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ABSTRACT 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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Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.
Assuntos
Fibrose Cística , Staphylococcus aureus Resistente à Meticilina , Humanos , Brasil , Fibrose Cística/tratamento farmacológico , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Staphylococcus aureus Resistente à Meticilina/metabolismo , Mutação , Qualidade de VidaRESUMO
A fibrose cística (FC) é uma doença genética que resulta em disfunção da proteína reguladora de condutância transmembrana da FC (CFTR), que é um canal de cloro e bicarbonato expresso na porção apical de células epiteliais de diversos órgãos. A disfunção dessa proteína resulta em manifestações clínicas diversas, envolvendo primariamente os sistemas respiratório e gastrointestinal com redução da qualidade e expectativa de vida. A FC ainda é uma patologia incurável, porém o horizonte terapêutico e prognóstico é hoje totalmente distinto e muito mais favorável. O objetivo destas diretrizes foi definir recomendações brasileiras baseadas em evidências em relação ao emprego de agentes farmacológicos no tratamento pulmonar da FC. As perguntas PICO (acrônimo baseado em perguntas referentes aos Pacientes de interesse, Intervenção a ser estudada, Comparação da intervenção e Outcome [desfecho] de interesse) abordaram aspectos relativos ao uso de moduladores de CFTR (ivacaftor, lumacaftor + ivacaftor e tezacaftor + ivacaftor), uso de dornase alfa, terapia de erradicação e supressão crônica de Pseudomonas aeruginosa, e erradicação de Staphylococcus aureus resistente a meticilina e do complexo Burkholderia cepacia. Para a formulação das perguntas, um grupo de especialistas brasileiros foi reunido e realizou-se uma revisão sistemática sobre os temas, com meta-análise quando aplicável. Os resultados encontrados foram analisados quanto à força das evidências compiladas, sendo concebidas recomendações seguindo a metodologia GRADE. Os autores acreditam que o presente documento represente um importante avanço a ser incorporado na abordagem de pacientes com FC, objetivando principalmente favorecer seu manejo, podendo se tornar uma ferramenta auxiliar na definição de políticas públicas relacionadas à FC.
Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.
Assuntos
Fibrose Pulmonar/tratamento farmacológico , Regulador de Condutância Transmembrana em Fibrose Cística , Fibrose Cística/genética , Staphylococcus aureus Resistente à Meticilina/efeitos dos fármacos , Metanálise em Rede , Antifibróticos/uso terapêutico , Anti-Infecciosos/uso terapêuticoRESUMO
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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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.
Assuntos
Fibrose Cística , Humanos , Fibrose Cística/diagnóstico , Complexo Antígeno L1 Leucocitário , Biomarcadores , Inflamação , Intestinos , Fezes , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
ABSTRACT Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.
RESUMO A fibrose cística (FC) é uma doença genética que resulta em disfunção da proteína reguladora de condutância transmembrana da FC (CFTR), que é um canal de cloro e bicarbonato expresso na porção apical de células epiteliais de diversos órgãos. A disfunção dessa proteína resulta em manifestações clínicas diversas, envolvendo primariamente os sistemas respiratório e gastrointestinal com redução da qualidade e expectativa de vida. A FC ainda é uma patologia incurável, porém o horizonte terapêutico e prognóstico é hoje totalmente distinto e muito mais favorável. O objetivo destas diretrizes foi definir recomendações brasileiras baseadas em evidências em relação ao emprego de agentes farmacológicos no tratamento pulmonar da FC. As perguntas PICO (acrônimo baseado em perguntas referentes aos Pacientes de interesse, Intervenção a ser estudada, Comparação da intervenção e Outcome [desfecho] de interesse) abordaram aspectos relativos ao uso de moduladores de CFTR (ivacaftor, lumacaftor + ivacaftor e tezacaftor + ivacaftor), uso de dornase alfa, terapia de erradicação e supressão crônica de Pseudomonas aeruginosa, e erradicação de Staphylococcus aureus resistente a meticilina e do complexo Burkholderia cepacia. Para a formulação das perguntas, um grupo de especialistas brasileiros foi reunido e realizou-se uma revisão sistemática sobre os temas, com meta-análise quando aplicável. Os resultados encontrados foram analisados quanto à força das evidências compiladas, sendo concebidas recomendações seguindo a metodologia GRADE. Os autores acreditam que o presente documento represente um importante avanço a ser incorporado na abordagem de pacientes com FC, objetivando principalmente favorecer seu manejo, podendo se tornar uma ferramenta auxiliar na definição de políticas públicas relacionadas à FC.
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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 , Pré-Escolar , Criança , AdolescenteRESUMO
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.