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OBJECTIVE: Thymidine-dependent small-colony variants (TD-SCVs) of Staphylococcus aureus are being isolated with increasing frequency from patients with cystic fibrosis (CF). The aim of this study was to evaluate the relationship between TD-SCV isolation and pulmonary function in patients with CF, as well as to determine whether the emergence of TD-SCVs was associated with trimethoprim-sulfamethoxazole (TMP-SMX) use and with coinfection with other microorganisms. METHODS: This was a retrospective case-control study including patients with CF who visited the Clinical Hospital Complex of the Federal University of Paraná, in Curitiba, Brazil, between 2013 and 2022. Demographic, clinical, and spirometric data, as well as information on TD-SCVs and other isolated microorganisms, were collected from the medical records of patients with CF and TD-SCVs (TD-SCV group; n = 32) and compared with those of a matched group of patients with CF without TD-SCVs (control group; n = 64). RESULTS: Isolation of TD-SCVs was positively associated with TMP-SMX use (p = 0.009), hospitalization (p < 0.001), and impaired pulmonary function (p = 0.04). CONCLUSIONS: The use of TMP-SMX seems to contribute to the emergence of TD-SCVs, the isolation of which was directly associated with worse pulmonary function in our sample.
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Fibrosis Quística , Infecciones Estafilocócicas , Staphylococcus aureus , Humanos , Fibrosis Quística/microbiología , Fibrosis Quística/fisiopatología , Estudios Retrospectivos , Masculino , Femenino , Estudios de Casos y Controles , Staphylococcus aureus/efectos de los fármacos , Infecciones Estafilocócicas/microbiología , Infecciones Estafilocócicas/tratamiento farmacológico , Infecciones Estafilocócicas/fisiopatología , Adulto , Adulto Joven , Adolescente , Timidina/análogos & derivados , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Combinación Trimetoprim y Sulfametoxazol/farmacología , Pulmón/fisiopatología , Pulmón/microbiología , Pulmón/efectos de los fármacos , Antibacterianos/uso terapéutico , Antibacterianos/farmacología , Niño , Brasil , Pruebas de Función RespiratoriaRESUMEN
Background: Newborn screening (NBS) for the early detection of inborn errors of immunity (IEI) has been implemented in a few countries. The objective of this study was to verify the situation and define obstacles to the implementation of NBS worldwide. Methods: A questionnaire was developed by the Inborn Errors of Immunity Committee of the World Allergy Organization (WAO) with 17 questions regarding NBS for IEI in the physician's workplace, NBS test type, problems hindering NBS implementation, reimbursement for IEI therapy, presence of a national IEI registry, referral centers, molecular diagnosis, hematopoietic stem cell transplantation centers, gene therapy, and immunoglobulin replacement therapy. The survey was sent by email once a week to doctors and others associated with WAO and the main immunology societies worldwide as a Google Form™ to be completed during September and October 2021. Results: Two hundred twenty-nine questionnaires were completed, of which 216 (94.3%) were completed by physicians. One hundred seventy-six (76.8%) physicians were both allergists and immunologists. The agreement between allergists/immunologists and non-allergists/non-immunologists for the question "Is there NBS for IEI in the country you work in?" was good (κ = 0,64: 95% CI 0.55-0.69). Ninety-eight (42.8%) participants were from Latin America, 35 (15.3%) from North America, 29 (12.6%) from Europe, 18 (7.9%) from Africa, 44 (19.2%) from Asia, and 5 (2.2%) from Oceania. More than half the participants (n = 124, 54.2%) regularly treated patients with IEI, followed by occasional treatment (n = 77, 33.6%), or never (n = 28, 12.2%). Of the respondents, 14.8% reported that their countries performed NBS for IEI, whereas 42.2% reported their countries did not. T-cell receptor excision circles was the most widely used technique in some countries, with 75 (59.9%) for the diagnosis of NBS for IEI, followed by combined use with kappa deleting-recombination excision circles. Only 13 participants (10.3%) underwent neonatal exon screening in their respective countries. Financial and technical issues were among the major obstacles to the implementation of NBS for IEI. Conclusions: This pilot study showed that few countries have implemented NBS for IEI, despite the presence of immunology referral centers and the availability of hematopoietic stem cell transplantation and intravenous immunoglobulin replacement therapy. The findings highlight the difficulties, mainly financial and technical, hindering wide application of NBS. Sharing experiences, technologies, and resources at the international level can help overcome these difficulties.
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Os anticorpos monoclonais são uma nova classe de medicamentos que representa um marco na evolução da terapia de doenças alérgicas graves. Além de possibilitar uma terapia imunológica alvo específico, proporciona maior controle de sintomas, redução de exacerbações, melhoria da qualidade de vida e da segurança. A eficácia e a segurança dos anticorpos monoclonais no tratamento de doenças alérgicas estão bem documentadas nos estudos clínicos pivotais, de extensão e de vida real. No Brasil, estão licenciados atualmente pela Agência Nacional de Vigilância Sanitária (ANVISA) imunobiológicos para asma, dermatite atópica (DA), esofagite eosinofílica (EoE), granulomatose eosinofílica com poliangeíte (GEPA), rinossinusite crônica com pólipo nasal (RSCcPN), síndromes hipereosinofílicas (SHE) e urticária crônica espontânea (UCE). Com a incorporação do uso dessas novas terapias no dia a dia do médico alergologista e imunologista, naturalmente emergem aspectos práticos que exigem orientações práticas perante as evidências científicas mais atuais, a fim de se manter a boa prática médica, com uso criterioso e consciente pelo especialista capacitado. Assim, nesse guia prático, abordaremos os imunobiológicos aprovados até o momento para doenças alérgicas graves, com objetivo de auxiliar o especialista em Alergia e Imunologia na prescrição e manejo dessas medicações, incluindo indicações, contraindicações, monitoramento da eficácia e segurança, notificação de eventos adversos, bem como aspectos associados aos cuidados com vacinas, populações especiais, acesso, transporte, armazenamento e aplicação domiciliar.
Monoclonal antibodies are a new class of drugs that represent a milestone in the evolution of therapy for severe allergic diseases. In addition to allowing targeted immunologic therapy, they can improve symptom control, reduce exacerbations, and increase quality of life and safety. The efficacy and safety of monoclonal antibodies in the treatment of allergic diseases are well documented in pivotal, extension, and real-life clinical studies. In Brazil, immunobiologic agents are currently licensed by the National Health Surveillance Agency (ANVISA) for use in asthma, atopic dermatitis (AD), eosinophilic esophagitis (EoE), eosinophilic granulomatosis with polyangiitis (EGPA), chronic rhinosinusitis with nasal polyps (CRSwNP), hypereosinophilic syndrome (HES), and chronic spontaneous urticaria (CSU). With the incorporation of these new therapies into the daily practice of the allergist and immunologist, practical aspects will naturally emerge and require practical guidelines in light of the most current scientific evidence in order to maintain good medical practice, with judicious and conscious use by a qualified specialist. Therefore, in this practical guide, we will address the immunobiologic agents currently approved for severe allergic diseases, aiming to assist allergy and immunology specialists in the prescription and practical management of these medications, including indications, contraindications, efficacy and safety monitoring, adverse event reporting, as well as health care factors associated with vaccination, special populations, access, transport, storage, and home use.
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HumanosRESUMEN
ABSTRACT Purpose: To determine normal corneal tomographic parameters in children and adolescents without corneal disease or atopy diagnosis. Methods: This descriptive cross-sectional study evaluated patients aged 8-16 years who underwent a complete slit-lamp biomicroscopic examination and tomographic corneal evaluation by a dual Scheimpflug analyzer, excluding those with ocular disease (including allergic conjunctivitis) or a positive prick test for systemic atopies. Results: A total of 170 patients were evaluated, and 34 patients (68 eyes) were analyzed once the exclusion criteria were applied. The sample's mean age was 10.76 ± 2.31 years; with 19 (55.9%) men and 15 (44.1%) women. The mean keratometry in the flat meridian (Kflat), steep meridian (Ksteep), and maximum (Kmax) were 42.37 ± 1.63D, 43.53 ± 1.65D, and 43.90 ± 1.73D, respectively. The mean values for corneal asphericity (ε2) and thinnest point were 0.28 ± 0.11 and 550.20 ± 37.90 μm, respectively. The inferior-superior asymmetry ratio (I-S) and coma were 0.74 ±0.59D and 0.28 ± 0.12D, respectively. Conclusion: The knowledge of normal corneal tomographic parameters and their variation in children and adolescents without corneal disease or atopy may be useful for diagnosing keratoconus and initiating early disease treatment.
RESUMO Objetivo: Identificar parâmetros tomográficos de normalidade em córneas de crianças e adolescentes sem a presença de atopias sistêmicas e alergias oculares. Métodos: Este estudo descritivo transversal avaliou pacientes com idade entre 8 e 16 anos que foram submetidos a exame biomicroscópico completo por lâmpada de fenda e avaliação tomográfica da córnea por tomógrafo dual Scheimpflug, excluindo pacientes com doença ocular (incluindo conjuntivite alérgica) ou prick test positivo para atopias sistêmicas. Resultados: Cento e setenta pacientes foram avaliados e após cumpridos os critérios de exclusão, 34 (68 olhos) foram analisados. A média etária da amostra foi 10,76 ± 2,31 anos; 19 (55,9%) eram meninos e 15 (44,1%) meninas. A média da ceratometria em dioptrias (D) no meridiano mais plano (Kflat), mais curvo (Ksteep) e máxima (Kmax) foram 42,37 ± 1,63D, 43,53 ± 1,65D e 43,90 ± 1,73D, respectivamente. Os valores médios da asfericidade corneana (ε2) e do ponto mais fino da córnea foram 0,28 ± 0,11 e 550,20 ± 37,90 micras (μm). A assimetria corneana inferior-superior (I-S) e coma foi em média 0,74 ± 0,59D e 0,28 ± 0,12D, respectivamente. Conclusão: O conhecimento dos valores médios e sua variação de parâmetros tomográficos da córnea em crianças e adolescentes sem atopias sistêmicas ou alergias oculares pode ser útil para o diagnóstico precoce do ceratocone e o seu tratamento em estágio inicial.
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Objective: To evaluate the relationship between the occurrence of contact urticaria in reaction to cows milk in infants and the diagnosis of cows 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 cows milk and underwent a total of 30 OFC. Baked cows 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 cows milk formula) were recorded on standardized forms. The challenge was conducted with reconstituted cows milk formula in increasing volumes every 1520 min. Results: 24 OFC were performed; 13/24 (54.2%) for diagnostic purpose and 11/24 (45.8%) to evaluate cows 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 cows milk with tolerance to oral ingestion. Reactions due to oral challenge were related to a larger wheal diameter on the SPT (AU)
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Humanos , Masculino , Femenino , Niño , Adolescente , Adulto Joven , Hipersensibilidad a la Leche/complicaciones , Hipersensibilidad a la Leche/dietoterapia , Urticaria/dietoterapia , Urticaria/etiología , Estudios TransversalesRESUMEN
Background: Hereditary 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. Methods: An 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. Results: Four 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 female, 289 (55.7%) were under 50 years of age, 286 (62.9%) graduated from Medical School more than 10 years previously, 83 (18.2%) held an MSc/PhD degree, and 253 (55.6%) were living in the Southeast Region of Brazil. The median number of correct answers to the questions related to HAE among A/I was 7 out of 12 (58.3%), with median ranging from 4.5 to 8 correct answers, while for N-A/I it was 3 (25%), with median ranging from 2.5 to 4 correct answers (p < 0.001). Conclusion: Knowledge about HAE among Brazilian pediatricians, whether board certified in Allergy and Immunology or not, was unsatisfactory. HAE is a rare disease, largely unknown among physicians; therefore, increasing awareness may lead to improvement in diagnosis and treatment.
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Rinitis Alérgica , Humanos , Brasil , Rinitis Alérgica/terapia , Desensibilización InmunológicaRESUMEN
Introdução: O conhecimento da aerobiologia local é fundamental para o alergista. Os aeroalérgenos são capazes de sensibilizar e levar ao desenvolvimento de doenças respiratórias alérgicas, portanto devem ser monitorados rotineiramente, tendo em vista possíveis mudanças locais conforme alterações climáticas, poluição e atividades agroindustriais. Objetivo: Verificar a presença e concentração do alérgeno principal da poeira da casca da soja (Gly m 1) na atmosfera da cidade de Maringá-PR e possíveis associações aos fatores climáticos. A escolha da soja deve-se a alta prevalência desta cultura no Brasil e nesta região do país. Até o presente momento, há apenas um estudo piloto feito por este mesmo grupo avaliando a presença deste alérgeno no Brasil. Métodos: Foram realizadas coletas de material atmosférico, durante o período de março de 2017 a março de 2018, durante 24 ou 48 horas distribuídas no decorrer do período, totalizando 70 amostras, das quais 10 foram excluídas por problemas técnicos de coleta. As amostras foram avaliadas pelo método ELISA (Enzyme linked immunosorbent assay ) para Gly m 1, sendo que todas as amostras apresentaram níveis detectáveis do alérgeno. Resultados: A mediana de concentração de Gly m 1 foi de 4,89 ng/m3. Os valores encontrados variaram de 0,66 ng/ m3 a 1826,1 ng/m3. Das 60 amostras analisadas, 23% delas apresentaram valores superiores a 90 ng/m3, sendo os meses de junho/2017 e março/2018 com concentrações mais elevadas. Houve correlação positiva das concentrações de Gly m 1 com as temperaturas máxima, média e mínima, umidade relativa, vento e insolação. Conclusão: Os dados evidenciam exposições constantes da população ao alérgeno do Gly m 1, por vezes em níveis elevados possivelmente capazes de gerar sensibilização e sintomas.
Introduction: Knowledge of local aerobiology is essential for allergists. Because airborne allergens can sensitize the population and lead to allergic respiratory diseases, they must be routinely monitored for the effects of climate change, pollution, and agroindustry. Objective: To verify the airborne presence and concentration of the main soy hull dust allergen (Gly m 1) in Maringá, PR, Brazil and possible associations with climatic factors. Soybeans were selected due to the high prevalence of this crop in this region. To date, only 1 pilot study (conducted by our group) has evaluated this allergen's presence in Brazil. Methods: Atmospheric material was collected between March 2017 and March 2018 in 24- or 48-hour intervals, totaling 70 samples, of which 10 were excluded due to technical problems. The samples were tested for Gly m 1 using enzyme-linked immunosorbent assay, and all samples showed detectable levels of the allergen. Results: The median concentration of Gly m 1 was 4.89 ng/m3, with values ranging from 0.66 ng/m3 to 1826.1 ng/m3. Of the 60 samples, 23% showed values > 90 ng/m3, with June 2017 and March 2018 having the highest concentrations. There was a positive correlation between Gly m 1 concentration and maximum, mean, and minimum temperatures, relative humidity, wind, and insolation. Conclusion: The data show that the population is constantly exposed to the Gly m 1 allergen, sometimes at high levels, which may lead to sensitization and symptoms.
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HumanosRESUMEN
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% 1,171,76). 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 nos previamente hígidos.
This study aims to investigate the association between electronic cigarette use and lung disease in adolescents. A systematic review was conducted in PubMed. We used the MeSH terms "Electronic Nicotine Delivery Systems" and "Lung Diseases" as well as synonyms in the title and abstract, with the age filter "child: birth - 18 years" to search for articles related to electronic cigarette use and lung disease in adolescents. The eligibility criteria consisted of adolescent users and exposure to e-cigarettes that resulted in lung disease. The articles were selected by independent assessment, reading first the titles and abstracts, then the full text of the selected articles. The Newcastle-Ottawa Scale was used to assess study quality, and the included studies received between 5 and 7 stars. Finally, the data were extracted for meta-analysis. Initially, 61 articles were found and 6 were considered eligible, all of which were cross-sectional and applied questionnaires. The meta-analysis found a significant association between electronic cigarette use and asthma exacerbation (adjusted OR 1.44 95% CI 1.17 - 1.76). However, no studies evaluated the association with other lung diseases, including electronic cigarette or vaping product use-associated lung injury in adolescents. The metaanalysis revealed a significant association between e-cigarette use and asthma exacerbation among adolescents with chronic asthma, as well as among their previously healthy peers.
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Humanos , Adolescente , Medical Subject HeadingsRESUMEN
Abstract 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.
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Trasplante de Células Madre Hematopoyéticas , Enfermedades Pulmonares , Niño , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/etiología , Tomografía Computarizada por Rayos X/efectos adversosRESUMEN
A utilização de agentes imunobiológicos em alergia e imunologia tem sido cada vez mais frequente nos últimos anos, emergindo como potencialmente eficazes para o tratamento de doenças alérgicas e de hipersensibilidade. O uso de imunobiológicos em doenças alérgicas está recomendado nas formas graves onde a eficácia, segurança e custo-efetividade estão comprovados. O objetivo deste artigo é sintetizar os efeitos adversos mais comuns ou significativos, incluindo as reações de hipersensibilidade aos principais anticorpos monoclonais aprovados para o tratamento de doenças alérgicas licenciados e comercializados no Brasil até o momento.
The use of immunobiological agents in allergy and immunology has increased in recent years, emerging as potentially effective strategies to treat allergic and hypersensitivity diseases. The use of immunobiological agents is recommended in the severe forms of allergic diseases, for which their efficacy, safety, and cost-effectiveness have been established. The purpose of this study was to summarize the most common or significant adverse effects, including hypersensitivity reactions to the main monoclonal antibodies approved for the treatment of allergic diseases that are currently licensed and marketed in Brazil.
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Humanos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , OmalizumabRESUMEN
A dermatite atópica é a doença inflamatória cutânea mais prevalente mundialmente. A via JAK/STAT tem papel importante no mecanismo da doença e as pequenas moléculas inibidores de JAK são fármacos com grande potencial de uso na dermatite atópica. Foi realizada uma revisão sistemática da literatura na base de dados PubMed, utilizando os termos "atopic dermatitis" e/ou "JAK inhibitors" e/ou "small molecules" entre 2017 e 2022. Foram incluídos os resultados disponíveis de estudos de fase 3, avaliando o uso de inibidores de JAK em apresentações tópicas e sistêmicas. Entre 646 estudos, foram selecionados 37 em humanos que avaliaram a eficácia e segurança dos inibidores de JAK. Os resultados do uso, quando bem indicados, mostraram-se positivos e em alguns casos superiores a outros tratamentos já preconizados para o controle da dermatite atópica, com um bom perfil de segurança.
Atopic dermatitis is the most common inflammatory skin disease worldwide. The JAK/STAT pathway plays an important role in the disease mechanism, and small-molecule JAK inhibitors are drugs with great potential for use in atopic dermatitis. We systematically reviewed PubMed using the search terms "atopic dermatitis" AND/OR "JAK inhibitors" AND/OR "small molecules" for studies published between 2017 and 2022. Results from phase III trials evaluating both topical and systemic application of JAK inhibitors were included. Of 646 studies retrieved, 37 evaluating the efficacy and safety of JAK inhibitors in humans were selected for analysis. When properly indicated, the use of JAK inhibitors yielded positive results, some of which were superior to those of recommended treatments for the control of atopic dermatitis, with a good safety profile.
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HumanosRESUMEN
Introdução: A asma é a doença crônica mais prevalente na infância. O controle da doença é desafiador, porém fundamental para evitar exacerbações graves e danos em longo prazo. Estudos em adultos já mostraram que a baixa adesão medicamentosa, bem como aos cuidados do ambiente, impactam no controle da doença. Objetivo: Conhecer a adesão ao tratamento da asma na população pediátrica e associá-lo ao controle da doença e outras variáveis clínicas. Métodos: Trata-se de um estudo observacional transversal onde foram incluídos 104 pacientes com asma, acompanhados no Serviço de Alergia, Imunologia e Pneumologia Pediátrica do Complexo Hospital de Clínicas da Universidade Federal do Paraná. Foram realizadas entrevistas com base em questionários sobre adesão ao uso de medicação, controle ambiental e crenças populares sobre a asma. Resultados: Foi possível identificar uma correlação positiva entre pacientes que acreditavam em um ou mais mitos sobre a asma e pior adesão ao uso da medicação (p = 0,025). Também foi possível identificar uma relação significativa, entre uma boa adesão à medicação e o controle total da asma (p = 0,038) medido pelo Asthma Control Test (ACT) de 25 pontos. Cinquenta e um por cento dos participantes entrevistados relatou boa e ótima adesão ao controle de ambiente. Conclusão: A adesão e o controle de ambiente avaliados foram satisfatórios na população de crianças asmáticas de um ambulatório de referência. As crenças populares mostraram influência na adesão e no controle da asma dos pacientes entrevistados. Os achados reforçam a importância da comunicação assertiva entre médico e paciente, bem como do papel da educação da asma também voltada para a população pediátrica.
Background: Asthma is the most common chronic disease in childhood. Disease control is challenging but critical to prevent severe exacerbations and long-term damage. Studies in adults have shown that poor adherence to medication and environmental control practices has an impact on disease control. Objective: To determine pediatric asthma treatment adherence and associate it with disease control and other clinical variables. Methods: This was a cross-sectional observational study of 104 patients with asthma followed up at the Pediatric Allergy, Immunology and Pulmonology Service of the Hospital de Clínicas Complex of the Federal University of Paraná, south of Brazil. Participants were interviewed using questionnaires about medication adherence, environmental control, and popular myths about asthma. Results: There was a positive correlation between patients who believed in 1 or more myths about asthma and poorer medication adherence (p=0.025). There was also a significant association between good medication adherence and total asthma control (p=0.038) measured by the 25-point Asthma Control Test. Good and excellent adherence to environmental control practices was reported by 51% of respondents. Conclusion: Medication adherence and environmental control were satisfactory in the population of asthmatic children from a specialized outpatient clinic. Popular beliefs influenced adherence and asthma control in these patients. The findings highlight the importance of assertive communication between physicians and patients, as well as of pediatric asthma education programs.
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Humanos , Preescolar , Niño , AdolescenteRESUMEN
Neste relato descrevemos as características clínicas, epidemiológicas e radiológicas da pneumonite de hipersensibilidade, uma causa rara de insuficiência respiratória em pediatria. Paciente masculino, com 8 anos de idade, proveniente da zona rural, admitido em serviço terciário por quadro de febre, vômitos, tosse seca, dispneia progressiva, anorexia e perda de peso há 15 dias, associado a taquipneia, esforço respiratório, hipóxia e estertores finos em base direita. Tomografia computadoriza de tórax demonstrou opacidades com atenuações em vidro fosco, com comprometimento difuso e distribuição predominantemente centrolobular e acinar, característicos de pneumonite por hipersensibilidade. Na revisão das condições e hábitos de vida, foi relatado pela responsável do paciente a presença de um aviário e convívio com aves de várias espécies na residência, reforçando a hipótese diagnóstica, após descartadas outras causas de insuficiência respiratória. Iniciado corticoterapia com metilprednisolona 1 mg/ kg/dia por 7 dias, seguido de redução progressiva nas semanas posteriores. Paciente evoluiu com melhora do quadro e alta hospitalar, após orientações sobre controle ambiental e importância do afastamento dos antígenos desencadeantes. A pneumonite por hipersensibilidade é uma síndrome incomum na população pediátrica, que pode levar à insuficiência respiratória e fibrose pulmonar, devendo ser considerada nos pacientes com epidemiologia positiva. Pela sua raridade e semelhança com outras infecções respiratórias, ressalta-se ainda a importância da coleta de dados sobre os hábitos de vida dos pacientes, destacando sua importância para a elucidação diagnóstica.
We report the clinical, epidemiological, and radiological features of hypersensitivity pneumonitis, a rare cause of respiratory failure in pediatrics. An 8-year-old male patient, from a rural area, was admitted to a tertiary care facility for fever, vomiting, dry cough, progressive dyspnea, anorexia, and weight loss for 15 days, associated with tachypnea, respiratory effort, hypoxia, and fine rales at the right base. Chest computed tomography showed ground-glass opacities, diffuse involvement, and predominantly centrilobular and acinar distribution, characteristic of hypersensitivity pneumonitis. In the review of living conditions and habits, the patient's guardian reported the presence of an aviary and interaction with birds of various species in the residence, supporting the presumptive diagnosis of hypersensitivity pneumonitis, after ruling out other causes of respiratory failure. Corticosteroid therapy was started with methylprednisolone 1 mg/kg/day for 7 days, followed by tapering over subsequent weeks. The patient's condition improved, and he was discharged home after receiving guidance on environmental control and the importance of removing the triggering antigens. Hypersensitivity pneumonitis is an uncommon syndrome in the pediatric population. It can lead to respiratory failure and pulmonary fibrosis and should therefore be considered in patients with a positive epidemiological history. Due to its rarity and similarity to other respiratory diseases, collecting data on patients' lifestyle habits is highlighted as an important diagnostic tool.
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Humanos , Masculino , NiñoRESUMEN
PURPOSE: To determine normal corneal tomographic parameters in children and adolescents without corneal disease or atopy diagnosis. METHODS: This descriptive cross-sectional study evaluated patients aged 8-16 years who underwent a complete slit-lamp biomicroscopic examination and tomographic corneal evaluation by a dual Scheimpflug analyzer, excluding those with ocular disease (including allergic conjunctivitis) or a positive prick test for systemic atopies. RESULTS: A total of 170 patients were evaluated, and 34 patients (68 eyes) were analyzed once the exclusion criteria were applied. The sample's mean age was 10.76 ± 2.31 years; with 19 (55.9%) men and 15 (44.1%) women. The mean keratometry in the flat meridian (Kflat), steep meridian (Ksteep), and maximum (Kmax) were 42.37 ± 1.63D, 43.53 ± 1.65D, and 43.90 ± 1.73D, respectively. The mean values for corneal asphericity (ε2) and thinnest point were 0.28 ± 0.11 and 550.20 ± 37.90 µm, respectively. The inferior-superior asymmetry ratio (I-S) and coma were 0.74 ±0.59D and 0.28 ± 0.12D, respectively. CONCLUSION: The knowledge of normal corneal tomographic parameters and their variation in children and adolescents without corneal disease or atopy may be useful for diagnosing keratoconus and initiating early disease treatment.
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O angioedema hereditário é uma doença autossômica dominante caracterizada por crises recorrentes de edema que acometem o tecido subcutâneo e o submucoso, com envolvimento de diversos órgãos. Os principais locais afetados são face, membros superiores e inferiores, as alças intestinais e as vias respiratórias superiores. Em decorrência da falta de conhecimento dessa condição por profissionais de saúde, ocorre atraso importante no seu diagnóstico, comprometendo a qualidade de vida dos indivíduos afetados. Além disso, o retardo no diagnóstico pode resultar em aumento da mortalidade por asfixia devido ao edema de laringe. A natureza errática das crises com variação do quadro clínico e gravidade dos sintomas entre diferentes pacientes, e no mesmo paciente ao longo da vida, se constitui em desafio no cuidado dos doentes que têm angioedema hereditário. O principal tipo de angioedema hereditário é resultante de mais de 700 variantes patogênicas do gene SERPING1 com deficiência funcional ou quantitativa da proteína inibidor de C1, porém nos últimos anos outras mutações foram descritas em seis outros genes. Ocorreram avanços importantes na fisiopatologia da doença e novas drogas para o tratamento do angioedema hereditário foram desenvolvidas. Nesse contexto, o Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH) em conjunto com a Associação Brasileira de Alergia e Imunologia (ASBAI) atualizou as diretrizes brasileiras do angioedema hereditário. O maior conhecimento dos diversos aspectos resultou na divisão das diretrizes em duas partes, sendo nessa primeira parte abordados a definição, a classificação e o diagnóstico.
Hereditary angioedema is an autosomal dominant disease characterized by recurrent attacks of edema that affect the subcutaneous tissue and the submucosa, involving several organs. The main affected sites are the face, upper and lower limbs, gastrointestinal tract, and upper airways. Because health professionals lack knowledge about this condition, there is a significant delay in diagnosis, compromising the quality of life of affected individuals. Furthermore, delayed diagnosis may result in increased mortality from asphyxia due to laryngeal edema. The erratic nature of the attacks with variations in clinical course and severity of symptoms among different patients and in one patient throughout life constitutes a challenge in the care of patients with hereditary angioedema. The main type of hereditary angioedema results from more than 700 pathogenic variants of the SERPING1 gene with functional or quantitative deficiency of the C1 inhibitor protein, but in recent years other mutations have been described in six other genes. Important advances have been made in the pathophysiology of the disease, and new drugs for the treatment of hereditary angioedema have been developed. In this context, the Brazilian Study Group on Hereditary Angioedema (GEBRAEH) in conjunction with the Brazilian Association of Allergy and Immunology (ASBAI) updated the Brazilian guidelines on hereditary angioedema. Greater knowledge of different aspects resulted in the division of the guidelines into two parts, with definition, classification, and diagnosis being addressed in this first part.
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Humanos , Terapéutica , Clasificación , Diagnóstico , Angioedemas Hereditarios , Calidad de Vida , Asfixia , Signos y Síntomas , Sociedades Médicas , Preparaciones Farmacéuticas , Glicoproteínas , Edema Laríngeo , Alergia e Inmunología , MutaciónRESUMEN
O tratamento do angioedema hereditário tem início com a educação dos pacientes e familiares sobre a doença, pois é fundamental o conhecimento da imprevisibilidade das crises, assim como os seus fatores desencadeantes. O tratamento medicamentoso se divide em terapia das crises e profilaxia das manifestações clínicas. As crises devem ser tratadas o mais precocemente possível com o uso do antagonista do receptor de bradicinina, o icatibanto ou o concentrado de C1-inibidor. É necessário estabeler um plano de ação em caso de crises para todos os pacientes. A profilaxia de longo prazo dos sintomas deve ser realizada preferencialmente com medicamentos de primeira linha, como concentrado do C1-inibidor ou o anticorpo monoclonal anti-calicreína, lanadelumabe. Como segunda linha de tratamento temos os andrógenos atenuados. Na profilaxia de curto prazo, antes de procedimentos que podem desencadear crises, o uso do concentrado de C1-inibidor é preconizado. Existem algumas restrições para uso desses tratamentos em crianças e gestantes que devem ser consideradas. Novos medicamentos baseados nos avanços do conhecimento da fisiopatologia do angioedema hereditário estão em desenvolvimento, devendo melhorar a qualidade de vida dos pacientes. O uso de ferramentas padronizadas para monitorização da qualidade de vida, do controle e da atividade da doença são fundamentais no acompanhamento destes pacientes. A criação de associações de pacientes e familiares de pacientes com angioedema hereditário tem desempenhado um papel muito importante no cuidado destes pacientes no nosso país.
The treatment of hereditary angioedema begins with the education of patients and their families about the disease, as it is essential to know the unpredictability of attacks as well as their triggering factors. Drug treatment is divided into attack therapy and prophylaxis of clinical manifestations. Attacks should be treated as early as possible with the bradykinin receptor antagonist icatibant or C1-inhibitor concentrate. An action plan needs to be established for all patients with attacks. Long-term prophylaxis of symptoms should preferably be performed with first-line drugs such as C1-inhibitor concentrate or the anti-kallikrein monoclonal antibody lanadelumab. Attenuated androgens are the second line of treatment. In short-term prophylaxis, before procedures that can trigger attacks, the use of C1-inhibitor concentrate is recommended. There are some restrictions for the use of these treatments in children and pregnant women that should be considered. New drugs based on advances in knowledge of the pathophysiology of hereditary angioedema are under development and are expected to improve patient quality of life. The use of standardized tools for monitoring quality of life and controlling disease activity is essential in the follow-up of these patients. The creation of associations of patients and families of patients with hereditary angioedema has played a very important role in the care of these patients in Brazil.
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Humanos , Quimioterapia , Angioedemas Hereditarios , Anticuerpos Monoclonales Humanizados , Antagonistas de los Receptores de Bradiquinina , Pacientes , Calidad de Vida , Terapéutica , Bradiquinina , Preparaciones Farmacéuticas , Calicreínas , Medicamentos de ReferenciaRESUMEN
The association between symptoms of asthma, rhinoconjunctivitis (RC) and allergic conjunctivitis (AC) is frequent, and AC is considered a comorbidity of asthma and allergic rhinitis (AR). Ocular symptoms are often underestimated and undertreated. Differences according to gender were reported, because girls present symptoms more frequently. The development of RC depends on genetic and environmental factors, and recent studies have indicated that gender, family history of atopy, early sensitization, food allergy, and atopic dermatitis are risk factors for allergic RC. There are six well-defined clinical forms of ocular allergy: seasonal AC, perennial AC, vernal keratoconjunctivitis, atopic keratoconjunctivitis, and contact blepharoconjunctivitis (AU)