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Pediatric Organ and Tissue Donation—A 10-Year Retrospective Study in Portugal
No full textOrgan and tissue donation can transform lives. One donor can ensure the survival of up to 8 people through their organs and improve the quality of life for dozens more through tissue donation. Portugal has an excellent transplantation rate, but deaths still occur while waiting for an organ. The study aimed to analyze pediatric organ and tissue donors nationally and evaluate brain deaths in a pediatric intensive care unit (PICU) over the past 10 years to identify any potential lost donors. We conducted a retrospective descriptive study of pediatric organ and tissue donors and diagnosed brain deaths from January 2011 to December 2021. Demographic and clinical data were analyzed, including those provided by the National Transplant Coordination. Over the past 10 years in Portugal, 121 pediatric donors (11.7 per million population) were collected, and 569 organs and tissues were collected. During the same period in the PICU, there were 125 deaths, including 20 brain deaths. Of this group, 4 were organ and tissue donors. In the non-donor group (n = 16), a potential lost donor case stands out. Pediatric specialists need to be more familiar with the donation process, which would enable the identification and optimization of all potential donors, thus minimizing the number of potentially lost organs.info:eu-repo/semantics/publishedVersio
Alterações na Variabilidade da Frequência Cardíaca Após Encerramento da Comunicação Interventricular em Crianças. Qual é a Nossa Posição? Mecanismo de Adaptação, Sequela ou Complicação?
No full textinfo:eu-repo/semantics/publishedVersio
Challenges and Knowledge Gaps with Immune Checkpoint Inhibitors Monotherapy in the Management of Patients with Non-Small-Cell Lung Cancer: a Survey of Oncologist Perceptions
No full textBackground: Immune checkpoint-inhibitors (ICIs) are changing outcomes in different cancer settings, notably for patients with non-small-cell lung cancer (NSCLC). There are, however, still important gaps of evidence for clinical practice when using these novel treatments. In this study, we assessed physicians' opinion and experience on challenges for clinical practice with ICIs monotherapy in NSCLC.
Methods: A survey was conducted on experienced physicians treating patients with NSCLC with ICIs. Two rounds of pilot tests were carried out for validation among a group of experts. Topics under analysis were in relation to treatment of elderly populations, performance status, brain metastases, use of steroids or antibiotics, the effects of gut microbiome, autoimmune diseases, human immunodeficiency virus infection, solid organ transplants, use of anti-programmed cell death protein 1 versus anti-programmed death-ligand 1 drugs, atypical tumour responses, predictors of response, duration of treatment and a final open question on additional relevant challenges.
Results: Two hundred and twenty-one answers were collected, including 106 (48%) valid answers from experts for final analysis (physicians who have treated at least 20 patients with NSCLC with ICIs). The vast majority agreed that the selected topics in this study are important challenges ahead and more evidence is needed. Moreover, predictors of response, treating brain metastasis, shorter duration of treatment, the effects of gut microbiome and concomitant use of steroids were voted the most important topics to be further addressed in prospective clinical research.
Conclusions: This survey contributed to understanding which are the main challenges for clinical practice with ICIs monotherapy in NSCLC. It can also contribute to guide further clinical research, considering the opinions and experience of those who regularly treat NSCLC patients with ICIs.info:eu-repo/semantics/publishedVersio
Novel Insights in the Management of Vernal Keratoconjunctivitis (VKC): European Expert Consensus Using a Modified Nominal Group Technique
No full textIntroduction: Vernal keratoconjunctivitis (VKC) is a rare, severe allergic ocular disease, typically occurring in children and adolescents, that can have a significant impact on quality of life and lead to visual impairment. Long-term treatment may be necessary to tackle chronic inflammation and topical corticosteroid dependency must be minimised due to the risk of complications. There is a need for unified clinical guidance to aid the assessment, diagnosis and management of VKC across Europe. The aim of this expert panel (the EUR-VKC Group) was to provide clear guidance for primary care physicians and general ophthalmologists involved in the diagnosis and management of VKC.
Methods: An expert group of seven European ophthalmologists was convened and a modified nominal group technique used to develop key recommendations on VKC management. The recommendations were subject to up to two rounds of voting using a 5-point Likert scale to ascertain consensus and the strength of each recommendation. Consensus was set at a predetermined threshold of ≥ 75.0% of experts selecting 'Strongly agree' or 'Agree'.
Results: A total of 47 recommendations were developed relating to the assessment of key of VKC, guidance on who and when to refer, as well as treatment-escalation pathways, long-term follow-up, and supportive care and education. All recommendations reached consensus after two rounds. The group emphasise how timely diagnosis and treatment initiation that is appropriate to disease severity are crucial to benefit patients with VKC. Patients with signs ('red flags') indicating severe VKC, or persistent mild-to-moderate VKC that is non-responsive following 2-4 weeks of treatment, should be referred to a sub-specialist.
Conclusion: The EUR-VKC Group provides recommendations on the assessment, diagnosis, management, referral and follow-up of patients with VKC. It also provides a framework to facilitate collaboration between primary care physicians, general ophthalmologists and sub-specialists to improve the outcomes for patients with VKC.info:eu-repo/semantics/publishedVersio
Real-World Inter-Observer Variability of the Sequential Organ Failure Assessment (SOFA) Score in Intensive Care Medicine: the Time Has come for an Update: Authors’ Reply
No full textinfo:eu-repo/semantics/publishedVersio
Rhinitis Associated with Asthma is Distinct from Rhinitis Alone: The ARIA‐MeDALL Hypothesis
No full textAsthma, rhinitis, and atopic dermatitis (AD) are interrelated clinical phenotypes that partly overlap in the human interactome. The concept of "one-airway-one-disease," coined over 20 years ago, is a simplistic approach of the links between upper- and lower-airway allergic diseases. With new data, it is time to reassess the concept. This article reviews (i) the clinical observations that led to Allergic Rhinitis and its Impact on Asthma (ARIA), (ii) new insights into polysensitization and multimorbidity, (iii) advances in mHealth for novel phenotype definitions, (iv) confirmation in canonical epidemiologic studies, (v) genomic findings, (vi) treatment approaches, and (vii) novel concepts on the onset of rhinitis and multimorbidity. One recent concept, bringing together upper- and lower-airway allergic diseases with skin, gut, and neuropsychiatric multimorbidities, is the "Epithelial Barrier Hypothesis." This review determined that the "one-airway-one-disease" concept does not always hold true and that several phenotypes of disease can be defined. These phenotypes include an extreme "allergic" (asthma) phenotype combining asthma, rhinitis, and conjunctivitis. Rhinitis alone and rhinitis and asthma multimorbidity represent two distinct diseases with the following differences: (i) genomic and transcriptomic background (Toll-Like Receptors and IL-17 for rhinitis alone as a local disease; IL-33 and IL-5 for allergic and non-allergic multimorbidity as a systemic disease), (ii) allergen sensitization patterns (mono- or pauci-sensitization versus polysensitization), (iii) severity of symptoms, and (iv) treatment response. In conclusion, rhinitis alone (local disease) and rhinitis with asthma multimorbidity (systemic disease) should be considered as two distinct diseases, possibly modulated by the microbiome, and may be a model for understanding the epidemics of chronic and autoimmune diseases.info:eu-repo/semantics/publishedVersio
Altos e Baixos no Mapeamento das Arritmias de Macro-Reentrada. Uma Revelação numa Centelha de Luz
No full textinfo:eu-repo/semantics/publishedVersio
Rare Primary Dyslipidaemias Associated with Low LDL and HDL Cholesterol Values in Portugal
No full textBackground: Dyslipidaemia represents a group of disorders of lipid metabolism, characterized by either an increase or decrease in lipid particles, usually associated with triglycerides, LDL cholesterol (LDL-C) and/or HDL cholesterol (HDL-C). Most hyperlipidaemias and HDL deficiencies confer an increased cardiovascular risk, while hypolipidaemia, such as abeta or hypobetalipoproteinemia, may present different manifestations ranging from poor weight progression to neurological manifestations. The aim of this study is to present 7 cases with rare dyslipidaemias associated with low LDL or low HDL cholesterol values, referred to our laboratory for the genetic identification of the cause of the dyslipidaemia. Methods: Lipid profile was determined for each individual in an automated equipment Integra Cobas (Roche). Molecular analysis was performed by NGS with a target panel of 57 genes involved in lipid metabolism (Sure select QXT, Agilent) and samples were run in a NextSEQ Sequencer (Illumina). Only genes associated to rare forms of low HDL-c or LDL-c were analysed for this work, namely: ABCA1, APOA1, LCAT, SCARB1, APOB, PCSK9, MTTP, SAR1B, and ANGPTL3. All rare variants (MAFA) and the other is a possible compound heterozygous for APOB variants c.2604+1G>A and c.4651C>T/p.(Gln1551*). In two patients only a variant in heterozygosity (c.3365delG/p.(Gly1122Vfs*62) and c.11095A>T/p.(Arg3699*)). In the remaining patient, no variants were identified. NGS proved to be a fundamental key for genetic testing of rare lipid disorders, allowing us to find the genetic cause of disease in 6/7 patients with low HDL-c and LDL-c. Patients with these rare conditions should be identified as early as possible in order to minimize or prevent clinical manifestations. The unsolved case is still under investigation.info:eu-repo/semantics/publishedVersio
Fatores de Risco de Burnout nos Profissionais de Saúde em Contexto Hospitalar
No full textIntrodução – O burnout é um estado de esgotamento físico, resultante de stress crónico no trabalho não gerido com êxito (OMS, 2019). Existem inúmeras definições de burnout, sendo a mais atual e comummente utilizada pelos grandes investigadores do fenómeno como Maslach e Jackson, a que caracteriza a síndrome como sendo uma crise de caráter psicológico com elevada exaustão emocional, despersonalização e baixa realização profissional, provocada pela fadiga, stress, exaustão e falta de realização no mundo do trabalho (Schaufeli, 2017).
Objetivo – Identificar os fatores de risco associados ao burnout nos profissionais de saúde em contexto hospitalar.
Metodologia – Revisão sistemática da literatura. Bases de dados: CINAHL Complete, MEDLINE Complete e MEDIC LATINA, na EBSCOhost (2017-2021). Descritores: Health Personnel; Burnout, Professional; Burnout, Psychological; Stress, Psychological; Occupational Stress; Risk Factors; Risk; Hospitals; Hospital Units; Hospital Departments; Health facility departments. N=542 artigos, selecionados 10.
Resultados – A síndrome de burnout constitui um processo multicausal, com fatores individuais, sociais e organizacionais.
Discussão/Conclusão – Os ambientes em que os profissionais de saúde se inserem, assim como as suas características sociodemográficas e pessoais, constituem-se como fatores que podem levar a doença mental. Urge implementar estratégias que melhorem o seu bem-estar físico e psicológico.info:eu-repo/semantics/publishedVersio
Preoperative Risk Score (PreopScore) to Predict Overall Survival After Resection for Hepatocellular Carcinoma.
No full textBackground: This study aimed to develop a holistic risk score incorporating preoperative tumor, liver, nutritional, and inflammatory markers to predict overall survival (OS) after hepatectomy for hepatocellular carcinoma (HCC).
Methods: Patients who underwent curative-intent surgery for HCC between 2000 and 2020 were identified using an international multi-institutional database. Preoperative predictors associated with OS were selected and a prognostic risk score model (PreopScore) was developed and validated using cross-validation.
Results: A total of 1676 patients were included. On multivariable analysis, preoperative parameters associated with OS included α-feto protein (hazard ratio [HR]1.17, 95%CI 1.03-1.34), neutrophil-to-lymphocyte ratio (HR2.62, 95%CI 1.30-5.30), albumin (HR0.49, 95%CI 0.34-0.70), gamma-glutamyl transpeptidase (HR1.00, 95%CI 1.00-1.00), as well as vascular involvement (HR3.52, 95%CI 2.10-5.89) and tumor burden score (medium, HR3.49, 95%CI 1.62-7.58; high, HR3.21, 95%CI 1.40-7.35) on preoperative imaging. A weighted PreopScore was devised and made available online (https://yutaka-endo.shinyapps.io/PrepoScore_Shiny/). Patients with a PreopScore 0-2, 2-3.5, and >3.5 had incrementally worse 5-year OS of 85.8%, 70.7%, and 52.4%, respectively (p < 0.001). The c-index of the test and validation cohort were 0.75 and 0.71, respectively. The PreopScore outperformed individual parameters and previous HCC staging systems.
Discussion: The PreopScore can be used as a better guide to preoperatively identify patients and individualize pre-/post-operative strategies