Spirometry with incentive games was applied to 207 2-5-year-old preschool children (PSC) with asthma in order to refine the quality-control criteria proposed by Aurora et al. (Am J Respir Crit Care Med 2004;169:1152-159). The data set in our study was much larger compared to that in Aurora et al. (Am J Respir Crit Care Med 2004;169:1152-159), where 42 children with cystic fibrosis and 37 healthy control were studied. At least two acceptable maneuvers were obtained in 178 (86%) children. Data were focused on 3-5-year-old children (n = 171). The proportion of children achieving a larger number of thresholds for each quality-control criterion (backward-extrapolated volume (Vbe), Vbe in percent of forced vital capacity (FVC, Vbe/FVC), time-to-peak expiratory flow (time-to-PEF), and difference (Delta) between the two FVCs (DeltaFVC), forced expiratory volume in 1 sec (DeltaFEV(1)), and forced expiratory volume in 0.5 sec (DeltaFEV(0.5)) from the two "best" curves) was calculated, and cumulative plots were obtained. The optimal threshold was determined for all ages by derivative function of rate of success-threshold curves, close to the inflexion point. The following thresholds were defined for acceptability: Vbe

. These newer statistical procedures provided us for the first time with a single equation for ages from 3 years through to 95 years. Measured values are converted to z-scores which describe how many standard deviations a measured value differs from the predicted value and these are independent of sex, age and height.

The life expectancy of patients with Duchenne muscular dystrophy (DMD) has increased. A cross-sectional study of DMD patients showed that 54 % of 13-year-old patients are obese and that 54 % of 18-year-old patients are underweight. We aimed to describe the natural evolution of weight status in DMD. This retrospective multi-centre audit collected body-weight measurements for seventy DMD patients born before 1992. The body-weight:age ratio (W:A) was used to evaluate weight status in reference to the Griffiths and Edwards chart. At the age of 13 years, 73 % were obese and 4 % were underweight. At maximal follow-up (age 15 -26 years, mean 18·3 (SD 2·3) years), 47 % were obese and 34 % were underweight. Obesity at the age of 13 years was associated with later obesity, whereas normal weight status and underweight in 13-year-old patients predicted later underweight. A W:A $151 % in 13-year-old patients predicted later obesity, and a W:A #126·5 % predicted later underweight. Our audit provides the first longitudinal information about the spontaneous outcome of weight status in DMD. Patients (13 years old) with a W:A $151 % were more likely to become obese in late adolescence, but obesity prevented later underweight. These data suggest that mild obesity in 13-year-old DMD patients (W:A between 120 and 150 %) should not be discouraged because it prevents later underweight.

BackgroundViruses are important triggers of asthma exacerbations. They are also detected outside of exacerbation. Alteration of anti-viral response in asthmatic patients has been shown although the mechanisms responsible for this defect remain unclear. The objective of this study was to compare in virus-infected and not-infected allergic asthmatic children, aged 6 to 16 years, admitted to hospital for a severe exacerbation, the innate immune response and especially the expression of pattern recognition receptor (PRR) and their function.MethodsVirus identification was performed both during the exacerbation and at steady state (eight weeks later). Data assessed at both periods included clinical features, anti-viral response and inflammation (in sputum and plasma), and PRR expression/function in blood mononuclear cells.ResultsViruses were identified in 46 out of 72 children (median age 8.9 years) during exacerbation, and among them, in 17 at steady state. IFN-β, IFN-γ and IL-29 levels in sputum and plasma were similar between infected and not infected patients at both times, as well as the expression of TLR3, RIG-I and MDA5 in blood monocytes and dendritic cells. Airway inflammation in infected patients was characterized by significantly higher IL-5 concentration and eosinophil count. Compared to patients only infected at exacerbation, the re-infected children significantly exhibited lower levels of IFN-γ in plasma and sputum at exacerbation associated with modifications in PRR expression and function in blood mononuclear cells. These re-infected patients also presented an airway neutrophilic inflammation at steady state.ConclusionOur results reports in asthmatic children that impaired anti-viral response during virus-induced exacerbation is more pronounced in a subgroup of patients prone to re-infection by virus. This subgroup is characterized by altered PRR function and a different pattern of airway inflammation.Trial registrationThis multicenter prospective study was approved by the regional investigational review board (ref: 08/07).Electronic supplementary materialThe online version of this article (10.1186/s12931-017-0672-0) contains supplementary material, which is available to authorized users.

These CI can detect and quantify the concavity of the descending limb of the MEFVL in preschool children with wheezing disorders, MEF(50%) /PEF having the highest sensitivity in detecting the concavity.

The aim of the present study was to define reference values for lung volumes and the lung transfer factor for carbon monoxide (TL,CO) for an adolescent population using thoracic volume index (TVI) and an index of pubertal stage in order to account for the variation in growth pattern between adolescents.TVI, pubertal stage by Tanner scale (PST), time since menarche, functional residual capacity measured using the helium-dilution technique, vital capacity, total lung capacity and TL,CO measured using a steady-state method were determined in 51 males (aged 13-20 yrs; PST T3-T5) and 52 females (aged 13-18 yrs; PST T2-T4; all but three had already undergone menarche).In male adolescents, height, weight, TVI, lung volumes and TL,CO increased with age. This was not the case in female adolescents. In males, the TVI was the independent variable that best correlated with pulmonary volumes. In females, height was the independent variable that best correlated with pulmonary volumes. In both sexes, the variable that best correlated with TL,CO was PST, associated with height in males.This cross-sectional study provides prediction equations for lung volumes and the lung transfer factor for carbon monoxide taking into account thoracic volume index and pubertal stage. It shows that, in adolescent males, lung and thoracic development occurs during and until the end of puberty. Conversely, in adolescent females, lung development is almost finished following menarche.

Traditional measures of respiratory function in children with Duchenne muscular dystrophy (DMD) are based on maximal inspiratory pressure (PImax) and vital capacity (VC). Sniff nasal inspiratory pressure (SNIP) measurements are easily performed by young children with neuromuscular disorders. The clinical value of SNIP in the longitudinal assessment of respiratory weakness remains to be assessed. The objective of the present study was to assess longitudinally the changes in SNIP, PImax and VC with age in DMD children. We hypothesised that their longitudinal assessment would show an earlier decline in SNIP than VC.A 3-year, prospective follow-up, at 6-month intervals of, 33 steroid-naïve, 5-20-year-old DMD patients was analysed using a linear mixed model. SNIP measurements were reliable (within-session coefficient of variation 8%). SNIP and VC increased until 10.5 and 12.5 years of age, respectively, and declined thereafter, while PImax did not change with age.SNIP was an earlier marker of decline in respiratory muscle strength (at 10.5 years) than VC (at 12.5 years) in young DMD patients. SNIP longitudinal assessment is useful in the detection of inspiratory strength decline in young DMD patients when VC values remain within normal values and as an outcome measure in clinical trials for emerging therapeutics in young DMD patients from the age of 5 years. @ERSpublications Earlier sniff nasal inspiratory pressure than vital capacity decline in follow-up of Duchenne muscular dystrophy children

Vestibular schwannoma (VS) growth in neurofibromatosis type 2 (NF2) can be responsible for brainstem compression and hearing loss. Surgical removal remains the standard therapy despite potential morbidity. Previous studies suggested that the inhibition of the VEGF-pathway with bevacizumab could result in hearing improvement, reduction of the tumor volume or both in adults. We retrospectively describe the French experience of bevacizumab treatment delivered for progressive VS in pediatric NF2 patients. Patients received Bevacizumab 5 or 10 mg/kg every 2 weeks according to the physician's choice. Follow-up included clinical assessment, audiometry and volumetric MRI every 3-6 months. Seven patients harboring 11 VS were included. The median age at inclusion was 15 years (11.4-18.8), and the median treatment duration was 11.3 months (3.2-55.6). At baseline, the median tumor volume was 1.2 cm(3) (0.52-13.5) and the median word recognition score was 90 % (0-100). We observed one major response, two minor responses and a decrease in the rate of tumor growth for the 4 other patients. The median annual growth rate before treatment was significantly higher than after 1 year of treatment (138 vs. 36 %, n = 5, p = 0.043). We noted one hearing improvement over the course of 1 year under treatment (hearing response rate was 14 %). Overall, the treatment was well tolerated. Our study supports that bevacizumab is an attractive therapeutic option for pediatric NF2 patients with growing VS. Thorough multidisciplinary evaluation is necessary to identify the best candidates prior to treatment. It is likely that a better functional outcome would be expected if targeted therapies were discussed early in the management of the disease.