Acquired Angioedema due to C1-Inhibitor Deficiency: A Challenging Condition.

BACKGROUND: Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema. METHODS: Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Clinical data collected included sex, date of birth, date of onset of symptoms, date of diagnosis, plasma levels of antigenic and/or functional C1-INH, levels of C4 and C1q, location and treatment of angioedema attacks, long-term prophylaxis, associated diseases, and definitive treatment. RESULTS: Fourteen patients were identified with AAE-C1-INH. Most patients (10/14; 71.4%) were female. The median age at onset of symptoms was 56.5 years (range, 14-74 years; interquartile range [IQR], 32-64 years), and median age at diagnosis was 58.0 years (range, 20-76 years; IQR, 38-65 years), with a median time until diagnosis of 2 years (range, 0-6 years; IQR, 1-3 years). The most common manifestations were cutaneous (face, eyelids, lips, trunk, hands, feet, and genitals). Most patient had low levels of C4 (13/14; 92.8%) and of antigenic C1-INH (8/14; 57.1%). Four had decreased functional activity of C1-INH (4/7; 57.1%) and C1q levels were low in 5 patients (5/12; 41.6%). Underlying diseases were identified in all 14 patients, with lymphoma of the splenic marginal zone and monoclonal gammopathy of undetermined significance being the most frequent. Nine patients (64.2%) needed long-term prophylactic treatment for recurrent angioedema and 5 patients (46.7%) required treatment for angioedema attacks. Most of them (12/14; 85.7%) had resolution of angioedema. CONCLUSION: Therapy of AAE-C1-INH aims to control symptoms; however, diagnosis and treatment of the underlying disease, when present, should be an important target and may lead to the resolution of angioedema in patients with AAE-C1-INH.

Outcome of SARS-CoV-2 Infection in 121 Patients with Inborn Errors of Immunity: A Cross-Sectional Study.

PURPOSE: There is still scarce data on SARS-CoV-2 infection in patients with Inborn Errors of Immunity (IEI) and many unresolved questions. We aimed to describe the clinical outcome of SARS-CoV-2 infection in Brazilian IEI patients and identify factors influencing the infection. METHODS: We did a cross-sectional, multicenter study that included patients of any age affected by IEI and SARS-CoV-2 infection. The variables studied were sex, age, type of IEI, comorbidities (number and type), treatment in use for IEI, clinical manifestations and severity of SARS-CoV-2 infection. RESULTS: 121 patients were included: 55.4% female, ages from six months to 74 yo (median age = 25.1 yo). Most patients had predominantly antibody deficiency (n = 53). The infection was mostly asymptomatic (n = 21) and mild (n = 66), and one child had multisystem inflammatory syndrome (MIS-C). We could not observe sex-related susceptibility, and there was a weak correlation between age and severity of infection. The number of comorbidities was higher in severe cases, particularly bronchiectasis and cardiopathy. There were no severe cases in hereditary angioedema patients. Six patients aged 2 to 74 years died, three of them with antibody deficiency. CONCLUSION: The outcome was mild in most patients, but the Case Fatality Ratio was higher than in the general population. However, the type of IEI was not a determining factor for severity, except for complement deficiencies linked to milder COVID-19. The severity of SARS-CoV-2 infection seems to be more related to older age, a higher number of comorbidities and type of comorbidities (bronchiectasis and cardiopathy).

[Update on perioperative hypersensitivity reactions: joint document of the Brazilian Society of Anesthesiology (SBA) and Brazilian Association of Allergy and Immunology (ASBAI) - Part I: post-crisis guidelines and treatment]. / Atualização sobre reações de hipersensibilidade perioperatória: documento conjunto da Sociedade Brasileira de Anestesiologia (SBA) e Associação Brasileira de Alergia e Imunologia (ASBAI) ­ Parte I: tratamento e orientação pós­crise.

Experts from the Brazilian Association of Allergy and Immunology (ASBAI) and the Brazilian Society of Anesthesiology (SBA) interested in the issue of perioperative anaphylaxis, and aiming to strengthen the collaboration between the two societies, combined efforts to study the topic and to prepare a joint document to guide specialists in both areas. The purpose of the present series of two articles was to report the most recent evidence based on the collaborative assessment between both societies. This first article will consider the updated definitions, treatment and guidelines after a perioperative crisis. The following article will discuss the major etiologic agents, how to proceed with the investigation, and the appropriate tests.

[Update on perioperative hypersensitivity reactions: joint document from the Brazilian Society of Anesthesiology (SBA) and Brazilian Association of Allergy and Immunology (ASBAI) - Part II: etiology and diagnosis]. / Atualização sobre reações de hipersensibilidade perioperatória: documento conjunto da Sociedade Brasileira de Anestesiologia (SBA) e Associação Brasileira de Alergia e Imunologia (ASBAI) ­ Parte II: etiologia e diagnóstico.

This second joint document, written by experts from the Brazilian Association of Allergy and Immunology (ASBAI) and Brazilian Society of Anesthesiology (SBA) concerned with perioperative anaphylaxis, aims to review the pathophysiological reaction mechanisms, triggering agents (in adults and children), and the approach for diagnosis during and after an episode of anaphylaxis. As anaphylaxis assessment is extensive, the identification of medications, antiseptics and other substances used at each setting, the comprehensive data documentation, and the use of standardized nomenclature are key points for obtaining more consistent epidemiological information on perioperative anaphylaxis.

Central obesity and other factors associated with uncontrolled asthma in women.

BACKGROUND: Asthma remains an uncontrolled disease. The Comorbidities, particularly obesity, and several other factors have been identified as being individually associated with asthma control, and these factors vary among different countries and between sexes. Studies have suggested that the harmful effects of these factors are greatest among women. The aim of the present study was to identify associated factors with uncontrolled asthma in women at the outpatient clinic of a Federal University Hospital in Rio de Janeiro, Brazil. METHODS: Cross-sectional study with asthmatic women, older than 18 years old. All subjects were included according to stringent criteria. The study used a structured questionnaire with data about demographic variables, education level, comorbid conditions, and disease history. Anthropometric and spirometric measurements were obtained. Asthma control was assessed by three different tools: the Asthma Control Test (ACT), the Asthma Control Questionnaire (ACQ) and the Global Initiative for Asthma (GINA) criteria. RESULTS: A total of 124 women were included, and 57%, 38% and 21% were totally controlled according to ACT, ACQ and GINA criteria, respectively. A total of 31.5% were obese. According to the CI (Conicity Index) and WC (waist circumference) respectively, 84% and 68% were centrally obese. There was no association between asthma control and age, education, duration of the disease or BMI (Body Mass Index) in the three models, but there was a significant association between central obesity and asthma control with the ACQ and GINA assessment tools, controlling for explanatory variables such as GERD (gastroesophageal reflux disease). Pre-bronchodilator percent predicted forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) were significantly associated with age and FVC was also associated with central obesity. CONCLUSIONS: Asthma remains uncontrolled in women despite treatment, and central obesity seems to have a negative influence on the control of the disease. We believe that women should be studied as a separate group and suggest prospective studies with assessment of fat distribution and biomarkers, controlling for possible comorbidities associated with asthma control.

Update on perioperative hypersensitivity reactions: joint document of the Brazilian Society of Anesthesiology (SBA) and Brazilian Association of Allergy and Immunology (ASBAI) - Part I: post-crisis guidelines and treatment / Atualização sobre reações de hipersensibilidade perioperatória: documento conjunto da Sociedade Brasileira de Anestesiologia (SBA) e Associação Brasileira de Alergia e Imunologia (ASBAI) - Parte I: tratamento e orientação pós-crise

Abstract Experts from the Brazilian Association of Allergy and Immunology (ASBAI) and the Brazilian Society of Anesthesiology (SBA) interested in the issue of perioperative anaphylaxis, and aiming to strengthen the collaboration between the two societies, combined efforts to study the topic and to prepare a joint document to guide specialists in both areas. The purpose of the present series of two articles was to report the most recent evidence based on the collaborative assessment between both societies. This first article will consider the updated definitions, treatment and guidelines after a perioperative crisis. The following article will discuss the major etiologic agents, how to proceed with the investigation, and the appropriate tests.

Resumo Especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e da Sociedade Brasileira de Anestesiologia (SBA) interessados no tema anafilaxia perioperatória reuniram-se com o objetivo de intensificar a colaboração entre as duas sociedades no estudo desse tema e elaborar um documento conjunto que possa guiar os especialistas de ambas as áreas. O objetivo desta série de dois artigos foi mostrar as evidências mais recentes alicerçadas na visão colaborativa entre as sociedades. Este primeiro artigo versará sobre as definições mais atuais, formas de tratamento e as orientações após a crise no perioperatório. No próximo artigo serão discutidos os principais agentes causais e a condução da investigação com testes apropriados.

Update on perioperative hypersensitivity reactions: joint document from the Brazilian Society of Anesthesiology (SBA) and Brazilian Association of Allergy and Immunology (ASBAI) - Part II: etiology and diagnosis / Atualização sobre reações de hipersensibilidade perioperatória: documento conjunto da Sociedade Brasileira de Anestesiologia (SBA) e Associação Brasileira de Alergia e Imunologia (ASBAI) - Parte II: etiologia e diagnóstico

Abstract This second joint document, written by experts from the Brazilian Association of Allergy and Immunology (ASBAI) and Brazilian Society of Anesthesiology (SBA) concerned with perioperative anaphylaxis, aims to review the pathophysiological reaction mechanisms, triggering agents (in adults and children), and the approach for diagnosis during and after an episode of anaphylaxis. As anaphylaxis assessment is extensive, the identification of medications, antiseptics and other substances used at each setting, the comprehensive data documentation, and the use of standardized nomenclature are key points for obtaining more consistent epidemiological information on perioperative anaphylaxis.

Resumo Este segundo documento, escrito por especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e da Sociedade Brasileira de Anestesiologia (SBA) interessados no tema anafilaxia perioperatória, tem por objetivo revisar os mecanismos fisiopatológicos, agentes desencadeantes (em adultos e crianças), assim como a abordagem diagnóstica durante e após o episódio. Por se tratar de uma avaliação abrangente, a identificação das medicações, antissépticos e outras substâncias usadas em cada região, registros detalhados, e nomenclatura padronizada são pontos fundamentais para a obtenção de dados epidemiológicos mais fidedignos sobre a anafilaxia perioperatória.

Atualização sobre reações de hipersensibilidade perioperatória: Documento conjunto da Sociedade Brasileira de Anestesiologia e Associação Brasileira de Alergia e Imunologia ­ Parte II: etiologia e diagnóstico / Update on perioperative hypersensitivity reactions: Joint document from the Brazilian Society of Anesthesiology and Brazilian Association of Allergy and Immunology ­ Part II: Etiology and diagnosis

A anafilaxia perioperatória é manifestação importante no contexto de eventos adversos relacionados à cirurgia. Embora frequentemente relacionada à indução anestésica, pode ocorrer por outros agentes administrados por outras vias. A anafilaxia pode se apresentar como colapso cardiovascular, obstrução da via aérea e/ou insuficiência respiratória com ou sem manifestação cutânea, com consequências fatais em muito casos. Apesar de considerada inevitável em alguns casos, a sua incidência poderia (e deveria) ser reduzida através da busca por fármacos mais seguros. A avaliação abrangente de um episódio é um dos elementos primordiais para tornar a exposição subsequente mais segura, com orientações derivadas dessa investigação. Entretanto, representa um desafio estatístico por ser reação rara, randômica e muitas vezes independente de exposições sucessivas dos pacientes a procedimentos de baixo risco. Neste documento são revisados os mecanismos fisiopatológicos, agentes desencadeantes (adultos e crianças), assim como a abordagem diagnóstica durante a crise e após o episódio. Uma avaliação abrangente, a identificação das medicações, antissépticos e outras substâncias usadas em cada região, registros detalhados e nomenclatura padronizada são pontos fundamentais para a obtenção de dados epidemiológicos mais fidedignos sobre a anafilaxia perioperatória.

Perioperative anaphylaxis is an important manifestation in the context of surgery-related adverse events. Although often related to anesthetic induction, it may be caused by other agents administered by other routes. Anaphylaxis may manifest as cardiovascular collapse, airway obstruction and/or respiratory failure with or without skin manifestation, resulting often in death. Although this reaction is considered inevitable in some cases, its incidence could (and should) be reduced by the search for safer drugs. Comprehensive assessment of an allergic reaction is a key element to make subsequent exposure safer, with guidance derived from this investigation. However, surveillance of perioperative anaphylaxis represents a statistical challenge because this is a rare, random reaction and often independent of successive patient exposures to low-risk procedures. This paper reviews pathophysiological mechanisms, triggering agents (adults and children), as well as therapeutic and diagnostic approach during and after an allergic reaction. Comprehensive assessment, identification of medications/antiseptics used in each region and detailed records with standardized terminology are key points for obtaining more reliable epidemiological data on perioperative anaphylaxis.

Abordagem das reações de hipersensibilidade perioperatória: Orientações da Sociedade Brasileira de Anestesiologia e da Associação Brasileira de Alergia e Imunologia - Parte II: etiologia e diagnóstico / Approach to perioperative hypersensitivity reactions: Guidelines from the Brazilian Society of Anesthesiology and the Brazilian Association of Allergy and Immunology - Part II: etiology and diagnosis

A anafilaxia perioperatória é manifestação importante no contexto de eventos adversos relacionados à cirurgia. Embora frequentemente relacionada à indução anestésica, pode ocorrer por outros agentes administrados por outras vias. A anafilaxia pode se apresentar como colapso cardiovascular, obstrução da via aérea e/ou insuficiência respiratória com ou sem manifestação cutânea, com consequências fatais em muito casos. Apesar de considerada inevitável em alguns casos, a sua incidência poderia (e deveria) ser reduzida através da busca por fármacos mais seguros. A avaliação abrangente de um episódio é um dos elementos primordiais para tornar a exposição subsequente mais segura, com orientações derivadas dessa investigação. Entretanto, representa um desafio estatístico por ser reação rara, randômica e muitas vezes independente de exposições sucessivas dos pacientes a procedimentos de baixo risco. Neste documento são revisados os mecanismos fisiopatológicos, agentes desencadeantes (adultos e crianças), assim como a abordagem diagnóstica durante a crise e após o episódio. Uma avaliação abrangente, a identificação das medicações, antissépticos e outras substâncias usadas em cada região, registros detalhados e nomenclatura padronizada são pontos fundamentais para a obtenção de dados epidemiológicos mais fidedignos sobre a anafilaxia perioperatória.

Perioperative anaphylaxis is an important manifestation in the context of surgery-related adverse events. Although often related to anesthetic induction, it may be caused by other agents administered by other routes. Anaphylaxis may manifest as cardiovascular collapse, airway obstruction and/or respiratory failure with or without skin manifestation, resulting often in death. Although this reaction is considered inevitable in some cases, its incidence could (and should) be reduced by the search for safer drugs. Comprehensive assessment of an allergic reaction is a key element to make subsequent exposure safer, with guidance derived from this investigation. However, surveillance of perioperative anaphylaxis represents a statistical challenge because this is a rare, random reaction and often independent of successive patient exposures to low-risk procedures. This paper reviews pathophysiological mechanisms, triggering agents (adults and children), as well as therapeutic and diagnostic approach during and after an allergic reaction. Comprehensive assessment, identification of medications/antiseptics used in each region and detailed records with standardized terminology are key points for obtaining more reliable epidemiological data on perioperative anaphylaxis.

Atualização sobre reações de hipersensibilidade perioperatória: Documento conjunto da Sociedade Brasileira de Anestesiologia e Associação Brasileira de Alergia e Imunologia ­ Parte I: Tratamento e orientação pós-crise / Update on perioperative hypersensitivity reactions: Joint document from the Brazilian Society of Anesthesiology and Brazilian Association of Allergy and Immunology - Part I: Post-event management and guidance

Especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e da Sociedade Brasileira de Anestesiologia (SBA), interessados no tema anafilaxia perioperatória, reuniram-se com o objetivo de intensificar a colaboração entre as duas sociedades no estudo desse tema e elaborar um documento conjunto que possa guiar ambos os especialistas. O objetivo desta série de dois artigos foi mostrar as evidências mais recentes alicerçadas na visão colaborativa entre as sociedades. Este primeiro artigo versará sobre as definições mais atuais, formas de tratamento e as orientações após a crise no perioperatório. No próximo artigo serão discutidos os principais agentes causais e a condução da investigação com testes apropriados.

Experts from the Brazilian Association of Allergy and Immunology (ASBAI) and Brazilian Society of Anesthesiology (SBA), interested in the topic of perioperative anaphylaxis, have met to strengthen collaboration between the two societies in the study of this topic and to draft a joint document that can provide guidance to members of both societies. The purpose of this series of two articles is to provide the latest evidence based on the collaborative view of both societies. This first article will cover the most current definitions, treatment modalities, and guidelines for management after a perioperative event. The second article will discuss major causative agents and whether investigation has been conducted with proper tests.

Impacto da Neuro-AIDS na infância / The influence of NEURO-AIDS in children

Introdução: a influência da infecção do sistema nervoso central no neurodesenvolvimento da criança com infecção pelo HIV-1 merece melhor compreensão. Objetivo: identificar as desordens neurológicas da infecção pelo HIV-1 em crianças e analisar possíveis fatores associados. Métodos: revisão de mais de 100 artigos recentemente publicados na literatura médica nacional e internacional referentes à patogênese da neuro-aids e sua implicação no desenvolvimento neurológico da criança com infecção pelo HIV-1. Resultados: o HIV-1 penetra precocemente no sistema nervoso central (SNC) e seu acometimento pode representar a manifestação inicial da aids em até 18% das crianças infectadas. Na neuropatogênese da aids na criança, destacam-se as interações virais com astrócitos. A diferença nos níveis de citocinas no líquor e a infecção ativa de neurônios são as principais diferenças da infecção do SNC pelo HIV-1 quando comparada com adultos. Após a era HAART, os quadros de encefalopatia diminuíram significativamente, permanecendo as alterações no neurodesenvolvimento, cuja prevalência varia de 8% a mais de 60%. Demonstra-se a associação com a gravidade da infecção, idade da criança, mas também com regimes de HAART eficientes. Os diferentes trabalhos analisados apresentam resultados contraditórios quanto à influência de fatores de risco no neurodesenvolvimento de crianças infectadas pelo HIV-1. Conclusão: os mecanismos da neuropatogênese da infecção pelo HIV são extremamente complexos e ainda pouco compreendidos. Ostrabalhos apontam que atraso no neurodesenvolvimento pode ser importante indicador de progressão da doença. Identificar precocemente, por meio de instrumentos de avaliação de neurodesenvolvimento, sinais associados a desordens do SNC em crianças infectadas pelo HIV-1 é essencial.

Introduction: the influence of the infection of the central nervous system in the neurodevelopment of the child infected with HIV-1 deserves better understanding. Objective: to evaluate neurological disorders in HIV-1 infected children and possible associated factors. Methods: review of more than 100 papers published recently in national and international medical literature concerning neuropathogenesis of AIDS and its influence in the neurological development of HIV infected children. Results: HIV-1 invades the central nervous system (CNS) early in infection and is the first AIDS-defining illness in as many as 18% of pediatric patients. In the neuropathogenesis of AIDS in children we must stress the viral interactions with astrocytes. Differences in the level of proinflammatory mediators in the cerebrospinal fluid (CSF), and the productive infection of neurons are the main differences in children. The reported prevalence of delay in neurodevelopment among HIV-infected children has varied from 8% to more than 60%. An increased prevalence is associated with the severity of the infection, increasing age, but also occur with children with efficient HAART regimens. Papers selected have distinct conclusions in relation to the risk factors associated with neurodevelpmental delays of HIV infected children. Conclusion: the molecular and cellular mechanisms of the neuropathogenesis of HIV-1 infection are extremely complex and not well understood. Neurodevel pmental delay is clearly associated with HIV-1 infection in children and can be an important indicator of the infection evolution. It is essential to idenfity, using neurodevelopmental assessment tools, early warning signs of HIV-1 associated neurological disorders in infected children.