Pathophysiology of non-cystic fibrosis bronchiectasis in children and adolescents with asthma: A protocol for systematic review and meta-analysis.

BACKGROUND: The pathophysiological mechanisms by which asthma and bronchiectasis are associated are still unclear. The association of these two diseases can result in more severe symptoms and a greater number of exacerbations. OBJECTIVE: The aim of this systematic review is to collect evidence of the pathophysiology of non-cystic fibrosis bronchiectasis with associated asthma in children and adolescents, aged 6-18 years old. METHODS: A systematic and comprehensive search will be performed using eight main databases, PubMed, PubMed PMC, BVS/BIREME, Scopus, EMBASE, Cochrane Library, Scielo and Web of Science. Articles will be searched from the earliest available time to July 2023. The studied population will be composed of children and adolescents with asthma and non-cystic fibrosis bronchiectasis. From the data obtained, all articles found will be transferred to the Rayyan platform. Study selection will follow the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Protocols Checklist (PRISMA P-2015). In addition, if sufficient data are available, a meta-analysis will be conducted. Two independent reviewers will conduct the studies selection, data extraction, and risk of bias assessment. The outcome measures will be to analyze if non-cystic fibrosis bronchiectasis is related to a specific inflammatory profile. DISCUSSION: A systematic review will provide better knowledge about the etiopathogenesis and causes of the association between asthma and bronchiectasis and its role in the severity and control of asthma. Identifying, selecting and critically evaluating studies on asthma and bronchiectasis, would be possible to illuminate the characteristics of children and adolescents with associated diagnoses and provide information to help individualized treatments in order to control and prevent complications. The findings of this study will be published in a peer-reviewed journal. SYSTEMATIC REVIEW REGISTRATION: The systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO) in July 2023 (registration number CRD42023440355).

The role of vitamin D in the severity and control of asthma in children and adolescents: A protocol for systematic review and meta-analysis.

BACKGROUND: Vitamin D plays an important role in the immune system and consequently in the inflammatory process of asthma. It acts directly on the regulation of helper T lymphocytes 1 (Th1) and helper T lymphocytes 2 (Th2) cells, and regulatory T lymphocytes. Evidence shows that vitamin D acts on dendritic cells, raising inflammatory mediators and increasing the imbalance between Th1, Th2, and helper T lymphocytes 17 (Th17). Evidence shows a strong association between vitamin D levels and asthma incidence, especially in patients with severe and uncontrolled asthma. METHODS: A systematic and comprehensive search will be performed using four main databases, PubMed, EMBASE, Cochrane Library, and Web of Science. Articles will be searched from the earliest available time to august 2022. The studied population will be composed of children and or adolescents with asthma. From the data obtained, all articles found will be transferred to the Rayyan platform. Study selection will follow the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Checklist (PRISMA P-2020). In addition, if sufficient data are available, a meta-analysis will be conducted. Two independent reviewers will conduct the studies selection, data extraction, and risk of bias assessment. The outcome measures will be to analyze the serum levels of vitamin D in patients with asthma and to relate this hormone to the control and severity of the disease and its anti- inflammatory effect. RESULTS: A systematic review will provide better knowledge regarding vitamin D and its role in the severity and control of asthma. CONCLUSIONS: The findings of this study will be published in a peer-reviewed journal.

CD40 Ligand Deficiency in Latin America: Clinical, Immunological, and Genetic Characteristics.

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.

Are there differences in the physical activity level and functional capacity among children and adolescents with and without asthma?

OBJECTIVE: The aim of this study was to analyze the physical activity level, using two tools, and the functional capacity of children and adolescents with asthma and with different levels of disease control, and to compare them to those of individuals without asthma. METHODS: Cross-sectional study with children and adolescents with (asthma group, AG) and without asthma (WAG), aged from 7 to 17 years. All participants performed the six-minute walk test (6MWT), the Glittre Activities of Daily Living test (Glittre-ADL), the International Physical Activity Questionnaire (IPAQ), and daily record of steps on a pedometer. RESULTS: The study included 145 individuals with asthma and 173 individuals without asthma. The WAG walked a greater distance in the 6MWT and performed the Glittre-ADL in less time than the AG. Individuals with uncontrolled, partially controlled, and controlled asthma presented the same functional capacity. A difference was observed in the IPAQ classification, with 13.9% of participants from the WAG being sedentary, compared with 26.2% in the AG. The mean quantity of steps measured by the pedometer was higher in the WAG. CONCLUSION: There was a difference in the performance of individuals with and without asthma in the physical activity and functional capacity tests. The AG presented worse performance in the physical activity tests and, regardless of the level of asthma control, presented worse functional capacity.

Obesity increases eosinophil activity in asthmatic children and adolescents.

BACKGROUND: A clear relationship between asthma and obesity has been reported, but the mechanisms remain unclear. The aim of this study was to evaluate the influence of obesity on eosinophil activity (chemotaxis and adhesion) in asthmatic children and adolescents compared with cells from healthy volunteers. METHODS: Asthmatic obese (AO), asthmatic non-obese (ANO), non-asthmatic obese (NAO) and non-asthmatic non-obese (NANO) individuals were included in the present study. The chemotaxis of eosinophils after stimulation with eotaxin (300 ng/ml), platelet-activating factor (10 µM; PAF) and RANTES (100 ng/ml) was performed using a microchemotaxis chamber. The eosinophil peroxidase activity was measured to determine the adhesion activity of eosinophils cultivated on fibronectin-coated plates. The serum leptin, adiponectin, TNF-α and IgE levels were quantified using ELISA assays. RESULTS: The serum IgE levels and eosinophil counts were significantly higher in asthmatic (obese and non-obese) individuals compared with non-asthmatic individuals (obese and non-obese). Spontaneous eosinophil chemotaxis was greater in the AO group compared with either the ANO or NANO groups. The activation of eosinophils using eotaxin and PAF increased eosinophil chemotaxis in the AO group. RANTES treatment increased eosinophil chemotaxis in the NAO group compared with the NANO or ANO groups. The activation of eosinophils using eotaxin significantly increased eosinophil adhesion in the AO group compared with other groups. The serum leptin and TNF-α levels were higher in obese subjects (asthmatic and non-asthmatic), whereas the levels of adiponectin did not significantly differ among these groups. CONCLUSION: This study is the first to show increased eosinophilic activity (chemotaxis and adhesion) associated with high serum leptin and TNF-α levels in atopic asthmatic obese children and adolescents compared with non-obese healthy volunteers.

Pulmonary deposition of inhaled tobramycin prior to and after respiratory therapy and use of inhaled albuterol in cystic fibrosis patients colonized with Pseudomonas aeruginosa.

OBJECTIVE: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF) and whether pulmonary deposition correlates with disease severity or genotype. METHODS: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25%. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low energy all purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1). One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2). RESULTS: We included 24 patients (12 males) aged 5-27 years (mean +/- SD: 12.85 +/- 6.64 years). The Shwachman score (SS) was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were DeltaF508 homozygotes, 13 were DeltaF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017) and in heterozygotes (p = 0.043). CONCLUSIONS: The use of a respiratory therapy technique and the administration of inhaled albuterol immediately prior to the use of inhaled tobramycin decreased the pulmonary deposition of the latter in CF patients, and this reduction correlates with disease severity and genotype.

Deposição pulmonar de tobramicina inalatória antes e após fisioterapia respiratória e uso de salbutamol inalatório em pacientes com fibrose cística colonizados por Pseudomonas aeruginosa / Pulmonary deposition of inhaled tobramycin prior to and after respiratory therapy and use of inhaled albuterol in cystic fibrosis patients colonized with Pseudomonas aeruginosa

OBJETIVO: Avaliar se a fisioterapia respiratória seguida do uso de salbutamol inalatório modifica a deposição pulmonar de tobramicina inalatória em pacientes com fibrose cística (FC) e se a deposição pulmonar apresenta correlação com a gravidade da doença ou com o genótipo. MÉTODOS: Um estudo prospectivo foi realizado com pacientes com FC maiores de 6 anos e colonizados por Pseudomonas aeruginosa. Os critérios de exclusão foram exacerbação pulmonar, mudança terapêutica entre as fases do estudo e FEV1 < 25 por cento. Todos os pacientes foram submetidos à cintilografia pulmonar com câmara de cintilação com um colimador low energy all purpose para avaliar a penetração da droga após a inalação de tobramicina marcada com tecnécio (99mTc-tobramicina), e à perfusão pulmonar com 99mTc-macroagregados de albumina (fase 1). Após um mês, foi realizado o mesmo procedimento precedido de fisioterapia respiratória e administração de salbutamol inalatório (fase 2). RESULTADOS: Foram incluídos 24 pacientes (12 masculinos) com idade variando de 5 a 27 anos (média ± dp: 12,85 ± 6,64 anos). O escore de Shwachman (ES) foi excelente/bom em 8 pacientes, moderado/regular em 8 e grave em 0. A genotipagem revelou que 7 pacientes eram ΔF508 homozigotos, 13 eram ΔF508 heterozigotos, e 4 apresentavam outras mutações. A deposição pulmonar da tobramicina foi menor na fase 2 em todos os pacientes, sendo menor nos pacientes com ES moderado/regular (p = 0,017) e também nos heterozigotos (p = 0,043). CONCLUSÕES: O uso de uma técnica de fisioterapia respiratória e a administração de salbutamol inalatório imediatamente antes do uso de tobramicina inalada diminuem a deposição pulmonar desta última em pacientes com FC, e esta redução tem correlação com a gravidade da doença e genótipo.

OBJECTIVE: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF) and whether pulmonary deposition correlates with disease severity or genotype. METHODS: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25 percent. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low energy all purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1). One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2). RESULTS: We included 24 patients (12 males) aged 5-27 years (mean ± SD: 12.85 ± 6.64 years). The Shwachman score (SS) was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were ΔF508 homozygotes, 13 were ΔF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017) and in heterozygotes (p = 0.043). CONCLUSIONS: The use of a respiratory therapy technique and the administration of inhaled albuterol immediately prior to the use of inhaled tobramycin decreased the pulmonary deposition of the latter in CF patients, and this reduction correlates with disease severity and genotype.

Estudo comparativo entre diferentes abordagens educativas no sistema de alojamento conjunto / Comparative study of different educational approaches in the rooming-in care system

O Sistema de Alojamento Conjunto tem como princípio básico prestar todos os cuidados assistenciais e de orientaçäo às mäes sobre a sua saúde e a de seus filhos. O objetivo deste estudo foi comparar a eficiência de duas açöes educativas, sobre aleitamento materno e cuidados com o recém-nascido, no Hospital e Maternidade Celso Pierro da PUC-Campinas. O estudo compreendeu a aplicaçäo de um questionário as mäes abordando aspectos básicos e práticos sobre os temas: aleitamento materno, desenvolvimento do recém-nascido e noçöes de puericultura. As mäes foram distribuídas em três grupos de acordo com a estratégia educacional. Grupo 1: as mäes foram entrevistadas em três momentos, recebendo orientaçöes individuais junto ao leito durante sua permanência neste Serviço; Grupo 2: o questionário foi aplicado uma única vez após a orientaçäo de alta, que é realizada em grupo; Grupo 3: o questionário foi aplicado uma única vez, antes que as mäes recebessem qualquer orientaçäo específica, somente àquela da rotina hospitalar (grupo controle). A abordagem quanto as técnicas de amamentaçäo foi eficaz nos dois grupos de intervençäo, revelando deficiência na rotina normal do Sistema de Alojamento Conjunto. Quanto às práticas como troca de fraldas e banho do recém-nascido, a freqüência de respostas satisfatórias só ocorreu no Grupo 1 que recebeu acompanhamento mais direto e específico, mostrando que a orientaçäo em grupo näo foi suficiente para oferecer segurança à mäe para desenvolver essas práticas adequadamente. Os resultados deste estudo demonstraram que os dois programas de intervençäo educacional avaliados promoveram impacto positivo no conhecimento das mäes sobre aleitamento materno e cuidados com o recém-nascido, quando comparados com o grupo que recebeu apenas a assistência da rotina hospitalar.

Estudo comparativo entre diferentes abordagens educativas no sistema de alojamento conjunto

O Sistema de Alojamento Conjunto tem como principio basico prestar todos os cuidados assistenciais e de orientacao as maes sobre a sua saude e a de seus filhos. O objetivo deste estudo foi comparar a eficiencia de duas acoes educativas, sobre aleitamento materno e cuidados com o recem-nascido, no Hospital e Maternidade Celso Pierro da PUC-Campinas. O estudo compreendeu a aplicacao de um questionario as maes abordando aspectos basicos e praticos sobre os temas: aleitamento materno, desenvolvimento do recem-nascido e nocoes de puericultura. As maes foram distribuidas em tres grupos de acordo com a estrategia educacional: Grupo 1: as maes foram entrevistadas em tres momentos, recebendo orientacoes individuais junto ao leito durante sua permanencia neste Servico; Grupo 2: o questionario foi aplicado uma unica vez apos a orientacao de alta, que e realizada em grupo; Grupo 3: o questionario foi aplicado uma unica vez, antes que as maes recebessem qualquer orientacao especifica, somente aquela da rotina hospitalar (grupo controle). A abordagem quanto as tecnicas de amamentacao foi eficaz nos dois grupos de intervencao, revelando deficiencia na rotina normal do Sistema de Alojamento Conjunto. Quanto as praticas como troca de fraldas e banho do recem-nascido, a frequencia de respostas satisfatorias so ocorreu no Grupo 1 que recebeu acompanhamento mais direto e especifico, mostrando que a orientacao em grupo nao foi suficiente para oferecer seguranca a mae para desenvolver essas praticas adequadamente. Os resultados deste estudo demonstraram que os dois programas de intervencao educacional avaliados promoveram impacto positivo no conhecimento das maes sobre aleitamento materno e cuidados com o recem-nascidos, quando comparados com o grupo que recebeu apenas a assistencia da rotina hospitalar.