Defeitos da β-Oxidação Mitocondrial: Um Caso Fatal de Deficiência em 3-Hidroxi-Acil-CoA Desidrogenase de Cadeia Longa. (LCHAD)

Descreve-se um caso fatal de deficiência em 3-hidroxi-acil-CoA desidrogenase de cadeia longa (LCHAD) diagnosticado numa criança de 8 meses de idade, segunda filha de um casal jovem, saudável e consanguíneo. Os primeiros sintomas manifestaram-se aos 6,5 meses de idade ao entrar em coma hipoglicémico não cetótico do qual recupera após infusão i.v. de glucose. Detectou-se hepatomegália e posteriormente cardiomegália. A presença de acidúria 3-hidroxi-dicarboxílica foi confirmada após teste de jejum prolongado de 13th e o estudo enzimático efectuado em cultura de fibroblastos de biópsia de pele confirmou o déficit em LCHAD

Streptococcus pneumoniae Vaccination in Children and Adolescents at High Risk of Invasive Pneumococcal Disease

Introdução: Em Portugal, a vacinação anti-pneumocócica é gratuita e recomendada pela Direção-Geral da Saúde na população pediátrica de alto risco para doença invasiva pneumocócica. O objetivo deste estudo foi analisar o cumprimento vacinal numa população pediátrica seguida em consulta hospitalar. Material e Métodos: Estudo observacional transversal, em crianças com diagnóstico de alto risco de doença invasiva pneumocócica e consulta num hospital nível três, entre julho e dezembro de 2014. Os dados foram obtidos através do processo clínico, Boletim Individual de Saúde e Plataforma de Dados da Saúde®. Resultados: Dos 122 participantes, 95,9% realizaram, pelo menos, uma dose de vacina mas, destes, só 64,8% efetuaram o esquema completo. O cumprimento do esquema vacinal foi melhor nos de idade inferior a cinco anos (p < 0,01). A proporção de crianças com esquema completo foi de: 100% nas hemoglobinopatias, 100% nas infeções por vírus da imunodeficiência humana, 66,7% nos prematuros com idade gestacional ≤ 28 semanas, 62,5% nos esplenectomizados e 54,7% na síndrome de Down. As crianças têm mais esquemas completos quando são seguidas em consulta de Infeciologia (100%) e de Pneumologia pediátricas (88,2%). O grupo com idade superior a cinco anos está mais vacinado com a vacina polissacarida 23-valente do que o dos 2-5 anos (74,5% vs 40,5%; p < 0,01). Discussão: A maioria da nossa população de alto risco para doença invasiva pneumocócica efetuou vacinação anti-pneumocócica, mas apenas dois terços completaram o esquema recomendado, sendo a maior falha na administração da vacina polissacarida 23-valente. Conclusões: Embora estes resultados sejam melhores do que em países europeus com recomendações semelhantes, é necessário explorar as causas das falhas observadas para otimizar a vacinação

Efficient extraction of the RuBisCO enzyme from spinach leaves using aqueous solutions of biocompatible ionic liquids

Ribulose-1,5-biphosphate carboxylase/oxygenase (RuBisCO) is the most abundant protein on the planet, being present in plants, algae and various species of bacteria, with application in the pharmaceutical, chemical, cosmetic and food industries. However, current extraction methods of RuBisCO do not allow high yields of extraction. Therefore, the development of an efficient and selective RuBisCOs’ extraction method is required. In this work, aqueous solutions of biocompatible ionic liquids (ILs), i.e., ILs derived from choline and analogues of glycine-betaine, were applied in the RuBisCO’s extraction from spinach leaves. Three commercial imidazolium-based ILs were also investigated for comparison purposes. To optimize RuBisCO’s extraction conditions, response surface methodology was applied. Under optimum extraction conditions, extraction yields of 10.92 and 10.57 mg of RuBisCO/g of biomass were obtained with the ILs cholinium acetate ([Ch][Ac]) and cholinium chloride ([Ch]Cl), respectively. Circular dichroism (CD) spectroscopy results show that the secondary structure of RuBisCO is better preserved in the IL solutions when compared to the commonly used extraction solvent. The obtained results indicate that cholinium-based ILs are a promising and viable alternative for the extraction of RuBisCO from vegetable biomass.publishe

Non-AIDS-related comorbidities in people living with HIV-1 aged 50 years and older: The AGING POSITIVE study.

OBJECTIVE: To characterize the profile of non-AIDS-related comorbidities (NARC) in the older HIV-1-infected population and to explore the factors associated with multiple NARC. METHODS: This was a multicentre, cross-sectional study including HIV-1-infected patients aged ≥50 years, who were virologically suppressed and had been on a stable antiretroviral therapy (ART) regimen for at least 6 months. A multiple regression model explored the association between demographic and clinical variables and the number of NARC. RESULTS: Overall, 401 patients were enrolled. The mean age of the patients was 59.3 years and 72.6% were male. The mean duration of HIV-1 infection was 12.0 years and the median exposure to ART was 10.0 years. The mean number of NARC was 2.1, and 34.7% of patients had three or more NARC. Hypercholesterolemia was the most frequent NARC (60.8%), followed by arterial hypertension (39.7%) and chronic depression/anxiety (23.9%). Arterial hypertension and diabetes mellitus were the most frequently treated NARC (95.6% and 92.6% of cases, respectively). The linear regression analysis showed a positive relationship between age and NARC (B=0.032, 95% confidence interval 0.015-0.049; p=0.0003) and between the duration of HIV-1 infection and NARC (B=0.039, 95% confidence interval 0.017-0.059; p=0.0005). CONCLUSIONS: A high prevalence of NARC was found, the most common being metabolic, cardiovascular, and psychological conditions. NARC rates were similar to those reported for the general population, suggesting a larger societal problem beyond HIV infection. A multidisciplinary approach is essential to reduce the burden of complex multi-morbid conditions in the HIV-1-infected population.info:eu-repo/semantics/publishedVersio

Selective ablating urologic cancers with cold atmospheric plasma

info:eu-repo/semantics/publishedVersio

Avaliação de acessos de melancia quanto a resistência ao fungo Didymella bryoniae.

A técnica de inoculação com injeção de suspensão de esporos se mostrou mais adequada para avaliação dos acessos de melancia

Unravelling the effect of SrTiO3 antiferrodistortive phase transition on the magnetic properties of La0.7Sr0.3MnO3 thin films

Epitaxial La0.7Sr0.3MnO3 (LSMO) thin films, with different thicknesses ranging from 20 to 330 nm, were deposited on (1 0 0)-oriented strontium titanate (STO) substrates by pulsed laser deposition, with their structure and morphology characterized at room temperature. The magnetic and electric transport properties of the as-processed thin films reveal an abnormal behaviour in the temperature dependent magnetization M(T) below the antiferrodistortive STO phase transition (TSTO), and also an anomaly in the magnetoresistance and electrical resistivity close to the same temperature. Films with thickness ≤100 nm show an in-excess magnetization and pronounced changes in the coercivity due to the interface-mediated magnetoelastic coupling with antiferrodistortive domain wall movement occurring below TSTO. However, in thicker LSMO thin films, an in-defect magnetization is observed. This reversed behaviour can be understood with the emergence in the upper layer of the film, of a columnar structure needed to relax the elastic energy stored in the film, which leads to randomly oriented magnetic domain reconstructions. For enough high-applied magnetic fields, as thermodynamic equilibrium is reached, a full suppression of the anomalous magnetization occurs, wherein the temperature dependence of the magnetization starts to follow the expected Brillouin behaviour.This work was supported by the Fundação para a Ciencia e Tecnologia and COMPETE/QREN/EU, through the project PTDC/CTM/099415/2008. The authors are very grateful to Maria Joao Pereira and Maria Rosario Soares from CICECO, University of Aveiro, for the HR-XRD measurements and discussion of the results. F Figueiras acknowledges FCT grant SFRH/BPD/80663/2011. The authors also acknowledge Projeto Norte-070124-FEDER-000070 and Professor J Fontcuberta for their fruitful discussions

Impact of montelukast as add on treatment to the novel coronavirus pneumonia (COVID-19): protocol for an investigator-initiated open labeled randomized controlled pragmatic trial

Background: Montelukast, a safe drug widely use in asthmatic patients, may be an adjuvant in the treatment of Covid-19, either by improving lung injury and inflammation, or by acting as an anti-viral drug. We aim to assess the efficacy and safety of montelukast as add-on treatment in patients with Covid-19. Methods: We propose a randomized, controlled, parallel, open-label trial involving 160 hospitalized adult patients with confirmed Covid-19. Patients will be randomly assigned in a 1:1 ratio to receive either montelukast 10 mg, once a day for 14 days, in addition to standard of care (SoC), or SoC alone. SoC will follow the best practice for treating these patients, according to updated recommendations. The primary outcome is time to recovery. Participants will be assessed using diary cards to capture data on treatment-related improvements in an 8-point ordinal scale. Secondary endpoints will include changes in respiratory and inflammatory parameters, and adverse events. This phase IV clinical trial will take place at the University Hospital of São João, Porto. EudraCT number: 2020-001747-21. Results: This study intends to generate scientific evidence on efficacy and safety of montelukast as add-on treatment in Covid-19. The results will be essential to improve clinical outcomes which remains to be determined. Conclusion: Montelukast has been suggested as a potential drug with 2 main actions on Covid-19. The validation of montelukast as an adjuvant treatment may improve lung injury, inflammation, and symptoms leading to a better prognosis. The use of this drug may fulfil the existing gap on therapeutic options