Nelson Rosario

MethodsObservational, descriptive, cross-sectional study which assessed demographics, PROMs knowledge, frequency of use, PROM preferences, satisfaction, physician training, attitudes, and barriers of using PROMs. Data collection was carried out in collaboration with research networks worldwide.ResultsOverall, 2528 physicians were surveyed, and 51.7%(n= 1308) reported using PROMs. Most of them (57.9%) were 30-49 years old (yo), attending both public and private settings (41.4%) and respiratory-related specialties (RRS, 41.1%). The use of PROMs in asthma was higher than in RA and RS (28.6%, 15.6%, and 8.9% respectively). The most frequently PROMs used were ACT and AQLQ, the RCAT, and SNOT22 (RS). The principal reason for use was to monitor control, treatment, and severity of disease (≈ 90%), time constraints were the principal barrier (≈ 76%), and most requested being trained in the …

Background:To assess the efficacy of applying the endoscopic reference score for EoE (EREFS) in children with symptoms of esophageal dysfunction naïve to proton pump inhibitor (PPI) therapy.Methods:An observational cross-sectional study was conducted by reviewing reports and photographs of upper gastrointestinal endoscopies (UGE) and esophageal biopsies of patients with symptoms of esophageal dysfunction. Patients who were treated with PPI or had other conditions that may cause esophageal eosinophilia were excluded.Results:Of the 2,036 patients evaluated, endoscopic findings of EoE were identified in 248 (12.2%) and more than one abnormality was observed in 167 (8.2%). Among all patients, 154 (7.6%) presented esophageal eosinophilia (≥ 15 eosinophils per high power field)(P< 0.01). In this group, 30 patients (19.5%) had normal endoscopy. In patients with EoE, edema (74% vs 6.5%, P< 0.01) and furrows (66.2% vs 2.4%, P< 0.01) were more prevalent than in the control group. Association of edema and furrows was more frequent in patients with EoE than in the control group (29.2% vs 1.6%, P< 0.01, OR= 24.7, CI= 15.0-40.5). The presence of more than one endoscopic finding had sensitivity of 80.5%, specificity of 93.4%, positive predictive value (PPV) of 50%, negative predictive value (NPV) of 98.3%, and accuracy of 92.4%.Conclusion:In conclusion, this study showed that endoscopic features suggestive of EoE had high specificity and NPV for diagnosing EoE in children naïve to PPI therapy. These findings highlight the importance of the EREFS in contributing to early identification of inflammatory and fibrostenosing …

Hereditary Angioedema (HAE) is a rare and severe genetic autosomal dominant disease of the immune system, caused by a deficiency of C1 esterase inhibitor (C1-INH). Classified into 3 subtypes: HAE with quantitative deficiency of the C1-INH inhibitor, C1-INH dysfunction, and normal C1-INH. The objective is to present a protocol examining the evidence on the relationship between mutations, clinical manifestations and prognosis in AEH. A systematic review will be conducted according to the steps: formulation of the clinical question (PICO), protocol with inclusion and exclusion criteria, conduction of a broad bibliographical search, extraction and summary of the included studies. This systematic review will follow the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Evidence will be obtained from the Pubmed, Embase, Web of Science and Scopus databases in the last 10 years. The selection of descriptors will be conducted using the Health Sciences Descriptors (DeCs), with the Boolean operators “AND and OR”, which will result in a search strategy for each proposed database. The selection will be conducted by two independent reviewers based on pre-determined criteria. In case of any discrepancy between the two reviewers, a third reviewer will mediate the conflict. Each study included will be assessed using the ROBINS-I, for the risk of bias, following the Oxford Evidence Base Center level of evidence and recommendation. Kappa Statistic will be used to assess inter-rater reliability. This systematic review will allow a greater understanding of HAE, and provide scientific evidence for healthcare professionals …

Rationale Real-life clinical data on severe asthma are needed for improving asthma management worldwide. This is a real-life multicentre cohort of patients with severe asthma in Brazil (REBRAG Study). The aim of this study is to describe the impact of the disease on patients with severe asthma in a real-life cohort, showing the basal clinical characteristics, treatment access, and retrospective data of clinical remission on biological therapy. Methods Patients with severe asthma (> 6 years) have been recruited from 23 Brazilian sites. Clinical data, lung function, phenotypic biomarkers, and therapies prescribed were collected in the baseline visit of the study, from July 2021 to September 2023. Pediatric, adult, and elderly patients were analyzed. Clinical remission was defined as control of disease and absence of severe exacerbations in the last 12 months in patients under biologic therapy for> 12 months. Results 417 …

Introduction Information and communication technologies (ICTs) improve patient‐centered care and are routinely used in Allergic Rhinitis (AR), but patients' preferences and attitudes are unexplored. This study examines AR‐related information preferences and ICT use by AR patients. Methods A survey‐based cross‐sectional study was carried out in Ecuador from July to September 2019 in seven centers of reference for allergic disease. Participants were 18 years or older, diagnosed with AR and had access to ICT and the Internet. Descriptive and binomial logistic regressions were performed. A value of less than 0.05 was considered statistically significant. Results 217 patients were included. 47% (n = 102) used ICTs to learn about AR, of which 38.2% (n = 83) found it useful. Most of participants (75%, n = 164) did not think that ICTs reduce their need to see a doctor. Individuals with poorer quality of life were …

MethodsAdult patients with≥ 15 eosinophils/HPF and esophageal dysfunction symptoms treated between August 2007 and November 2021 were enrolled. EoE was diagnosed based on 2018 consensus. Causes for esophageal eosinophilia other than EoE (eosinophilic gastroenteritis, achalasia, Barrett's esophagus, inflammatory bowel disease, esophageal neoplasm, and caustic or radiation esophagitis) were excluded. Baseline characteristics and endoscopic findings were collected. Symptoms, EREFS features and scores, and peak eosinophil counts (PECs) were evaluated before and after treatment, and the correlation between the EREFS score and PECs was assessed. An optimal EREFS threshold for histologic (< 15 eosinophil/HPF) and symptomatic response was determined based on sensitivity, specificity, and area under the curve (AUC).ResultsOverall, 102 EoE patients were enrolled (74.5% male …

MethodsA Brazilian registry for HAE was established, with demographic, clinical, treatment and genetic data included in the REDCap platform by the treating physicians.ResultsData from the first 283 Brazilian patients (69.3% female) enrolled in the registry were analyzed. 216 (76.6%) patients have HAE with C1-INH deficiency (65 patients with identified disease-causing SERPING1 variants) and 66 (23.4%) have HAE with normal C1-INH (54 with identified F12 disease-causing variants). 260 (91.9%) were symptomatic, of whom 179 (70.8%) have experienced at least 1 severe attack. Most affected areas were abdomen (87.4%) and extremities (71, 1%); most common triggering factor were stress 170 (67.5%) and trauma (54%). The mean time from onset of symptoms to diagnosis of HAE was 12.5±12.8 years; however, among the 41 patients under 18years-old, the mean age at diagnosis was only 0.8±3.3 years. Of …

MethodsAs a result, the UCARE PROMUSE project attempted to discover, through a cross-sectional study, which PROMs are utilized by physicians caring for patients with angioedema, as well as the frequency of usage, barriers, and fields of learning that physicians desire to enhance their management. The questionnaire was sent to 45 specialized centers belonging to the UCARE and ACARE Network. The study’s IRB approval No. HCK-CEISH-21-002.ResultsOnly 370 (15%) of 2,534 responders use PROMs for angioedema. PROM usage was similar among men and women (p= 0.677). The AAS was the most used (49%), followed by the AE-QoL (44%) and the AECT (28%). Forty percent used PROMs “sometimes” and only 15% used “always”. Nine out of every 10 surveyed used PROMs for disease control, severity, and decision-making. Clinicians noted many PROM usage barriers: Time restrictions (78%), lack of …

Background: In phase-3 clinical trials, dupilumab significantly improved disease severity in patients with moderate-to-severe atopic dermatitis (AD). Systemic drugs are used in children with AD, however, their real-world impact across anatomical regions is unknown.Methods: PEDISTAD (NCT03687359) is an ongoing, international, observational study in patients aged< 12 years with moderate-to-severe AD receiving systemic AD medications or otherwise candidates for systemic treatment. The effect of three most frequently used systemic AD therapies (dupilumab, methotrexate, cyclosporine) on various anatomical regions was assessed using Eczema Area and Severity Index (EASI) region subcomponents (therapy start to last assessment).Results: At baseline, 82.2% of patients had head and neck AD involvement (< 2 years age group: 89.3%; 2 to< 6 years: 82.2%; 6 to< 12 years: 80.7%). 144 patients received …

MethodsOrdinal logistic regressions were used to predict CU in individuals with no alexithymia, possible alexithymia, or alexithymia. The model predicted TAS-20 based on UAS7 (Urticaria Activity Score), also controlling for gender, age, postsecondary education, marital status, cardiovascular, autoimmune, cancer, allergy, and mental comorbidities; diagnosis time; first-or second-generation antihistaminic medication use, omalizumab and corticoid use.Results423 people from Ecuador, Peru, Chile, Argentina, Mexico, Peru, Brazil, Denmark, Germany, India, and Russia took part in this study, and 48.05% of them were men. The average age and disease years were 43.6 and 5.2. 42.08% of patients presented with alexithymia and 21.51% may have been affected (TAS-20). Patients with well-controlled CU had a considerably increased risk of developing alexithymia (OR= 1.74) after adjusting for covariates. ORs were 2 …

Objectives Fecal calprotectin is an inflammatory marker used for monitoring intestinal diseases. It has been studied as a marker of intestinal inflammation in cystic fibrosis (CF), a multi-systemic genetic disease caused by alterations to the CFTR gene. Manifestations of the disease favor a systemic inflammation not limited to the respiratory tract, therefore, calprotectin is a non-invasive and effective diagnostic method. The aim of the study was to perform a systematic review of the literature with a qualitative synthesis of studies. Sources The articles were selected from PubMed, Web of Science, Scielo and Lilacs. Summary of the findings Nine studies were selected for that qualitative synthesis, one was a randomized clinical trial, and eight were case-control or cohort designs. Most studies have indicated that calprotectin is a marker of systemic inflammation in CF and not just intestinal inflammation. Calprotectin is an aid in monitoring inflammatory bowel conditions in patients with cystic fibrosis. Conclusion Further studies should be conducted to investigate the role of this marker in the systemic inflammation of CF.

Objective To evaluate the relationship between the occurrence of contact urticaria in reaction to cow’s milk in infants and the diagnosis of cow’s milk allergy (CMA) established by supervised oral food challenge. Methods In this cross-sectional study, we identified 184 children who had undergone 221 oral food challenge (OFC) at a CMA reference center between July 2015 and August 2019. Of these, 23 (12.5%) had a history of contact urticaria to cow’s milk and underwent a total of 30 OFC. Baked cow’s milk OFC were excluded, and 21 children were included in the study. All data from clinical history and allergy tests (serum-specific IgE dosages and skin prick test [SPT] with reconstituted cow’s milk formula) were recorded on standardized forms. The challenge was conducted with reconstituted cow’s milk formula in increasing volumes every 15–20 min. Results 24 OFC were performed; 13/24 (54.2%) for diagnostic purpose and 11/24 (45.8%) to evaluate cow’s milk tolerance. Allergy tests were positive in 14 patients (87.5%). Positive oral challenge outcomes were identified in 7/24. One patient had a late flare of atopic dermatitis and five children presented with immediate urticaria (two generalized urticaria and three perioral urticaria). The median papule diameter was 6.5 mm in patients with a positive OFC and 3.75 mm in the negative group (P = 0.02). Conclusion The occurrence of contact urticaria might be the only manifestation among infants sensitized to cow’s milk with tolerance to oral ingestion. Reactions due to oral challenge were related to a larger wheal diameter on the SPT.

MethodsAir samples were obtained using an impactor of total particulate, placed in urban Maringá-PR (Brazil), on latitude 23.41 o S, longitude 51.97 o W, 537 m above sea level and 2.5 km from the large processing soybean industry. Samples of atmospheric material were collected (24 or 48 hours), from March/2017 to March/2018, totaling 70 samples. Concentrations of Gly m 1 were determined by ELISA (Enzyme linked immunosorbent assay) and metereological data were obtained from INMET (National Institute of Meterology).ResultsAll samples showed detectable levels of Gly m 1. There were no statistical differences between filters at 24 and 48 hours. The values ranged from 0.66 ng/m 3 to 1826.1 ng/m 3 (median 4.89 ng/m 3). 23% of samples had values above 90 ng/m 3, months of June/2017 and March/2018 having the highest concentrations. There was a positive correlation between the concentrations of …

Introduction The deficiency of ADA2 (DADA2) is a rare autoinflammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic management whereas bone marrow transplantation is considered for refractory or severe phenotypes. Data from Brazil is scarce and this multicentric study reports 18 patients with DADA2 from Brazil. Patients and methods This is a multicentric study proposed by the Center for Rare and Immunological Disorders of the Hospital 9 de Julho - DASA, São Paulo - Brazil. Patients of any age with a confirmed diagnosis of DADA2 were eligible for this project and data on clinical, laboratory, genetics and treatment were collected. Results Eighteen patients from 10 different centers are reported here. All patients had disease onset at the pediatric age (median of 5 years) and most of them from the state of São Paulo. Vasculopathy with recurrent stroke was the most common phenotype but atypical phenotypes compatible with ALPS-like and Common Variable Immunodeficiency (CVID) was also found. All patients carried pathogenic mutations in the ADA2 gene. Acute management of vasculitis was not satisfactory with steroids in many patients and all those who used anti-TNF had favorable responses. Conclusion The low number of patients diagnosed with DADA2 in Brazil reinforces the need for disease awareness for this condition. Moreover, the absence of guidelines for diagnosis and management is also …

Este trabalho tem como objetivo investigar a associação entreo o uso dos cigarros eletrônicos e doenças pulmonares em adolescentes. Foi realizada uma revisão sistemática na base de dados PubMed. Os termos Mesh incluídos na busca foram" Electronic Nicotine Delivery Systems" e" Lung Diseases" e sinônimos no título e abstract, com o filtro de idade" child birth-18 years", para buscar artigos relacionados ao uso de cigarros eletrônicos e doenças pulmonares em adolescentes. Os critérios de elegibilidade consistiram em usuários adolescentes, exposição ao cigarro eletrônico e doença pulmonar como desfecho. Os artigos foram selecionados por uma revisão pareada de maneira independente, primeiramente com a leitura dos títulos e resumos, seguida da leitura integral dos artigos selecionados, os quais foram analisados pela ferramenta New Castle-Ottawa quanto sua qualidade, e receberam entre 5 e 7 estrelas. Os dados encontrados foram extraídos para a realização da metanálise. Inicialmente foram encontrados 61 artigos, sendo seis considerados elegíveis, todos transversais e com aplicação de questionários. Na metanálise foi encontrada uma associação significativa entre o uso de cigarro eletrônico e exacerbação de asma (OR ajustado 1, 44; IC 95% 1, 17 1, 76). Não foram encontrados estudos que avaliassem a associação do cigarro eletrônico e outras doenças pulmonares, incluindo EVALI (E-cigarette or Vaping product use-Associated Lung Injury), em adolescentes. Na metanálise foi encontrada uma associação significativa entre exacerbações de asma e uso de cigarros eletrônicos em adolescentes com asma crônica e …

Epidemiological studies show that allergic rhinitis (AR) is observed in 10–40% of the world’s population. This disease significantly compromises the quality of life, impairing development in children and professional activities in adults. AR is also frequently associated with allergic asthma (AA) 1, 2. It has been observed that 15–38% of patients with AR develop concomitant AA. This relationship between AR and AA is based on robust pathophysiological mechanisms, which are consistent with the united airways theory. This model states that environmental exposure to allergenic molecules in genetically predisposed individuals directs the production of specific cytokines responsible for the development of the allergic inflammatory process in the nasal mucosa and lungs1, 3. The association between AR and AA or atopic dermatitis (AD) is very common, usually developing since childhood, representing a phenomenon called the atopic march. Therefore, patients with AR should be evaluated in a broad and systemic way due to the implications and interactions of this disease that is part of a broad allergic process that can affect the upper airways, lower airways, skin, and mucous membranes. These diseases, classified as atopic diseases, are characterized by the presence of a specific, genetically directed immune response after exposure to allergens1, 2, 4, 5. In Brazil, the components derived from the house dust mites Dermatophagoides farinae

Forty-two per cent of patients with chronic urticaria in our study had alexithymia. Female patients had approximately 48% higher odds of having alexithymia. Patients with uncontrolled urticaria had 58% higher odds of having alexithymia. Among those with mild urticaria activity, the likelihood of having alexithymia was twice that of urticaria-free individuals. The use osf older first-generation antihistamines doubled the odds of having alexithymia. Patients with chronic urticaria with cardiovascular comorbidities had a 2.5-fold increased risk of alexithymia.

Rhinitis is a chronic inflammatory process of the nasal mucosa that can progress to structural alterations characterized as airway remodeling such as observed in asthmatic patients [1]. Remodeling is the result of recovering from an inflammatory damage and represents the relationship between an initial inflammation and the subsequent tissue damage. Although not well defined in rhinitis it could be related to increased thickness of basement membrane, pseudofibrosis and epithelial detachment in hystologic samples of nasal mucosa [2].The nasal mucosa is a physical barrier to particles, allergens and pollutants and a conditioner for inspired air. The nasal pseudostratified columnar epithelium is separated from the lamina propria by a continuous basement membrane. Chronic inflammation could induce structural changes in the epithelium or lamina própria [3].

BackgroundHereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of subcutaneous or mucosal edema caused by excess bradykinin. The aim of the present study was to assess the knowledge of pediatricians about hereditary angioedema.MethodsAn online survey with 12 HAE-related and 14 demographics-related questions was e-mailed to all pediatricians who were members of the Brazilian Society of Pediatrics (n = 17 145) once a week during the months of June and July 2021. The electronic questionnaire assessed clinical manifestations, diagnosis, and treatment of hereditary angioedema in children and adolescents.ResultsFour hundred and fifty-five pediatricians responded to the questionnaire (2.6%), of whom 55 (12.1%) were board certified in Allergy and Immunology (A/I), while 400 (87.9%) were not (N-A/I). Three hundred and sixty-eight (80.9%) were …

Ataxia-telangiectasia (AT) is a rare neurodegenerative genetic disorder leading to neurological defects, telangiectasias and immunodeficiency. We aimed to study the clinical and immunological features of Latin American patients with AT and analyze the factors associated with AT-related mortality. Referral centers (n= 46) from 9 Latin American countries participated in this retrospective cohort study. AT was defined with ESID Criteria. Designated physicians in each healthcare center reviewed medical records of 218 patients with AT. Data from 218 patients with AT were analyzed. Mean±standard deviation ages at symptom onset and diagnosis were 1.6±1.1 and 5.7±3.5 years, respectively. Most (66.9%) patients presented recurrent airway infections, which was significantly associated with IgA deficiency. Humoral deficiencies included IgA deficiency in 60.8% of patients and IgG deficiency in 28.6%. Lymphopenia was present in most cases, mainly affecting T and B cells. Around half of patients used antibiotic prophylaxis (57.7%) and immunoglobulin replacement (49.1%). No complications due to live viral vaccines were reported. Their mean survival was 24.2 years and Kaplan-Meier 20-year-survival rate was 52.6%. Low IgG levels were associated with decreased life expectancy (hazard ratio 2.1; 95% CI, 1.11–3.93), whereas male sex was a protecting factor (hazard ratio 0.52; 95% CI, 0.27–0.99). There was a high frequency of recurrent infections and immunologic abnormalities in our sample of patients with AT. Higher mortality was associated with female gender and low IgG levels. These findings suggest that immunologic status should be …