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Background Various bacterial and viral assemblages composing Cystic Fibrosis (CF) lung microbiota contribute to long-term lung function decline over time. Yet, the impact of individual microorganisms on pulmonary functions remains uncertain in children with CF. Methods As part of the 'Mucoviscidosis, respiratory VIruses, intracellular Bacteria and fastidious organisms'' project, children with CF were longitudinally followed in a Swiss multicentric study. Respiratory samples included mainly throat swabs and sputa samples for bacterial culture and 16S rRNA metagenomics and nasopharyngeal swabs for respiratory virus detection by molecular assays. Percentage of predicted Forced Expiratory Volume in one second (FEV1%) and Lung Clearance Index (LCI) were recorded. Results Sixty-one children, of whom 20 (32.8%) presented with at least one pulmonary exacerbation, were included. Almost half of the 363 nasopharyngeal swabs tested by RT-PCR were positive for a respiratory virus, mainly rhinovirus (26.5%). From linear mixed-effects regression models, P. aeruginosa (-11.35, 95%CI [-17.90; -4.80], p = 0.001) was significantly associated with a decreased FEV1%, whereas rhinovirus was associated with a significantly higher FEV1% (+4.24 95%CI [1.67; 6.81], p = 0.001). Compared to conventional culture, 16S rRNA metagenomics showed a sensitivity and specificity of 80.0% and 85.4%, respectively for detection of typical CF pathogens. However, metagenomics detected a bacteria almost twice more often than culture. Conclusions As expected, P. aeruginosa impacted negatively on FEV1% while rhinovirus was surprisingly associated with better FEV1%. Culture-free assays identifies significantly more pathogens than standard culture, with disputable clinical correlation.

Respiratorische Symptome sind bei körperlicher Aktivität häufig, bei Elitesportlern ebenso wie bei Freizeitsportlern. Beschrieben werden vor allem Atemnot bei Belastung, Husten, Thoraxschmerzen oder Giemen. Diese Symptome können nicht nur die Gesundheit des Kindes/Jugendlichen negativ beeinflussen, sondern auch seine Freude an körperlicher Betätigung und am Training, sowie auch seine sportlichen Leistungen beeinträchtigen.

Les symptômes respiratoires durant l’exercice sont fréquents, tant chez les athlètes de compétition que les sportifs récréatifs. Principalement, l’essoufflement à l’effort, la toux, les douleurs thoraciques ou encore le wheezing sont décrits. Ces symptômes peuvent non seulement avoir un impact négatif sur la santé de l’enfant/adolescent, mais aussi altérer son plaisir à pratiquer une activité physique, sa capacité à s’entraîner voire ses performances sportives.

Lactic acidosis is a common complication of status asthmaticus in adults. However, data is sparse in children. The aim of this study was to describe the prevalence and risk factors for lactic acidosis in children hospitalised for acute moderate or severe asthma. A total of 154 children 2–17 years of age were enrolled in a prospective observational study conducted in a tertiary hospital. All had capillary blood gas assessment 4 h after the first dose of salbutamol in hospital. The primary endpoint was the prevalence of lactic acidosis. Potential contributing factors such as age, sex, BMI, initial degree of asthma severity, type of salbutamol administration (nebuliser or inhaler), steroids, ipratropium bromide, and glucose-containing maintenance fluid represented secondary endpoints. All in all, 87% of patients had hyperlactatemia (lactate concentration > 2.2 mmol/l). Lactic acidosis (lactate concentration > 5 mmol/l and anion gap ≥ 16 mmol/l) was observed in 26%. In multivariate analysis, age more than 6 years (OR = 2.8, 95% CI 1.2–6.6), glycemia above 11 mmol/l (OR = 3.2 95% CI 1.4–7.4), and salbutamol administered by nebuliser (OR = 10, 95% CI 2.7–47) were identified as risk factors for lactic acidosis in children with moderate or severe asthma.Conclusion: Lactic acidosis is a frequent and early complication of acute moderate or severe asthma in children. What is Known:• Lactic acidosis during acute asthma is associated with b2-mimetics administration.• Salbutamol-related lactic acidosis is self-limited but important to recognise, as compensatory hyperventilation of lactic acidosis can be mistaken for respiratory worsening and lead to inappropriate supplemental bronchodilator administration.What is New:• Lactic acidosis is a frequent complication of acute asthma in the paediatric population.• Age older than 6 years, hyperglycaemia, and nebulised salbutamol are risk factors for lactic acidosis during asthma.

Background Lung disease can develop within the first year of life in infants with cystic fibrosis (CF). However, the frequency and severity of respiratory symptoms in infancy are not known. Methods We assessed respiratory symptoms in 50 infants with CF and 50 healthy matched controls from two prospective birth cohort studies. Respiratory symptoms and respiratory rate were documented by standardized weekly interviews throughout the first year. Infants performed multiple breath washout in the first weeks of life. Results We analyzed 4552 data points (2217 in CF). Respiratory symptoms (either mild or severe) were not more frequent in infants with CF (OR:1.1;95% CI:[0.76, 1.59]; p=0.6). Higher lung clearance index and higher respiratory rate in infants with CF were not associated with respiratory symptoms. Conclusions We found no difference in respiratory symptoms between healthy and CF infants. These data indicate that early CF lung disease may not be captured by clinical presentation alone.

Aim To summarize lung function characteristics of athletic swimmers and discuss mechanisms explaining these changes while puting forward the lack of a clear understanding of the precise physiological factors implicated. Methods Literature search until 07.2021 on Medline and EMBASE using key words swimming, athletes, respiratory physiology, lung development, lung function tests. Relevant articles in french and english were reviewed. Results We found insufficient data to perform a meta-analysis. However, there are evidences that swimmers have better expiratory flows and increased baseline lung volumes than non-athletes or non-swimmers. Although these features can result from changes in lung development following intense training over the years, the contribution of a genetic predisposition and positive selection cannot be totally excluded. Conclusion Disentangling the participation of constitutional factors and years of hard training to explain the larger lung volumes of athletic swimmers is in favour of an adaptative response of the lungs to early swim training through modification of the pathway of lung development. There seems to be an optimal window of opportunity before the end of growth for these adaptational changes to occur. Precise mechanisms, and contribution of adaptative change on lung physiology, remain to be further studied.

Lactic acidosis is a common complication of status asthmaticus in adults. However, data is sparse in children. The aim of this study was to describe the prevalence and risk factors for lactic acidosis in children hospitalised for acute moderate or severe asthma. A total of 154 children 2-17 years of age were enrolled in a prospective observational study conducted in a tertiary hospital. All had capillary blood gas assessment 4 h after the first dose of salbutamol in hospital. The primary endpoint was the prevalence of lactic acidosis. Potential contributing factors such as age, sex, BMI, initial degree of asthma severity, type of salbutamol administration (nebuliser or inhaler), steroids, ipratropium bromide, and glucose-containing maintenance fluid represented secondary endpoints. All in all, 87% of patients had hyperlactatemia (lactate concentration2.2 mmol/l). Lactic acidosis (lactate concentration5 mmol/l and anion gap ≥ 16 mmol/l) was observed in 26%. In multivariate analysis, age more than 6 years (OR = 2.8, 95% CI 1.2-6.6), glycemia above 11 mmol/l (OR = 3.2 95% CI 1.4-7.4), and salbutamol administered by nebuliser (OR = 10, 95% CI 2.7-47) were identified as risk factors for lactic acidosis in children with moderate or severe asthma.Conclusion: Lactic acidosis is a frequent and early complication of acute moderate or severe asthma in children. What is Known: • Lactic acidosis during acute asthma is associated with b2-mimetics administration. • Salbutamol-related lactic acidosis is self-limited but important to recognise, as compensatory hyperventilation of lactic acidosis can be mistaken for respiratory worsening and lead to inappropriate supplemental bronchodilator administration. What is New: • Lactic acidosis is a frequent complication of acute asthma in the paediatric population. • Age older than 6 years, hyperglycaemia, and nebulised salbutamol are risk factors for lactic acidosis during asthma.

Objectives: The contribution of intracellular and fastidious bacteria in Cystic fibrosis (CF) pulmonary exacerbations, and progressive lung function decline remains unknown. This project aimed to explore their impact on bacterial microbiota diversity over time in CF children. Methods: Sixty-one children enrolled in the MUCOVIB multicentre prospective cohort provided 746 samples, mostly nasopharyngeal swabs, throat swabs and sputa which were analysed using culture, specific real-time qPCRs and 16S rRNA amplicon metagenomics. Results: Chlamydia pneumoniae (n = 3) and Mycoplasma pneumoniae (n = 1) were prospectively documented in 6.6% of CF children. Microbiota alpha-diversity in children with a documented C. pneumoniae was highly variable, similarly to children infected with Staphylococcus aureus or Pseudomonas aeruginosa. The transition from routine follow-up visits to pulmonary exacerbation (n = 17) yielded variable changes in diversity indexes with some extreme loss of diversity. Conclusions: The high rate of C. pneumoniae detection supports the need for regular screenings in CF patients. A minor impact of C. pneumoniae on the microbial community structure was documented. Although detected in a single patient, M. pneumoniae should also be considered as a possible aetiology of lung infection in CF subjects., This study documented Chlamydia pneumoniae in 4.9% of children with cystic fibrosis, stressing the need for routine screening, and investigated its impact on airway microbiota by 16S amplicon-based metagenomics.

INTRODUCTION Normalized phase III slope (Sn ) indices from multiple breath washout (MBW) estimate ventilation inhomogeneity. Alternate (*) protocols for Sn indices exist, however the utility of these outcomes in children with mild-to-moderate cystic fibrosis (CF) is unknown. METHODS We measured nitrogen MBW and spirometry in 135 children (43 controls) aged 4-18 years. We assessed validity, practicability, and reliability of Sn protocols. Outcomes included the ability to detect abnormal lung function, test agreement, measurement duration, intra-test repeatability, and quality. RESULTS Lung clearance index (LCI) was abnormal in 80 (87%), Scond in 55 (60%), Scond* in 17 (19%), Sacin in 10 (11%), Sacin* in 11 (12%), and FEV in 28 (30%). Alternate protocols reduced measurement duration. Agreement of indices to detect abnormal lung function was poor. The quality of analysis and repeatability deteriorated with the alternate technique compared to standard. CONCLUSION In children with mild-to-moderate CF lung disease, alternate protocols seem practical but clinimetric properties of standard Sn protocols are preferable.

BACKGROUND Increased (abnormal) ventilation inhomogeneity in individuals with mild Cystic Fibrosis (CF) lung disease may become a treatable trait for small-molecule therapeutics improving Cystic Fibrosis Transmembrane Regulator (CFTR) function. The relationship between CFTR function and ventilation inhomogeneity is unknown. We aimed to identify and quantify increased ventilation inhomogeneity in relation to CFTR function. METHODS This was an international, multi-center, cross-sectional study. We collated data from individuals aged 3-25 years with minimal (CFTR-MF) or residual (CFTR-RF) function of a variety of CFTR genotypes and FEV1 ≥ 70% predicted. We measured lung function using nitrogen multiple-breath washout and spirometry. We compared lung clearance index (LCI) and FEV1 between individuals with CFTR-MF vs CFTR-RF using a mixed effects multi-variable linear regression model to account for study differences and a logistic model based on propensity-score matching to adjust for possible confounding. RESULTS We included 141 with CFTR-MF and 35 with CFTR-RF. LCI (> 1.96 z-score) was elevated in 71.6% individuals with CFTR-MF and in 40.0% with CFTR-RF. FEV1 (< -1.96 z-score) was reduced in 11.3% individuals with CFTR-MF and in 5.7% with CFTR-RF. The mean difference (95% CI) of LCI and FEV1 between CFTR-MF and CFTR-RF was 3.71 (1.63 to 5.79) and -0.40 (-0.83 to 0.02) z-score. The LCI differences were similar after adjustment for confounders and in individuals with normal FEV1. CONCLUSION Increased ventilation inhomogeneity is associated with less CFTR function. In individuals with mild CF lung disease, LCI can identify and quantify increased ventilation inhomogeneity, a candidate treatable trait.

Exercise induced dyspnea is a common complaint among adolescents, whether sedentary or competitive athletes. This complaint may be associated with reduced athletic performance and general fatigue. It can be caused by various etiologies, sometimes concomitant. It is important for clinicians to obtain a specific history and then perform the appropriate additional tests. The three most common diagnostic entities are exercise-induced asthma/bronchoconstriction, exercise-induced laryngeal obstruction, and physical deconditioning, each with distinctive characteristics. Therapeutic management, including environmental measures and appropriate drug treatment, must be individualized and depends on the etiology.La dyspnée d’effort est une plainte fréquente chez l’adolescent, qu’il soit sédentaire ou sportif de compétition. Cette plainte peut être associée à une baisse des performances sportives et à une fatigue générale. Elle peut être causée par diverses étiologies, parfois concomitantes. Il est important pour les cliniciens d’obtenir une anamnèse spécifique pour ensuite réaliser les examens complémentaires appropriés. Les trois entités diagnostiques les plus fréquentes sont l’asthme/bronchoconstriction induite par l’effort, l’obstruction laryngée induite par l’exercice, et le déconditionnement physique, présentant chacune des caractéristiques distinctives. La prise en charge thérapeutique doit être individualisée et dépend de l’étiologie retrouvée, incluant des mesures environnementales et un traitement médicamenteux approprié.

Objective Patients with cystic fibrosis are more susceptible than members of the general population to lung infections. Infections with Pseudomonas aeruginosa require particular attention, because they may accelerate the deterioration of lung function if not adequately treated. This study assessed the eradication rate of P. aeruginosa primoinfections, with a protocol of inhaled tobramycin and oral ciprofloxacin over a 3 months’ period. Results Retrospective single-center study from June 1st, 2007 to December 31st, 2015. Inclusion of 28 pediatric patients (11 females, 17 males), with a total of 49 primoinfections. Overall success rate of 67.3%, which is similar or even inferior to figures published in the literature.

Numerous benefits are attributed to omega-3 fatty acids (OM3) especially in cardiovascular health. However, bioavailability and clinical efficacy depend on numerous factors, including OM3 form, food matrix effects (especially the lipid content of the diet), and metabolic capacity. Here, we show in humans that a &ldquo, pre-digested&rdquo, OM3-sn-1(3)-monoacylglycerol lipid structure (OM3-MAG) has a significantly greater absorption at high therapeutic doses (2.9 g/day) than the most commonly OM3-ethyl ester (3.1 g/day) form (used for the treatment of hypertriglyceridemia), and a comparable profile to other pre-digested OM3 free fatty acids (OM3-FFA) structure (3.2 g/day). Nutritional supplement doses of MAG resulted in similar increases in OM3 blood level, compared to OM3 triacylglycerols (OM3-TAG) supplements in obese subjects (1.2 g/day) under low fat diet, and in children with cystic fibrosis (1.0 g/day). These results suggest that both forms of pre-digested OM3-MAG and OM3-FFA are effectively absorbed and re-incorporated effectively into triacylglycerols inside the enterocytes, before being exported into the chylomicrons lipid transport system. The pre-digested OM3-MAG might provide a more effective therapy in severe cardiovascular conditions where high doses of OM3 are required and a low-fat diet is indicated, which limited digestive lipase activity.

Drowning is a significant and neglected public health problem mostly affecting young children. The definition of drowning has recently been modified and the notions of « dry drowning », « secondary drowning » and « near drowning » are no longer accepted or used. However, recent articles in the media describing dry drowning of children have been reported. This entity seems to be absent of the actual scientific literature and worried parents can be reassured. Increased vigilance is the key as prevention is the most efficient intervention to reduce drowning mortality. To provide more guidance to the pediatrician and the general practitioner we are reviewing the most recent literature on the subject, as well as facts in the mater of drowning prevention.La noyade est un problème de santé publique grave et négligé touchant principalement les enfants en bas âge. La définition de la noyade a été revue récemment et les notions de noyade « sèche », « secondaire » ou « presque-noyade » ne sont plus reconnues ou utilisées. On observe néanmoins la mention récente dans les médias de noyades sèches d’enfants. L’entité décrite semble absente de la littérature médicale actuelle et les parents inquiets peuvent être rassurés. La vigilance reste toutefois de mise, car la prévention est l’intervention la plus efficace pour réduire la mortalité par noyade. Pour guider au mieux le pédiatre et le médecin de premier recours, nous revoyons les connaissances actuelles sur le sujet ainsi que les preuves en matière de prévention de la noyade.

Tuberculous dacryoadenitis occurs rarely. It should be suspected in children presenting with unremitting inflammation of the lacrimal gland despite standard antibiotic and/or anti-inflammatory treatment, and a thorough systemic evaluation should be undertaken to identify a primary infectious site. We present an adolescent girl in whom bilateral dacryoadenitis was the first sign of pulmonary tuberculosis.

Introduction: Optimal management of parapneumonic pleural effusion in children is a mater of controversy. To unify the care strategy at our institution, a multidisciplinary standardised protocol was elaborated. Aim: To evaluate the change in management after implementation of our decision-tree (DT). Method: Case control retrospective study of children 2 months to 18 years old admitted for pneumonia with pleural effusion before and after introducing the DT to compare management and outcome. Results: 81 patients eligible for complete chart review, 52 before DT (group I, median age 4.5y, IQR 1.7-14.3) and 29 after (group II, median age 4.4y, IQR 0.8-16.8). Proportion of patients undergoing chest tube insertion did not change between groups (21% vs. 17%, p=0.6) and was low. Number of chest CT decreased without statistical significance (13 vs 6, p=0.4). Duration of antibiotic treatment (15d, IQR 11-32 vs. 14d, IQR 12-21) and hospital stay (6d, IQR 2-12 vs. 6.2d, IQR 3-13.5) did not significantly alter. There was a significant increase in pain control in group II compared to group I (69% vs. 31%, p Conclusion: The implementation of a multidisciplinary DT for the management of parapneumonic effusion has not decreased the number of invasive procedures, which is overall low. However, it led to a significant improvement in pain control and nutritional outcome. As the incidence of complicated pneumonia is rising, it is of great interest to improve patient care.

Background: Nitrogen multiple breath washout (MBW) is an established method to detect ventilation inhomogeneity (VI) in cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). A specific marker for convection-dependent inhomogeneity (CDI) is Scond. In adults with severe lung disease (FEV 1 below -1.64 z-scores), phase III slope fitting became distorted and lead to a ceiling effect of Scond. Therefore Scond*, calculated from an abbreviated MBW protocol, was proposed. We determined if the ceiling effect also occurs in younger patients with less severe lung disease, and if Scond* could also be used in these patients, with the additional benefit of shorter measurement duration. Method: In a prospective cross-sectional study, we applied MBW in 132 children and adults with CF (n= 92) or PCD (n=40). Patients showed mild to moderate lung disease with mean FEV 1 of -1.1 z-scores. We calculated lung clearance index (LCI), Scond/Scond* and Sacin/Sacin*. The (*) indices were derived between 0-3 lung turn over. Abnormal ventilation heterogeneity was defined as >1.64 z-scores from our control group measured with the same settings. Primary endpoints were prevalence of abnormal ventilation heterogeneity for Scond* and the agreement with Scond and LCI. Results: Mean (SD) age was 12.4 (4.4) years. In the whole population, LCI was abnormal in 106 (80 %), Scond in 105 (80%), Scond* in 34 (26%), Sacin in 14 (11%), and Sacin* in 14 (11%). Agreement between VI estimated by Scond* was poor compared with Scond and LCI in CF and PCD. Conclusion: Scond* does not reliably capture CDI in CF or PCD patients with mild to moderate lung disease.

Background: Little is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment. Aim: This study was undertaken to provide an international snapshot of this issue. Design: An online survey was distributed across three continents. Setting: Distribution to the medical directors of the cystic fibrosis centres affiliated with the US Cystic Fibrosis Foundation, Cystic Fibrosis Australia (inclusion of New Zealand) and to every clinician member of the European Cystic Fibrosis Society. Results: More than 200 cystic fibrosis patients not considered to be terminally ill and, who voluntarily ceased treatment, were reported by the clinicians surveyed. Detailed data were reported in 102 patients (4 children, 25 adolescents and 73 adults). Only one child, six adolescents and one adult were judged by clinicians not to be competent to make the decision to stop treatment. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued. Resignation was the main reported reason for discontinuing treatment, followed by reactive depression and lack of familial support. A total of 69% of the patients received palliative care and 72% died in the 6 months following cessation of treatment. Conclusion: Death of cystic fibrosis patients, not considered to be terminally ill, is reported in Europe, the United States and Australia due to voluntary cessation of treatment.

The impact of a moderate consumption of an instant coffee on the general composition of the human intestinal bacterial population was assessed in this study. Sixteen (16) healthy adult volunteers consumed a daily dose of 3 cups of coffee during 3 weeks. Faecal samples were collected before and after the consumption of coffee, and the impact of the ingestion of the product on the intestinal bacteria as well as the quantification of specific bacterial groups was assessed using nucleic acid-based methods. Although faecal profiles of the dominant microbiota were not significantly affected after the consumption of the coffee (Dice's similarity index=92%, n=16), the population of Bifidobacterium spp. increased after the 3-week test period (P=0.02). Moreover, in some subjects, there was a specific increase in the metabolic activity of Bifidobacterium spp. Our results show that the consumption of the coffee preparation resulting from water co-extraction of green and roasted coffee beans produce an increase in the metabolic activity and/or numbers of the Bifidobacterium spp. population, a bacterial group of reputed beneficial effects, without major impact on the dominant microbiota.

BACKGROUND: Late-onset noninfectious pulmonary complications (LONIPCs) are life-threatening complications of bone marrow transplantation (BMT). Several pathological patterns are described in the literature with different prognoses, and with different relationships to graft-versus-host disease (GVHD). The role of high-resolution CT (HRCT) is not yet well established. OBJECTIVE: To illustrate different patterns of LONIPCs on HRCT in allogeneic versus autologous BMT in order to investigate the correlation with chronic GVHD (cGVHD). MATERIALS AND METHODS: A total of 67 HRCT scans were performed in 24 patients with noninfectious pulmonary disease at least 3 months after BMT (16 allogeneic, 8 autologous). Abnormality patterns and extension on HRCT images were correlated with the clinical outcome and with the severity of cGVHD. RESULTS: Of 24 patients, 9 showed LONIPCs (1 autologous, 8 allogeneic). There was a significant association between abnormalities on HRCT and severe cGVHD (P = 0.038), with no specific pattern. Prognosis seemed to be related to the severity of cGVHD and not to the extent of abnormalities on HRCT. CONCLUSION: The significant association between abnormalities on HRCT and severe GVHD suggests that LONIPCs can be a pulmonary manifestation of the disease. HRCT is a useful tool when combined with clinical data.

BACKGROUND: Cytological composition of bronchoalveolar lavage (BAL) fluid in pediatric bone marrow transplant (BMT) recipients with pulmonary complications has not been comprehensively described and BAL specific markers of pulmonary GVHD are lacking. The aim of this retrospective study was to assess the role of BAL in the diagnosis of pulmonary GVHD by comparing BAL cytological findings between pediatric allogenic BMT patients with pulmonary complications and oncology children receiving chemotherapy alone. METHODS: Retrospective analysis of BAL specimens for cytology, total and differential cell counts and presence of infections. RESULTS: Seventeen BMT and 13 chemotherapy BAL were analyzed. BAL total cell count was increased but similar between groups (96.9 x 10(4) vs. 98.2 x 10(4), P = NS). BAL cellular composition differed considerably between groups with a significantly higher number of lymphocytes (18% vs. 6.25%, P = 0.03) and a significantly lower number of neutrophils (25.9% vs. 58%, P = 0.02) in BMT BAL specimens. Atypical epithelial cells were significantly more frequent (75% vs. 30.8%, P = 0.027), and significantly more severe (P = 0.01) in BMT patients. The presence and severity of atypia was not associated with infection or pneumotoxic drug exposure (P = NS). CONCLUSION: BAL cytology differs significantly between BMT and chemotherapy patients. The presence BAL lymphocytosis and severe epithelial cell atypia concomitantly to respiratory symptoms and GVHD in other organs may suggest the diagnosis of pulmonary GHVD. Prospective studies assessing the reliability of this finding combined with markers such as epithelial cell apoptosis and increased cytokines are needed.

Fructooligosaccharides (FOS) are considered prebiotics because of their ability to promote growth of specific beneficial gut bacteria, such as bifidobacteria. Some studies reported potential immune-modulating properties. The aim of this study was to investigate the effect of FOS:inulin mix on murine response to Salmonella vaccine and evaluate the relevance toward protection against Salmonella infection. Balb/c mice were fed a diet containing 5% FOS:inulin mix or a control diet 1 wk before oral immunization with a suboptimal dose of live attenuated Salmonella typhimurium vaccine. Four weeks after vaccination, mice were infected with LD100 of virulent S. typhimurium. Specific blood Salmonella immunoglobulin G and fecal immunoglobulin A significantly increased in mice fed the diet containing prebiotics compared with control mice 4 wk postimmunization. Peritoneal macrophage phagocytic activity also significantly increased in FOS:inulin-fed mice at 1 wk postimmunization compared with control mice. No detectable effects were observed on the percentage of lymphoid cell subsets in the spleen. However, production of cytokines, interferon-gamma, interleukin-12, and tumor necrosis factor alpha, was numerically increased in spleen cell cultures stimulated with mitogens from FOS:inulin-fed mice 1 and 4 wk postimmunization. Salmonella translocation to lymphoid organs was not affected by feeding FOS:inulin. However, the improved response to Salmonella vaccine was concomitant with an increase in the survival rate of FOS:inulin-fed mice upon challenge with virulent Salmonella. No detectable effects were observed on the composition or the metabolic activity of the microbiota. Overall, the data suggest that a diet supplemented with FOS:inulin mix stimulates mucosal immunity and seems to improve efficacy of an oral vaccine.

It is not known at what age lung function impairment may arise in children with cystic fibrosis (CF). We assessed lung function shortly after birth in infants with CF diagnosed by newborn screening.We performed infant lung function measurements in a prospective cohort of infants with CF and healthy controls. We assessed lung clearance index (LCI), functional residual capacity (FRC) and tidal breathing parameters. The primary outcome was prevalence and severity of abnormal lung function (±1.64 z-scores) in CF.We enrolled 53 infants with CF (mean age 7.8 weeks) and 57 controls (mean age 5.2 weeks). Compared to controls, LCI and FRC were elevated (mean difference 0.30, 95% CI 0.02–0.60; p=0.034 and 14.5 mL, 95% CI 7.7–21.3 mL; pShortly after birth, abnormal lung function is prevalent in CF infants. Ventilation inhomogeneity or hyperinflation may serve as noninvasive markers to monitor CF lung disease and specific treatment effects, and could thus be used as outcome parameters for future intervention studies in this age group.

Aims: The primary objectives was to confirm the bifidogenic effects of fructooligosaccharides in elderly subjects (increase equal or higher than 1 log endogenous bifidobacteria per gram of faeces), and to make an exploratory investigation on non-specific immune defense parameters, such as phagocytosis and changes in lymphocyte subpopulations, in relation to the increase in endogenous bifidobacteria. Methods: The study was a pretest/posttest study of 19 elderly nursing home patients, with one period of 3 weeks of 8 g fructooligosaccharides (FOS) given in portions of 4 g, twice a day. Faecal bacteria composition was investigated using viable counts, lymphocyte subpopulation was analysed using a FACS scan, and relative expression of interleukin-6 (IL-6) by measuring levels of IL-6 mRNA in peripheral blood monocytes. Results: Bacterial counts for bifidobacteria increased by a mean of 2.8 ± 0.57 log10CFU/g faeces after 3 weeks of supplementation, and decreased by a mean of 1.1 log10CFU/g faeces after the period without FOS (post-test). Unexpected changes in non-specific immunity were observed: decreased phagocytic activity of granulocytes and monocytes, as well as a decreased expression of interleukin-6 mRNA in peripheral blood monocytes. These results suggest a possible decrease in inflammatory process in elderly subjects after FOS supplementation. Conclusion: The results confirm the bifidogenic effect of FOS with a 2 log increase in bifidobacteria counts and the frail elderly subjects showed low counts at the beginning of study. A diminution in inflammatory process is suggested by the decreased expression of IL-6 mRNA in peripheral blood monocytes. These results need confirmation in further studies.

Background: Desquamative interstitial pneumonia is a rare form of interstitial lung disease in children. Respiratory symptoms appear progressively, are often subtle, and diagnosis is often delayed by a mean of 6 months after onset. High resolution chest computed tomography is the most sensitive imaging technique for demonstrating and identifying interstitial pneumonia. The typical histologic pattern of desquamative interstitial pneumonia, with prominent clustered alveolar macrophages, diffuse reactive alveolar epithelial hyperplasia and globular proteinaceous material, is diagnostic. Desquamative interstitial pneumonia in children can be idiopathic, though it is mostly related to an inborn error of surfactant metabolism. Case presentation: We present the complex clinical course and pathologic findings of a 30-months-old Mauritian and Senegalese girl with idiopathic desquamative interstitial pneumonia and multiple extrapulmonary manifestations. To our knowledge, this is the first case report of desquamative interstitial pneumonia to occur as part of a syndrome with multiple organ involvement. Conclusion: We believe that desquamative interstitial pneumonia is not always associated with mutations of the surfactant proteins, and can still be idiopathic, especially when occurring as part of a syndrome with multiple organ involvement, as described in other interstitial lung diseases.

Chest CT is very sensitive in assessing pulmonary damage in bronchopulmonary dysplasia (BPD) and radiological findings in BPD are well described. Validated CT scores are available to assess BPD, as available in other pulmonary diseases such as cystic fibrosis.To investigate whether there is a correlation between radiological pulmonary lesions and relevant BPD clinical data (gestational age, type and duration of mechanical ventilation, and severity of BPD) and assess the usefulness of a CT score in evaluating clinical severity.Retrospective study of 19 premature infants with BPD born between 1998 and 2007 who underwent at least one chest CT during their first year of life. A total of 29 CT were blindly evaluated by two radiologists for the presence or absence of pulmonary parenchymal abnormalities described in BPD (areas of decreased attenuation, presence of bullae/emphysema, bronchial wall thickening, bronchiectasis, linear, and subpleural opacities). This score was then compared with the most relevant clinical data.All CT scans showed abnormalities. The most frequent lesion was bronchial wall thickening observed in all patients, followed by linear (89.5%) and subpleural (89.5%) opacities. Areas of decreased attenuation were found in 68.4%. Bullae/emphysema and bronchiectasis were the less frequent item described (26.3% and 21.1%, respectively). The presence of areas of decreased attenuation significantly correlated with BPD severity (P = 0.03). However, there was no significant correlation between the CT score and clinical data.This study demonstrates the potential usefulness of chest CT score to assess the severity of BPD. Areas of decreased attenuation seem the most sensitive item to predict BPD severity. More patients are needed to validate this approach and to evaluate the long-term usefulness of CT scan.

Chest physiotherapy (CP) using passive expiratory manoeuvres is widely used in Western Europe for the treatment of bronchiolitis, despite lacking evidence for its efficacy. We undertook an open randomised trial to evaluate the effectiveness of CP in infants hospitalised for bronchiolitis by comparing the time to clinical stability, the daily improvement of a severity score and the occurrence of complications between patients with and without CP. Children

To report early blood exchange transfusion in malignant pertussis and a favorable clinical outcome.A pediatric intensive care unit in a tertiary hospital in Geneva, Switzerland.A descriptive case report.An 8-wk-old girl was diagnosed with malignant pertussis (extreme leukocytosis, seizures, pneumonia, and secondary severe hypoxic respiratory failure associated with pulmonary hypertension). After administration of a one-volume blood exchange transfusion, a rapid decrease in white blood cell count (from 119,000/mm(3) to 36,500/mm(3)) was observed and followed by clinical improvement and favorable outcome despite the initial presence of all described risk factors associated with a high mortality.The use of exchange blood transfusion early in the course of the disease might help to prevent a fatal outcome of malignant pertussis.

Sleep related breathing disorders (SDB) in children has a major negative impact on their neurocognitive development and should be identified and treated early as to decrease morbidity. Children do not usually present daytime fatigue and sleepiness like adults, but rather show abnormal behavior patterns and learning disabilities. The presence of sustained nightime snoring is a good screening toll for SDB. Polysomnography is the gold standard for diagnosis even though nightime oxymetry, with its high positive predictive value, can also be used. The most frequent SDB in childhood is sleep obstructive apnea syndrome (SOAS), which generally requires surgical treatment with adenotonsillectomy. Long term evolution of pediatric SOAS still has to be defined, as the role of other diagnostic tools such as outpatient polygraphy.

Systemic onset juvenile rheumatoid arthritis is the most common rheumatologic disorder of childhood. Pleuropulmonary manifestations are rare in children in this multiorgan disease, and are usually not severe. The diagnosis of systemic onset juvenile rheumatoid arthritis is made by exclusion, in the presence of clinical findings constellation. We present the case of an 8-year-old girl who developed acute hypoxic respiratory failure as the first manifestation of systemic onset juvenile rheumatoid arthritis, then severe respiratory relapse 16 months later. Clinical and radiological improvement were achieved at both times after high dose pulse methylprednisolone therapy.

Probiotics and antioxidants could be alternatives to antibiotics in the prevention of bacterial infections in cirrhosis. The aim of the present study was to determine the effect of Lactobacillus johnsonii La1 and antioxidants on intestinal flora, endotoxemia, and bacterial translocation in cirrhotic rats.Twenty-nine Sprague-Dawley rats with cirrhosis induced by CCl(4) and ascites received Lactobacillus johnsonii La1 10(9)cfu/day in vehicle (antioxidants: vitamin C+glutamate) (n=10), vehicle alone (n=11), or water (n=8) by gavage. Another eight non-cirrhotic rats formed the control group. After 10 days of treatment, a laparotomy was performed to determine microbiological study of ileal and cecal feces, bacterial translocation, endotoxemia, and intestinal malondialdehyde (MDA) levels as index of intestinal oxidative damage.Intestinal enterobacteria and enterococci, bacterial translocation (0/11 and 0/10 vs. 5/8, P0.01), and ileal MDA levels (P0.01) were lower in cirrhotic rats treated with antioxidants alone or in combination with Lactobacillus johnsonii La1 compared to cirrhotic rats receiving water. Only rats treated with antioxidants and Lactobacillus johnsonii La1 showed a decrease in endotoxemia with respect to cirrhotic rats receiving water (P0.05).Antioxidants alone or in combination with Lactobacillus johnsonii La1 can be useful in preventing bacterial translocation in cirrhosis.

Background Exhaled nitric oxide (FeNO) is a well described marker of airway inflammation in asthma and is also known to increase after chronic exposure to inhaled allergens. It is not known whether monitoring FeNO could be useful during food challenges to detect early or subclinical reactions. Methods Forty children aged 3 to 16 years undergoing an allergen-food challenge at two centres were prospectively recruited for this study. FeNO was assessed before and repeatedly after the food-challenge. Results Data were obtained from a total of 53 challenges (16 positive, 37 negative) and were compared between the two groups. Half of the patients with a positive food challenge exhibited clinical upper respiratory symptoms. The FeNO significantly decreased in 7 of 16 patients with a positive challenge test within 60 to 90 minutes after the first symptoms of an allergic reaction. Conclusion Our results show a significant decrease in FeNO after a positive food challenge suggesting involvement of the lower airways despite absence of clinical and functional changes of lower airways. Prospective blinded studies are needed to confirm these results.