Hepatorenal tyrosinaemia (HT1) is an autosomal recessive disorder of tyrosine degradation resulting in hepatic and renal dysfunction, neurological sequelae may occur in some patients. The use of nitisinone (NTBC) has revolutionised treatment and outcome of this disorder. NTBC has to be combined with a low protein diet. While NTBC modulates the disease course in HT1 patients, several issues are open. Optimal dosage, doses per day, therapeutic range of NTBC concentration, mode of protein restriction and biomarkers are not well defined. HCC and neurocognitive deficits are long-term sequelae. Early diagnosis and treatment are essential to minimise the risk for these complications. Clinical guidance for management of HT1-patients is required. Randomised clinical studies are difficult in the presence of therapeutic options. We discussed these issues in a consensus group of 10 paediatricians, 1 adult hepatologist, 1 geneticist, 2 dieticians, 2 newborn screening specialists with experience in HT1, 1 psychologist and 2 representatives of a patient group from the German-speaking countries (DACH). Recommendations were based on scientific literature and expert opinion, also taking into account recent experience with newborn screening. There was strong consensus that newborn screening using succinylacetone (SA) and early treatment are essential for a good outcome. The dose of NTBC should be as low as possible without losing metabolic control. This has to be accompanied by a low protein diet, in some patients a simplified diet without calculation of protein intake. Specific education and psychosocial support are recommended. Indications for liver transplantation were defined. Monitoring shall include clinical findings, levels of SA, tyrosine, phenylalanine and NTBC in (dried) blood.

• MeSH
• cyklohexanony * terapeutické užití   MeSH
• heptanoáty MeSH
• konsensus MeSH
• lidé MeSH
• nitrobenzoany * terapeutické užití   MeSH
• nízkoproteinová dieta MeSH
• novorozenec MeSH
• novorozenecký screening * metody   MeSH
• transplantace jater MeSH
• tyrosinemie * diagnóza   terapie   MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Geografické názvy
• Německo MeSH

• MeSH
• cystektomie MeSH
• diverze moči * MeSH
• lidé MeSH
• nádory močového měchýře * chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Německo MeSH

INTRODUCTION: Advance care planning (ACP) ensures that patients receive medical care aligned with their values, goals, and preferences, especially regarding end-of-life decisions in serious chronic illnesses. OBJECTIVE: This project aimed to introduce and promote evidence-based ACP in oncology and palliative care at a midsized hospital near Berlin, Germany, during the COVID-19 pandemic. METHODS: This project was guided by the JBI Evidence Implementation Framework and used a mixed methods audit cycle. A baseline audit was conducted using qualitative interviews and workshops with representatives from all the health care disciplines involved in oncology and palliative care at the hospital. The findings were compared with eight best practice recommendations. Targeted strategies aimed at the key stakeholders involved in ACP practice were then implemented. Finally, a semi-quantitative questionnaire was used in a follow-up audit with the same participants as in the baseline audit. RESULTS: The baseline audit revealed a high level of familiarity with the concept of ACP. However, there was a lack of a uniformly accepted definition and understanding of ACP among the health care professionals, leading to a lack of coordination in task distribution. The follow-up audit revealed improvements with regard to education and training in ACP (Criterion 1: 50% to 100%) and organizational support to facilitate ACP conversations (Criterion 3: 87.5% to 100%). Other audit criteria compliance rates remained unchanged. CONCLUSION: Clinical education and team-based process analysis can facilitate ACP implementation across disciplines in oncology and palliative care facilities. However, the project did not succeed in implementing lasting changes in clinical processes and best practice ACP due to the COVID-19 pandemic. Such an endeavor would demand considerable resources and time, both of which were constrained during the pandemic. SPANISH ABSTRACT: http://links.lww.com/IJEBH/A236.

• MeSH
• COVID-19 * epidemiologie   MeSH
• lékařská onkologie * MeSH
• lékařská praxe založená na důkazech MeSH
• lidé MeSH
• paliativní péče * organizace a řízení   MeSH
• pandemie MeSH
• předběžné plánování péče * MeSH
• SARS-CoV-2 MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Německo MeSH

OBJECTIVE: The aim of this study was to determine the prevalence of potentially inappropriate medications (PIMs) in nursing home residents across eight countries and investigate differences between residents with and without cognitive impairment, as well as those with and without life expectancy of six months or less. METHODS AND DEIGN: The study utilized the second edition of the STOPP-Frail criteria to operationalize PIMs in the baseline assessment of nursing home residents participating in the Services and Health for Elderly in Long TERm care (SHELTER) project. The data were collected between 2009 and 2012. The project was conducted in eight countries: Czech Republic, England, Finland, France, Germany, Italy, the Netherlands, and Israel. Cognitive impairment was measured by the cognitive performance scale (CPS). The presence of end-stage disease with a life expectancy of six months or less was recorded. The study included residents aged 60 years or older who underwent a valid medication assessment. RESULTS: Among the 3,832 eligible residents, 87.9% had at least one PIM. Specifically, 24.3%, 23.5%, 18.8%, and 19.3% of residents had one, two, three, and four or more PIMs, respectively. On average, each person was prescribed 2.16 PIMs. Cognitively impaired residents (n = 1999) had an average of 1.96 PIMs (SD 1.49) per person, while residents with a low CPS score (n = 1783) had an average of 2.40 PIMs (SD 1.57) per person, showing a statistically significant difference (P < 0.001). Similarly, NH residents with life expectancy of six months or less had an average of 1.66 PIMs (SD 1.30), whereas those without had an average of 2.17 PIMs (SD 1.55) (p < 0.001). The average number of PIMs varied across countries, ranging from 3.23 in Finland to 2.15 in the UK (P < 0.001). Anti-platelets and aspirin were the most prescribed PIMs, accounting for over 38.0% of prescriptions. CONCLUSIONS: This study highlights the high prevalence of PIMs among nursing home residents. However, PIMs were somewhat lower in residents with cognitive impairment and life expectancy of six months or less. Efforts must continue to improve the rationale behind prescribing practices in nursing homes.

• MeSH
• domovy pro seniory MeSH
• křehký senior MeSH
• lidé středního věku MeSH
• lidé MeSH
• nevhodné předepisování statistika a číselné údaje   MeSH
• pečovatelské domovy * MeSH
• prevalence MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• seznam potenciálně nevhodných léčiv * MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Německo MeSH

PURPOSE: The significance of dental status and oral hygiene on a range of medical conditions is well-recognised. However, the correlation between periodontitis, oral bacterial dysbiosis and visceral surgical outcomes is less well established. To this end, we study sought to determine the influence of dental health and oral hygiene on the rates of postoperative complications following major visceral and transplant surgery in an exploratory, single-center, retrospective, non-interventional study. METHODS: Our retrospective non-interventional study was conducted at the Department of General, Visceral, and Transplant Surgery, University Hospital Heidelberg, Germany. Patients operated on between January 2018 and December 2019 were retrospectively enrolled in the study based on inclusion (minimum age of 18 years, surgery at our Department, intensive care / IMC treatment after major surgery, availability of patient-specific preoperative dental status assessment, documentation of postoperative complications) and exclusion criteria (minor patients or legally incapacitated patients, lack of intensive care or intermediate care (IMC) monitoring, incomplete documentation of preoperative dental status, intestinal surgery with potential intraoperative contamination of the site by intestinal microbes, pre-existing preoperative infection, absence of data regarding the primary endpoints of the study). The primary study endpoint was the incidence of postoperative complications. Secondary study endpoints were: 30-day mortality, length of hospital stay, duration of intensive care stay, Incidence of infectious complications, the microbial spectrum of infectious complication. A bacteriology examination was added whenever possible (if and only if the examination was safe for the patient)for infectious complications. RESULTS: The final patient cohort consisted of 417 patients. While dental status did not show an influence (p = 0.73) on postoperative complications, BMI (p = 0.035), age (p = 0.049) and quick (p = 0.033) were shown to be significant prognostic factors. There was significant association between oral health and the rate of infectious complications for all surgical procedures (p = 0.034), excluding transplant surgery. However, this did not result in increased 30-day mortality rates, prolonged intensive care unit treatment or an increase in the length of hospital stay (LOS) for the cohort as a whole. In contrast there was a significant correlation between the presence of oral pathogens and postoperative complications for a group as a whole (p < 0.001) and the visceral surgery subgroup (p < 0.001). Whilst this was not the case in the cohort who underwent transplant surgery, there was a correlation between oral health and LOS in this subgroup (p = 0.040). Bacterial swabs supports the link between poor oral health and infectious morbidity. CONCLUSIONS: Dental status was a significant predictor of postoperative infectious complications in this visceral surgery cohort. This study highlights the importance preoperative dental assessment and treatment prior to major surgery, particularly in the case of elective surgical procedures. Further research is required to determine the effect of oral health on surgical outcomes in order to inform future practice. TRIAL REGISTRATION: Trial registered under the ethics-number S-082/2022 (Ethic Committee of the University Heidelberg).

• MeSH
• délka pobytu statistika a číselné údaje   MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• orální hygiena MeSH
• orální zdraví MeSH
• pooperační komplikace * epidemiologie   etiologie   MeSH
• retrospektivní studie MeSH
• senioři MeSH
• transplantace orgánů škodlivé účinky   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH
• Geografické názvy
• Německo MeSH

• Klíčová slova
• Contergan,
• MeSH
• 2. světová válka MeSH
• dějiny 20. století MeSH
• farmaceutický průmysl dějiny   MeSH
• lékaři dějiny   MeSH
• lidé MeSH
• nacionální socialismus dějiny   MeSH
• thalidomid chemická syntéza   dějiny   škodlivé účinky   MeSH
• vakcíny proti tyfu a paratyfu chemická syntéza   dějiny   MeSH
• vyvíjení léků * dějiny   MeSH
• zločin dějiny   MeSH
• zločinci dějiny   MeSH
• Check Tag
• dějiny 20. století MeSH
• lidé MeSH
• Geografické názvy
• Německo MeSH

Aim: Individual experiences shape perceptions of illness. Post-Soviet migrants have different expectations of chronic disease treatment than German patients. This study aimed to delve into perceptions of diabetes mellitus, a topic of increasing importance in global migration. Design: Qualitative, semi-structured interviews were conducted with 26 post-Soviet migrants with diabetes and three Russian-speaking diabetes counselors, mainly recruited through a snowball system. Methods: The research data were analyzed using a hermeneutic approach. The categories of the common-sense self-regulation model and developed subcategories were scrutinized by means of content analysis, ensuring a comprehensive understanding of the data. Results: The perception of diabetes mellitus varied depending on the country where the diagnosis was made. This included the individual recording of symptoms, the expected time course, and the allocation of different competencies. Opportunities for access to the local healthcare system in the former Soviet Union influenced these expectations. Conclusion: The study's findings underscore the importance of healthcare access in the country of origin and the patient's knowledge of diabetes mellitus in shaping their perception and treatment. These factors, influenced by the social environment and the patient's level of education, continue to play a role even after immigration to Germany.

• MeSH
• diabetes mellitus 2. typu * psychologie   MeSH
• emigranti a imigranti * statistika a číselné údaje   MeSH
• komunikace MeSH
• lidé středního věku MeSH
• lidé MeSH
• odborná způsobilost MeSH
• percepce MeSH
• poskytování zdravotní péče MeSH
• průzkumy a dotazníky MeSH
• rozhovory jako téma MeSH
• senioři MeSH
• sociální prostředí MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Geografické názvy
• Německo MeSH

This study examines the personality patterns of solo founders in both high-tech and non-high-tech sectors during the first seven years of their entrepreneurial journey to emphasize the patterns' implications during policymaking, investment decisions, and self-assessments. IAB/ZEW startup panel microdata for the sector classification of 4470 solo entrepreneurs in Germany were analyzed to identify Big Five trait patterns influenced by risk propensities, innovation inclination, and gender. The entrepreneurial profiles indicate positive openness, emotional resilience, and sector-specific clusters. Conscientiousness suggests flexibility, and while variations in extraversion and agreeableness exist, negative neuroticism was predominantly found, except for gender-related differences and multidimensional service innovators. Big Five traits provide information about important foundational profile patterns to describe unique solo entrepreneur types influenced by risk, innovation, and gender. Originality and value: Risk propensity characterizes 'Adaptive Services,' 'Dynamic Knowledge Innovators,' and 'Strategic Risk Navigators.' Additionally, 'Multidimensional Service Innovators' and 'Focused Tech Innovators' signify innovation understanding. The Big Five profiles show openness and emotional stability across sectors, providing crucial insights for effective entrepreneurial support and investment strategies.

• MeSH
• dospělí MeSH
• lidé MeSH
• osobnost * MeSH
• podnikání * MeSH
• průmysl MeSH
• riziko MeSH
• sexuální faktory MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Německo MeSH

OBJECTIVES: To determine how social factors influence career decisions of dental service providers, particularly focusing on examining the impact of dentists' origins. METHODS: Online survey of Hessian panel dentists, with pairwise comparisons to a set of factors impacting their decision-making process. An Analytic Hierarchy Process examined the weighting of influencing drivers in career choice. RESULTS: Dentists from rural backgrounds were more likely to establish practices in rural areas than those from urban origins. Origin correlated with entrepreneurial intentions and a strong association of rural origin. Dentists who grew up in rural areas were 4.19 times more likely to start a business. CONCLUSION: These findings may support efficient resource allocation and support for rural dental businesses.

• MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• malé podnikání MeSH
• odborná praxe - umístění MeSH
• průzkumy a dotazníky MeSH
• venkovské obyvatelstvo MeSH
• volba povolání * MeSH
• zdravotnické služby pro venkov MeSH
• zubní lékaři statistika a číselné údaje   psychologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Německo MeSH

BACKGROUND: This study outlines the development of a highly interoperable federated IT infrastructure for academic biobanks located at the major university hospital sites across Germany. High-quality biosamples linked to clinical data, stored in biobanks are essential for biomedical research. We aimed to facilitate the findability of these biosamples and their associated data. Networks of biobanks provide access to even larger pools of samples and data even from rare diseases and small disease subgroups. The German Biobank Alliance (GBA) established in 2017 under the umbrella of the German Biobank Node (GBN), has taken on the mission of a federated data discovery service to make biosamples and associated data available to researchers across Germany and Europe. METHODS: In this context, we identified the requirements of researchers seeking human biosamples from biobanks and the needs of biobanks for data sovereignty over their samples and data in conjunction with the sample donor's consent. Based on this, we developed a highly interoperable federated IT infrastructure using standards such as Fast Healthcare Interoperability Resources (HL7 FHIR) and Clinical Quality Language (CQL). RESULTS: The infrastructure comprises two major components enabling federated real-time access to biosample metadata, allowing privacy-compliant queries and subsequent project requests. It has been in use since 2019, connecting 16 German academic biobanks, with additional European biobanks joining. In production since 2019 it has run 4941 queries over the span of one year on more than 900,000 biosamples collected from more than 170,000 donors. CONCLUSION: This infrastructure enhances the visibility and accessibility of biosamples for research, addressing the growing demand for human biosamples and associated data in research. It also underscores the need for improvements in processes beyond IT infrastructure, aiming to advance biomedical research and similar infrastructure development in other fields.

• MeSH
• banky biologického materiálu * MeSH
• biomedicínský výzkum MeSH
• databáze faktografické MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH
• Německo MeSH