Continuous Sedation in Palliative Care in Portugal: A Prospective Multicentric Study.

Objective: This study aimed to survey the practice of palliative sedation in Portugal, where data on this subject were lacking. Methods: This was a prospective multicentric study that included all patients admitted to each team that agreed to participate. Patients were followed until death, discharge, or after 3 months of follow-up. Results: The study included 8 teams: 4 as palliative care units (PCU), 1 as a hospital palliative care team (HPCT), 2 as home care (HC), and 1 as HPCT and HC. Of the 361 patients enrolled, 52% were male, the median age was 76 years, and 285 (79%) had cancer. Continuous sedation was undergone by 49 (14%) patients: 26 (53%) were male, and the median age was 76. Most patients, 46 (94%), had an oncological diagnosis. Only in a minority of cases, the family, 16 (33%), or the patient, 5 (10%), participated in the decision to sedate. Delirium was the most frequent symptom leading to sedation. The medication most used was midazolam (65%). In the multivariable analysis, only age and the combined score were independently associated with sedation; patients <76 years and those with higher levels of suffering had a higher probability of being sedated. Conclusions: The practice of continuous palliative sedation in Portugal is within the range reported in other studies. One particularly relevant point was the low participation of patients and their families in the decision-making process. Each team must have a deep discussion on this aspect.

Effects of Aging and Nerve Growth Factor on Neuropeptide Expression and Cholinergic Innervation of the Rat Basolateral Amygdala.

The basolateral amygdala (BLA) contains interneurons that express neuropeptide Y (NPY) and vasoactive intestinal polypeptide (VIP), both of which are involved in the regulation of functions and behaviors that undergo deterioration with aging. There is considerable evidence that, in some brain areas, the expression of NPY and VIP might be modulated by acetylcholine. Importantly, the BLA is one of the brain regions that has one of the densest cholinergic innervations, which arise mainly from the basal forebrain cholinergic neurons. These cholinergic neurons depend on nerve growth factor (NGF) for their survival, connectivity, and function. Thus, in this study, we sought to determine if aging alters the densities of NPY- and VIP-positive neurons and cholinergic varicosities in the BLA and, in the affirmative, if those changes might rely on insufficient trophic support provided by NGF. The number of NPY-positive neurons was significantly reduced in aged rats, whereas the number of VIP-immunoreactive neurons was unaltered. The decreased NPY expression was fully reversed by the infusion of NGF in the lateral ventricle. The density of cholinergic varicosities was similar in adult and old rats. On the other hand, the density of cholinergic varicosities is significantly higher in old rats treated with NGF than in adult and old rats. Our results indicate a dissimilar resistance of different populations of BLA interneurons to aging. Furthermore, the present data also show that the BLA cholinergic innervation is particularly resistant to aging effects. Finally, our results also show that the reduced NPY expression in the BLA of aged rats can be related to changes in the NGF neurotrophic support.

Liver Transplantation for Treatment of Unresectable Spontaneous Ruptured Hepatocellular Adenoma: A Rare Indication.

Hepatocellular Adenomas (HA) are rare benign tumors of the liver which occur predominantly in young women. Although benign, HA may have complications such as hemorrhage and malignant transformation. So, sometimes conservative management is not enough. We report a case of a 26-year-old woman on oral contraceptives who presented with acute abdominal pain and signs of hemodynamic shock. She underwent transarterial embolization due to the presence of multiple HA with rupture and active hemorrhage. This minimally invasive treatment failed to control the disease. The patient presented a progressive increase in the size of the masses with signs of recent hemorrhage, and the HA became unresectable, so she underwent liver transplantation. Liver transplantation is rarely indicated for the treatment of HA; however, in unresectable masses, it should be considered to prevent potential rupture with hemorrhage or malignant transformation.

Vaccine-Induced Thrombotic Thrombocytopenia: A Case Report.

While the number of people who have been vaccinated against coronavirus disease 2019 (COVID-19) in Portugal keeps rising, the risk of complications, although rare, keeps rising too. We report a case of vaccine-induced thrombotic thrombocytopenia (VITT) in a 30-year-old previously healthy male after vaccination with Ad26.COV2.S. The patient presented to the emergency department (ED) with abdominal pain and headache. Laboratory tests revealed thrombocytopenia, high D-dimer levels, and fibrinogen consumption. Thoracoabdominal CT scan showed a thrombus in the portal mesenteric venous axis. A positive PF4 heparin enzyme-linked immunosorbent assay confirmed the VITT diagnosis, and the patient was started on intravenous immunoglobulin. Both clinical complaints and laboratory findings resolved within six days, and he was discharged to follow-up. This case shows that general symptoms after vaccination should not be depreciated, highlights the importance of early diagnosis and treatment, and raises new questions about the follow-up and further study of these patients.

What Basophil Testing Tells Us About CSU Patients - Results of the CORSA Study.

Basophil testing is the most effective single approach for diagnosing type-IIb autoimmune chronic spontaneous urticaria (TIIbaiCSU). A positive basophil test has been linked to long disease duration, higher disease activity, a poor response to antihistamines and omalizumab, and a better response to cyclosporine and fenebrutinib. As of now it is unclear what other features are connected to a positive basophil test in chronic spontaneous urticaria (CSU). We aimed to identify features of basophil test-positive CSU patients. We performed a cross-sectional study of 85 CSU patients. Basophil testing was done with the basophil activation test (BAT) and the basophil histamine release assay (BHRA). Data were analysed using SPSS: Student's t-test, Chi-square test, Odds Ratio, Spearman's correlation test. Of 85 CSU patients, 44% and 28% tested positive with the BAT and BHRA, respectively. These patients showed higher disease activity and impact, lower levels of disease control and total serum IgE, as well as higher rates of having a positive autologous serum skin test (ASST), angioedema, nocturnal symptoms, symptoms for >5 days/week, and thyroid autoantibodies. The ASST, by itself, was not a good predictor of basophil test results, but it predicted a positive basophil test in up to 100% of cases when combined with angioedema, thyroid autoantibodies or low IgE. In conclusion, a positive basophil test is linked to known features of TIIbaiCSU and novel characteristics including nocturnal symptoms. Further studies on basophil test-positive and -negative CSU patients can help to better understand CSU endotypes and to develop better management approaches.

Molecular diagnosis and immunotherapy selection in a Portuguese population sensitized to grass and olive pollens / Diagnóstico molecular e seleção de imunoterapia numa população portuguesa sensibilizada ao pólen de gramíneas e de oliveira

Introduction: Phleum pratense (Phl p) and Olea europaea (Ole e) are common allergenic pollen. Objectives: To describe the sensitization patterns to Phl p and Ole e allergens in a subset of allergic rhinitis patients with positive skin prick tests (SPTs) to these pollens and compare the allergen immunotherapy (AIT) choice before and after determination of molecular components. Methods: Candidates to pollen immunotherapy with positive SPTs to both Phl p and Ole e were recruited. All of them underwent an SPT with a panel of aeroallergens and measurements of serum specific IgE (sIgE) to Phl p, Ole e, Phl p1, Phl p5, Phl p7, Phl p12, Ole e1, Ole e7, and Bet v2. Results: Forty adults were included. Of these, 83% and 65% were sIgE-positive to Phl p and Ole e, using the 0.35 kUA/L and 0.70 kUA/L cut-offs, respectively. Moreover, 42.5% of patients had positive sIgE to Phl p1 and/or Phl p5, 2.5% only to Ole e1, and 47.5% to both (0.35 kUA/L cutoff). By increasing the cut-off to 0.7 kUA/L, 55% of patients were sensitized to Phl p1 and/or Phl p5, and no patient was sensitized only to Ole e1. After component-resolved diagnosis, AIT choice was changed in 15 (37.5%) patients, with a decrease in the number of prescriptions of AIT with both grass and olive pollens and with olive alone, together with an increase in the prescriptions of AIT with grass pollen alone. Conclusion: Genuine sensitization to Olea europaea was reduced, and the sensitization patterns were heterogeneous. Knowledge of pattern of sensitization to molecular components changed immunotherapy prescription in more than one third of the patients.

Introdução: Os polens de Phleum pratense (Phl p) e de Olea europaea (Ole e) são fontes alergênicas comuns. Objetivos: Descrever os padrões de sensibilização aos alergênios destes dois polens num subconjunto de pacientes com rinite alérgica polínica e comparar a escolha de imunoterapia, antes e depois da determinação de alergênios moleculares para Phl p e Ole e. Métodos: Foram recrutados candidatos para imunoterapia com polens, com testes cutâneos positivos para Phl p e Ole e. Todos realizaram um painel de testes em picada a aeroalergênios e determinação de IgE séricas específicas para Phl p, Ole e, rPhl p1, rPhl p5, rPhl p7, rPhl p 12, rOle e 1, nOle e 7, rBet v2. Resultados: Foram incluídos 40 adultos. Em relação à sIgE para Phl p e Ole e, 83% e 65% dos pacientes apresentaram positividade para ambos, usando o cut-off de 0,35 kUA/L e 0,70 kUA/L, respectivamente. A positividade para Phl p1 e/ou Phl p 5 foi encontrada em 42,5%, para Ole e 1 apenas em 2,5%, enquanto 47,5% apresentaram sIgE positivo para ambos (cut-off corte de 0,35 kUA/L). Aumentando o cut-off para 0,7 kUA/L, 55% foram sensibilizados para Phl p1 e/ou Phl p5, nenhum paciente foi sensibilizado apenas para Ole e 1. Após a determinação dos alergênios para os componentes moleculares, a escolha de imunoterapia foi alterada em 15 (37,5%) pacientes, com uma diminuição no número de vacinas para Phleum + Olea e apenas para Olea e um aumento na prescrição de vacinas para Phleum. Conclusão: A sensibilização genuína do Olea europaea foi reduzida e os padrões de sensibilização foram heterogêneos. O conhecimento da sensibilização aos componentes moleculares dos alergênios mudou a prescrição de imunoterapia em mais de um terço dos pacientes.

Kounis syndrome - Report of 4 cases / Síndrome de Kounis - Relato de 4 casos

Kounis syndrome is described as the occurrence of myocardial injury following a hypersensitivity reaction triggered by allergen exposure. The actual incidence is unknown, as most of the information comes from case reports and there are no international recommendations. Kounis syndrome does not seem to be rare but rather underdiagnosed. We report and discuss the clinical presentation and management of 4 cases of Kounis syndrome. All patients presented with anaphylaxis and chest pain. Patient age ranged from 44 to 83 years. Anaphylaxis triggers were nonsteroidal anti-inflammatory drugs and antibiotics. It is important to recognize Kounis syndrome in order to conduct an adequate investigation and prevent further events. A major difficulty is that the treatment of the 2 entities (hypersensitivity reaction and acute coronary syndrome) may worsen each other. Large prospective studies are needed to establish definitive treatment guidelines for these patients.

A síndrome de Kounis caracteriza-se pela ocorrência de uma síndrome coronária aguda no contexto de uma reação alérgica concomitante desencadeada por exposição a um alergênio. A sua incidência real é desconhecida e não há consenso relativamente à abordagem, uma vez que a maioria dos dados provem de relatos de casos. A síndrome de Kounis não parece ser uma entidade rara, mas é infrequentemente diagnosticada. Apresentamos quatro casos, a sua apresentação clínica e abordagem diagnóstica e terapêutica. Todos os doentes, com idades entre os 44 e os 83 anos, se apresentaram com anafilaxia e dor torácica. Os fatores desencadeantes foram fármacos: anti-inflamatórios não esteroides e antibióticos. Torna-se importante reconhecer a síndrome de Kounis, de modo a que possa ser feita investigação adequada e prevenidos novos eventos. A grande dificuldade reside no fato de que o tratamento das duas entidades (Reação de hipersensibilidade e Síndrome coronária aguda), pode agravar uma à outra. São necessários estudos prospetivos alargados de modo a estabelecer diretrizes definitivas para o tratamento destes doentes.

Educação e democracia: do risco de desarticulação a uma recomposição crítica / Education and democracy: from the risk of disarticulation to a critical recomposition / Educación y democracia: del riesgo de desarticulación a una recomposición crítica / éducation et démocratie: du risque de désarticulation à une recomposition critique

Resumo Retornando a um tema que é um clássico em teoria da educação, o presente artigo procura respostas fundamentadas para um problema atual: a erosão da relação entre educação e democracia e o risco de assistirmos, se nada for feito, a uma desarticulação de ambas nos contextos em que a neoliberalização levou mais longe a economização de esferas e atividades, incluindo as não diretamente económicas. Voltado para as instituições escolares, e partindo da análise criteriosa desse fenómeno, o artigo aponta para uma recomposição crítica do alinhamento entre educação e democracia, especificando o que isso implica no percurso educativo dos mais novos nas escolas realmente existentes.

Abstract Returning to a theme that is a classic in the theory of education, this article seeks grounded answers to a current problem: the erosion of the relationship between education and democracy and the risk of witnessing, if nothing is done, a disarticulation of both in the contexts where neoliberalization has taken economization of spheres and activities further, including those not directly economic. Focused on school institutions, and based on a careful analysis of this phenomenon, the article points to a critical recomposition of the alignment between education and democracy, specifying what this implies in the educational path of the youngest in the really existing schools.

Resumen Volviendo a un tema que es un clásico en la teoría de la educación, el presente artículo busca respuestas razonadas a un problema actual: la erosión de la relación entre educación y democracia y el riesgo de presenciar, si no se hace nada, una desarticulación de ambas en los contextos donde la neoliberalización ha llevado más lejos la economización de esferas y actividades, incluidas las que no son directamente económicas. Dirigido a las instituciones escolares y basado en un análisis cuidadoso de este fenómeno, el artículo señala una recomposición crítica del alineamiento entre educación y democracia especificando lo que esto implica en el itinerario educativo de los más jóvenes en las escuelas realmente existentes.

Résumé Revenant sur un thème qui est déjà un classique de la théorie de l'éducation, cet article cherche des réponses concrètes à un problème actuel : l'érosion de la liaison entre éducation et démocratie et le risque d'assister, si rien n'est fait, à leur désarticulation dans les contextes où la néolibéralisation a poussé plus loin l'économisation de sphères et d'activités, y compris celles qui ne sont pas directement économiques. Centré sur les institutions scolaires et basé sur une analyse approfondie de ce phénomène, l'article pointe une recomposition critique de l'alignement entre éducation et démocratie et precise les implications dans le parcours scolaire des plus jeunes dans les écoles réellement existantes.

Diagnosis, treatment, and work impact of iron deficiency anemia in a Portuguese urban community.

INTRODUCTION: Little is known about iron deficiency anemia (IDA)'s treatment in Portugal. We aim to estimate the proportion of anemia, IDA, and iron deficiency without anemia; characterize the diagnostic procedures and prescription patterns; assess anemia's impact over work absenteeism, in a Local Health Unit. MATERIAL AND METHODS: Cross-sectional study that evaluated complete blood counts, iron-containing prescriptions, comorbidities, economic failure, and disability certificates issued in 2015 at the Local Health Unit. RESULTS: We evaluated 62,794 complete blood count. The proportion of anemia was 16.5%, higher in patients with economic failure, pregnant women, and patients with congestive heart failure. Of the patients with anemia 87.8% had not serum iron and/or ferritin dosing, and of those with serum iron/ferritin levels tested 50.6% had IDA. IDA was higher in pregnant women, women aged ≥15 years and in patients with congestive heart failure. Approximately 56.2% of patients with IDA did not receive iron-containing medication, and in 38% of the cases the prescribed dose was subtherapeutic. Of the total iron prescriptions 44.1% were association therapies. Anemia accounted for 5.2% of the disability certificates issued in 2015 (1749 workdays lost). DISCUSSION: Most patients with anemia are not being adequately evaluated and a major proportion does not undergo treatment or has subtherapeutic doses of iron. These results may explain the anemia's impact on work capacity. CONCLUSION: This is one of the largest studies on anemia in Portugal. An effort to adapt to the established recommendations is urged, to minimize the consequences of this disease.

Protein-conformational diseases in childhood: Naturally-occurring hIAPP amyloid-oligomers and early ß-cell damage in obesity and diabetes.

BACKGROUND AND AIMS: This is the first time that obesity and diabetes mellitus (DM) as protein conformational diseases (PCD) are reported in children and they are typically diagnosed too late, when ß-cell damage is evident. Here we wanted to investigate the level of naturally-ocurring or real (not synthetic) oligomeric aggregates of the human islet amyloid polypeptide (hIAPP) that we called RIAO in sera of pediatric patients with obesity and diabetes. We aimed to reduce the gap between basic biomedical research, clinical practice-health decision making and to explore whether RIAO work as a potential biomarker of early ß-cell damage. MATERIALS AND METHODS: We performed a multicentric collaborative, cross-sectional, analytical, ambispective and blinded study; the RIAO from pretreated samples (PTS) of sera of 146 pediatric patients with obesity or DM and 16 healthy children, were isolated, measured by sound indirect ELISA with novel anti-hIAPP cytotoxic oligomers polyclonal antibody (MEX1). We carried out morphological and functional studied and cluster-clinical data driven analysis. RESULTS: We demonstrated by western blot, Transmission Electron Microscopy and cell viability experiments that RIAO circulate in the blood and can be measured by ELISA; are elevated in serum of childhood obesity and diabetes; are neurotoxics and works as biomarkers of early ß-cell failure. We explored the range of evidence-based medicine clusters that included the RIAO level, which allowed us to classify and stratify the obesity patients with high cardiometabolic risk. CONCLUSIONS: RIAO level increases as the number of complications rises; RIAOs > 3.35 µg/ml is a predictor of changes in the current indicators of ß-cell damage. We proposed a novel physio-pathological pathway and shows that PCD affect not only elderly patients but also children. Here we reduced the gap between basic biomedical research, clinical practice and health decision making.

A First Sign Not to be Missed: Cutaneous Metastasis from Breast Cancer.

Cutaneous metastasis has a frequency of 1 to 10% among all metastatic cancer forms and breast cancer accounts for 30% of all cases. We report the case of a 73-year-old woman who presented with 4 skin lesions distributed across the upper trunk and abdomen; these had developed over a period of 12 months. Over the previous 6 months she had also developed anorexia, asthenia and weight loss. Upon investigation, a nodular mass was found in the left breast. Skin and breast mass biopsy were performed. Histology confirmed the diagnosis: infiltrating lobular breast cancer with cutaneous metastasis. The patient underwent hormonal treatment, mastectomy and radiotherapy. In rare cases, cutaneous metastasis appears as the first clinical manifestation of breast cancer. It is therefore crucial for patients and health professionals alike to be aware of new skin lesions. Cutaneous metastasis is a diagnostic sign of cancer that, it must be emphasised, is not restricted exclusively to later forms of the disease. LEARNING POINTS: Although breast cancer has the highest cutaneous metastasis rate among internal malignancies, cutaneous metastases actually occur in only a small minority of breast cancer patients. In rare cases, skin metastasis may appear as the first clinical manifestation of breast cancer.The clinical presentation of cutaneous metastasis from breast cancer can be varied, and therefore warrants greater awareness in clinical practice.Cutaneous metastasis from breast cancer typically carries a less unfavourable prognosis than cutaneous metastasis from other internal malignancies.

Contribution of molecular diagnosis to bee venom allergic patients with systemic reactions during the build-up phase of bee venom immunotherapy / Contribuição do diagnóstico molecular em doentes alérgicos ao veneno de abelha com reações sistêmicas durante o ultra-rush

Introduction: Bee venom (BV) allergy is one of the most common causes of severe anaphylaxis. Venom immunotherapy (VIT) is considered the most effective treatment, but systemic reactions may occur. This study aimed to characterize the sensitization profile by molecular components of patients with BV anaphylaxis under VIT and to evaluate whether systemic reactions during the build-up phase of VIT protocol are related to different sensitization patterns. Methods: A retrospective study of 30 patients under VIT for 1 year. The group of patients who reacted during the build-up phase (group A) was compared with the group with no reactions (group B). Specific IgE (sIgE) and IgG4 (sIgG4) for BV and recombinants (rApi m1, rApi m2, rApi m3, rApi m5, and rApi m10) were evaluated before and 1 year after VIT. Statistical analysis was performed using GraphPad Prism v5.01. Results: Men accounted for 80% of the sample, and mean age was 47 years (14-74 years). Group A consisted of 10 patients, and group B of 20 patients. Before VIT, sIgE to rApi m1 was detected in 86.7% of patients, rApi m2 in 46.7%, rApi m3 in 16.7%, rApi m5 in 43.3%, and rApi m10 in 70%. Positive results to at least 1 BV allergen were detected in 100%; 73% of patients were sensitized to >1 allergen, and 13.3% to all allergens. The profile of the two groups did not differ significantly before VIT, but group B showed a significant decrease in whole BV extract (p=0.045), rApi m 3 (p=0.017), and rApi m 10 (p=0.021) 1 year after VIT. Regarding sIgG4, there was a significant increase in rApi m1, which was not observed in other allergens, such as rApi m3 and rApi m10. Conclusion: The analysis of a panel of BV recombinants can improve diagnostic sensitivity, when compared to rApi m1 alone. There was no association between systemic reactions during the build-up phase of VIT and molecular sensitization profile. Nevertheless, it is important to study a greater number of patients.

Introdução: A alergia ao veneno de abelha (VA) é uma das causas mais comuns de anafilaxia grave. A imunoterapia com veneno de abelha (VIT) é considerada o tratamento mais eficaz, mas reações sistêmicas podem ocorrer. O objetivo deste estudo foi caracterizar o perfil de sensibilização por componentes moleculares de doentes com anafilaxia a VA e avaliar se reações sistêmicas durante o ultrarush estão relacionadas com diferentes padrões de sensibilização. Métodos: Estudo retrospectivo incluindo 30 doentes submetidos a VIT durante 1 ano. Considerou-se dois grupos: grupo de doentes que reagiu durante o ultra-rush (Grupo A), que foi comparado com o grupo sem reação (Grupo B). Foram avaliadas as IgE (sIgE) e IgG4 (sIgG4) específicas para VA(i1) e componentes moleculares: rApi m1, rApi m2, rApi m3, rApi m5 e rApi m10 antes e 1 ano após VIT. Os testes estatísticos foram realizados com Graph-PadPrism v5.01. Resultados: 80% sexo masculino, média de idade 47 anos (14-74). Grupo A com 10 doentes, Grupo B com 20 doentes. Previamente à VIT, sIgE para rApi m1 foi detectada em 86,7%; rApi m2 em 46,7%; rApi m3 em 16,7%; rApi m5 em 43,3%; e rApi m10 em 70%. Resultados positivos para pelo menos um alergênio de VA foram detectados em 100%. 73% dos doentes eram sensibilizados a mais de um alergênio, e 13,3% a todos os alergênios. Não houve diferenças estatisticamente significativas no perfil dos dois grupos antes da VIT, porém verificouse uma diminuição significativa: p = 0,045; p = 0,017 e p = 0,021 de i1, rApi m3 e rApi m10, respectivamente, no grupo B um ano após VIT. Relativamente à sIgG4, observou-se um aumento significativo de rApi m1, não observado nos restantes alergênios como rApi m3 e rApi m10. Conclusão: A análise de um painel de recombinantes de VA pode melhorar a sensibilidade diagnóstica, quando comparado com rApi m1 isolado. Não se verificou associação entre a ocorrência de reações sistêmicas durante o ultra-rush e o perfil de sensibilização molecular. No entanto, é importante para estudar um maior número de doentes.

Chlorhexidine: a hidden life-threatening allergen.

Chlorhexidine is a commonly used antiseptic and disinfectant in the health-care setting. Anaphylaxis to chlorhexidine is a rare but potentially life-threatening complication. Epidemiologic data suggest that the cases of chlorhexidine allergy appears to be increasing. In this article we report a life-threatening anaphylactic shock with cardiorespiratory arrest, during urethral catheterization due to chlorhexidine. The authors also performed a literature review of PubMed library of anaphylactic cases reports due to this antiseptic between 2014 and 2018, demonstrating the increase in the number of cases occurring worldwide and the importance of detailed anamnesis and appropriate diagnostic workup of allergic reactions to disinfectants.

Hospitalizations due to Angioedema without Urticaria in a Portuguese Center: Five Year Retrospective Study.

INTRODUCTION: Hospitalizations due to angioedema are important especially in debilitating or life-threatening situations. The aim of this study was to evaluate the frequency and etiology of angioedema without urticaria in hospital admissions. MATERIAL AND METHODS: The admissions between 2009 and 2013 in Centro Hospitalar Lisboa Norte with a diagnosis grouped under the ICD9 codes of angioedema were retrospectively analysed. The episodes of angioedema with urticaria were excluded. The admissions were categorized into 2 groups: A - hospitalizations motivated by the angioedema; B - hospitalizations in which the angioedema was an incidental finding. RESULTS: There were 169 hospitalizations (52% females, 96% adults, mean age 52 ± 20.8 years), distributed by 23 hospital departments, 51% in the Immunoallergology department. The mean annual angioedema admission rate was 72/100 000. In 68% of the cases, angioedema was the cause for the admission; in 32% an incidental finding. In 38% there was upper airway involvement. The etiologies were: hereditary angioedema in 24%, angiotensin converting enzyme inhibitor induced angioedema in 31%, idiopathic angioedema in 21%, thrombolysis induced angioedema in 13%, nonsteroidal anti-inflammatory drug-induced angioedema in 5%. DISCUSSION: The main etiology was angiotensin converting enzyme inhibitor angioedema, followed by hereditary angioedema and thrombolysis induced angioedema, and these findings concur with the international literature. CONCLUSION: The mean annual angioedema admission rate was 72/100 000 and there was airway involvement in 38% of hospitalizations.

Introdução: Os internamentos por angioedema são importantes, sobretudo, nas situações incapacitantes ou de risco de vida. O objetivo deste estudo foi avaliar a frequência e etiologia dos internamentos por angioedema sem urticária. Material e Métodos: Estudo retrospetivo dos internamentos com os códigos CID9 para angioedema, entre 2009 e 2013, no Centro Hospitalar Lisboa Norte. Foram excluídos os episódios de angioedema com urticária. Categorizaram-se os internamentos em dois grupos: A ­ Internamentos motivados pela crise de angioedema; B ­ Internamentos em que o angioedema foi uma intercorrência. Resultados: Foram incluídos 169 internamentos (53% mulheres, 96% adultos, idade média 52 ± 20,8 anos), distribuídos por 23 serviços hospitalares (51% na Imunoalergologia), com uma taxa média anual de internamentos de 72/ 100 000. Em 68% o angioedema foi o motivo de internamento, em 32% uma intercorrência. Em 38% houve envolvimento das vias aéreas superiores. As etiologias foram: em 24% angioedema hereditário, em 31% angioedema induzido por inibidores da enzima conversora da angiotensina, 21% foram idiopáticos, em 13% o angioedema surgiu após trombólise, em 5% induzido por anti-inflamatórios não esteroides e 7% outras etiologias. Discussão: A principal etiologia foi o angioedema induzido por inibidores da enzima conversora da angiotensina, seguido do angioedema hereditario e angioedema associado a trombolise, sendo estes dados semelhantes a outras revisões internacionais. Conclusão: A taxa média de internamentos por angioedema foi de 72/100 000 e a frequência de envolvimento das vias aéreas foi de 38%.

Esofagite eosinofílica numa consulta de alergia alimentar: caracterização e comparação entre idade pediátrica e idade adulta / Eosinophilic esophagitis at a food allergy appointment: characterization and comparison between pediatric and adult ages

Introdução: Esofagite eosinofílica (EoE) é uma doença inflamatória crônica do esôfago, mediada imunologicamente e caracterizada por sintomas relacionados com disfunção esofágica e infiltração da mucosa esofágica por eosinófilos (Eo). Os objetivos foram caracterizar os doentes com diagnóstico de EoE e analisar as diferenças entre doentes com diagnóstico em idade pediátrica (Cr, < 18 anos) e adulta (Ad, ≥ 18 anos). Métodos: Estudo observacional retrospetivo dos doentes seguidos no serviço de Imunoalergologia, no período de Fev/2009 a Jul/2017, com diagnóstico de EoE. Foram divididos em dois grupos, Cr e Ad, caracterizados de acordo com dados demográficos, história de atopia, sintomas, sensibilizações alimentares, IgE Total, eosinofilia, achados na endoscopia digestiva alta e biópsias. Avaliou-se a correlação entre sensibilização alimentar, clínica grave (ClinG), ou seja, idas ao serviço de urgência ou internamento por complicações de EoE ou histologia grave (HistG), biópsia com Eo > 50 e/ou microabcessos. Resultados: 74 pacientes (81% sexo masculino, média de idades 27±17 anos), 36 Cr e 38 Ad. Os sintomas mais frequentemente reportados foram, no grupo Cr disfagia (73%) e refluxo gastroesofágico (46%), enquanto no grupo Ad impactação (85%) e disfagia (56%). Foram referidos antecedentes de atopia em 96% das Cr, e 67% dos Ad. Em 77% das Cr e 69% dos Ad havia sensibilização alimentar. Os achados endoscópicos mais frequentes no grupo Cr foram estriação (65%) e placas brancas (50%), enquanto que no grupo Ad foram placas brancas (42%) e anéis esofágicos (35%). HistG (46%) associou-se a ClinG (35%), p = 0,001, nas Cr, mas o mesmo não foi objetivado no grupo Ad [ClinG (22%) e HistG (17%), p = 0,5]. Conclusão: Os nossos resultados estão de acordo com o descrito na literatura, observando-se um predomínio do sexo masculino e uma maior frequência de história de atopia e sensibilização alimentar no grupo Cr. As situações graves de impactação e estenose esofágica foram mais frequentes nos Ad, e objetivou-se uma associação de histologia grave com clínica grave, apenas nas Cr.

Background: Eosinophilic esophagitis (EoE) is an immunologically mediated chronic inflammatory disease of the esophagus characterized by symptoms related to esophageal dysfunction and eosinophilic infiltration in the esophageal mucosa. This study aimed to characterize patients with a diagnosis of EoE and analyze differences between patients with EoE diagnosed at pediatric age (Ch, <18 years) and at adult age (Ad, ≥18 years). Methods: This was a retrospective study of patients with a diagnosis of EoE who were followed in the immunoallergology department from February 2009 to July 2017. Patients were divided into Ch and Ad groups and characterized according to demographic data, history of atopy, symptoms, food sensitization, total IgE, eosinophils (Eo), upper digestive endoscopy (UDE) findings, and biopsy findings. Correlations were assessed between food sensitization, clinical severity (SClin; determined by number of ER visits or hospital admissions for EoE complications), and severe histology (SHist; defined as biopsy with Eo >50 and/or microabscesses). Results: 74 patients (81% male, mean age 27±17 years), 36 Ch and 38 Ad. The most commonly reported symptoms were dysphagia (73%) and gastroesophageal reflux (46%) in the Ch group, and impaction (85%) and dysphagia (56%) in the Ad group. History of atopy was reported in 96% of Ch vs 67% of Ad. Food sensitization was found in 77% of Ch vs 69% of Ad. The most common UDE findings were striation (65%) and white plaques (50%) in the Ch group, and white plaques (42%) and esophageal rings (35%) in the Ad group. SHist (46%) was associated with SClin (35%) in Ch (p=0.001), but not in Ad (SClin [22%] and SHist [17%], p=0.5). Conclusion: Our results are consistent with those reported in the literature, with a male predominance and a higher prevalence of atopy and food sensitization in Ch. Severe impaction and esophageal stenosis were more frequent in Ad, but an association between SHist and SClin was found only in Ch.

Psychiatric Symptoms and Benzodiazepine Use Mask a Patent Foramen Ovale-Related Stroke: A Cautionary Tale.

Neurological and psychiatric diseases often present with overlapping features. Patients are sometimes seen by internal medicine, neurology and psychiatry clinicians and time can be important for determining whether a condition is organic or not. The authors present the case of a 31-year-old woman admitted to the emergency department with altered mental status and incomprehensible speech. She had a previous history of anxiety and smoking and was under therapy with benzodiazepines and combined hormonal contraceptives. After an acute neurological disorder was ruled out, the patient was transferred to the psychiatry department, where she was re-evaluated after 12 hours of observation and benzodiazepine washout. As speech impairment and confusion were still present, she was transferred to the Stroke Unit. A bilateral thalamic lesion containing a small focal haemorrhage and a moderate grade right-to-left shunt were identified. A patent foramen ovale was confirmed leading to the final diagnosis of acute ischaemic infarction secondary to a paradoxical embolism. Medical therapy was preferred over percutaneous closure. Risk factors were removed (smoking and hormonal contraceptives), and after the patient recovered completely acetylsalicylic acid 100 mg daily was initiated. LEARNING POINTS: Psychiatric symptoms and medication often mask organic conditions, so observation and re-evaluation are key.There are new guidelines for the treatment of patent foramen ovale-related strokes, but not all patients fit the guidelines.A relationship between stroke in the young adult, smoking and hormonal contraceptives should always be considered.

The Perfect Storm: A Case of Non-Uremic Calciphylaxis.

Calciphylaxis is a severe condition associated with chronic kidney disease. We describe a rare case of non-uremic calciphylaxis in a 79-year-old woman with prosthetic aortic and mitral valves, chronic kidney disease and anaemia, who presented with painful skin lesions on her left leg. She was taking warfarin, calcium and iron supplements, and had been recently diagnosed with oligoarthritis and started on prednisolone, all factors associated with calciphylaxis. Despite treatment, the lesions rapidly progressed to large ulceration and sepsis, and pain management was challenging. Calciphylaxis was confirmed following a second skin biopsy but the patient died from septic shock and multi-organ failure. This case highlights the challenges associated with the diagnosis and treatment of calciphylaxis despite a high level of suspicion. LEARNING POINTS: The number of reported cases of calciphylaxis, once considered very rare, is increasing.Physicians should be aware of new information concerning the condition.Treatment includes a low antibiotic threshold, comprehensive pain management and efficient wound care.

Listeria monocytogenes as a Cause of Spontaneous Bacterial Peritonitis.

Spontaneous bacterial peritonitis is a common and often serious complication of long standing ascites in the presence of advanced liver disease. We report a case of a 74-year-old woman with alcoholic cirrhosis admitted to our emergency department with a 1-week history of diffuse abdominal pain and increased abdominal perimeter. On physical examination, she had a significant abdominal distention and a positive fluid wave consistent with ascites. A diagnosis of spontaneous bacterial peritonitis was made and empiric therapy with ceftriaxone was started but the patient's condition worsened. Four days later Listeria monocytogenes was detected in peritoneal fluid culture and ampicillin was initiated according to in vitro sensibility test. The patient completed 14 days of treatment with clinical and laboratory improvement. Listeria is an uncommon cause of spontaneous bacterial peritonitis. Clinicians should be aware of the uncommon agents of spontaneous bacterial peritonitis, mainly when there is not a proper response to therapy.