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1.
Rehabil Psychol ; 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38647452

RESUMO

PURPOSE/OBJECTIVE: To investigate the occurrence of behavioral problems 7 years after severe pediatric traumatic brain injury (TBI), and their evolution from 3 months to 7 years postinjury. METHOD/DESIGN: Thirty-four participants, 38% girls, M (SD) age at injury 7.6 (4.7) years, age at assessment 15 (4.6) years, underwent comprehensive assessments 7 years after severe TBI from March 2014 to March 2016 and were matched to a control group by age, gender, and parental education. A subgroup of 20 participants had available behavioral assessments at 3, 12, and 24 months postinjury. Internalizing, externalizing, and total behavioral problems were assessed with self- and parent reports of the Achenbach's Behavioral Checklist. Additional data included sociodemographic background, initial injury severity, and specific outcomes assessed concurrently 7 years postinjury. RESULTS: Compared to controls: (a) a significant proportion of participants with severe TBI fell above the clinical cutoff for self- (42%) and parent-reported (36%) externalizing problems, but not for self- (33%) or parent-reported (45%) internalizing problems; (b) withdrawn/depressed, intrusive behavior, and somatic complaints were significantly higher in self-reports; and (c) rule-breaking behavior, attention, and social problems were significantly higher in parent reports. Parent-reported internalizing problems were associated with older age at injury, whereas externalizing problems correlated with greater injury severity and concurrent levels of greater overall disability, lower intellectual ability, and poorer family functioning. In multiple hierarchical regression analyses, overall disability and worse family functioning significantly predicted externalizing problems. Parent-reported internalizing and externalizing problems persisted over time. CONCLUSIONS/IMPLICATIONS: These results highlight the importance of long-term follow-up and individualized behavioral interventions for children who sustained severe TBI. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

2.
J Bodyw Mov Ther ; 37: 360-365, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38432829

RESUMO

INTRODUCTION: Effects on strength performance and muscle activation in the contralateral limb have been observed after training with the ipsilateral limb (IL). Cross fatigue effects in the contralateral limb (CL) can occur at intervals of up to 48 h after a training session performed with the ipsilateral limb. The objective of this study was to verify the effect of a training session in the ipsilateral limb, on the strength and activation of the muscles in the contralateral limb also submitted to the training protocol. METHODS: 10 untrained men (mean ± SD: age = 23.7 ± 4.9 years) performed unilateral knee extension with both limbs in equated protocols, in two conditions with different intervals between limbs - 20 min and 24 h. RESULTS: There were no differences in the comparison of the force produced between the pre x post interventions with the CL limb, as well as in the activation of the quadriceps during its performance. These results were similar for the two different intervals between the protocols. CONCLUSION: It was concluded that when the CL member performs the protocol after the IL, the responses in the CL depend mainly on the requirement imposed on the protocol performed by this member, without influence of the training protocol performed previously with the IL member.


Assuntos
Exercício Físico , Fadiga , Masculino , Humanos , Adolescente , Adulto Jovem , Adulto , Articulação do Joelho , Músculo Quadríceps , Descanso
3.
Brain Inj ; 38(6): 467-478, 2024 May 11.
Artigo em Inglês | MEDLINE | ID: mdl-38379310

RESUMO

OBJECTIVE: To investigate reported burden by the Primary Family Caregiver (PFC) 7-years after severe pediatric traumatic brain injury in the TGE (Traumatisme Grave de l'Enfant) longitudinal study. METHODS: Subjective burden was estimated with the Zarit Burden Inventory (ZBI) in 36 PFC (parents), who rated their own health status (Medical Outcome Study Short Form-12), family functioning and their child's level of care and needs (Pediatric/Adult Care And Needs Scale [PCANS/CANS]). Data collection included: child and PFC sociodemographic characteristics, injury-related factors, 'objective' (e.g. overall level of disability: Glasgow Outcome Scale - Extended, GOS-E/GOS-E-Peds) and 'subjective' outcomes (e.g. participation, behavior, executive functions, quality of life and fatigue). RESULTS: 25% of PFC reported mild-moderate burden, and 19% moderate-severe burden. Higher burden correlated with worse outcomes in all 'subjective' PFC-rated outcomes, and with self-reported participation. The ZBI correlated strongly with CANS/PCANS and GOS-E/GOS-E-Peds. Overall level of disability and PFC-reported executive functioning explained 62% of the ZBI variance. For equal levels of disability, burden was higher when PFC reported a 'negative' picture of their child. CONCLUSION: Significant PFC-reported burden 7-years post-injury was associated with overall disability and 'subjective' PFC-rated outcomes. Factors influencing parental burden in the long term should be identified and psychological support implemented over time.


Assuntos
Lesões Encefálicas Traumáticas , Lesões Encefálicas , Adulto , Humanos , Criança , Estudos Longitudinais , Qualidade de Vida/psicologia , Cuidadores/psicologia , Lesões Encefálicas Traumáticas/complicações , Lesões Encefálicas/complicações
4.
Rev Port Cardiol ; 2024 Feb 08.
Artigo em Inglês, Português | MEDLINE | ID: mdl-38336222

RESUMO

INTRODUCTION AND OBJECTIVES: Concerns regarding the consequences of ionizing radiation (IR) have been increasing in the field of interventional cardiology (IC). There is little information on reported national and local radiation diagnostic reference levels (DRLs) in catheterization laboratories in Portugal. This study was designed to assess the IR dose exposure during complex percutaneous coronary intervention (PCI), and to set the respective DRLs and future achievable doses (ADs). METHODS: This was a retrospective cohort study which took place between 2019 and 2020, including patients who underwent complex PCI. Complex PCI was defined as all procedures that encompass treatment of chronic total occlusions (CTO) or left main coronary artery. DRLs were defined as the 75th percentile of the distribution of the median values of air kerma area product (PKA) and cumulative air kerma (Ka,r). ADs were set at the 50th percentile of the study dose distribution. Multivariate analysis was performed using linear regression to identify predictors significantly associated with radiation dose (Ka,r). RESULTS: A total of 242 patients were included in the analysis. Most patients underwent a CTO procedure (146, 60.3%). Patients were aged 67.9±11.2 years and mostly male (81.4%). DRLs were set in Ka,r (3012 mGy) and PKA (162 Gy cm2) for complex PCI. ADs were also set in Ka,r (1917 mGy) and PKA (101 Gy cm2). Independent predictors of Ka,r with a positive correlation were PKA (0.893, p<0.001), fluoroscopy time (0.520, p<0.001) and PCI time (0.521, p<0.001). CONCLUSIONS: This study reports the results of IR in complex PCI. DRLs were set for IR dose exposure measured in Ka,r (3012 mGy) and PKA (162 Gy cm2). ADs, values to be achieved in future assessment, were set to Ka,r (1917 mGy) and PKA (101 Gy cm2).

5.
Rev Port Cardiol ; 43(3): 131-138, 2024 Mar.
Artigo em Inglês, Português | MEDLINE | ID: mdl-38244775

RESUMO

INTRODUCTION AND OBJECTIVES: Coronary chronic total occlusions (CTOs) of the right coronary artery (RCA) are a relatively common finding in the context of coronary angiography. However, the benefit of revascularization remains controversial. METHODS: A single-center retrospective cohort analysis prospectively collected outcomes of CTO patients undergoing percutaneous coronary intervention (PCI) in 2019 and 2020. Patients were divided into two groups according to the CTO vessel treated (left coronary artery [LCA]-CTO or RCA-CTO). The primary outcome was defined as the recurrence of angina and/or heart failure (HF) symptoms and secondary outcomes were myocardial infarction (MI) and all-cause mortality. RESULTS: A total of 177 patients (82.5% male) were included in the analysis, with a mean age of 65±11 years. The primary outcome occurred in 28 (16.6%) patients and was significantly more frequent in RCA-CTO patients (19, 24.7%, p=0.010) in a mean follow-up of 18 months. This was mainly driven by recurrence of HF symptoms (12, 15.6%, p=0.013). Treated RCA-CTO was an independent predictor of the primary outcome (p=0.019, HR 2.66, 95% CI 1.17-6.05). MI and mortality rates were no different between groups (RCA-CTO with 1.3%, p=0.361 and 2.6%, p=0.673, respectively, on survival analysis). Left ventricular ejection fraction was an independent predictor of mortality (p=0.041, HR 0.93, 95% CI 0.87-0.99). CONCLUSIONS: Revascularization of CTO lesions by PCI was associated with low rates of symptom recurrence, and clinical outcomes showed no differences regardless of which artery was treated. Recanalization of RCA-CTO was less beneficial in reducing the recurrence of HF symptoms.


Assuntos
Oclusão Coronária , Insuficiência Cardíaca , Infarto do Miocárdio , Intervenção Coronária Percutânea , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Feminino , Vasos Coronários , Oclusão Coronária/cirurgia , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda
6.
Materials (Basel) ; 17(2)2024 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-38255591

RESUMO

This work aims to obtain recycled carbon fibre and develop an application for this new material. The carbon fibres were obtained by recycling aerospace prepreg waste via the pyrolysis process. The recycled fibres were combined with an Araldite LH5052/Aradur LY5053 epoxy resin/hardener system using manual lay-up and vacuum bagging processes. For comparison, the same resin/hardener system was used to produce a composite using commercial carbon fibre. The recycled and commercial composites were subjected to flexural, tensile and Mode I testing. Fracture aspects were analysed via scanning electron microscopy (SEM). The pyrolysis process did not affect the fibre surface as no degradation was observed. The fracture aspect showed a mixture of failure in the recycled composite laminate and interlaminar/translaminar failure near the surface of the commercial composite caused by flexural stress. Flexural and tensile tests showed a loss of mechanical strength due to the recycling process, but the tensile values were twice as high. The sand ladder platform was the project chosen for the development of a product made with recycled carbon fibres. The product was manufactured using the same manufacturing process as the specimens and tested with a 1243 kg car. The method chosen to design, manufacture and test the prototype sand ladder platform made of recycled carbon fibre was appropriate and gave satisfactory results in terms of high mechanical strength to bending and ease of use.

7.
Rev Port Cardiol ; 43(4): 167-174, 2024 Apr.
Artigo em Inglês, Português | MEDLINE | ID: mdl-38141872

RESUMO

INTRODUCTION AND OBJECTIVES: Coronary chronic total occlusions (CTOs) are relatively common findings in patients with type 2 diabetes mellitus (T2DM). However, the indication for percutaneous coronary intervention (PCI) and its clinical benefit in these patients remain controversial. METHODS: A single-center retrospective cohort study with prospectively collected outcomes was carried out with CTO patients undergoing PCI in 2019 and 2020. Patients were divided into two groups according to previous T2DM diagnosis (T2DM and non-T2DM). The primary outcome was recurrence of angina and/or heart failure symptoms and secondary outcomes were myocardial infarction and all-cause mortality. RESULTS: A total of 177 patients (82.5% male) were included in the analysis, with a mean age of 65±11 years. The primary outcome (total symptom recurrence) occurred in 16.6% of the sample, with no difference between groups (non-T2DM 13.6% vs. T2DM 21.2%, p=0.194) in a two-year follow-up. Angina recurrence was significantly more frequent in T2DM patients (15.2%, p=0.043). The presence of T2DM was not an independent predictor of symptom recurrence (p=0.429, HR 1.37, 95% CI 0.62-2.98). Myocardial infarction and all-cause mortality were also not different between groups (T2DM 1.5%, p=0.786 and 4.5%, p=0.352, respectively, on survival analysis). Independent predictors of all-cause mortality were left ventricular function and creatine clearance (p=0.039, HR 0.92, 95% CI 0.85-0.99 and p=0.013, HR 0.96, 95% CI 0.93-0.99, respectively). CONCLUSIONS: T2DM did not influence outcomes in CTO patients undergoing PCI, and its presence should not be a limiting factor in deciding on CTO revascularization.


Assuntos
Oclusão Coronária , Diabetes Mellitus Tipo 2 , Infarto do Miocárdio , Intervenção Coronária Percutânea , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Feminino , Intervenção Coronária Percutânea/efeitos adversos , Oclusão Coronária/cirurgia , Oclusão Coronária/complicações , Oclusão Coronária/diagnóstico , Diabetes Mellitus Tipo 2/complicações , Estudos Retrospectivos , Doença Crônica , Infarto do Miocárdio/etiologia , Resultado do Tratamento , Fatores de Risco
8.
Opt Express ; 31(24): 40041-40055, 2023 Nov 20.
Artigo em Inglês | MEDLINE | ID: mdl-38041314

RESUMO

The pursuit of high-power solar laser systems with high efficiency and capacity for large tracking error compensation is determinant for the applicability of this renewable technology. A side-pumped dual-rod Ce:Nd:YAG solar laser was developed and tested at the focus of a 2 m diameter parabolic concentrator. Maximum continuous-wave total solar laser power of 58 W was measured. To the best of our knowledge, this is the highest laser power from a Ce:NdYAG solar laser. Moreover, wide tracking error compensation width of 5.1° in the azimuthal direction was reached, being 4.25 times higher than the previous measurement without solar tracking assistance.

9.
Rev Port Cardiol ; 2023 Nov 10.
Artigo em Inglês, Português | MEDLINE | ID: mdl-37952927

RESUMO

INTRODUCTION AND OBJECTIVES: Concerns surrounding the consequences of ionizing radiation (IR) have increased in interventional cardiology (IC). Despite this, the ever-growing complexity of diseases as well as procedures can lead to greater exposure to radiation. The aim of this survey, led by Portuguese Association of Interventional Cardiology (APIC), was to evaluate the level of awareness and current practices on IR protection among its members. METHODS: An online survey was emailed to all APIC members, between August and November 2021. The questionnaire consisted of 50 questions focusing on knowledge and measures of IR protection in the catheterization laboratory. Results were analyzed using descriptive statistics. RESULTS: From a response rate of 46.9%, the study obtained a total sample of 159 responses (156 selected for analysis). Most survey respondents (66.0%) were unaware of the radiation exposure category, and only 60.4% reported systematically using a dosimeter. A large majority (90.4%) employed techniques to minimize exposure to radiation. All participants used personal protective equipment, despite eyewear protection only being used frequently by 49.2% of main operators. Ceiling suspended shields and table protectors were often used. Only two-thirds were familiar with the legally established limit on radiation doses for workers or the dose that should trigger patient follow-up. Most of the survey respondents had a non-certified training in IR procedures and only 32.0% had attended their yearly occupational health consultation. CONCLUSIONS: Safety methods and protective equipment are largely adopted among interventional cardiologists, who have shown some IR awareness. Despite this, there is room for improvement, especially concerning the use of eyewear protection, monitoring, and certification.

10.
Hellenic J Cardiol ; 2023 Nov 11.
Artigo em Inglês | MEDLINE | ID: mdl-37956770

RESUMO

BACKGROUND: Pretreatment antithrombotic strategies in non-ST elevation acute coronary syndromes (NSTE-ACS) during hospitalization is still a matter of contention within the cardiology community. Our aim was to analyze in-hospital and one-year follow-up outcomes of patients with NSTE-ACS pretreated with dual antiplatelet therapy (DAPT) versus single antiplatelet therapy (SAPT). METHODS: A retrospective study was carried out with NSTE-ACS patients planned to undergo an invasive strategy and were included in the Portuguese Registry of ACS between 2018-2021. A composite primary outcome (in-hospital re-infarction, stroke, heart failure, hemorrhage, death) was compared regarding antiplatelet strategy (DAPT versus SAPT). Secondary outcomes were defined as one-year all-cause mortality and one-year cardiovascular rehospitalization. RESULTS: A total of 1469 patients were included, with a mean age of 66±12 years and 73.9% were male. DAPT regime was used in 38.2% and SAPT in 61.8% of patients. NSTE myocardial infarction was the most frequent presentation (88.5%). Revascularization after 24h occurred in 44.8% patients (63% of these after 48h). Enoxaparin was the anticoagulant more frequently used (45.1%). The primary outcome was more frequently observed in the SAPT group (10.4%, p=0.033), mainly driven by more ischemic events. Time until revascularization > 48h and SAPT regime were independent predictors of the primary outcome (OR 1.66, p=0.036 and OR 2.03, p=0.008, respectively). CONCLUSION: NSTE-ACS patients pretreated with SAPT had worse in-hospital outcomes. This difference can be probably explained by a delay in time until revascularization. Pretreatment DAPT strategy and crossover between heparins is still frequently used in clinical practice.

11.
Ecol Evol ; 13(10): e10624, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37869432

RESUMO

Agricultural commodity production is one the main drivers of deforestation in Legal Brazilian Amazonia resulting in a deforested and/or fragmented landscape formed by forest remnants of different sizes and shape embedded within the agricultural matrix. As an ecosystem engineer and a crucial seed predator, white-lipped peccaries (Tayassu pecari) play a pivotal role in forest structure, biodiversity, and nutrient cycling. However, they are highly sensitive to habitat fragmentation and hunting pressure. White-lipped peccaries are, therefore, a wide-ranging "landscape species," the spatial and ecological requirements of which can be used to guide conservation planning in human-modified landscapes. Using data from GPS-tracked individuals in large-scale mechanized agriculture landscapes in the state of Mato Grosso, Brazil's largest soybean and maize producer, we investiated the home range size and resource selection during both the crop and non-crop season. We observed a seasonal variation in home range size and an increased selection for croplands during the crop season. White-lipped peccaries favored native vegetation patches and also exhibited avoidance of locations distant from perennial water bodies and distant cropland locations far from forest remmants. This study can contribute to inform effective conservation strategies and land management practices aimed at preserving suitable habitats and promoting wildlife coexistence with working agricultural landscapes.

12.
J Sports Med Phys Fitness ; 63(11): 1194-1201, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37675501

RESUMO

BACKGROUND: Magnetic resonance imaging (MRI) is a well-used technique to assess muscle size and can be acquired on different planes. Pectoralis major (PM) and triceps brachii (TB) muscles are often acquired and analyzed on the axial plane, however it is unknown if anatomical cross-sectional area (CSA) calculated from different planes will affect the muscle size-strength relationship. Thus, the first aim of the present study was to identify if the CSA of the PM and TB measured on different planes presents a similar muscle size-strength relationship. A secondary aim was to investigate if the quantification of CSA of the PM and TB muscles are similar between sagittal and axial plane. METHODS: Fifteen males underwent an MRI examination, and after that, one-repetition maximum (1RM) test was performed. RESULTS: There was a significant relationship between 1RM and PM CSA measured on the axial and sagittal plane (r≤0.81), while the relationship with TB CSA was only good on the axial plane (r=0.65) and not significant on the sagittal plane (r=0.27). ICC between planes was excellent for PM CSA (0.96) with Bland-Altman procedure showing agreement between planes (d=0.376; P=0.612). Contrarily, TB CSA ICC was week (0.07), with Bland-Altman procedure showing no agreement between planes (d=-24.49; P=0.022). CONCLUSIONS: CSA measured at axial plane from PM and TB muscles showed a significant relationship with 1RM, while only PM CSA on the sagittal plane showed a significant relationship with 1RM. Finally, it was demonstrated that PM images showed a great reliability between planes, which was not true for TB muscle.


Assuntos
Músculo Esquelético , Músculos Peitorais , Masculino , Humanos , Músculos Peitorais/diagnóstico por imagem , Músculos Peitorais/fisiologia , Reprodutibilidade dos Testes , Músculo Esquelético/fisiologia , Braço , Força Muscular/fisiologia , Imageamento por Ressonância Magnética
13.
Conserv Biol ; 2023 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-37650432

RESUMO

Protected and Conserved Areas (PCAs) are key ecosystem management tools for conserving biodiversity and sustaining ecosystem services and social co-benefits. As countries adopt a 30% target for protection of land and sea under the Global Biodiversity Framework of the United Nations Convention on Biological Diversity, a critical question emerging is, "which 30%?". One approach to an answer is risk-based: we should protect the 30% that returns the greatest reductions in risks of species extinction and ecosystem collapse. IUCN Red List protocols provide practical methods for assessing these risks. All species, including humans, depend on the integrity of ecosystems for their well-being and survival. Africa is strategically important for ecosystem management due to convergence of high ecosystem diversity, intense pressures and high levels of human dependency on nature. We reviewed the outcomes of a symposium at the inaugural African Protected Areas Congress convened to discuss applications of the IUCN Red List of Ecosystems to the design and management of PCAs. We found significant recent progress in red listing, with 920 ecosystem types assessed across 21 countries. While these span a diversity of environments across the continent, the greatest thematic gaps are in freshwater, marine and subterranean realms and large geographic gaps exist in north Africa and parts of west and east Africa. The projects are implemented by a diverse community of government agencies, NGOs and researchers. Already, they are having impact on policy and management, informing extensions to, and management of formal Protected Area networks, supporting decision making for sustainable development and informing ecosystem conservation and threat abatement, both within boundaries of PCAs and in surrounding landscapes and seascapes. We recommend further integration of risk assessments into environmental policy and enhanced investment in ecosystem red listing to fill current gaps. This article is protected by copyright. All rights reserved.

14.
Mol Ecol Resour ; 23(7): 1641-1655, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37464467

RESUMO

The coastline of Sub-Saharan Africa hosts highly diverse fish communities of great conservation value, which are also key resources for local livelihoods. However, many costal ecosystems are threatened by overexploitation and their conservation state is frequently unknown due to their vast spatial extent and limited monitoring budgets. Here, we evaluated the potential of citizen science-based eDNA surveys to alleviate such chronic data deficiencies and assessed fish communities in Mozambique using two 12S metabarcoding primer sets. Samples were either collected by scientific personnel or trained community members and results from the two metabarcoding primers were combined using a new data merging approach. Irrespective of the background of sampling personnel, a high average fish species richness was recorded (38 ± 20 OTUs per sample). Individual sections of the coastline largely differed in the occurrence of threatened and commercially important species, highlighting the need for regionally differentiated management strategies. A detailed comparison of the two applied primer sets revealed an important trade-off in primer choice with MiFish primers amplifying a higher number of species but Riaz primers performing better in the detection of threatened fish species. This trade-off could be partly resolved by applying our new data-merging approach, which was especially designed to increase the robustness of multiprimer assessments in regions with poor reference libraries. Overall, our study provides encouraging results but also highlights that eDNA-based monitoring will require further improvements of, for example, reference databases and local analytical infrastructure to facilitate routine applications in Sub-Saharan Africa.


Assuntos
Ciência do Cidadão , DNA Ambiental , Animais , Ecossistema , Código de Barras de DNA Taxonômico/métodos , Biodiversidade , Monitoramento Ambiental/métodos , Peixes/genética , Espécies em Perigo de Extinção
15.
Appl Opt ; 62(10): 2697-2706, 2023 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-37132823

RESUMO

Solar laser technology typically requires a highly accurate solar tracking system that operates continuously, which increases energy consumption and reduces the system's lifetime. We propose a multi-rod solar laser pumping approach to enhance solar laser stability under non-continuous solar tracking conditions. Using a heliostat, solar radiation is redirected toward a first-stage parabolic concentrator. At its focus, an aspheric lens further concentrates the solar rays onto five Nd:YAG rods positioned within an elliptical-shaped pump cavity. Numerical analysis using Zemax and LASCAD software showed that the tracking error width at 10% laser power loss for the five 6.5 mm diameter and 15 mm length rods was 2.20°, which is 50% higher than that of the solar laser in previous non-continuous solar tracking experiments. 2.0% solar-to-laser conversion efficiency was also attained.

16.
Future Cardiol ; 19(1): 39-43, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36695159

RESUMO

Fabry disease is a rare lysosomal storage disorder caused by mutations in the GLA gene, resulting in reduced or absent α-Gal A activity. Migalastat is an oral chaperone therapy for Fabry patients with amenable GLA variants. We previously reported a case of a 60-year-old male patient with a classic phenotype of Fabry disease, presenting with two GLA variants: p.R356Q and p.G360R. Herein, we report that, although these two missense variants are individually classified as amenable to migalastat in the validated in vitro human embryonic kidney-293 cell-based assay, their combination precludes the patient to be treated with this oral chaperone. This case illustrates how therapeutic decisions may be challenging and how a good genotypic characterization of Fabry patients is critical for the selection of the correct therapeutic strategy.


Fabry disease is a rare genetic disease that is part of a group of conditions called lysosomal storage diseases. It is characterized by an abnormal accumulation of glycosphingolipids, a subclass of glycolipids which are important components of the body's cell membranes. This accumulation is caused by a reduction in, or absence of, enzyme α-Gal A activity, which normally breaks glycosphingolipids down into smaller units, avoiding their accumulation. The absence or reduction in the α-Gal A enzyme activity is caused by mutations (changes in the normal DNA sequence) in the GLA gene. Migalastat is an oral treatment for Fabry patients with GLA mutations that respond to this treatment. We report a case of a 60-year-old male patient with Fabry disease, presenting with two GLA mutations (p.R356Q and p.G360R). Although these mutations are individually amenable to migalastat, their combination and interaction in the same chromosome precludes response to this treatment. This case illustrates how therapeutic decisions for treating Fabry disease can be challenging depending on the mutations causing the disease and how genetic material is decisive for therapy selection.


Assuntos
Doença de Fabry , Masculino , Humanos , Pessoa de Meia-Idade , alfa-Galactosidase/uso terapêutico , 1-Desoxinojirimicina/efeitos adversos , Mutação
17.
Omega (Westport) ; 86(3): 769-787, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33530891

RESUMO

This study examined the psychometric properties of a Brazilian adapted version of the Coronavirus Anxiety Scale (CAS-BR) in a sample of adults in Brazil. Confirmatory factor analyses demonstrated that the CAS-BR produces a reliable (α = .84), unidimensional construct whose structure was shown to be invariant across gender, race, and age. However, some items of the CAS-BR were stronger indicators of the coronavirus anxiety construct for women and younger adults. Although the CAS-BR demonstrated evidence of discrimination ability for functional impairment (AUC = .77), Youden indexes were low to identify a clinical cut-score. Construct validity was demonstrated with correlations between CAS-BR scores and measures of functional impairment, generalized anxiety, and depression. Exploratory analyses revealed that CAS-BR total scores were higher among women and participants with a history of anxiety disorder. These findings are consistent with previous investigations and support the validity of CAS-BR for measuring coronavirus anxiety with Brazilian adults.


Assuntos
Coronavirus , Adulto , Humanos , Feminino , Brasil , Psicometria , Ansiedade/diagnóstico , Transtornos de Ansiedade/diagnóstico , Reprodutibilidade dos Testes , Inquéritos e Questionários
18.
Sci Total Environ ; 857(Pt 3): 159589, 2023 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-36270379

RESUMO

Unprecedented human induced changes to the climate system have already contributed to a variety of observed impacts to both ecosystems and populations. Decision-makers demand impact assessments at the regional-to-local scale to be able to plan and define effective climate action measures. Integrated socio-ecological assessments that properly consider system uncertainties require the use of prospective scenarios that project potential climate impacts, while accounting for sectoral exposure and adaptive capacity. Here we provide an integrated assessment of climate change to the whale watching sector by: 1) extending the European Shared Socio-economic Pathways (Eur-SSPs) and developing four whale watching SSP narratives (WW-SSPs) and 2) characterize each key element comprised in the WW-SSPs for the time period 2025-2055. We applied this approach in a case study for the Macaronesia region where we developed scenarios which integrate the socio-economic (WW-SSPs), climate (RCPs) and ecological (species' thermal suitability responses) dimensions of whale watching. These scenarios were used by local stakeholders to identify the level of preparedness of the whale watching sector. When confronted with scenarios that combine this ecological dimension with projected climate changes and the four different socioeconomic narratives, stakeholders assessed the whale watching sector in Macaronesia as being somewhat prepared for a Sustainable World and a Fossil Fuel Development World, but somewhat unprepared for a Rivalry World. No consensus was reached regarding the sector's preparedness level under an Inequality World scenario. Our study demonstrates the importance of considering multiple dimensions when assessing the potential challenges posed by climate change and provides a needed resource to help the whale watching sector in Macaronesia, and elsewhere, in its effort to devise efficient climate action policies and strategies.


Assuntos
Ecossistema , Baleias , Animais , Humanos , Estudos Prospectivos , Mudança Climática , Fatores Socioeconômicos
19.
J Electromyogr Kinesiol ; 68: 102724, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36399915

RESUMO

The first aim was to investigate the impact of different electromyography (EMG) parameters as a reference to normalize the EMG amplitude of the superficial quadriceps femoris muscles across different sets of a knee extension exercise. The second aim is to examine the reliability between days of the EMG parameters used as a reference. Eleven young males attended the laboratory on 4 different days and performed one repetition maximum test, maximumvoluntary isometric contractions, and a resistance training protocol until failure. Surface EMG was placed over the rectus femoris, vastus lateralis, and vastus medialis muscles. Seven EMG parameters were calculated from the tasks and used to normalize EMG amplitude measured during the resistance training protocol. A repeated-measures two-way ANOVA was used (normalized EMG amplitude × set) to compare normalized EMG across sets, while an intraclass correlation coefficient, coefficient of variation, and Bland-Altman plots were used to calculate the intra-day reliability of the EMG parameters. The present investigation showed that normalized EMG amplitude of the superficial muscles of the quadriceps measured during a knee extension exercise is influenced by the EMG parameter and depends on the muscle. While rectus femoris and vastus lateralis normalized EMG amplitude presented one parameter among seven showing similar value to the other parameters, VM showed two. Lastly, all EMG parameters for all muscles presented an overall excellent reliability and agreement between days.


Assuntos
Músculo Esquelético , Treinamento de Força , Masculino , Humanos , Eletromiografia/métodos , Músculo Esquelético/fisiologia , Reprodutibilidade dos Testes , Músculo Quadríceps/fisiologia
20.
Sci Total Environ ; 860: 160376, 2023 Feb 20.
Artigo em Inglês | MEDLINE | ID: mdl-36423844

RESUMO

Over the last decades, global warming has contributed to changes in marine species composition, abundance and distribution, in response to changes in oceanographic conditions such as temperature, acidification, and deoxygenation. Experimentally derived thermal limits, which are known to be related to observed latitudinal ranges, have been used to assess variations in species distribution patterns. However, such experiments cannot be undertaken on free-swimming large marine predators with wide-range distribution, like cetaceans. An alternative approach is to elicit expert's knowledge to derive species' thermal suitability and assess their thermal responses, something that has never been tested in these taxa. We developed and applied a methodology based on expert-derived thermal suitability curves and projected future responses for several species under different climate scenarios. We tested this approach with ten cetacean species currently present in the biogeographic area of Macaronesia (North Atlantic) under Representative Concentration Pathways 2.6, 4.5 and 8.5, until 2050. Overall, increases in annual thermal suitability were found for Balaenoptera edeni, Globicephala macrorhynchus, Mesoplodon densirostris, Physeter macrocephalus, Stenella frontalis, Tursiops truncatus and Ziphius cavirostris. Conversely, our results indicated a decline in thermal suitability for B. physalus, Delphinus delphis, and Grampus griseus. Our study reveals potential responses in cetaceans' thermal suitability, and potentially in other highly mobile and large predators, and it tests this method's applicability, which is a novel application for this purpose and group of species. It aims to be a cost-efficient tool to support conservation managers and practitioners.


Assuntos
Balaenoptera , Golfinho Nariz-de-Garrafa , Stenella , Baleias Piloto , Animais , Ecossistema , Clima , Cachalote , Aquecimento Global , Mudança Climática
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