Síndroma Ovo-Ave na Criança:Um Caso Clínico

A síndroma ovo -ave é uma entidade clínica rara, em especial em idade pediátrica. Descreve -se o caso de criança de sexo masculino, 5 anos, que habita zona rural, com clínica sugestiva de alergia ao ovo e à carne de frango desde os 7 meses. Aos 2,5 anos apresenta queixas de asma brônquica, rinite alérgica e eczema atópico. Os testes cutâneos foram positivos para extractos de clara e gema de ovo. Dosearam -se IgE específi cas para clara e gema de ovo (>100 kU/L), carne de frango (1,0 kU/L), α -livetina (0,7 kU/L), penas de frango (15,3 kU/L). O estudo de immunoblotting evidenciou ligação de IgE a bandas com peso molecular entre 30-66 kDa e 32-45 kDa para clara e gema, respectivamente, 38/39/42 kDa para carne de frango e 33-45 kDa para penas de frango. Em doentes com alergia a carne de aves, expostos a factores ambientais de risco e sensibilização elevada a gema de ovo, dever -se -á suspeitar da síndroma ovo-ave

Abernethy Malformation and Hepatocellular Carcinoma: a Serious Consequence of a Rare Disease

Congenital portosystemic shunts (CPSS) are a rare vascular consequence of embryogenetic vascular alterations or the persistence of the fetal circulation elements, first described by John Abernethy in 1793 and classified by Morgan and Superina, into complete and partial portosystemic shunts. Its prevalence to this day has not been defined. We present a patient series of a 44-year-old and 47-year-old man and woman, with this rare congenital malformation and underlining hepatocellular carcinoma (HCC) treatment strategies. Over half of the individuals with CPSS have benign or malignant liver tumours, ranging from nodular regenerative hyperplasia, focal nodular hyperplasia, adenomas, HCC and hepatoblastomas. Additionally, it is known that half of individuals with Abernethy malformation type Ib will develop one or multiple types of tumours. There seems to be a direct association with tumorigenesis and CPSS, which is the primary consequence of absent portal flow. Surgery is the treatment of choice, either as a curative resection or orthotopic liver transplantation if recommended as per the criteria, in which replacing the hepatic parenchyma in the setting of an Abernathy malformation will correct the underlining hyper-arterialisation.info:eu-repo/semantics/publishedVersio

Barriers and facilitators to the recruitment of disabled people to clinical trials: a scoping review

Introduction Underrepresentation of disabled groups in clinical trials results in an inadequate evidence base for their clinical care, which drives health inequalities. This study aims to review and map the potential barriers and facilitators to the recruitment of disabled people in clinical trials to identify knowledge gaps and areas for further extensive research. The review addresses the question: ‘What are the barriers and facilitators to recruitment of disabled people to clinical trials?’. Methods The Joanna Briggs Institute (JBI) Scoping review guidelines were followed to complete the current scoping review. MEDLINE and EMBASE databases were searched via Ovid. The literature search was guided by a combination of four key concepts from the research question: (1) disabled populations, (2) patient recruitment, (3) barriers and facilitators, and (4) clinical trials. Papers discussing barriers and facilitators of all types were included. Papers that did not have at least one disabled group as their population were excluded. Data on study characteristics and identified barriers and facilitators were extracted. Identified barriers and facilitators were then synthesised according to common themes. Results The review included 56 eligible papers. The evidence on barriers and facilitators was largely sourced from Short Communications from Researcher Perspectives (N = 22) and Primary Quantitative Research (N = 17). Carer perspectives were rarely represented in articles. The most common disability types for the population of interest in the literature were neurological and psychiatric disabilities. A total of five emergent themes were determined across the barriers and facilitators. These were as follows: risk vs benefit assessment, design and management of recruitment protocol, balancing internal and external validity considerations, consent and ethics, and systemic factors. Conclusions Both barriers and facilitators were often highly specific to disability type and context. Assumptions should be minimised, and study design should prioritise principles of co-design and be informed by a data-driven assessment of needs for the study population. Person-centred approaches to consent that empower disabled people to exercise their right to choose should be adopted in inclusive practice. Implementing these recommendations stands to improve inclusive practices in clinical trial research, serving to produce a well-rounded and comprehensive evidence base

CD26/DPPIV and response to hepatitis B vaccination

The prevention of hepatitis B is important, since it is responsible for significant morbidity and mortality around the world. Unfortunately, hepatitis B vaccine does not always induce protective immunity. The lack of immune response to vaccine (non-responders) can depend on individual characteristics. The objective of this study was to correlate the CD26/DPPIV cellular expression and DPPIV serum activity with HBV vaccine response and its possible role as an indicator of immune competence acquisition. We also determined the cellular expression of CD3, CD19, CD56 and CD25 in peripheral blood T lymphocytes. Blood samples were obtained from 28 healthy human volunteers who were enrolled with a vaccination program. There were "responders" (RM = 13) and "non-responders" (NRM = 15), after vaccination. The lymphocyte populations were identified by flow cytometry. DPPIV serum activity was measured fluorimetrically. CD26 expression in responders (55.9 +/- 7.7%) versus in non-responders (51.9 +/- 7.0%) did not show a significant difference. The DPPIV serum activity in responders compared to in non-responder subgroup (59.9 +/- 8.4/50.3 +/- 10.6U/L) showed, however, a significant difference (P < 0.05). The expression of CD3, CD19 and CD56 on peripheral lymphocytes was similar between responders and non-responders. The expression of CD3CD26 (52.2 +/- 8.6%) and CD3CD25 (10.9 +/- 3.8%) in responders versus the expression of CD3CD26 (48.0 +/- 5.7%) and CD3CD25 (8 +/- 4.6%) in non-responders did not show statistically significant difference. CD25 referred as a marker of T lymphocyte activation was increased in responders (15.8 +/- 4.5%) versus in non-responders (10.1 +/- 4.8%), showing a significant difference (P = 0.003). It was, however, impossible to demonstrate an increase in CD3CD25 and CD3CD26 in the responder subgroup. This suggests that different lymphocyte subsets other than T cells are implicated in the response to hepatitis B vaccination

Severe Influenza B Pneumonia Virus in a Newborn

A infecção por vírus influenza B é rara no período neonatal com uma incidência desconhecida. Relata-se o caso de uma recém-nascida de termo, reinternada ao nono dia de vida por quadro de má perfusão periférica, gemido, dificuldade alimentar e dificuldade respiratória com necessidade de ventila ção mecânica, óxido nítrico inalado e surfactante. A radiografia de tórax no primeiro dia apresentava infiltrado intersticial ligeiro, difuso. Esteve sob ventilação invasiva durante 11 dias e oxigenoterapia 15 dias, tendo tido alta ao 20º dia, clinicamente bem. É fundamental pensar em infecção por vírus influenza B quando existe história de possível contágio, e em mães sem imunização anti-influenza. Não há terapêutica aprovada neste grupo etário, devendo ser tomadas medidas de suporte, de contenção e prevenção da disseminação da infecção

In silico and expression analyses of fasciclin-like arabinogalactan proteins reveal functional conservation during embryo and seed development

Fasciclin-like arabinogalactan proteins (FLAs) are a subclass of arabinogalactan proteins (AGPs), which contain fasciclin-like domains in addition to typical AGP domains. FLAs are present across all embryophytes, and despite their low overall sequence similarity, conserved regions that define the fasciclin functional domain (FAS) have been identified, suggesting that the cell adhesion property is also conserved. FLAs in Arabidopsis have been organized into four subgroups according to the number and distribution of functional domains. Recent studies associated FLAs with cell wall-related processes where domain organization seemed to be related to functional roles. In Arabidopsis, FLAs containing a single FAS domain were found to be important for the integrity and elasticity of the plant cell wall matrix, and FLAs with two FAS domains and two AGP domains were found to be involved in maintaining proper cell expansion under salt stress conditions. The main purpose of the present work was to elucidate the expression pattern of selected FLA genes during embryo and seed development using RT-qPCR. AtFLA8 and AtFLA10, two Arabidopsis genes that stood out in previous microarray studies of embryo development, were further examined using promoter-driven gene reporter analyses. We also studied the expression of cork oak FLA genes and found that their expression partially parallels the expression patterns of the putative AtFLA orthologs. We propose that the functional organization of FLAs is conserved and may be related to fundamental aspects of embryogenesis and seed development across angiosperms. Phylogenetic studies were performed, and we show that the same basic four-subgroup organization described for Arabidopsis FLA gene classification is valid for most Arabidopsis FLA orthologs of several plant species, namely poplar, corn and cork oak

Reflection on Risk Factors, Ashtma and Tobacco Smoke Exposure

O impacto da asma brônquica nas últimas décadas, nomeadamente em idade pediátrica, associando prevalências significativas a uma tendência, gravidade e custos crescentes, tem levado a que se efectuem múltiplos estudos para esclarecer causas, avaliando riscos, permitindo a elaboração de programas de prevenção. Estudos epidemiológicos bem desenhados, aplicados a amostras populacionais significativas, permitem identificar determinantes independentes da asma, viabilizando a actuação. Se muito se tem avançado no conhecimento das bases fisiopatológicas da doença alérgica, é com alguma preocupação que sentimos que a comunidade médica, mesmo a especializada, não sente as evidências epidemiológicas como aplicáveis à sua população de asmáticos. “Precisamos de mais estudos”, é declaração comum. Com este trabalho, centrado em três rastreios efectuados com a coordenação dos autores, pretendemos demonstrar que existem factores preveníveis, moduláveis, que podem permitir reduzir a morbilidade da doença asmática. Entre estes, o tabagismo passivo assume uma relevância ímpar, por ser o principal factor de risco para a gravidade da asma pediátrica em Portugal

Development Process and Cognitive Testing of CARATkids - Control of Allergic Rhinitis and Asthma Test for Children

Background: Allergic rhinitis and asthma (ARA) are chronic inflammatory diseases of the airways that often coexist in children. The only tool to assess the ARA control, the Control of Allergic Rhinitis and Asthma Test (CARAT) is to be used by adults. We aimed to develop the Pediatric version of Control of Allergic Rhinitis and Asthma Test (CARATkids) and to test its comprehensibility in children with 4 to 12 years of age. Methods: The questionnaire development included a literature review of pediatric questionnaires on asthma and/or rhinitis control and two consensus meetings of a multidisciplinary group. Cognitive testing was carried out in a cross-sectional qualitative study using cognitive interviews. Results: Four questionnaires to assess asthma and none to assess rhinitis control in children were identified. The multidisciplinary group produced a questionnaire version for children with 17 questions with illustrations and dichotomous (yes/no) response format. The version for caregivers had 4-points and dichotomous scales. Twenty-nine children, 4 to 12 years old, and their caregivers were interviewed. Only children over 6 years old could adequately answer the questionnaire. A few words/expressions were not fully understood by children of 6 to 8 years old. The drawings illustrating the questions were considered helpful by children and caregivers. Caregivers considered the questionnaire complete and clear and preferred dichotomous over the 4-points scales. The proportion of agreement between children and their caregivers was 61%. The words/expressions that were difficult to understand were amended. Conclusion: CARATkids, the first questionnaire to assess a child’s asthma and rhinitis control was developed and its content validity was assured. Cognitive testing showed that CARATKids is well-understood by children 6 to 12 years old. The questionnaire’s measurement properties can now be assessed in a validation study

Graphene oxide topical administration: Skin permeability studies

Nanostructured carriers have been widely used in pharmaceutical formulations for der-matological treatment. They offer targeted drug delivery, sustained release, improved biostability, and low toxicity, usually presenting advantages over conventional formulations. Due to its large surface area, small size and photothermal properties, graphene oxide (GO) has the potential to be used for such applications. Nanographene oxide (GOn) presented average sizes of 197.6 ± 11.8 nm, and a surface charge of -39.4 ± 1.8 mV, being stable in water for over 6 months. 55.5% of the mass of GOn dispersion (at a concentration of 1000 µg mL-1 ) permeated the skin after 6 h of exposure. GOn dispersions have been shown to absorb near-infrared radiation, reaching temperatures up to 45.7¿ C, within mild the photothermal therapy temperature range. Furthermore, GOn in amounts superior to those which could permeate the skin were shown not to affect human skin fibroblasts (HFF-1) morphology or viability, after 24 h of incubation. Due to its large size, no skin permeation was observed for graphite particles in aqueous dispersions stabilized with Pluronic P-123 (Gt–P-123). Altogether, for the first time, Gon’s potential as a topic administration agent and for delivery of photothermal therapy has been demonstrated.This work was financed by FEDER funds through the COMPETE 2020–Operacional Programme for Competitiveness and Internationalisation (POCI), Portugal 2020, and by national funds (PIDDAC) through FCT/MCTES in the framework of the project POCI-01-0145-FEDER-031143, and Base Funding-UIDB/00511/2020 of the Laboratory for Process Engineering, Environment, Biotechnology and Energy–LEPABE. Additional funding included FCT/MCTES in the framework of the project “Institute for Research and Innovation in Health Sciences” (UID/BIM/04293/2019). Authors would also like to thank the support of i3S Scientific Platforms and respective funding: HEMS, member of the national infrastructure PPBI–Portuguese Platform of Bioimaging: POCI-01-0145-FEDER-022122; and Biointerfaces and Nanotechnology (BN) Laboratory, Portuguese Funds through FCT, UID/BIM/04293/2019. Artur Pinto thanks the Portuguese Foundation for Science and Technology (FCT) for the financial support of his work contract through the Scientific Employment Stimulus-Individual Call–[CEECIND/03908/2017]. Soraia Pinto (SFRH/BD/144719/2019) would like to thank FCT, Portugal for financial support