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Severe combined immunodeficiency, SCID, is a pediatric emergency that represents the most critical group of inborn errors of immunity (IEI). Affected infants present with early onset life-threatening infections due to absent or non-functional T cells. Without early diagnosis and curative treatment, most die in early infancy. As most affected infants appear healthy at birth, newborn screening (NBS) is essential to identify and treat patients before the onset of symptoms. Here, we report 47 Brazilian patients investigated between 2009 and 2020 for SCID due to either a positive family history and/or clinical impression and low TRECs. Based on clinical presentation, laboratory finding, and genetic information, 24 patients were diagnosed as typical SCID, 14 as leaky SCID, and 6 as Omenn syndrome; 2 patients had non-SCID IEI, and 1 remained undefined. Disease onset median age was 2 months, but at the time of diagnosis and treatment, median ages were 6.5 and 11.5 months, respectively, revealing considerable delay which affected negatively treatment success. While overall survival was 51.1%, only 66.7% (30/45) lived long enough to undergo hematopoietic stem-cell transplantation, which was successful in 70% of cases. Forty-three of 47 (91.5%) patients underwent genetic testing, with a 65.1% success rate. Even though our patients did not come from the NBS programs, the diagnosis of SCID improved in Brazil during the pilot programs, likely due to improved medical education. However, we estimate that at least 80% of SCID cases are still missed. NBS-SCID started to be universally implemented in the city of São Paulo in May 2021, and it is our hope that other cities will follow, leading to early diagnosis and higher survival of SCID patients in Brazil.

CD40 ligand (CD40L) deficiency is a rare inborn error of immunity presenting with heterogeneous clinical manifestations. While a detailed characterization of patients affected by CD40L deficiency is essential to an accurate diagnosis and management, information about this disorder in Latin American patients is limited. We retrospectively analyzed data from 50 patients collected by the Latin American Society for Immunodeficiencies registry or provided by affiliated physicians to characterize the clinical, laboratory, and molecular features of Latin American patients with CD40L deficiency. The median age at disease onset and diagnosis was 7 months and 17 months, respectively, with a median diagnosis delay of 1 year. Forty-seven patients were genetically characterized revealing 6 novel mutations in the CD40LG gene. Pneumonia was the most common first symptom reported (66%). Initial immunoglobulin levels were variable among patients. Pneumonia (86%), upper respiratory tract infections (70%), neutropenia (70%), and gastrointestinal manifestations (60%) were the most prevalent clinical symptoms throughout life. Thirty-five infectious agents were reported, five of which were not previously described in CD40L deficient patients, representing the largest number of pathogens reported to date in a cohort of CD40L deficient patients. The characterization of the largest cohort of Latin American patients with CD40L deficiency adds novel insights to the recognition of this disorder, helping to fulfill unmet needs and gaps in the diagnosis and management of patients with CD40L deficiency.

Pneumonia is a kind of inflammatory disease characterized by pathogen infection of lower respiratory track. Lipopolysaccharide (LPS) is the main bioactive component of Gram-negative bacteria responsible for inflammatory response. Recently, coniferyl aldehyde (CA) has been reported to play a crucial role because of its anti-inflammatory activity. However, the effect and mechanisms of CA in ameliorating symptoms of acute pneumonia remain unknown. Evaluating and identifying the value and exploring the mechanisms of CA on LPS-mediated WI-38 apoptosis and inflammation were the aims of this study. Here, CCK-8 cell viability assay was applied on WI-38 after treatment with or without LPS at different doses of CA to verify that CA can increase LPS-induced cell viability. Then, quantitative polymerase chain reaction (qPCR) and enzyme-linked-immunosorbent serologic assays (ELISA) suggested that LPS treatment dramatically decreased the expression level of IL-10 (anti-inflammatory factor) while strikingly increasing the expression levels of IL-1β, IL-6, and TNF-α (tumor necrosis factor-α; proinflammatory factor) whereas CA treatment attenuates LPS-induced inflammation of WI-38. Further, flow cytometry and Western blot assay verified that LPS treatment dramatically promoted apoptosis of WI-38 cells, while administration of CA notably inhibited apoptosis of WI-38 cells. Moreover, the Western blot assay hinted that CA could inactivate LPS-induced JAK2–STAT1 signaling pathway. These findings indicated that CA could alleviate LPS-mediated WI-38 apoptosis and inflammation injury through JAK2–STAT1 pathway in acute pneumonia.

Objectives To describe the ontogeny of the immune system and the adaptive mechanisms of the immune system in the neonatal period, with an emphasis on transplacental antibody transport and breastfeeding. Source of data Non-systematic literature review in the PubMed database. Summary of the findings The last two decades have witnessed a great advance in the knowledge of the immune system since conception. Several investigation tools have provided insight on phenomena that were previously inadequately understood. Still expanding, the functional and molecular investigation of various aspects of the immune system will make it possible to understand how intra-uterus maternal-fetal exchanges, the maternal microbiota interacting with the fetus and newborn, and the acquisition of immunological competence occur in healthy and disease scenarios. Conclusions In-depth knowledge of the development of the immune system and of the adaptive mechanisms that allow a safer transition to the extrauterine environment are fundamental components of optimizing maternal and young infant vaccination, as well as the strategies associated with full postnatal development, and the early diagnosis and treatment of innate errors of immunity.

Introduction and objectives: Atopic individuals are characterized by increased IgE production and Th2 response if exposed to certain antigens. It is known that the mother transfers antimite antibodies to the fetus and newborn, IgG thru the placenta, and IgA thru breastfeeding, but it is not clear whether there is a protective mechanism mediated by them concerning the development of future allergies. This study aimed to compare the levels of IgA, IgG, and IgE antibodies specific to Der p 1 and Der p 2 between atopic and healthy individuals.Methods: Serum samples of 98 patients and 44 healthy controls were subjected to quantification for specific IgE, IgG, and IgA antibodies against Der p 1 and Der p 2 by ImmunoCap® and ELISA, and subjected to statistical analysis as indicated.Results: Atopic patients had higher serum levels of IgE, IgG, and IgA specific to Der p 1 and Der p 2. The correlation was more robust between IgE and IgG antibodies.Conclusions: Allergic patients produce higher levels of antibodies against Der p 1 and Der p 2 compared with healthy individuals. The mechanisms involved still require detailed studies.

Ataxia-telangiectasia (A-T) is an autosomal recessive neurodegenerative disease caused by mutations in the ataxia-telangiectasia mutated (ATM) gene. The parents do not manifest the disease but are ...

We would like to report our experience in the deployment of an Immunology outpatient clinic that over 14 years became a Reference Center for Inborn Errors of Immunity (IEI) in Brazil, which is located in the city of Taubaté, in the region of Vale do Paraíba, state of São Paulo, responsible for serving a region of approximately 2.5 million inhabitants. During this period, we participated in the pilot project to implement neonatal screening for severe combined immunodeficiency and initiated courses for physicians from basic health units, implementation of classes on clinical immunology in the undergraduate program of medicine, implementation of an outpatient clinic at the city's Faculty of Medicine, in addition to the assessment of patients hospitalized in emergency and intensive care units. Thus, the implementation of a Reference Center for IEI in Latin America proved to be viable and can serve as a model for other services to be initiated. Based on our experience, we suggest some itens for additional IEI centers to emerge.

Background: Ataxia-telangiectasia (A-T) is a DNA repair disorder characterized by changes in several organs and systems. Advances in clinical protocols have resulted in increased survival of A-T patients, however disease progression is evident, mainly through metabolic and liver changes. We aimed to identify the frequency of significant hepatic fibrosis in A-T patients and to verify association with metabolic alterations and degree of ataxia. Results: Dyslipidemia was observed in 16/25 (64%), diabetes in 4/22 (18%), insulin resistance in 5/17 (29%), hepatic steatosis in 13/20 (65%) and suggestive of significant hepatic fibrosis in 5/25 (20%). Patients in the group with significant hepatic fibrosis were older (pConclusions: A suggestive diagnosis of significant hepatic fibrosis was observed in 20% of A-T patients which was associated with changes in liver enzymes, ferritin, increased HOMA-AD and with severity of ataxia compared to patients without hepatic fibrosis.

INTRODUCTION Mucopolysaccharidoses (MPSs) are a group of rare diseases caused by an intralysosomal accumulation of glycosaminoglycans, resulting in a multisystemic clinical condition characterized by variable degrees of physical-functional impairment. OBJECTIVE To evaluate the functional capacity (FC) of MPS patients and compare with a healthy control group. METHODS This is a cross-sectional study of 6- to 39-year-old patients followed at a medical reference center and compared with their control peers, matched by age and sex. FC was assessed using the Sit-to-Stand Test (SST) and Incremental Shuttle Walk Test (ISWT). Heart rate (HR) and Borg rating of perceived exertion were measured before and after ISWT. HR recovery (HRR) was defined as the HR at the end of the test minus the HR in the second minute after ISWT. RESULTS Nineteen (19) MPS patients, 69% with type II MPS and mean age 17 ± 11 years were evaluated. Every patient was under enzyme replacement therapy. The time to perform the SST was longer in the MPS group (10.6 ± 2.5 s vs. 6.7 ± 1.2 s; p

BackgroundPatients with antibody deficiency may experience exceptionally long diagnostic delays, increasing the risk of life-threatening infections, end-organ damage, mortality, and health costs.ObjectiveThis study aimed to analyze serum protein electrophoresis and verify the correlation between calculated globulin (CG, total protein minus albumin levels) or electrophoretically determined serum gamma globulin fraction (Gamma) with IgG levels in children and adolescents under 18 years old (yo).MethodsWe analyzed serum protein electrophoresis (GC or Gamma) and IgG levels from 1215 children and adolescents under 18 yo, classified into 5 age groups. We verified the correlation between CG or Gamma with serum IgG levels.ResultsSerum IgG levels varied according to age groups (from 4.3 ± 2.3 g/l in children under 6 months old to 11.4 ± 3.2 g/l in adolescents in the 10-ConclusionBoth CG and Gamma levels may be of utility as a screening tool for earlier diagnosis of antibody deficiency in children and adolescents under 18 yo.

Objective Allergic sensitization is one of the key components for the development of allergies. Polysensitization seems to be related to the persistence and severity of allergic diseases. Furthermore, allergic sensitization has a predictive role in the development of allergies. The aim of this study was to characterize the pattern of sensitization of atopic patients treated at different pediatric allergy referral centers in Brazil. Methods A nation-wide transversal multicenter study collected data on patients attended in Brazil. Peripheral blood samples were collected to determine the serum levels of allergen-specific IgE. If allergen-specific IgE was higher than 0.1 kUA/L, the following specific components were quantified. Results A total of 470 individuals were enrolled in the study. Mite sensitization was the most frequent kind in all participants. A high frequency of sensitization to furry animals and grasses featured in the respiratory allergies. Regarding components, there was a predominance of sensitization to Der p 1 and Der p 2. It has been verified that having a food allergy, atopic dermatitis, or multimorbidity are risk factors for the development of more severe allergic disease. Conclusion Studies on the pattern of allergic sensitization to a specific population offer tools for the more effectual prevention, diagnosis, and treatment of allergic diseases. Sensitization to dust mites house was the most prevalent in the evaluated sample. High rates of sensitization to furry animals also stand out. Patients with food allergy, atopic dermatitis, or multimorbidity appear to be at greater risk for developing more severe allergic diseases.

Background: Fabry disease (FD) is a rare X-linked storage disorder resulting from the deficient activity of the lysosomal hydrolase α-galactosidase A (α-Gal A). Here we describe a 23-year-old man with FD possessing a novel mutation in the GLA gene, the evaluation of his family, and the functional characterization of the novel variant. Methods: Two generations of a family were screened for FD by clinical symptoms and low enzymatic activity. This step was followed by DNA sequencing that showed a novel GLA missense mutation. To confirm the pathogenicity potential of the mutation, we employed site-directed polymerase chain reaction mutagenesis. GLA wild-type and mutant plasmids were transfected into mammalian cells; RNA and proteins were extracted for expression and analysis of enzymatic activity. Results: The patient presents the variant p.Asn34Asp in the GLA and had several manifestations of FD since adolescence. The investigation of the deficiency of α-Gal A was initiated due to stage 4 of chronic kidney disease. All family members carrying the novel mutation presented early symptoms, including index case’s mother, who received a renal transplant when she was 35 years old. In silico and in vitro analysis confirmed the pathogenic potential of the mutation p.Asn34Asp showing that the enzyme had only 4% of residual activity due to protein misfolding. The ability of the pharmacological chaperone 1-deoxygalactonojirimycin to recover the mutant was confirmed, producing 37.5% of residual activity. Conclusion: In this work, we present a novel missense mutation in GLA that leads to the production of a catalytically competent α-Gal A, which is degraded before its delivery to the lysosome, promoting severe manifestations of FD, with a very similar disease course in affected men and women.

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).

The Phadiatop Infant® (PhInf) is a panel developed to assess allergic sensitization (immunoglobulin E [IgE]) in children aged 13 years having allergy (95%). The PhInf and sIgE agreement between patients (Kappa = 0.94, P < 0.001) and controls (Kappa = 0.84, P < 0.001) was high. PhInf and DP agreement in patients aged >13 years was excellent (Kappa = 0.936, P < 0.001). Compared with sIgE dosage, PhInf had high sensitivity (97%) and specificity (93%). Positivity of PhInf test in this population was high and had an excellent correlation with the allergens comprising the panel. It is a useful method for screening children suspected of having allergic diseases in a non-European country.

Background Ataxia telangiectasia is one of the most common causes of autosomal recessive cerebellar ataxias. However, absence of telangiectasia, normal levels of alpha-fetoprotein and negative genetic test may direct to alternative diagnosis with similar phenotypes such as ataxia telangiectasia-like disorders (ATLD). Cases We report two instructive cases of ATLD: the first case with ataxia telangiectasia-like disorder type 1 related to MRE11A gene, and the second case with ataxia telangiectasia-like disorder type 2 related to PCNA gene. Literature review ATLD is an unusual group of autosomal recessive diseases that share some clinical features and pathophysiological mechanisms with ataxia telangiectasia (AT). ATLD may be associated with mutations in the MRE11A (ATLD type 1) and PCNA (ATLD type 2) genes. ATLD belongs to the group of chromosomal instability syndromes. The reason for the term ATLD is related to the similar pathophysiological mechanisms observed in AT, which is characterized by chromosomal instability and radiosensitivity. Conclusions In this review, the main clinical features, biomarkers, brain imaging and genetics of ATLD are discussed. Mutations in the MRE11A and PCNA genes should be included in the differential diagnosis for early onset cerebellar ataxia with absence of telangiectasia and normal levels of alpha-fetoprotein.

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.

Objectives: Inborn Errors of Immunity (IEI), also known as primary immunodeficiencies, correspond to a heterogeneous group of congenital diseases that primarily affect immune response components. The main clinical manifestations comprise increased susceptibility to infections, autoimmunity, inflammation, allergies and malignancies. The aim of this article is to review the literature on combined immunodeficiencies (CIDs) focusing on the diagnosis and treatment and the particularities of the clinical management of these patients. Source of data: Critical integrative review, aimed to present articles related to primary immunodeficiencies combined with a searchin the PubMed and SciELO databases, with evaluation of publications from the last twenty years that were essential for the construction of knowledge on this group of diseases. Summary of data: We highlight the main characteristics of CIDs, dividing them according to their pathophysiological mechanisms, such as defects in the development of T cells, TCR signaling, co-stimulatory pathways, cytokine signaling, adhesion, migration and organization of the cytoskeleton, apoptosis pathways, DNA replication and repair and metabolic pathways. In CIDs, clinical manifestations vary widely, from sinopulmonary bacterial infections and diarrhea to opportunistic infections, caused by mycobacteria and fungi. Neonatal screening makes it possible to suspect these diseases before clinical manifestations appear. Conclusions: The CIDs or IEI constitute a complex group of genetic diseases with T-cell involvement. Neonatal screening for these diseases has improved the prognosis of these patients, especially in severe ones, known as SCIDs.

Would STAT1 GOF prevent severe forms of COVID-19?To the Editor:As the world faces the current SARS-CoV-2 pandemic, the scientific community struggles to answer questions about the disease that have not been fully clarified yet. It is well known that individuals with certain chronic diseases are at higher risk of having worse prognosis when infected with this virus. Although the risk of a severe disease is still a matter of conjecture in subjects with Inborn errors of Immunity (IEI), these patients may present more COVID-19 complications than the general population, either by community acquired or nosocomial infection1.In our immunology referral clinic at the Federal University of Sao Paulo (UNIFESP), so far, 16 IEI patients have been diagnosed with COVID-19, with a large spectrum of presentation, ranging from mild disease to severe acute respiratory syndrome and death. One of them, however, had a favorable outcome, with no complications, after being infected with the SARS-CoV-2, despite his several serious comorbidities.We report the case of an 11- year -old boy, diagnosed with Type 1 Diabetes Mellitus at the age of 3 years. At that time, he presented persistent watery diarrhea, 3 to 4 episodes a day. One year later, productive cough, persistent fever, conjunctival hyperemia and generalized lymph node enlargement emerged. Histoplasma sp. was isolated in ocular secretion. Acute Disseminated Histoplasmosis (pulmonary, ocular and ganglionic) was diagnosed and he received Itraconazole for one year.In 2018, at the age of 9 years, we started to follow him at our immunology clinic. He received all vaccines for his age with no adverse events. The family reported recurrent skin infections, repeated pneumonia, mucocutaneous and esophageal candidiasis, and arthralgia. He presented hepatomegaly and failure to thrive. The main diagnostic hypothesis was a disease of immune dysregulation. Screening for inborn errors of immunity and inflammatory lab tests were performed. White blood cell count, immunophenotyping and lymphocyte proliferation, serum immunoglobulins and dihydrorodamine (DHR) assay were in normal range. HIV serologies were negative. Whole exome sequencing was performed and the variant c.1675G> C (de novo variant) appeared in the STAT1 gene (Figure 1)3, in heterozygosis. To date, no data on this genetic variant has been published. This new finding is probably considered pathogenic and, due to the patient’s clinical characteristics, STAT1-GOF is very likely to be the diagnosis of our boy.

Introduction: Ataxia-Telangiectasia (A-T) is a multi-system disorder that may be associated with endocrine changes, oxidative stress in addition to inflammation. Studies suggest that selenium (Se) is a trace element related to protection against damage caused by oxidative stress; it is postulated that adequate consumption reduces the risk of some chronic diseases. Objective: To describe the concentrations of Se and glutathione peroxidase (GPx) in patients with A-T, to relate them to markers of the lipid profile. Methods: We evaluated, through a controlled cross-sectional study, 22 A-T patients matched by sex and age with healthy individuals, conjointly evaluating: nutritional status, food intake, serum selenium, glutathione peroxidase (activity), lipid metabolism biomarkers, inflammation and lipid. Results: The median age in the A-T group was 12.2 years. A-T patients had greater impairment of lean body mass and GPx activity as well as lower abdominal circumference. A more atherogenic lipid profile was observed with higher concentrations of total cholesterol, non-HDL cholesterol, LDLox, Apo B, Apo B / Apo A-1 and LDL / HDL ratio; while a lower value was observed in the Apo A-1 / HDL ratio. It was also in the A-T group that statistical difference was detected in the three markers of liver function AST, ALT and GGT. In regard to food intake, A-T patients had lower values ​​of carbohydrate, protein, monounsaturated fat, trans fat, and Se. Conclusion: The study showed cardiovascular risk in A-T patients. A-T patients appear to be at increased risk of reduced nutritional status, impaired liver function, dyslipidemia and inflammation.

Objective: Despite anti-smoking prevention programs, many adolescents start smoking at school age. The main objectives of this study were to determine the prevalence and risk factors associated with smoking in adolescents living in Uruguaiana, RS, Brazil. Methods: A prospective study was conducted in adolescents (12–19 years), enrolled in municipal schools, who answered a self-administered questionnaire on smoking. Results: 798 adolescents were enrolled in the study, with equal distribution between genders. The tobacco experimentation frequency (ever tried a cigarette, even one or two puffs) was 29.3%; 14.5% started smoking before 12 years of age and 13.0% reported smoking at least one cigarette/day last month. Having a smoking friend (OR: 5.67, 95% CI: 2.06–7.09), having cigarettes offered by friends (OR: 4.21, 95% CI: 2.46–5.76) and having easy access to cigarettes (OR: 3.82, 95% CI: 1.22–5.41) was identified as factors associated with smoking. Having parental guidance on smoking (OR: 0.67, 95% CI: 0.45–0.77), having no contact with cigarettes at home in the last week (OR: 0.51, 95% CI: 0.11–0.79) and knowing about the dangers of electronic cigarettes (OR: 0.88, 95% CI: 0.21–0.92) were identified as protection factors. Conclusion: The prevalence of smoking among adolescents in Uruguaiana is high. The implementation of measures to reduce/stop tobacco use and its new forms of consumption, such as electronic cigarettes and hookah, are urgent and imperative in schools. Resumo: Objetivo: Apesar dos programas de prevenção antitabagista, muitos adolescentes começam a fumar na idade escolar. Foram objetivos do estudo determinar a prevalência e os fatores de risco associados ao consumo de tabaco em adolescentes moradores do município de Uruguaiana RS, Brasil. Métodos: Estudo transversal, realizado em adolescentes de 12 a 19 anos, matriculados em escolas do município, que responderam questionário autoaplicável sobre tabagismo. Resultados: Participaram 798 adolescentes com igual distribuição entre os gêneros. A frequência de experimentação de tabaco (Alguma vez tentou fumar um cigarro, mesmo que uma ou duas tragadas) foi 29,3%, sendo que 14,5% começaram fumar antes dos 12 anos de vida e 13,0% deles afirmaram terem fumado pelo menos um cigarro/dia no último mês. Foram identificados como associados ao tabagismo: ter amigo tabagista (OR:5,67, IC95%:2,06-7,09), ter oferta de cigarro pelo amigo (OR:4,21, IC95%:2,46-5,76) e facilidade de conseguir cigarros (OR:3,82, IC95%:1,22-5,41). Ter orientações dos pais sobre tabagismo (OR:0,67, IC95%:0,45-0,77), não ter contato com cigarro em casa na última semana (OR:0,51, IC95%:0,11-0,79) e saber os malefícios do cigarro eletrônico (OR:0,88, IC95%:0,21-0,92) foram identificados como de proteção. Conclusões: A prevalência de tabagismo entre os adolescentes de Uruguaiana é alta. A implantação de medidas nas escolas para reduzir ou acabar o consumo de tabaco e de suas novas modalidades, como os cigarros eletrônicos e o narguilé é urgente e imperiosa. Keywords: Tobacco, Adolescent, Risk factors, Cigarette, Palavras-chave: Tabaco, Adolescente, Fatores de risco, Cigarro

The clinical history is of importance in the investigation of allergic diseases but does have limitations. Many allergic conditions will be over-diagnosed if anamnesis alone is used for diagnostic criteria. Serum total immunoglobulin E (TIgE) quantification, as well as panels containing allergens prevalent in the studied population, may serve as screening tests and facilitate the diagnosis of allergic disease or its exclusion. We assessed the positivity of two versions of these tests, Phadiatop Europe® (PhEU) and Phadiatop Infant® (PhInf), as well as total IgE (TigE) values in patients with a medical diagnosis of allergic disease and non-allergic individuals. Methods A cross-sectional study performed in eleven Brazilian pediatric allergy centers with patients divided into groups according to the primary condition and a group of assessed control subjects. They were submitted to TIgE measurement and screening tests (PhEu and PhInf). Results TIgE mean serum levels were significantly higher among allergic patients, especially those with asthma/rhinitis or atopic dermatitis. The positivity of the screening tests, considering the total population, was 63.8% for PhEU and 72.6% for PhInf. These increased when we evaluated only the allergic subjects. The concordance index of the two tests was Kappa = 0.7 and higher among those of greater age. Conclusions In the assessed population, there were significantly higher levels among those with positive screening tests and PhInf showed better performance in the identification of sensitized individuals, regardless of age. This is the first study to evaluate Phadiatop and Phadiatop Infant in the same population.