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Purpose: There is still scarce data on SARS-CoV-2 infection in patients with Inborn Errors of Immunity (IEI) and many questions. We aimed to describe the clinical outcome of SARS-CoV-2 infection in Brazilian IEI patients and to identify factors influencing the outcome of 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 presented mostly as asymptomatic (n=21) and mild (n=66), and one child had multisystem inflammatory syndrome (MIS-C). We could not observe sex related susceptibility and observed 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. Patients with complement deficiencies had milder COVID-19. However, the type of IEI was not a determining factor for severity. The severity of SARS-CoV-2 infection seems to be more related to older age, higher number of comorbidities and type of comorbidities (bronchiectasis and cardiopathy).

Background Primary angioedema (PA) is a complex disorder, presenting multiple hereditary (hereditary angioedema) and acquired subtypes (acquired angioedema). Despite a very similar clinical presentation among subtypes, the differential diagnosis is limited by the difficulty to identify bradykinin-mediated PA and the lack of specific biomarkers. Objectives To report the clinical and genetic features of Brazilian patients with PA. Methods Brazilian patients referred from 50 centers were diagnosed on the basis of clinical symptoms, C1 inhibitor (C1-INH) and C4 plasma measurements, and DNA sequencing of genes associated with hereditary angioedema. Results We characterized 92 patients with acquired angioedema and 425 with HAE: 125 with C1-INH deficiency, 180 with F12 mutations, and 120 of unknown cause. Thirty-one different mutations were identified in SERPING1 and 2 in F12, in addition to 2 mutations of uncertain significance in the ANGPT1 gene. The molecular diagnosis was decisive for 34 patients with HAE without family history, and for 39% of patients with inconsistent biochemical measurements. The median delay in diagnosis was 10 years, with a maximum of 18 years for HAE with C1-INH deficiency. Androgens and tranexamic acid were the most used drugs for long-term prophylaxis in all the PA subtypes, and they were used on demand by 15% of patients. Only 10% of patients reported the use of specific medication for HAE during attacks. Conclusions Our analysis exposes a broad picture of PA diagnosis and management in a developing country. Complement measurements presented considerable inconsistencies, increasing the diagnosis delay, while patients with PA with normal C1-INH remain with an inaccurate diagnosis and unspecific treatment.

Introduction: Asthma, allergic rhinitis and atopic eczema are among the most common chronic diseases in children. Objective: To determine the prevalence of asthma, rhinitis and atopic eczema in schoolchildren of the Vitoria, ES, Brazil. Methods: This cross sectional study used the ISAAC (International Study of Asthma and Allergies in Childhood) written questionnaire in children aged 6 and 7 years (n = 2452) attending public and private schools, from October 2008 to July 2009. Results: Wheezing prevalence rate in the previous year and physician-diagnosed asthma was 26.5% and 22.7%, respectively. Prevalence rate of rhinitis symptoms was 46.7% and rhino conjunctivitis 20%, while 36.2% of the children were diagnosed with rhinitis. Symptoms of eczema and atopic eczema on common sites were found in 16% and 10.5% of the children, respectively. A significant association between asthma and rhinitis was observed (RP = 3,8, IC95% = 3,1-4,6; p

Results Data from 51 patients, 29 (57%) females and 22 (43%) males, from 5 to 88 years old (mean: 32 years) was evaluated. Patients belonged to 7 families, 20 of them from the same family. The mean age of onset was 10 years and of diagnosis 26 years. Fifty (98%) patients were symptomatic, and 28 (55%) had experienced laryngeal edema. Deaths by laryngeal edema had occurred in 6 families. Crisis triggering factors were identified in 44 (86%) patients. Forty-five (88%) patients presented HAE due to quantitative deficit of C1-INH. Maintenance treatment was required for 32 (63%) patients, of whom 26 (81%) used Danazol, 5 (16%) Tranexamic acid, and 1 (3%) both. Thirteen (28%) patients needed icatibant to treat 23 crises.

Methods From 2003 to 2009 the following actions were implemented by the local health system: training of health professionals; creation of a referral outpatient clinic with a multidisciplinary team and free pharmacological services. The number of asthma related hospitalizations was collected in the period from 2002 to 2013 and rates and percentages of hospitalizations were calculated and linear regression model performed to determine the trend over this period of time. Data concerning the dispensing of medications which are included in State List of Exceptional Medicines (budesonide + formoterol 12/400 mcg) and in the State Ordinance 054-R, 12/05/ 2009 (salmeterol + fluticasone 25/125 and 50 / 250, 200 mcg budesonide, montelukast 4, 5 and 10 mg and omalizumab 150 mg) were also evaluated.