Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility.The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function.Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage.This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.

Neither single nor combined symptoms and signs have satisfactory performance in predicting pediatric OSA. Alternative diagnostic models are necessary to improve the accuracy.

The main objectives of this study were to determine the incidence of Listeria spp. in Portuguese domestic refrigerators and to evaluate some of the hygienic practices in the domestic environment that might contribute to the persistence of the organisms. It was found that L. monocytogenes was present in 3 domestic refrigerators of the 86 investigated. L. grayi and L. innocua were also isolated from 4 and 1 refrigerators, respectively.Overall, the information obtained from our survey demonstrates the need for consumer education in Portugal regarding safe food handling practices. For the refrigerators investigated, 71% were operating at a temperature higher than 6.1°C, 87% were cleaned only monthly or less frequently, and only 8% were cleaned with appropriate proprietary cleaning products available in supermarkets.

Congenital diaphragmatic hernia survivors are a group of patients that requires long term periodic follow up in a multidisciplinary setting to provide adequate support and improve their quality of life.

Introduction: Pediatric pulmonary embolism (PE) is rare but associated with adverse outcomes. We aimed to characterize PE cases admitted in a tertiary hospital and to evaluate sensitivity of selected PE diagnostic prediction tools.Methods: Retrospective, descriptive study of PE cases admitted from 2008 to 2020 using data collected from hospital records. Patients were grouped according to PE severity and setting (outpatients vs. inpatients). Links and correlation with demographic characteristics, risk factors, clinical presentation, management, and outcomes were analyzed. PE diagnostic prediction tools were applied.Results: Twenty-nine PE episodes occurred in 27 patients, 62.9% female, mean age 14.1 years. Most PE were central and split between massive or submassive. One was diagnosed in autopsy. Twenty outpatients, all adolescents, were admitted for classic PE symptoms; in half of them the diagnosis had been previously missed. Risk factors included contraceptives (65%), thrombophilia (35%), obesity (20%) and autoimmunity (20%). Eight inpatients, diagnosed during cardiorespiratory deterioration (n = 5), or through incidental radiological findings (n = 3), were younger and had immobilization (87.5%), complex chronic diseases (75%), infections (75%) and central venous catheter (62.5%) as risk factors. Retrospectively, D-dimer testing and adult scores performed better than pediatric scores (sensitivity 92.9%-96% vs. 85.7%-92.9%). Both pediatric scores missed a case with a positive family history.Discussion: Pediatric PE diagnosis is often delayed or missed. Development of pediatric prediction tools from validated adult scores merits being explored. We argue clinical presentation and risk factors may be different in inpatients and outpatients and propose broader reliance on family history.

Our results show that alveolar macrophages from wheezy infants are activated to release increased amounts of tumour necrosis factor-α, as in asthma, and suggest that infants with recurrent wheezing may eventually benefit from treatment with glucocorticoids.

To test the hypothesis that alveolar macrophages (AM) from wheezy infants release increased amounts of eicosanoids, as do AM from adults with asthma, we compared eicosanoid release by unstimulated- and ionophore-A23187-stimulated AM from 13 wheezy and six nonwheezy infants and analyzed its regulation by dexamethasone in vitro. Alveolar macrophages from wheezy infants released greater amounts of thromboxane A2 (TxA2) and leukotriene B4 (LTB4) under resting conditions and of TxA2 upon stimulation than did those from control subjects. Dexamethasone induced a dose-dependent inhibition of the spontaneous and A23187-stimulated release of TxA2, but not of the A23187-stimulated release of lipoxygenase products. The inhibition of TxA2 formation was maintained when free arachidonic acid was added during A23187 stimulation, demonstrating that dexamethasone acted mainly at a postphospholipase A2 site. AM exposed to acetylsalicylate and then incubated overnight exhibited de novo cyclooxygenase synthesis, suggesting the presence of the inducible cyclooxygenase as a target for inhibition by dexamethasone. In conclusion, our findings suggest that AM from wheezy infants are activated in vivo to release eicosanoids, as are AM from asthmatic adults, and they support the therapeutic indications of glucocorticoids in severe recurrent wheezing of infancy.

Resumo O principal objetivo deste estudo foi avaliar a importância da segurança alimentar em ambientes domésticos. A prevalência e identificação de bactérias de origem alimentar foi levada a cabo através da recolha de amostras em várias localizações de 15 casas, tais como maçanetas de portas, puxadores do frigorífico e máquina de lavar louça, botões de fogão, superfícies de preparação de alimentos, torneiras e toalhas de cozinha, bem como das patas de animais domésticos que usualmente têm acesso à área da cozinha, e ainda puxadores e torneiras de WC. Um questionário foi também preparado e efetuado ao responsável pelas tarefas domésticas de modo a avaliar a experiência em práticas de higiene alimentar. A deteção e quantificação de microrganismos de origem alimentar foram realizadas de acordo com os métodos descritos na International Standards Organization (ISO), resultando num total de 125 isolados de Enterobacteriaceae spp. (19 isolados de Salmonella spp., 46 de Escherichia coli e 60 de outras Enterobacteriaceae), 86 de Staphylococcus coagulase-positive, 5 de Listeria spp. e 13 de Escherichia coli. No entanto, nas 175 amostras analisadas não foi detetado Campylobacter spp.. A resistência aos antibióticos ampicilina, cloranfenicol, ciprofloxacina, gentamicina, ácido nalidíxico, tetraciclina, trimetropin e nitrofurantoína foi avaliada nos 3 grandes grupos dos 125 isolados de Enterobacteriaceae spp. (19 isolados de Salmonella spp., 46 de Escherichia coli e 60 de outras Enterobacteriaceae). Escherichia coli e Salmonella spp. demonstraram resistência à ampicilina, cloranfenicol, tetraciclina, ácido nalidíxico e nitrofurantoína, enquanto outras Enterobacteriaceae apresentaram resistência apenas à ampicilina, trimetropin e nitrofurantoína. Resistência múltipla aos antibióticos descritos ocorreu maioritariamente nos isolados de Escherichia coli mas também em isolados de Salmonella spp. e de outras Enterobacteriaceae; no entanto, todos os isolados mostraram sensibilidade a antibióticos de grande importância clínica, como as fluoroquinolonas e os aminoglicosídeos.