Reuma.pt/vasculitis - the Portuguese vasculitis registry

BACKGROUND: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. RESULTS: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. CONCLUSIONS: Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.publishersversionpublishe

Perspectivas atuais sobre Síndrome dos Ovários Policísticos: abordagens diagnósticas e terapêuticas

Introduction: Polycystic ovarian syndrome (PCOS) is a complex endocrine condition characterized by ovarian dysfunction and androgen hypersecretion. Objective: To evaluate the pathophysiological bases associated with polycystic ovary syndrome, highlighting its multifactorial complexity, and to examine therapeutic strategies. Methodology: This is a bibliographic review that included original articles and systematic reviews in English and Portuguese, which addressed pathophysiological aspects and PCOS management, published between 2016 and 2024, selected from the PubMed, Scopus and SciELO databases. After careful selection, 18 articles were chosen to compose this bibliographic review. Results: PCOS is influenced by epigenetic mechanisms, exposure to toxic compounds, hyperinsulinemia and hyperandrogenism, which directly affect ovulation and metabolism. Obesity also contributes to PCOS. Treatment involves lifestyle changes and medications to control symptoms and restore ovulation. Considerations: PCOS is a complex condition, involving genetic, epigenetic, environmental and metabolic factors. Treatment aims to address underlying symptoms and mechanisms, with lifestyle changes and individualized pharmacological therapy. A multidisciplinary approach is essential to improve patients' quality of life.Introdução: A síndrome do ovário policístico (SOP) é uma condição endócrina complexa caracterizada por disfunção ovariana e hipersecreção de andrógenos. Objetivo: Avaliar as bases fisiopatológicas e tratamentos associados à síndrome do ovário policístico. Metodologia: Trata-se de uma revisão bibliográfica que incluiu artigos originais e revisões sistemáticas em inglês e português, que abordaram aspectos fisiopatológicos e manejo SOP, publicados entre 2016 e 2024, selecionados nas bases de dados PubMed, Scopus e SciELO. Após a seleção criteriosa, foram escolhidos 18 artigos para compor esta revisão bibliográfica. Resultados: A SOP é influenciada por mecanismos epigenéticos, exposição a compostos tóxicos, hiperinsulinemia e hiperandrogenismo, que afetam diretamente a ovulação e metabolismo. A obesidade também contribui para a SOP. O tratamento envolve mudanças no estilo de vida e medicamentos para controlar sintomas e restaurar ovulação. Considerações: A SOP é uma condição complexa, envolvendo fatores genéticos, epigenéticos, ambientais e metabólicos. O tratamento visa abordar sintomas e mecanismos subjacentes, com mudanças no estilo de vida e terapia farmacológica individualizada. Uma abordagem multidisciplinar é essencial para melhorar a qualidade de vida dos pacientes

Perspectivas atuais sobre síndrome de cushing: bases diagnósticas e terapêuticas

Introduction: Cushing's syndrome (CS) is a medical condition resulting from chronic and excessive exposure to high levels of cortisol, whether of endogenous or exogenous origin. Common symptoms include central obesity, redistribution of body fat, muscle weakness, high blood pressure, increased blood glucose, skin fragility and susceptibility to infections. Objective: To evaluate the diagnostic and therapeutic aspects of Cushing's syndrome. Methodology: This is a bibliographic review that included original articles and systematic reviews in English and Portuguese, which addressed the diagnosis and treatment of CS, published between 2012 and 2024, selected from the PubMed, Scopus and SciELO databases. After careful selection, 21 articles were chosen to compose this bibliographic review. Results: CS is a complete condition and can use several diagnostic methods to discover the condition and etiology, such as measuring nocturnal salivary cortisol, urinary free cortisol, low-dose dexamethasone suppression test and imaging tests. Treatment varies depending on the etiology and includes transsphenoidal surgery, surgical excision of the extrapituitary tumor, or adrenalectomy. Pharmacological measures can be applied, such as inhibitors of cortisol synthesis and ACTH secretion. Considerations: CS presents a wide range of clinical symptoms, and biochemical confirmation of hypercortisolism and measurement of ACTH is crucial, in addition to the use of imaging tests. Management aims to resolve the underlying cause, using surgical and pharmacological interventions, with the aim of promoting a better quality of life for patients.Introdução: A síndrome de Cushing (SC) é uma condição médica resultante da exposição crônica e excessiva aos níveis elevados de cortisol, seja de origem endógena ou exógena. Os sintomas comuns incluem obesidade central, redistribuição de gordura corporal, fraqueza muscular, hipertensão arterial, aumento da glicose sanguínea, fragilidade cutânea e suscetibilidade a infecções. Objetivo: Avaliar os aspectos diagnósticos e terapêuticos da síndrome de cushing. Metodologia: Trata-se de uma revisão bibliográfica que incluiu artigos originais e revisões sistemáticas em inglês e português, que abordaram os diagnóstico e tratamento da SC, publicados entre 2012 e 2024, selecionados nas bases de dados PubMed, Scopus e SciELO. Após a seleção criteriosa, foram escolhidos 21 artigos para compor esta revisão bibliográfica. Resultados: A SC é um quadro completo e pode utilizar de diversos métodos diagnósticos para descoberta do quadro e etiologia, como medição do cortisol salivar noturno, cortisol livre urinário, teste de supressão de dexametasona em baixa dose e exames de imagem. O tratamento varia a depender da etiologia e inclui cirugia transesfenoidal, excisão cirúrgica do tumor extra-hipofisário, ou adrenalectomia. Medidas farmacológicas podem ser aplicadas, como os inibidores da síntese de cortisol e de secreção de ACTH. Considerações: A SC apresenta uma ampla gama de sintomas clínicos, e a confirmação bioquímica do hipercortisolismo e a mensuração do ACTH é crucial, além da utilização de exames de imagem. O manejo visa resolver a causa subjacente, utilizando de intervenções cirúrgicas e farmacológicas, com intuito de promover melhor qualidade de vida aos pacientes

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear un derstanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5–7 vast areas of the tropics remain understudied.8–11 In the American tropics, Amazonia stands out as the world’s most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepre sented in biodiversity databases.13–15 To worsen this situation, human-induced modifications16,17 may elim inate pieces of the Amazon’s biodiversity puzzle before we can use them to understand how ecological com munities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple or ganism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region’s vulnerability to environmental change. 15%–18% of the most ne glected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lostinfo:eu-repo/semantics/publishedVersio

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear understanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5,6,7 vast areas of the tropics remain understudied.8,9,10,11 In the American tropics, Amazonia stands out as the world's most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepresented in biodiversity databases.13,14,15 To worsen this situation, human-induced modifications16,17 may eliminate pieces of the Amazon's biodiversity puzzle before we can use them to understand how ecological communities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple organism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region's vulnerability to environmental change. 15%–18% of the most neglected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lost

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear understanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5,6,7 vast areas of the tropics remain understudied.8,9,10,11 In the American tropics, Amazonia stands out as the world's most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepresented in biodiversity databases.13,14,15 To worsen this situation, human-induced modifications16,17 may eliminate pieces of the Amazon's biodiversity puzzle before we can use them to understand how ecological communities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple organism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region's vulnerability to environmental change. 15%–18% of the most neglected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lost

Catálogo Taxonômico da Fauna do Brasil: setting the baseline knowledge on the animal diversity in Brazil

The limited temporal completeness and taxonomic accuracy of species lists, made available in a traditional manner in scientific publications, has always represented a problem. These lists are invariably limited to a few taxonomic groups and do not represent up-to-date knowledge of all species and classifications. In this context, the Brazilian megadiverse fauna is no exception, and the Catálogo Taxonômico da Fauna do Brasil (CTFB) (http://fauna.jbrj.gov.br/), made public in 2015, represents a database on biodiversity anchored on a list of valid and expertly recognized scientific names of animals in Brazil. The CTFB is updated in near real time by a team of more than 800 specialists. By January 1, 2024, the CTFB compiled 133,691 nominal species, with 125,138 that were considered valid. Most of the valid species were arthropods (82.3%, with more than 102,000 species) and chordates (7.69%, with over 11,000 species). These taxa were followed by a cluster composed of Mollusca (3,567 species), Platyhelminthes (2,292 species), Annelida (1,833 species), and Nematoda (1,447 species). All remaining groups had less than 1,000 species reported in Brazil, with Cnidaria (831 species), Porifera (628 species), Rotifera (606 species), and Bryozoa (520 species) representing those with more than 500 species. Analysis of the CTFB database can facilitate and direct efforts towards the discovery of new species in Brazil, but it is also fundamental in providing the best available list of valid nominal species to users, including those in science, health, conservation efforts, and any initiative involving animals. The importance of the CTFB is evidenced by the elevated number of citations in the scientific literature in diverse areas of biology, law, anthropology, education, forensic science, and veterinary science, among others

Characterisation of microbial attack on archaeological bone

As part of an EU funded project to investigate the factors influencing bone preservation in the archaeological record, more than 250 bones from 41 archaeological sites in five countries spanning four climatic regions were studied for diagenetic alteration. Sites were selected to cover a range of environmental conditions and archaeological contexts. Microscopic and physical (mercury intrusion porosimetry) analyses of these bones revealed that the majority (68%) had suffered microbial attack. Furthermore, significant differences were found between animal and human bone in both the state of preservation and the type of microbial attack present. These differences in preservation might result from differences in early taphonomy of the bones. © 2003 Elsevier Science Ltd. All rights reserved

Núcleos de Ensino da Unesp: artigos 2008

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq