Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease. The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year. The Experts in Clinical Immunology and Allergy of the "Associação Brasileira de Alergia e Imunopatologia -ASBAI" developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.

OBJECTIVES: The aims of the Online Latin American Survey of Anaphylaxis (OLASA) were to identify the main clinical manifestations, triggers, and treatments of severe allergic reactions in patients who were seen by allergists from July 2008 to June 2010 in 15 Latin American countries and Portugal (n =634). RESULTS: Of all patients, 68.5% were older than 18 years, 41.6% were male, and 65.4% experienced the allergic reaction at home. The etiologic agent was identified in 87.4% of cases and predominantly consisted of drugs (31.2%), foods (23.3%), and insect stings (14.9%). The main symptom categories observed during the acute episodes were cutaneous (94.0%) and respiratory (79.0%). The majority of patients (71.6%) were treated initially by a physician (office/emergency room) within the first hour after the reaction occurred (60.2%), and 43.5% recovered in the first hour after treatment. Most patients were treated in an emergency setting, but only 37.3% received parenteral epinephrine alone or associated with other medication. However, 80.5% and 70.2% were treated with corticosteroids or antihistamines (alone or in association), respectively. A total of 12.9% of the patients underwent reanimation maneuvers, and 15.2% were hospitalized. Only 5.8% of the patients returned to the emergency room after discharge, with 21.7% returning in the first 6 hours after initial treatment. CONCLUSION: The main clinical manifestations of severe allergic reactions were cutaneous. The etiologic agents that were identified as causing these acute episodes differed according to age group. Following in order: drugs (31.2%), foods (23.3% and insect stings (14.9%) in adults with foods predominance in children. Treatment provided for acute anaphylactic reactions was not appropriate. It is necessary to improve educational programs in order to enhance the knowledge on this potentially fatal emergency.

OBJETIVO: Avaliar o conhecimento de pediatras sobre alergia alimentar. MÉTODOS: Dados obtidos de questionário padronizado, postado e respondido por pediatras filiados à Sociedade Brasileira de Pediatria (SBP). Digitação dos dados em planilha Excel e análise de freqüência de respostas afirmativas em porcentagem. RESULTADOS: Foram analisados 895 questionários preenchidos por pediatras de todo o país, com predomínio da região Sudeste (61,6%). Segundo os pediatras entrevistados, as manifestações diagnósticas de alergia alimentar são: respiratórias, cutâneas e sistêmicas, em iguais proporções. Ainda segundo estes pediatras, leite de vaca (98,9%), clara de ovo (58,7%) e amendoim (50,9%) são os principais alimentos associados a essas manifestações. Embora 74,8% dos respondedores tivessem identificados os corantes e aditivos alimentares como responsáveis pela alergia alimentar, apenas 19,4% conheciam o código de identificação da tartrazina. CONCLUSÕES: Os dados apresentados reforçam a necessidade de ampliação dos conhecimentos dos profissionais de saúde sobre o diagnóstico e tratamento da alergia alimentar, com o objetivo de garantir o uso de critérios diagnósticos e terapêuticos mais adequados.

OBJECTIVE: To evaluate the knowledge of Brazilian pediatricians about food allergy. METHODS: Data was obtained from a sent back posted written questionnaire. It was filled in by Brazilian pediatricians, affiliated to the Brazilian Society of Pediatrics. Data was transcript to an Excel spread sheet and the frequency of affirmative responses was reported as percentages. RESULTS: Data from 895 written questionnaire of pediatricians from all over Brazil, (mainly from the southeastern region - 61.6%), were analyzed. The main clinical expressions of food allergy determined by the pediatricians were: respiratory, cutaneous and systemic symptoms (equal proportions). According to these pediatricians, cow's milk (98.9%), egg white (58.7%) and peanuts (50.9%) were the main allergens related to food allergy symptoms. Although 74.8% of the responders have indicated food dyes and food additives as associated to food allergies, only 19.4% of them knew the identification code of tartrarzine. CONCLUSIONS: The presented data reinforce the need to improve the pediatricians' knowledge about the diagnosis and treatment of food allergy, in order to assure the use of appropriate diagnostic and treatment criteria.

OBJETIVO: Avaliar o papel da sensibilização cutânea a diferentes aeroalérgenos em pacientes com ceratoconjuntivite vernal e a correlação entre esta e os aspectos clínicos da doença. MÉTODOS: Vinte pacientes do setor de doenças externas e córnea foram aleatoriamente convidados para participar deste estudo. Os pacientes foram avaliados e a eles foi atribuído um escore clínico variando de 0 a 20 de acordo com sinais e sintomas presentes no exame oftalmológico. Todos os pacientes foram submetidos a testes cutâneos de hipersensibilidade imediata contra aeroalérgenos padronizados como os ácaros domiciliares D. pteronyssinus, D. farinae e Blomia tropicalis, assim como também a alérgenos de epitélio de gato, epitélio de cão, mistura de fungos e mistura de penas. RESULTADOS: Setenta e cinco por cento dos pacientes tiveram teste de hipersensibilidade imediata positivo contra pelo menos um dos antígenos testados. Os ácaros domiciliares foram responsáveis pela maioria destes casos (75%). Houve uma pobre correlação entre o escore clínico e a hipersensibilidade cutânea aos alérgenos (r= -0,119 para fungos; r= -0,174 para epitélio de cão; r= -0,243 para ácaros domiciliares; r= -0,090 para mistura de penas). Houve correlação significativa apenas contra epitélio de gatos (r= -0,510; p=0,024). CONCLUSÃO: O estudo demonstrou uma pobre correlação entre os testes cutâneos de hipersensibilidade imediata e os achados clínicos nos pacientes com ceratoconjuntivite vernal. Os testes cutâneos de hipersensibilidade imediata contra aeroalérgenos não foi parâmetro eficaz na identificação dos casos de maior gravidade e cronicidade de ceratoconjuntivite vernal.

PURPOSE: The purpose of this study was to analyze the cutaneous sensitivity to a variety of allergens in patients with vernal keratoconjunctivitis (VKC) and to demonstrate the relation between skin response and clinical aspects of the disease. METHODS: Twenty patients with vernal keratoconjunctivitis were randomly chosen from the External Disease and Cornea Sector. They were clinically evaluated, and a score ranging from 0 to 20 was applied based on signs and symptoms on ophthalmic examination. All subjects underwent a skin prick test against standardized allergens, such as house dust mites D. pteronyssinus, D. farinae, and Blomia tropicalis, as well as allergens from cat, dog, fungi and feather. RESULTS: Seventy-five per cent of patients were positive for at least one of the allergens tested. House dust mites were responsible for the majority of the cases (75%). There was a poor correlation between the clinical score and sensitivity to allergens (r= - 0.119 for fungi; r= - 0.174 for dog; r= - 0.243 for house dust mites; r= - 0.090 for feather). A significant correlation was found only for cat allergen extract (r = - 0.510; p=0.024). CONCLUSIONS: Our study demonstrated poor correlation between cutaneous hypersensitivity tests and clinical findings in patients with vernal keratoconjuntivitis. We concluded that skin response to inhalant allergens is not a useful test to identify clinical severity and chronicity of inflammatory process in this disease.