Predicted health care profile after transition to adult care in Turner syndrome children—experience of single center

IntroductionTurner Syndrome (TS) is caused by the complete or partial loss of one of the X chromosomes in all or some female cell lines. The variable genotypes are responsible for a large phenotypic diversity, nevertheless most studies emphasize a weak correlation between genotype and phenotype. The study aimed to assess the occurrence of defects and diseases depending on the karyotype in patients with TS and correlation with the predicted health care profile after the transition to adulthood.Materials and methods45 patients of the Department of Endocrinology and Pediatrics of the Medical University of Warsaw in 1990–2002 were analyzed. Girls were divided into 2 subgroups: “A”, which included 16 patients with the karyotype 45,X, and “B”, which included 29 girls with mosaic karyotypes. Based on the literature data, characteristic phenotypic features and the typical defects or diseases accompanying TS were selected, and the frequency of their occurrence was compared in both subgroups. Accordingly to this data, the predicted medical care profile was determined.ResultsIn our study, patients with complete monosomy of the X chromosome had more characteristic phenotypic features. They needed sex hormone replacement therapy more often and started to menstruate spontaneously much less frequently (only 18.18% in monosomy vs. 73.91% in mosaic patients, p = 0.006). In patients with monosomy, congenital defects of the circulatory system were found more often (46.67% vs. 30.77%). The diagnosis in patients with mosaic karyotype was more often delayed, therefore the optimal time of growth hormone therapy was shorter. In our study, the X isochromosome determined the higher prevalence of autoimmune thyroiditis (83.33% vs. 12.5%, p = 0.049). We didn't find a correlation between the type of karyotype and health care profile after the transition, most of the patients needed more than 2 specialists. Most often, they required: gynecologists, cardiologists, and orthopedics.ConclusionsAfter the transition from pediatric to adulthood, patients with TS need multidisciplinary care, but not all need the same kind of assistance. The phenotype and comorbidities determine the profile of patients' health care, however it wasn't directly related to the type of karyotype in our study

Habitat - idea, sztuka, filozofia

Publikacja recenzowana / Peer-reviewed publicationZE WSTĘPU: Architektura a habitat. Wyzwaniem na dziś nie jest chyba tylko kształt architektonicznych ikon, ale to jak w najbliższej przyszłości projektanci, architekci i urbaniści mają sobie radzić z problemami demograficznymi, socjalnymi i ekonomicznymi. Zapewne habitat będzie się zmieniał i przybierał coraz to inne formy, które zharmonizowane zostaną jako proekologiczny organizm współgrający z wszystkimi samowystarczającymi systemami. Znaczącą jednak funkcję pełnić będzie zawsze sztuka budowania miejsc i nieodłączną w tym znaczeniu formą i funkcją. Łącząc piękno, piękno życia i przeżywania, piękno naturalne i stworzone przez człowieka, będziemy mieli zawsze moralny obowiązek ochrony i pomnażania tego piękna. Ta wielka kompozycja o nieograniczonej liczbie możliwości i wariantów będzie udziałem Nowego Człowieka solidarnie budującego i przeżywającego Nową Postać Świata

Competences 4.0 in the logistics sector against the challenges of sustainable digital transformation of the economy in the Polish perspective

Purpose: The aim of the paper was the identification of 4.0 competences that should be characterized by logistics workers in the era of the fourth industrial revolution. Design/methodology/approach: First step was the desk research analysis of source material (monographic studies, publications and reports). The second step were primary (fragmentary) explanatory, descriptive and explanatory, focused on the implementation of one research goal. The method used in the course of the study was the "user-centric" CAWI (Computer Assisted Web Interview). Findings: Technologization and robotization require from employees to acquire new digital competences. Studies show that logistics workers are aware of the needed change. There is a need to acquire competences 4.0 which can consist of cognitive, informative and technical competences. Research limitations/implications: The main limitation of the research was the availability of employees in logistics companies and the precise division of enterprises in which the respondents were employed. In the available statistical summaries and address databases, there is no division taking into account the place of logistics as an element of business activities in enterprises. Practical implications: Research can be also a signpost for employers of what kind of competences should be developed in the future in the face of upcoming economy 5.0 and society 5.0. Originality/value: The article can be a road-map for sustainable digital transformation taking into account not only machines but also human beings

Early effects of Nivolumab and Ipilimumab combined immunotherapy in the treatment of metastatic melanoma in Poland : a multicenter experience

Nivolumab and ipilimumab combination became the first-line standard in advanced melanoma. We assessed its efficacy in a real-life study in Poland. In a one-year follow-up, we evaluated the medical records of 50 melanoma patients treated with that modality in five oncology centers. We recorded therapy outcomes and adverse events (AEs) after 3 and 12 months of therapy. At the first checkpoint, the disease control rate (DCR) was recorded in 58% (n = 29) of patients, but the same number of patients (n = 29, 58%) stopped immunotherapy due to disease progression (PD, n = 14, 48.3%), toxicity (n = 11, 37.9%) or death (n = 4, 13.8%). Among patients with DCR after the induction phase, 8 (27.6%) terminated due to toxicity, and 21 (72.4%) continued. However, at the 12-month checkpoint, only 14 patients (27% of all) were still receiving immunotherapy. In 7 (33.3%) it was discontinued due to PD (n = 2), toxicity (n = 2, 28.6% each), or death (n = 3, 42.9%). AEs occurred in 66.7% (n = 34) of patients; severe (grade 3 or 4) in half of them. Interestingly, those with AEs had an 80% lower risk of death (hazard ratio [HR] 0.2, 95% confidence interval [CI] 0.07–0.57, p = 0.001) and PD (HR 0.2, 95%CI 0.09–0.47, p < 0.0001). In the entire group of patients, after a 12-month follow-up, the median overall survival was not reached (NR, range: 6.8 months-NR) and progression-free survival was 6.3 (range: 3-NR) months. Our results demonstrate that combined immunotherapy is less effective in real-life than in pivotal trials. However, early responders will likely continue the therapy after a one-year follow-up. AEs occurrence might be a predictor of clinical effectiveness