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Introduction: Hereditary angioedema (HAE) with C1 inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. Although the first symptoms can appear in childhood, the diagnosis’s delay has a strong impact on the patient’s quality of life. We analyzed clinical and laboratory characteristics and the drug therapy of pediatric patients with HAE in Brazil. Methods: Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH. Results: A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients. Conclusions: This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.

Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Intestinal angioedema is another important and incapacitating presentation that may be the main or only manifestation during an attack. In this article, a group of experts from the “Associação Brasileira de Alergia e Imunologia (ASBAI)” and the “Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH)” has updated the Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.

Background Asthma in the elderly population (60 years of age and older) is frequently underdiagnosed, as well as atopy. Atopy, although more prevalent in younger patients, can be a major cause of asthma in the elderly. Chronic obstructive pulmonary disease (COPD) and cardiovascular disease are common differential diagnoses, especially in elderly smokers. Objective The objective of this study was to assess atopy and comorbidities in elderly patients with asthma. Methods This was an observational and retrospective study involving elderly asthmatic patients followed up at a tertiary center. Patients were assessed for severity of asthma, frequency of atopy, and frequency of comorbidities concomitant with asthma. Then, they were classified according to their age at asthma onset and the groups compared with each other for atopy, spirometric parameters, and comorbidities. Results This study included 243 elderly asthmatic patients, 71.8% of them presenting severe disease and 82.3% forced expiratory volume in 1 second (FEV1) Conclusions Most elderly asthmatic patients followed up in our tertiary center were atopic and higher values of total serum IgE suggest atopy. Atopy was inversely correlated with age of asthma onset. The diagnosis of allergic asthma in the elderly population is essential to treat patients more properly, improving their quality of life and decreasing asthma morbidity and mortality.

Background: Identification of asthma phenotypes enables a better understanding and management of this heterogeneous disease. Studies have reported associations between human leukocyte antigens (HLA) and asthma in different populations, but the results have been inconclusive and they have rarely considered the distinct disease phenotypes. Our objective was to characterize allergic and nonallergic asthma phenotypes and evaluate possible associations with the HLA system. Methods: A total of 109 patients with asthma were prospectively followed during 2 years. They were divided into 2 groups, i.e., allergic and nonallergic asthma, according to their clinical history and skin prick test and serum-specific immunoglobulin E (IgE) results. The control group comprised 297 deceased donors of solid organs. Patients' features and HLA class I and II genotypes were assessed and compared. Results: This study showed different features between asthma phenotypes. Nonallergic patients were older at the onset of asthma symptoms and had a higher rate of intolerance to nonsteroidal anti-inflammatory drugs. Allergic patients had higher total serum IgE levels, reported atopic dermatitis and rhinoconjunctivitis more frequently, and, unexpectedly, had a greater disease severity. New associations between the HLA genotypes and allergic and nonallergic asthma were identified. The HLA-B*42, HLA-C*17, HLA-DPA1*03, and HLA-DPB1*105 genotypes were associated with allergic asthma and the HLA-B*48 genotype with the nonallergic phenotype. The presence of the haplotype HLA-DPA1*03 DQA*05 was associated with allergic asthma, and the presence of HLA-DPA1*03 and the absence of HLA-DQA*05 with nonallergic asthma. Conclusions: Allergic and nonallergic asthma have distinct phenotypic and genotypic features. New associations between asthma phenotypes and HLA class I and II were identified.

Upper and lower airways are considered a unified morphological and functional unit, and the connection existing between them has been observed for many years, both in health and in disease. There is strong epidemiologic, pathophysiologic, and clinical evidence supporting an integrated view of rhinitis and asthma: united airway disease in the present review. The term "united airway disease" is opportune, because rhinitis and asthma are chronic inflammatory diseases of the upper and lower airways, which can be induced by allergic or nonallergic reproducible mechanisms, and present several phenotypes. Management of rhinitis and asthma must be jointly carried out, leading to better control of both diseases, and the lessons of the Allergic Rhinitis and Its Impact on Asthma initiative cannot be forgotten.

Background The prevalence of asthma is high, the worldwide average being estimated at 10%, which makes it a public health problem. Many studies show a clear relationship between asthma and specific allergens. With sensitization to aeroallergens identified as a dominant risk factor for asthma. Objective The present study of asthma reports the allergic sensitization of patients with severe persistent asthma followed in the Division of Clinical Immunology and Allergy of University of São Paulo Medical School. Methods A total of 61 patients with severe persistent asthma defined according to the criteria of the Global Initiative for Asthma (GINA) were enrolled. Total IgE levels (IU/mL) were measured in serum and levels up to 120 IU/mL were considered within normal range. A battery of 7 aeroantigens (Dermatophagoides pteronyssinus, Blomia tropicalis, Aspergillus fumigatus, Penicillium nonatum, Lolium perenne, Felis domesticus, Canis familiaris, Blatela germanica and Periplaneta americana) was used in skin prick tests (SPTs), which were performed in each subject, on the volar side of the forearm. Histamine hydrochloride and normal saline solutions were used as positive and negative controls, respectively. The SPTs were read after 15 minutes and, a wheal at least 3 mm greater than the negative control was considered positive. Results The asthmatic patients had a mean age of 48 years and 75% were female. We found that mean total serum IgE levels were 518.4 IU/mL (between 17 and 4720 UI/mL). SPTs positivity was 91.8% for D pteronyssinus, 67.2% for Blomia tropicalis, 4.9% for P nonatum and A fumigatus, 6.5% L perene and Felis domesticus, 16.3% for Canis familiaris, 21.3% Blatela germanica, 13.1% for Periplaneta americana. Twelve patients were monosensitized and 23 patients were polysensitized to 3 or more allergens. Conclusions Most patients with severe allergic asthma were polysensitized, and dust mites, followed by cockroaches, were the main allergens.