47 research outputs found
Subsidence across the Antler foreland of Montana and Idaho: Tectonic versus eustatic effects
Devonian and Mississippian sedimentary rocks of western Montana and east-central Idaho were deposited on a cratonic platform that faced a deep basin to the west. The deep basin in Idaho was a northern extension of the Antler foredeep and formed as a flexural response to loading of the ancient North American continental margin by an inferred arc and thrust belt complex. Subsidence analyses of the Devonian-Mississippian strata indicate episodic subsidence events in the proximal foredeep and adjacent cratonic platform, an area approximately 800 km (500 mi) wide (palinspastic). Isopach maps for this sequence illustrate that many depocenters and paleohighs were geographically coincident across the foreland through time. The Devonian-Mississippian foreland structures had cross-sectional wavelengths of 50-200 km (30-120 mi) and amplitudes of about 50-350 m (160-1,150 ft). Some of these structures were tectonically inverted (i.e., paleohighs became depocenters and vice versa) several times during the 50-60 m.y. represented by this stratigraphic sequence. Many of these generally east-west-trending paleostructures were oriented at high angles to the north-south-trending axis of the Antler foredeep and the inferred strike of the Antler orogenic belt. These foreland structures coincide geographically with structural trends produced during Proterozoic extension, suggesting that the Proterozoic faults were reactivated during Antler convergence. The isopach maps also show progressive southeastward migration of Antler foredeep depocenters from Late Devonian to Early Pennsylvanian time. The southeastward migration of the foredeep depocenter suggests that the maximum thrust load moved progressively southeastward from Late Devonian to Early Pennsylvanian time. The complex patterns of subsidence across the Montana-Idaho foreland do not fit into simple flexural models for vertical loading of unbroken elastic plates. Instead, differential subsidence of the foreland may be related to several mechanisms: (1) flexure of mechanically independent, fault-bounded segments of the foreland produced by areally limited thrust loads (subregional vertical loading); (2) transmission of compressive in-plane stresses through the foreland lithosphere (regional horizontal loading) that may have reactivated Proterozoic fault systems; and (3) waxing and waning of in-plane compressive stresses resulting from the episodic nature of Antler convergence. Results from this study suggest that, in settings where the foreland lithosphere is broken by ancient fault systems, the foreland may exhibit complex patterns of differential subsidence that probably reflect a composite response to both vertical and horizontal loads. Also, the simultaneous pulses of subsidence documented across large parts of the Antler foreland suggest that it may be possible to date episodes of convergence along ancient continental margins, even when the ancient thrust belt complex is poorly preserved
Subsidence across the Antler foreland of Montana and Idaho: Tectonic versus eustatic effects
Devonian and Mississippian sedimentary rocks of western Montana and east-central Idaho were deposited on a cratonic platform that faced a deep basin to the west. The deep basin in Idaho was a northern extension of the Antler foredeep and formed as a flexural response to loading of the ancient North American continental margin by an inferred arc and thrust belt complex. Subsidence analyses of the Devonian-Mississippian strata indicate episodic subsidence events in the proximal foredeep and adjacent cratonic platform, an area approximately 800 km (500 mi) wide (palinspastic). Isopach maps for this sequence illustrate that many depocenters and paleohighs were geographically coincident across the foreland through time. The Devonian-Mississippian foreland structures had cross-sectional wavelengths of 50-200 km (30-120 mi) and amplitudes of about 50-350 m (160-1,150 ft). Some of these structures were tectonically inverted (i.e., paleohighs became depocenters and vice versa) several times during the 50-60 m.y. represented by this stratigraphic sequence. Many of these generally east-west-trending paleostructures were oriented at high angles to the north-south-trending axis of the Antler foredeep and the inferred strike of the Antler orogenic belt. These foreland structures coincide geographically with structural trends produced during Proterozoic extension, suggesting that the Proterozoic faults were reactivated during Antler convergence. The isopach maps also show progressive southeastward migration of Antler foredeep depocenters from Late Devonian to Early Pennsylvanian time. The southeastward migration of the foredeep depocenter suggests that the maximum thrust load moved progressively southeastward from Late Devonian to Early Pennsylvanian time. The complex patterns of subsidence across the Montana-Idaho foreland do not fit into simple flexural models for vertical loading of unbroken elastic plates. Instead, differential subsidence of the foreland may be related to several mechanisms: (1) flexure of mechanically independent, fault-bounded segments of the foreland produced by areally limited thrust loads (subregional vertical loading); (2) transmission of compressive in-plane stresses through the foreland lithosphere (regional horizontal loading) that may have reactivated Proterozoic fault systems; and (3) waxing and waning of in-plane compressive stresses resulting from the episodic nature of Antler convergence. Results from this study suggest that, in settings where the foreland lithosphere is broken by ancient fault systems, the foreland may exhibit complex patterns of differential subsidence that probably reflect a composite response to both vertical and horizontal loads. Also, the simultaneous pulses of subsidence documented across large parts of the Antler foreland suggest that it may be possible to date episodes of convergence along ancient continental margins, even when the ancient thrust belt complex is poorly preserved
Zmiany histopatologiczne w biopsji mięśnia u 31 chorych z mutacjami w genie kodującym kalpainę 3
Background and purpose
At present, more than 20 different forms of limb-girdle muscular dystrophies (LGMDs) are known (at least 7 autosomal dominant and 14 autosomal recessive). Although these different forms show some typical phenotypic characteristics, the existing clinical overlap makes their differential diagnosis difficult. Limb-girdle muscular dystrophy type 2 (LGMD2A) is the most prevalent LGMD in many European as well as Brazilian communities and is caused by mutations in the gene CAPN3. Laboratory testing, such as calpain immunohistochemistry and Western-blot analysis, is not totally reliable, since up to 20% of molecularly confirmed LGMD2A show normal content of calpain 3 and a third of LGMD2A biopsies have normal calpain 3 proteolytic activity in the muscle. Thus, genetic testing is considered as the only reliable diagnostic criterion in LGMD2A.
Material and methods
In an attempt to find a correlation between genotype and muscle pathology in limb-girdle muscular dystrophy 2A we performed histopathological investigation of a group of 31 patients subdivided according to the type of pathologic CAPN3 gene mutation.
Results
In all biopsies typical features of muscular dystrophy such as fiber necrosis and regeneration, variation in fiber size and fibrosis were noted. Lobulated fibers were often encountered in the muscle biopsies of LGMD2A patients. Such fibers were more frequent in patients with 550delA mutation.
Conclusions
These findings may be helpful in establishing diagnostic strategies in LGMD.Wstęp i cel pracy
Dotychczas opisano ponad 20 różnych form dystrofii obręczowo-kończynowej (limb girdle muscular dystrophy – LGMD) (co najmniej 7 rodzajów o dziedziczeniu autosomalnym dominującym oraz 14 o dziedziczeniu autosomalnym recesywnym). Pomimo że część z tych chorób można różnicować na podstawie obrazu klinicznego, diagnostykę utrudnia często podobieństwo objawów. Dystrofia obręczowo–kończynowa typu 2A (limb-girdle muscular dystrophy type 2 – LGMD2A), najczęstsza dystrofia mięśniowa w wielu społecznościach (np. w Europie i Brazylii), spowodowana jest przez mutacje w genie kalpainy 3 (CAPN3). Badanie immunohisto-chemiczne kalpainy czy też metodą Western blot nie są wystarczające do ustalenia właściwego rozpoznania (w odpowiednio 1/3 i 20% potwierdzonych genetycznie LGMD2A badania te wypadają prawidłowo). Podstawę rozpoznania tej miopatii stanowi badanie genetyczne.
Materiał i metody
W pracy przedstawiono wyniki badania zależności między genotypem a analizą histopatologiczną biopsji mięśnia u 31 chorych na LGMD2A. Chorzy podzieleni zostali na grupy według wyników badania genetycznego genu CAPN3 odpowiedzialnego za tę chorobę.
Wyniki
We wszystkich badanych biopsjach stwierdzano typowe zmiany dystroficzne, takie jak obecność włókien martwiczych i regenerujących, zróżnicowaną wielkość włókien oraz włóknienie. Włókna o nierównomiernym rozkładzie barwień na enzymy oddechowe (lobulated fibers) były często obserwowane w biopsjach chorych z LGMD2A. Tego typu włókna szczególnie często występowały u chorych z mutacją 550delA.
Wnioski
Wyniki pracy wnoszą nowe informacje ułatwiające diagnostykę LGMD
Early onset facioscapulohumeral dystrophy - a systematic review using individual patient data
Infantile or early onset is estimated to occur in around 10% of all facioscapulohumeral dystrophy (FSHD) patients. Although small series of early onset FSHD patients have been reported, comprehensive data on the clinical phenotype is missing. We performed a systematic literature search on the clinical features of early onset FSHD comprising a total of 43 articles with individual data on 227 patients. Additional data from four cohorts was provided by the authors. Mean age at reporting was 18.8 years, and 40% of patients were wheelchair-dependent at that age. Half of the patients had systemic features, including hearing loss (40%), retinal abnormalities (37%) and developmental delay (8%). We found an inverse correlation between repeat size and disease severity, similar to adult-onset FSHD. De novo FSHD1 mutations were more prevalent than in adult-onset FSHD. Compared to adult FSHD, our findings indicate that early onset FSHD is overall characterized by a more severe muscle phenotype and a higher prevalence of systemic features. However, similar as in adults, a significant clinical heterogeneity was observed. Based on this, we consider early onset FSHD to be on the severe end of the FSHD disease spectrum. We found natural history studies and treatment studies to be very scarce in early onset FSHD, therefore longitudinal studies are needed to improve prognostication, clinical management and trial-readiness
Global Impact of the COVID-19 Pandemic on Cerebral Venous Thrombosis and Mortality
Background and purpose: Recent studies suggested an increased incidence of cerebral venous thrombosis (CVT) during the coronavirus disease 2019 (COVID-19) pandemic. We evaluated the volume of CVT hospitalization and in-hospital mortality during the 1st year of the COVID-19 pandemic compared to the preceding year.
Methods: We conducted a cross-sectional retrospective study of 171 stroke centers from 49 countries. We recorded COVID-19 admission volumes, CVT hospitalization, and CVT in-hospital mortality from January 1, 2019, to May 31, 2021. CVT diagnoses were identified by International Classification of Disease-10 (ICD-10) codes or stroke databases. We additionally sought to compare the same metrics in the first 5 months of 2021 compared to the corresponding months in 2019 and 2020 (ClinicalTrials.gov Identifier: NCT04934020).
Results: There were 2,313 CVT admissions across the 1-year pre-pandemic (2019) and pandemic year (2020); no differences in CVT volume or CVT mortality were observed. During the first 5 months of 2021, there was an increase in CVT volumes compared to 2019 (27.5%; 95% confidence interval [CI], 24.2 to 32.0; P<0.0001) and 2020 (41.4%; 95% CI, 37.0 to 46.0; P<0.0001). A COVID-19 diagnosis was present in 7.6% (132/1,738) of CVT hospitalizations. CVT was present in 0.04% (103/292,080) of COVID-19 hospitalizations. During the first pandemic year, CVT mortality was higher in patients who were COVID positive compared to COVID negative patients (8/53 [15.0%] vs. 41/910 [4.5%], P=0.004). There was an increase in CVT mortality during the first 5 months of pandemic years 2020 and 2021 compared to the first 5 months of the pre-pandemic year 2019 (2019 vs. 2020: 2.26% vs. 4.74%, P=0.05; 2019 vs. 2021: 2.26% vs. 4.99%, P=0.03). In the first 5 months of 2021, there were 26 cases of vaccine-induced immune thrombotic thrombocytopenia (VITT), resulting in six deaths.
Conclusions: During the 1st year of the COVID-19 pandemic, CVT hospitalization volume and CVT in-hospital mortality did not change compared to the prior year. COVID-19 diagnosis was associated with higher CVT in-hospital mortality. During the first 5 months of 2021, there was an increase in CVT hospitalization volume and increase in CVT-related mortality, partially attributable to VITT
Państwo, gospodarka, społeczeństwo w integrującej się Europie TOM 3
Ze wstępu: "1 maja 2004 przyniesie radykalną zmianą sytuacji dotychczasowych
kandydatów do Unii Europejskiej. Z roli aplikanta i petenta przekształcą się
we współdecydenta. Już dziś z przyszłymi członkami konsultuje się większość
kwestii wymagających strategicznych decyzji. Przez ostatnie dziesięć lat wysiłek
polityczny i intelektualny był skierowany na uzyskanie członkostwa
Unii, a w ostatnim okresie negocjacji - na osiągnięcie najlepszych według
polityków i ekonomistów warunków akcesji. 1 ten etap mamy już za sobą. Pora
zacząć patrzeć przed siebie, lecz niejako petent, ale kraj współodpowiedzialny
za dalsze funkcjonowanie i rozwój powiększonej Unii.
Z tej perspektywy istotnajest analiza gospodarki europejskiej, z którąjuż dziś
gospodarka państw kandydackich, także Polski, jest silnie powiązana. Wiedza
na ten temat jest uboga i ograniczona do przeglądu bieżących wskaźników
makroekonomicznych. Zarówno w ośrodkach rządowych, jak i pozarządowych
dominuje podejście analizujące, co z konkretnego wydarzenia
w innym kraju wynika dla gospodarki polskiej. Stanowczo nie wystarczy to
do pełnienia odpowiedzialnej roli współdecydenta. Potrzebna jest pogłębiona
wiedza na temat gospodarki europejskiej jako całości i poszczególnych
krajów, a także najważniejszych partnerów handlowych i gospodarczych zjednoczonej
Europy. Konieczne są pogłębione prace studialne dotyczące mechanizmów
międzynarodowych, gdyż organy unijne będą się zajmować
w najbliższych latach dalszym rozwojem europejskiego jednolitego Rynku,
rywalizacją gospodarczą z USA i krajami azjatyckimi, liberalizacjąhandlu światowego."(...
Administracja, zarządzanie i handel zagraniczny w warunkach integracji. Materiały konferencyjne - Zarządzanie bezpieczeństwem
Ze wstępu: "Bezpieczeństwo jest w coraz wyższej cenie. Zajmują się nim naukowcy
oraz praktycy z różnych dziedzin. W najszerszym wymiarze
pojęcie „bezpieczeństwo” sprowadzić można do słów: stan niezagrożenia,
spokoju, pewności. Takie ogólne ujęcie problematyki bezpieczeństwa
sprawia, że różne podmioty podchodzą wybiórczo do tych bardzo
ważnych zarówno dla pojedynczych ludzi, jak i społeczeństwa zagadnień.
Inaczej postrzegają i interpretują bezpieczeństwo politycy, prawnicy,
ekonomiści, wojskowi, policjanci, lekarze, pedagodzy, a inaczej
zwyczajni ludzie. W ich ujęciu bezpieczeństwo to:
1) stan świadomości człowieka, w którym czuje się on wolny od
jakichkolwiek zagrożeń, pociągający za sobą poczucie spokoju i komfortu;
2) niczym niezakłócone współistnienie człowieka z innymi ludźmi
i środowiskiem przyrodniczym;
3) stan bez lęku i niepokoju o siebie i innych, pewność jutra;
4) brak zagrożenia utraty zdrowia, mienia i życia, komfort psychiczny
umożliwiający realizację życiowych marzeń i celów;
5) sytuacja, w której człowiekowi nic nie zagraża, a w nagłych
i nieprzewidzianych sytuacjach może liczyć na pomoc i wsparcie innych."(...
Global Impact of the COVID-19 Pandemic on Cerebral Venous Thrombosis and Mortality.
BACKGROUND AND PURPOSE: Recent studies suggested an increased incidence of cerebral venous thrombosis (CVT) during the coronavirus disease 2019 (COVID-19) pandemic. We evaluated the volume of CVT hospitalization and in-hospital mortality during the 1st year of the COVID-19 pandemic compared to the preceding year. METHODS: We conducted a cross-sectional retrospective study of 171 stroke centers from 49 countries. We recorded COVID-19 admission volumes, CVT hospitalization, and CVT in-hospital mortality from January 1, 2019, to May 31, 2021. CVT diagnoses were identified by International Classification of Disease-10 (ICD-10) codes or stroke databases. We additionally sought to compare the same metrics in the first 5 months of 2021 compared to the corresponding months in 2019 and 2020 (ClinicalTrials.gov Identifier: NCT04934020). RESULTS: There were 2,313 CVT admissions across the 1-year pre-pandemic (2019) and pandemic year (2020); no differences in CVT volume or CVT mortality were observed. During the first 5 months of 2021, there was an increase in CVT volumes compared to 2019 (27.5%; 95% confidence interval [CI], 24.2 to 32.0; P<0.0001) and 2020 (41.4%; 95% CI, 37.0 to 46.0; P<0.0001). A COVID-19 diagnosis was present in 7.6% (132/1,738) of CVT hospitalizations. CVT was present in 0.04% (103/292,080) of COVID-19 hospitalizations. During the first pandemic year, CVT mortality was higher in patients who were COVID positive compared to COVID negative patients (8/53 [15.0%] vs. 41/910 [4.5%], P=0.004). There was an increase in CVT mortality during the first 5 months of pandemic years 2020 and 2021 compared to the first 5 months of the pre-pandemic year 2019 (2019 vs. 2020: 2.26% vs. 4.74%, P=0.05; 2019 vs. 2021: 2.26% vs. 4.99%, P=0.03). In the first 5 months of 2021, there were 26 cases of vaccine-induced immune thrombotic thrombocytopenia (VITT), resulting in six deaths. CONCLUSIONS: During the 1st year of the COVID-19 pandemic, CVT hospitalization volume and CVT in-hospital mortality did not change compared to the prior year. COVID-19 diagnosis was associated with higher CVT in-hospital mortality. During the first 5 months of 2021, there was an increase in CVT hospitalization volume and increase in CVT-related mortality, partially attributable to VITT
The use of XML language for digital recording of construction
Zaprezentowano możliwości wykorzystania języka XML w procesie tworzenia cyfrowego formatu zapisu konstrukcji w obszarze inżynierii mechanicznej. Przedstawiona metodyka pozwala na efektywny udział inżynierów mechaników w tym procesie.The aim of this work was presenting opportunities of using XML Language in process of creating digital format of recording of construction in mechanical engineering. Presented methodology allows mechanical engineers to participate effectively in this process