligatory and must be implemented. This has the implication that the principles of general elections include the principles of direct, general, free, confidential, honest and fair, and must be held periodically for five years. In this case, the principle of periodicity occupies an important position in the holding of general elections.

Periodic Principles in General Elections: Orientation and Implications in Indonesia

The general election is one part of the practice of democracy in Indonesia. In this case, the general election is a manifestation of people’s sovereignty. In general, the principles of elections include the principles of direct, general, free, confidential, honest, and fair. This study seeks to explore the legal construction related to the periodic principle of general elections which must be held periodically every five years to become a separate principle in elections. The issue of periodization in general elections really needs to be emphasized as one of the principles in general elections as the main basis for creating a cycle of replacement and filling of public positions in the state administration system. This research is a normative legal research with statutory and conceptual approaches. The results of the study confirm that the orientation of legal construction on the periodic principle has actually been expressly stated in the 1945 Constitution of the Republic of Indonesia and is based on a systematic interpretation. In this case, the orientation of the periodic aspect is part of the general election principle which is obligatory and must be implemented. This implies that the principles of general elections include the principles of direct, general, free, confidential, honest, and fair, and must be held periodically for five years. In this case, the periodic principle becomes very important to be recognized and explicitly stated in the general election law as a new principle in the technical implementation of general elections. this has implications for the implementation of elections that are inconsistent and tend to change in each implementation. Whereas in holding general elections, the periodic principle will uniformize the holding of elections simultaneously, both national elections and elections at the regional level by setting forth and affirming in the general election law that periodization is a technical principle in holding general elections in Indonesia.

Ophthalmological Manifestations in People with HIV from Northeastern Romania.

Background and Objectives: Although ocular disorders can occasionally impact people with HIV over the course of their illness, HIV/AIDS is unmistakably a multisystem disorder. A physician can rule out a wide range of ophthalmic problems with the assistance of an ophthalmologist, from adnexal disorders to posterior segment diseases, including those affecting the optic tract and optic nerve. Materials and Methods: Based on patient medical data from the "St. Parascheva" Clinical Hospital of Infectious Diseases in Iasi, we carried out a retrospective clinical investigation on patients with HIV/AIDS and ophthalmological conditions who were hospitalized in northeastern Romania. We seek to draw attention to the characteristics and ophthalmological comorbidities of HIV/AIDS patients. The studied period was between 1 January 1991 and 31 December 2022. Results: There were a total of 38 recorded cases of ophthalmological manifestations in the HIV-infected patients. The research group's average age was 37.31 years old (standard deviation 9.5693917). Males were primarily impacted, having lower total CD4+ T-lymphocyte levels based on sex and CD4+ T-lymphocyte levels overall. The HIV viral load was 999 268.13 copies/mL on average (standard deviation 1,653,722.9). Of all the patients, we found out that 17 had congenital eye diseases (44.73%) and the others (21, 55.26%) developed ophthalmological diseases. CMV Retinitis was found most frequently, in eight patients (21.05%), followed by Myopia in seven patients (18.42%). Conclusions: The key to the management of HIV-positive patients is a multidisciplinary approach and access to antiretroviral therapy. Anyone who is HIV-positive and experiences ocular symptoms at any time should be directed to seek professional ophthalmologic treatment as soon as feasible. A therapeutic holdup could result in irreversible vision loss. Long-term coordination is required to combat this disease, improving communication between the ophthalmology and infectious disease fields.

PENGGUNAAN MEDIA SEBAGAI SUMBER INFORMASI PEMILU PILPRES DAN PILEG 2024 DI KALANGAN MAHASISWA PERGURUAN TINGGI KEAGAMAAN NEGERI DI PALANGKA RAYA

General election is a manifestation of democracy. Since Indonesia independence, general election has been carried out using various electoral systems. After the reform in 1998, the electoral system involved direct people's participation as a manifestation of people's sovereignty. General election in Indonesia is held every five years. Currently, the General Selection Commission is preparing everything regarding the general election in 2024, both the legislative and presidential elections. Information regarding general election is indeed important for the people, and the media plays an important role in providing various information regarding general election. In other words, the media acts as a means of political communication. This research aimed to describe the perspectives or opinions of students of three State Religious Institutions in Palangka Raya regarding general election information and the media used as the source of information. This research was a descriptive qualitative research, the data was collected by using in-depth interview and documentation. A total of 10 students became the key informants who were selected and determined based on purposive sampling. The results of the research showed that the majority of students thought that the media was an important source of information regarding general election, and in general, social media platforms were used as the main source of information besides the types of mainstream media or internet media. Based on the types of election information categories, the majority of informants stated that information regarding candidates and election processes or mechanisms was the information they wanted to know the most. The informants also stated that they had participated directly in general election, even though only as beginner voters because they believed that as good citizens they should have used their right to vote responsibly.

Possibilities of Forming Empathic Reactions in Children with ASD

The purpose of this work was to analyze the possibilities of formation and manifestation of empathy in people with ASD. According to the Diagnostic and Statistical Manual of Mental Disorders (DSM-V), the deficit of socio-emotional reciprocity, which includes a reduced exchange of interests, emotions and affection, is defined as one of the diagnostic criteria of ASD.The analysis of papers covering this topic has shown that a decrease in empathic manifestations in people with ASD may be associated with unexplored relationships between the affective stimuli and corresponding empathic reactions. To correct this mismatch, a training program aimed at the formation of appropriate vocal and motor reactions in response to affectively charged situations presented was developed. Diagnostics of the initial level of empathic response showed that the main deficiency behind the lack or weak development of empathy is the inability to correlate the context of the situation and the relevance of a certain reaction (even if this reaction is present in the behavioral repertoire).The implementation of our program showed that 7–8-year-old children with autism can learn to demonstrate socially significant empathic response skills in pre-game conditions, and that these skills can extend to situations and toys not intended for learning, as well as situations in natural conditions. It is connected with the possibility of correlating the context and the relevance of a certain reaction. In other words, we can conclude that empathic response is available for children with ASD.At the same time, we came to a conclusion that people with ASD are characterized by an inconsistency in manifestation of affective reactions, including empathic ones, which predetermines difficulties in other people’s perceiving of such manifestations. Consistency in the manifestation of empathic response implies the contextual unity of eye contact, facial expressions, intonation accompaniment, and vocal and motor manifestations. As the results of this study show, we can help children and adults with ASD to show contextual components of the empathic response. The integration of empathic manifestations into a coordinated affective response was not observed in this study. To what extent such integration is fundamentally achievable and what are the ways and prospects for the formation of a coordinated empathic response is a question for future research.

A Functional and Neuroanatomical Model of Dehumanization.

The dehumanization of others is a major scourge of mankind; however, despite its significance, physicians have little understanding of the neurobiological mechanisms for this behavior. We can learn much about dehumanization from its brain-behavior localization and its manifestations in people with brain disorders. Dehumanization as an act of denying to others human qualities includes two major forms. Animalistic dehumanization (also called infrahumanization) results from increased inhibition of prepotent tendencies for emotional feelings and empathy for others. The mechanism may be increased activity in the inferior frontal gyrus. In contrast, mechanistic dehumanization results from a loss of perception of basic human nature and decreased mind-attribution. The mechanism may be hypofunction of a mentalization network centered in the ventromedial prefrontal cortex and adjacent subgenual anterior cingulate cortex. Whereas developmental factors may promote animalistic dehumanization, brain disorders, such as frontotemporal dementia, primarily promote mechanistic dehumanization. The consideration of these two processes as distinct, with different neurobiological origins, could help guide efforts to mitigate expression of this behavior.

CFTR Inhibitors Display In Vitro Antiviral Activity against SARS-CoV-2

Several reports have indicated that SARS-CoV-2 infection displays unexpected mild clinical manifestations in people with cystic fibrosis (pwCF), suggesting that CFTR expression and function may be involved in the SARS-CoV-2 life cycle. To evaluate the possible association of CFTR activity with SARS-CoV-2 replication, we tested the antiviral activity of two well-known CFTR inhibitors (IOWH-032 and PPQ-102) in wild type (WT)-CFTR bronchial cells. SARS-CoV-2 replication was inhibited by IOWH-032 treatment, with an IC50 of 4.52 μM, and by PPQ-102, with an IC50 of 15.92 μM. We confirmed this antiviral effect on primary cells (MucilAirTM wt-CFTR) using 10 μM IOWH-032. According to our results, CFTR inhibition can effectively tackle SARS-CoV-2 infection, suggesting that CFTR expression and function might play an important role in SARS-CoV-2 replication, revealing new perspectives on the mechanisms governing SARS-CoV-2 infection in both normal and CF individuals, as well as leading to potential novel treatments.

Supporting Behavioral and Psychological Challenges in Alzheimer Using Technology: A Systematic Review

ABSTRACT The aging of the population is associated with an increase in the prevalence of neurodegenerative diseases such as dementia. Our research aim was to determine how technology can help to improve the support for behavioral and psychological challenges of dementia. A systematic review of the literature was performed following PRISMA guidelines. Papers meeting the inclusion criteria and reporting technological devices aimed to the management of these challenges. After screening 1085 papers, 18 studies were eligible for inclusion in the review. Technologies identified in this search (e.g., robots, mobile, apps, computer, software, GPS, wearables, assistive technology) suggest that this non-pharmacological approach may be useful in projects aimed to the management and control of behavioral and psychological manifestations in people living with Alzheimer. The manifestations that are most successfully supported by means of technological applications and devices are depression, sleep disorders, anxiety, apathy, motor activity, and agitation.

Mencari Pemimpin Negarawan Membagun Demokrasi Berkeadaban Dan Dinamika Pemilihan Umum Presiden 2024 Menuju Indonesia Emas

Besides having been ordered by God Almighty, the statesman leader was also not formed immediately. A statesman leader will always place professionalism and morals as the main standard. Leaders of statesmen whose policies depart from the collective interests of the people and minimize the domination of collegial interests originating from the group interested sts and encouragement. Leaders of statesmen never mathe ke empty promises. If you promise, the promithe se is a debt that will be accounted for vertically and horizontally. General elections are one of the vehicles for democracy as a manifestation of people's sovereignty. From the above expectations, we should be able to build a civilized democracy, a naresearchocracy that brings goodness and dignity and welfare to society (welfare state), far from SARA intrigues or identity politics. The role of the younger generation as the future leadership relay must be positioned, seated, and understood about future statesman leadersingle-spacedf 11-pointl parties is not only to produce a political leader but also to be able to produce a statesman leader.

Ocular Manifestations in People Living with HIV/AIDS (PLWHA) at the Kerti Praja Foundation, Denpasar, Bali

Background and purpose: People living with Human Immunodeficiency Virus - acquired immunodeficiency syndrome (PLWHA) could present various ocular manifestations according to the CD4+ lymphocyte count. This study aims to determine the socio-demographic characteristics and ocular manifestations of PLWHA. Methods: This study is an observational study with a cross-sectional design and consecutive sampling, involving a total of 118 samples, conducted in August 2021 at Kerti Praja Foundation Denpasar. Data were collected through history taking and ocular examinations, which included the variables of gender, age, occupation, residence, working status, level of education, duration of ARV treatment, treatment compliance, visual acuity, anterior segment manifestation, posterior segment manifestation, and CD4 T-cell count. Data analysis was performed with a statistical software. Results: A total of 118 samples were involved in this study, with 53 respondents (44.9%) experienced ocular manifestations and 55.1% did not experience ocular manifestations. The most frequent ocular manifestation identified was dry eye (68.5% found in right eye and 54.9% found in left eye), while only 2 patients have posterior-segment manifestation. Conclusion: The most common ocular manifestation in HIV-infected people on anti-retroviral therapy was dry eye. Screening for dry eye is recommended in PLWHA.

RESULTS OF THEORETICAL ASPECTS OF FORMATION OF CARTOGRAPHY

Technological progress has touched practically all spheres of human activity. In the twenty first century, it is important to discover the world without a laptop or a smartphone, without software security, which allows you to quickly and effectively manage your business needs. Cartography has changed a lot and today. Making geographical discoveries, mankind from very ancient maps and protoplans came to mathematical cartography, and technological progress made it possible to create electronic maps, without which no modern person can do today. Cartography, as a science, is closely related to geography, and geographic insights have long been a tool for cartography. As a prime example of what Columbus declared to the New World, that cardinal change in the manifestation of people about the world. The achievements of modern cartography are based on technological progress. Far from skin science can boast of such a rich history of its formation as cartography, even though it is the root of ancient hours and skin historical period of the formation of cartography as a science of appearances with prominent examples of humankind and skin in its own way for culture. The path of the formation of cartography as a science can be mentally subdivided into several periods: cartography of the ancient world; cartography of the period of great geographic appearances of seafarers; cartography, which is based on technical progress and mathematical basis; the latest cartography with the best modern technologies. Cartography appeared, and the people learned how to designate coordinates. Modern cartography is a collapsible technological process based on the fundamental achievements of science and technology. Previously, the cards were hung on stones, created behind an additional penzle and parchment, but now the process has been completed, which allows you to take an electronic card and visualization using various modern technologies that are practically accessible to the skin.

ЛІНГВОКУЛЬТУРОЛОГІЧНЕ ТЛУМАЧЕННЯ ОЙКОНІМІВ: У ПОШУКАХ ІСТИНИ

The article is devoted to the review and analysis of the conceptual foundations and results of the study by V. V. Kotovych, set out in the book «The Oiconym of Ukraine as a linguistic and cultural phenomenon: monograph» (2020). The study aims at studing the names of settlements (mostly urban) in Ukraine in the onomastic and cultural aspect, decoding linguistic and extralinguistic information reflected in the basics of the analyzed concepts. In four chapters of the monograph are analyzed scientific works devoted to the study of Ukrainian oikonymy in different aspects; the linguistic and cultural information contained in the oikonyms of Ukraine (first of all the proper names of cities) is characterized; the Ukraine оikonyms is described as a multifaceted linguistic and cultural phenomenon through the prism of reflection in specific names of various manifestations of people’s lives, their ideas about space and time, spirituality, certain symbols, etc. Unfortunately, in many cases V. V. Kotovych explains the names of settlements outside their historical forms, and analyzes the creative bases superficially, neglecting their word-forming structure and semantics. This approach often leads to erroneous conclusions both in the interpretation of the derivation model of oikonyms and in establishing their etymology. For example, the author derives the oikonym Zhmerynka in Eastern Podillya on behalf of *Zhmeryn + the suffix -k-a. However, the original name of the city is not Zhmerynka, but Bokiivtsi, known since the end of the XVI century: 1598 — Bokijowce. The name Zhmerynka is later. By the way, she was preceded by the Zhmerynka uniform. In addition, the suffix -k-a does not perform a possessive function. Thus, linguistic and cultural research of toponyms has prospects only if it is based on professional structural-semantic and etymological analysis of the respective geographical names, taking into account their historical forms and possible phonetic changes in the time plane.

Analysis of the genotypic profile and its relationship with the clinical manifestations in people with cystic fibrosis: study from a rare disease registry

BackgroundCystic fibrosis (CF) has a vast and heterogeneous mutational spectrum in Europe. This variability has also been described in Spain, and there are numerous studies linking CFTR variants with the symptoms of the disease. Most of the studies analysed determinate clinical manifestations or specific sequence variants in patients from clinical units. Others used registry data without addressing the genotype–phenotype relationship. Therefore, the objective of this study is to describe the genetic and clinical characteristics of people with CF and to analyse the relationship between both using data from the rare disease registry of a region in southeastern Spain.MethodsA cross-sectional study was carried out in people with a confirmed diagnosis of CF registered in the Rare Diseases Information System (SIER) of the Region of Murcia (Spain). The patients were classified into two genotypes according to the functional consequence that the genetic variants had on the CFTR protein.ResultsThere were 192 people diagnosed with CF reported in the Region of Murcia as of 31 December 2018. Seventy-six genotypes and 49 different variants were described, with c.1521_1523delCTT (p. Phe508del) being the most common in 58.3% of the CF patients and 37.0% of the alleles. In addition, 67% of the patients were classified as a high-risk genotype, which was associated with a lower percentage of FEV1 (OR: 5.3; 95% CI: 1.2, 24.4), an increased risk of colonization by Pseudomonas aeruginosa (OR: 7.5; 95% CI: 1.7, 33.0) and the presence of pancreatic insufficiency (OR: 28.1; 95% CI: 9.3, 84.4) compared to those with a low-risk genotype.ConclusionsThis is the first study in Spain that describes the mutational spectrum and its association with clinical manifestations in patients with CF using data from a rare disease registry. The results obtained allow planning for the health resources needed by people with this disease, thus contributing to the development of personalized medicine that helps to optimize health care in CF patients.

Справедливость: старые новые ракурсы проблемы

The purpose of the study. The author aims, on the one hand, to point out the universal problems that make justice an extremely difficult topic, and on the other hand, to show which subjects of justice have come to the fore today, what they have brought to the classic set of questions about justice. Methodological basis of the research. Justice/injustice is seen by the author of the article as a value-communicative attitude built around the plot "equality-inequality", "behavior-retribution", and carrying a huge charge of subjectivity: what is considered fair depends on the leading values. Research results. Universal difficulties of studying the topic, according to the author, are: first of all, the inability to establish justice without the desire of adherents of different values (interests, goals) to find a value agreement, to find a "sphere between" with another. The article notes that the leading theme of justice/injustice has historically always been social and material inequality, camouflaged by the imitation of equality of all before the market in a market society. However, in these days of revaluation of values, the theme of justice/injustice is also being transferred to new areas. So the doubt about the value of a stable sexual identity generates a debate around whether it is fair to transgender people to call a woman a woman and a man a man. The revision of the value of the will of the majority leads to the demand for "fair power" for minorities. The postmodern rejection of the value of truth and truth declares "just" a radical revision of history with the overthrow of former heroes and idols. The field of total injustice is becoming the media, which refused evidence, without which justice is impossible. Ignoring the value of qualitative assessment, the widespread transition to purely quantitative assessments in scores, ratings and occupied places is also an acute problem. The article also discusses the question of the abstractness of normative theories of justice, which ignore the extra-rationality of many manifestations of people, their tendency to passion, bitterness, resentment and the desire to win instead of justice at all costs.   Prospects for the study. The technization and informatization of society, as well as new types of social relations, form a demand for the formation of new approaches to the definition and study of social justice in the modern world.