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Background Asthma treatment should be modified according to symptom control and future risk, but there are scarce data on what drives treatment adjustments in routine tertiary care. Objective We studied factors that drive asthma treatment adjustment in pediatric outpatient clinics. Methods We performed a cross-sectional analysis of the Swiss Paediatric Airway Cohort, a clinical cohort of 0- to 16-year-old children seen by pediatric pulmonologists. We collected information on diagnosis, treatment, lung function, and FeNO from hospital records; and on symptoms, sociodemographic, and environmental factors from a parental questionnaire. We used reported symptoms to classify asthma control and categorized treatment according to the 2020 Global Initiative for Asthma guidelines. We used multivariable logistic regression to study factors associated with treatment adjustment (step-up or down vs no change). Results We included 551 children diagnosed with asthma (mean age, 10 years; 37% female). At the clinical visit, most children were prescribed Global Initiative for Asthma step 3 (35%). Compared with previsit treatment, 252 children remained on the same step (47%), 227 were stepped up (42%), and 58 were stepped down (11%). Female sex (adjusted odds ratio [aOR] = 1.61; 95% confidence interval [CI], 1.05-2.47), poor asthma control (aOR = 3.08; 95% CI, 1.72-5.54), and lower FEV1 Z-score (aOR = 0.70; 95% CI, 0.56-0.86 per one Z-score increase) were independently associated with treatment step-up, and low FeNO (aOR = 2.34; 95% CI, 1.23-4.45) was associated with treatment step-down, with marked heterogeneity between clinics. Conclusions In this tertiary care real-life study, we identified main drivers for asthma treatment adjustment. These findings may help improve both asthma management guidelines and clinical practice.

BackgroundExercise-induced breathing problems with similar clinical presentations can have different aetiologies. This makes distinguishing common diagnoses such as asthma, extrathoracic and thoracic dysfunctional breathing (DB), insufficient fitness, and chronic cough difficult.ObjectiveWe studied which parent-reported, exercise-induced symptoms (EIS) can help distinguish diagnoses of EIS in children seen in respiratory outpatient clinics.MethodsThis study was nested in the Swiss Paediatric Airway Cohort (SPAC), an observational study of children aged 0-17 years referred to paediatric respiratory outpatient clinics in Switzerland. We studied children aged 6-17 years and compared information on EIS from parent-completed questionnaires between children with different diagnoses. We used multinomial regression to analyse whether parent-reported symptoms differed between diagnoses (asthma as base).ResultsAmong 1109 children, EIS were reported for 732 (66%) (mean age 11 years, 318 of 732 [43%] female). Among the symptoms, dyspnoea best distinguished thoracic DB (relative risk ratio [RRR] 5.4, 95%CI 1.3-22) from asthma. Among exercise triggers, swimming best distinguished thoracic DB (RRR 2.4, 95%CI 1.3-6.2) and asthma plus DB (RRR 1.8, 95%CI 0.9-3.4) from asthma only. Late onset of EIS was less common for extrathoracic DB (RRR 0.1, 95%CI 0.03-0.5) and thoracic DB (RRR 0.4, 95%CI 0.1-1.2) compared with asthma. Localisation of dyspnoea (throat vs. chest) differed between extrathoracic DB (RRR 2.3, 95%CI 0.9-5.8) and asthma. Reported respiration phase (inspiration or expiration) did not help distinguish diagnoses.ConclusionParent-reported symptoms help distinguish different diagnoses in children with EIS. This highlights the importance of physicians obtaining detailed patient histories.Highlights boxWhat is already known about this topic?Experts suggest that information about the symptoms and their onset and duration can assist accurate diagnosis of children with exercise-induced respiratory problems, but no original studies have tested this. (29/35 words)What does this article add to our knowledge?Exercise-induced symptoms reported by parents and further information about their onset, triggers, and effects of treatment help differentiate diagnoses in children with exercise-induced respiratory problems. (25/35 words)How does this study impact current management guidelines?Our results emphasize the importance of taking detailed symptom histories of children with exercise-induced problems, and suggest which questions are most helpful.

Background Nanoduct™ is a simple and practical sweat analysis system measuring conductivity in situ. It requires only three microlitres of sweat, making it especially applicable to newborns. Methods We measured conductivity in 260 healthy term infants at the age of four days, and again at four weeks to determine the proportion of successful tests, test duration, and normal values for sweat conductivity in newborns. Results Sufficient sweat was collected in 159/260 of four-day olds (61%), and in 225/239 of four-week olds (94%). Mean (sd) test duration was 27 (5) and 25 (5) min. Mean (sd, range) conductivity was 53mmol/l (16, 8–114) at age four days, and 36 (9, 12–64) at four weeks. Conclusions Determination of sweat conductivity using Nanoduct™ cannot be recommended for four-day old newborns. However, at the age of four weeks the success rate is high (94%), and conductivity values at that age are comparable to older healthy children.

Introduction: Several diagnostic tests are used in children suspected for asthma, but the accuracy of these tests to diagnose asthma is unclear. Aim: We aimed to assess the diagnostic accuracy of reported respiratory symptoms and clinical tests to diagnose asthma in school-aged children under investigation for asthma. Methods: We studied cross-sectionally children aged 6-16 years referred to 2 pulmonary outpatient clinics with suspicion of asthma. All children underwent clinical evaluation including spirometry, fractional exhaled nitric oxide (FeNO), skin prick tests, and bronchial provocation tests (BPT) by exercise, methacholine, and mannitol. Symptoms were reported in a parental questionnaire. Asthma was diagnosed by the physicians based on medical history, clinical examination, and all clinical test results. We calculated sensitivity, specificity, positive and negative predictive value and area under the curve to assess the diagnostic accuracy of symptoms and clinical tests. Results: Of the 111 participants, 68 (61%) were diagnosed with asthma. The combined sensitivity and specificity to diagnose asthma was highest for wheeze without colds ((sensitivity/specificity) 0.76/0.65), wheeze (0.87/0.51), wheeze triggered by pollen (0.59/0.79) and more than 3 attacks of wheeze (0.49/0.88). Of the diagnostic tests, the area under the curve was highest for FeNO (0.81) and BPT by exercise (0.79) and lowest for FEV1 (0.52) and FEV1/FVC (0.58). Conclusion: This study, which needs validation in a larger sample, suggests that specific questions about triggers and severity of wheeze, FeNO and BPT by exercise are more useful to diagnose asthma in school-aged children than spirometry, skin prick tests and other BPTs.

Congenital lung anomalies are a group of rare malformations, often diagnosed during the prenatal period. Guidelines on how to manage these patients are currently under debate, especially with regard to prophylactic surgery in asymptomatic patients, or how to proceed with conservative follow-up. Currently, there is no clear consensus on management strategies. A Swiss congenital lung anomaly national database and biobank was created in 2016 to enable data recording and collection of surgical lung samples in order to help define the most appropriate management strategies. This national observational cohort study represents an important step towards a better understanding of the pathophysiology and clinical course of the diseases included under congenital lung anomalies, especially in the context of a small country like Switzerland.

INTRODUCTION The contribution of clinical investigations to prediction of long-term outcomes of children investigated for asthma is unclear. AIM We performed a broad range of clinical tests and investigated whether they helped to predict long-term wheeze among children referred for evaluation of possible asthma. METHODS We studied children aged 6 to 16 years referred to two Swiss pulmonary outpatient clinics with a history of wheeze, dyspnea, or cough in 2007. The initial assessment included spirometry, fractional exhaled nitric oxide, skin prick tests, and bronchial provocation tests by exercise, methacholine, and mannitol. Respiratory symptoms were assessed with questionnaires at baseline and at follow-up 7 years later. Associations between baseline factors and wheeze at follow-up were investigated by logistic regression. RESULTS At baseline, 111 children were examined in 2007. After 7 years, 85 (77%) completed the follow-up questionnaire, among whom 61 (72%) had wheeze at baseline, while at follow-up 39 (46%) reported wheeze. Adjusting for age and sex, the following characteristics predicted wheeze at adolescence: wheeze triggered by pets (odds ratio, 4.2; 95% CI, 1.2-14.8), pollen (2.8, 1.1-7.0), and exercise (3.1, 1.2-8.0). Of the clinical tests, only a positive exercise test (3.2, 1.1-9.7) predicted wheeze at adolescence. CONCLUSION Reported exercise-induced wheeze and wheeze triggered by pets or pollen were important predictors of wheeze persistence into adolescence. None of the clinical tests predicted wheeze more strongly than reported symptoms. Clinical tests might be important for asthma diagnosis but medical history is more helpful in predicting prognosis in children referred for asthma.

OBJECTIVES In the national newborn screening programme for CF in Switzerland, we compared the performance of two sweat test methods, by investigating the feasibility and diagnostic performance of the Macroduct collection method (with chloride mesurement) and Nanoduct test (measuring conductivity) for diagnosing CF. STUDY-DESIGN We included all newborns with a positive screening result between 2011 and 2015 who were referred to a CF-centre for sweat testing. In the CF-centre, a Macroduct and Nanoduct sweat test were performed simultaneously. If sweat test results were positive or borderline, a DNA analysis was performed. Final diagnosis was based on genetic mutations. RESULTS Over 5 years, 445 children were screened positive and in 413 (114 with CF) at least one sweat test was performed (median age at first test, 22 days); both tests were performed in 371 children. A sweat test result was more often available with the Nanoduct compared to the Macroduct (79 vs 60%, P

Primary Ciliary Dyskinesia (PCD) is a rare hereditary, multi-organ disease caused by defects in ciliary structure and function. It results in a wide range of clinical manifestations, most commonly in the upper and lower airways. Central data collection in national and international registries is essential to studying the epidemiology of rare diseases and filling in gaps in knowledge of diseases such as PCD. For this reason, the Swiss Primary Ciliary Dyskinesia Registry (CH-PCD) was founded in 2013 as a collaborative project between epidemiologists and adult and paediatric pulmonologists.The registry records patients of any age, suffering from PCD, who are treated and resident in Switzerland. It collects information from patients identified through physicians, diagnostic facilities, and patient organisations. The registry dataset contains data on diagnostic evaluations, lung function, microbiology and imaging, symptoms, treatments, and hospitalizations.By May 2018, CH-PCD has contacted 566 physicians of different specialties and identified 134 patients with PCD. At present this number represents an overall 1 in 63,000 prevalence of people diagnosed with PCD in Switzerland. Prevalence differs by age and region; it is highest in children and adults younger than 30 years, and in Espace Mittelland. The median age of patients in the registry is 25 years (range 5-73), and 49 patients have a definite PCD diagnosis based on recent international guidelines. Data from CH-PCD are contributed to international collaborative studies and the registry facilitates patient identification for nested studies.CH-PCD has proven to be a valuable research tool that already has highlighted weaknesses in PCD clinical practice in Switzerland. Development of centralised diagnostic and management centres and adherence to international guidelines are needed to improve diagnosis and management—particularly for adult PCD patients.

Chronic respiratory symptoms, such as cough, wheeze and dyspnoea, are common in children; however, most research has, with the exception of a few large-scale clinical cohort studies, been performed in the general population or in small, highly-selected samples. The Swiss Paediatric Airway Cohort (SPAC) is a national, prospective clinical cohort of children and adolescents who visit physicians for recurrent conditions, such as wheeze and cough, and exercise-related respiratory problems. The SPAC is an observational study and baseline assessment includes standardised questionnaires for families and data extracted from hospital records, including results of clinically indicated investigations, diagnoses and treatments. Outcomes are assessed through annual questionnaires, monthly symptom reporting via mobile phone and follow-up visits. The SPAC will address important questions about clinical phenotypes, diagnosis, treatment, and the short- and long-term prognosis of common respiratory problems in children. The cohort currently consists of 347 patients from four major hospitals (Bern, Zurich, Basel and Lucerne), with 70–80 additional patients joining each month. More centres will join and the target sample size is a minimum of 3000 patients. The SPAC will provide real-life data on children visiting the Swiss healthcare system for common respiratory problems and will provide a research platform for health services research and nested clinical and translational studies., The Swiss Paediatric Airway Cohort (SPAC) is a unique research platform for common respiratory problems in children http://ow.ly/Y1v030lDnji

IntroductionThere are few data on the usefulness of different tests to diagnose asthma in children.AimWe assessed the contribution of a detailed history and a variety of diagnostic tests for diagnosing asthma in children.MethodsWe studied children aged 6–16 years referred consecutively for evaluation of suspected asthma to two pulmonary outpatient clinics. Symptoms were assessed by parental questionnaire. The clinical evaluation included skin-prick tests, measurement of exhaled nitric oxide fraction (FeNO), spirometry, bronchodilator reversibility and bronchial provocation tests (BPT) by exercise, methacholine and mannitol. Asthma was diagnosed by the physicians at the end of the visit. We assessed diagnostic accuracy of symptoms and tests by calculating sensitivity, specificity, positive and negative predictive values and area under the curve (AUC).ResultsOf the 111 participants, 80 (72%) were diagnosed with asthma. The combined sensitivity and specificity was highest for reported frequent wheeze (more than three attacks per year) (sensitivity 0.44, specificity 0.90), awakening due to wheeze (0.41, 0.90) and wheeze triggered by pollen (0.46, 0.83) or by pets (0.29, 0.99). Of the diagnostic tests, the AUC was highest forFeNOmeasurement (0.80) and BPT by methacholine (0.81) or exercise (0.74), and lowest for forced expiratory volume in 1 s (FEV1) (0.62) and FEV1/forced vital capacity ratio (0.66), assessed by spirometry.ConclusionThis study suggests that specific questions about triggers and severity of wheeze, measurement ofFeNOand BPT by methacholine or exercise contribute more to the diagnosis of asthma in school-aged children than spirometry, bronchodilator reversibility and skin-prick tests.

BACKGROUND Nitrogen multiple-breath washout (N2 MBW) using 100% oxygen (O2 ) has regained interest to assess efficiency of tracer gas clearance in, for example, children with Cystic Fibrosis (CF). However, the influence of hyperoxia on the infants' respiratory control is unclear. We assessed safety and impact on breathing pattern from hyperoxia, and if exposure to 40% O2 first induces tolerance to subsequent 100% O2 for N2 MBW. METHODS We prospectively enrolled 39 infants aged 3-57 weeks: 15 infants with CF (8 sedated for testing) and 24 healthy controls. Infants were consecutively allocated to the protocols comprising of 100% O2 or 40/100% O2 administered for 30 breaths. Lung function was measured using an ultrasonic flowmeter setup. Primary outcome was tidal volume (VT ). RESULTS None of the infants experienced apnea, desaturation, or bradycardia. Both protocols initially induced hypoventilation. VT temporarily declined in 33/39 infants across 10-25 breaths. Hypoventilation occurred independent of age, disease, and sedation. In the new 40/100% O2 protocol, VT returned to baseline during 40% O2 and remained stable during 100% O2 exposure. End-tidal carbon dioxide monitored online did not change. CONCLUSION The classical N2 MBW protocol with 100% O2 may change breathing patterns of the infants. The new protocol with 40% O2 induces hyperoxia-tolerance and does not lead to changes in breathing patterns during later N2 washout using 100% O2 . Both protocols are safe, the new protocol seems an attractive option for N2 MBW in infants. Pediatr Pulmonol. © 2013 Wiley Periodicals, Inc.

Acute bronchiolitis is the most common lower respiratory tract infection in infants and there is no evidence that drug treatment alters its natural course. Despite this, most Swiss paediatricians reported in 2001 prescribing bronchodilators and inhaled corticosteroids (ICS). This situation led to the creation of national guidelines followed by a tailored implementation programme. The aim of this study was to examine if treatment practices changed after the implementation of the new guidelines.A questionnaire on treatment of bronchiolitis was sent to all Swiss paediatricians before (2001) and after (2006) creation and implementation of national guidelines (2003-2005). Guidelines were created in collaboration with all paediatric pulmonologists and implemented carefully using a multifaceted approach.Questionnaires were returned by 541 paediatricians (58%) in 2001 and by 639 (54%) in 2006. While both surveys showed a wide variation in the treatment of bronchiolitis between physicians, reported drug prescription decreased significantly between the two surveys. For outpatients, general use (for all patients) of bronchodilators dropped from 60% to 23%, and general use of ICS from 34% to 6%. For inpatients, general use of bronchodilators and ICS dropped from 55% to 18% and from 26% to 6%, respectively (all p0.001). The decrease was evident in all regions, among hospital and primary care physicians, and among general paediatricians and paediatric pulmonologists.National guidelines together with a tailored implementation programme can have a major impact on medical management practices in a country.

Effects of smoke-free legislation on paediatric RTIs: more evidence that child health can be easily improved http://ow.ly/OPcWd

Background: Major haemoptysis occurs in approximately 1% of children with cystic fibrosis (CF). This report describes management and follow-up of these children at a tertiary centre in Australia. Methods: Retrospective review of medical records from 1980–1999. Results: Fifty-one children (45% female) had major haemoptysis (102 episodes). Mean age at first episode was 15 years (range 7–19) and mean FEV1 was 56% predicted (range 14–98). Massive life-threatening haemoptysis was not confined to those with severe lung disease (FEV1

To review the authors' 15-year experience with bronchial artery embolization (BAE) for treatment of hemoptysis in young patients with cystic fibrosis.By searching the 1985-1999 radiology database, the authors identified 23 young patients who had been referred to the radiology department for angiography. Twenty of these patients underwent BAE. The 23 medical records were retrospectively reviewed with regard to embolization agents used, embolization success rates, number of repeat embolizations, survival times, and causes of death.BAE was performed on 38 occasions in 20 patients. The mean age of patients at first BAE was 15 years (age range, 7-19 years). The majority (n = 34 [89%]) of BAEs were performed by using polyvinyl alcohol. The immediate success rate after BAE (ie, no recurrent bleeding within 24 hours) was 95% (36 of 38 BAEs). Eleven (55%) patients required more than one BAE, and the median time between first and second embolizations was 4 months (range, 5 days to 61 months). Three patients died as a consequence of severe hemoptysis during induction of anesthesia with intermittent positive pressure ventilation in preparation for BAE. The median survival duration after the first BAE (Kaplan-Meier estimate) was 84 months (average follow-up, 61 months; range, 5 days to 169 months).BAE had a high success rate for short-term control of bleeding; however, more than half the patients required repeat embolization during the long-term follow-up.

Airway masses are uncommon in children. The majority of bronchial tumors are granulomata secondary to an inhaled foreign body. However, other rare diseases like primary bronchopulmonary tumors should always be considered in the evaluation of a bronchial granuloma in children. The differential diagnosis of bronchial granuloma is presented. We report a 7-year-old girl with a 3-year history of recurrent cough and fevers who was found to have a bronchial granuloma in the left upper lobe bronchus. The diagnosis of foreign body-related granuloma was eventually made after combined and repeated rigid and flexible bronchoscopy. This case highlights the need to search aggressively for a foreign body in the presence of an airway granuloma in children, even in the absence of a history of aspiration.

BackgroundFractional exhaled nitric oxide (FENO), a non-invasive marker of eosinophilic airway inflammation, is increasingly used for diagnostic and therapeutic decisions in adult and paediatric asthma. Standardized guidelines for the measurement of FENO recommend performing FENO measurements before rather than after bronchial provocation tests.ObjectiveTo investigate whether FENO levels decrease after a Mannitol dry powder (MDP) challenge in a clinical setting, and whether the extent of the decrease is influenced by number of MDP manoeuvres, baseline FENO, atopy and doctor diagnosed asthma.MethodsChildren aged 6-16 years, referred for possible reactive airway disease to a respiratory outpatient clinic, performed an MDP challenge (Aridol®, Pharmaxis, Australia). FENO was measured in doublets immediately before and after the challenge test using the portable NIOX MINO® device (Aerocrine, Stockholm, Sweden). We analysed the data using Kruskal-Wallis rank tests, Wilcoxon signed rank tests and multivariable linear regressions.ResultsOne hundred and seven children completed both tests (mean±SD age 11.5±2.8 years). Overall, median (interquartile range) FENO decreased slightly by -2.5 ppb (-7.0, -0.5), from 18.5 ppb (10.5, 45.5) before the MDP challenge to 16.5 ppb thereafter (8.5, 40.5; pConclusionMDP challenge test influences FENO values but differences are small and clinically barely relevant.

Rationale Mannitol dry powder (MDP) challenge is an indirect bronchial provocation test, which is well studied in adults but not established for children. Objective We compared feasibility, validity, and clinical significance of MDP challenge with exercise testing in children in a clinical setting. Methods Children aged 6–16 years, referred to two respiratory outpatient clinics for possible asthma diagnosis, underwent standardized exercise testing followed within a week by an MDP challenge (Aridol™, Pharmaxis, Australia). Agreement between the two challenge tests using Cohen's kappa and receiving operating characteristic (ROC) curves was compared. Results One hundred eleven children performed both challenge tests. Twelve children were excluded due to exhaustion or insufficient cooperation (11 at the exercise test, 1 at the MDP challenge), leaving 99 children (mean ± SD age 11.5 ± 2.7 years) for analysis. MDP tests were well accepted, with minor side effects and a shorter duration than exercise tests. The MDP challenge was positive in 29 children (29%), the exercise test in 21 (21%). Both tests were concordant in 83 children (84%), with moderate agreement (κ = 0.58, 95% CI 0.39–0.76). Positive and negative predictive values of the MDP challenge for exercise-induced bronchoconstriction were 68% and 89%. The overall ability of MDP challenge to separate children with or without positive exercise tests was good (area under the ROC curve 0.83). Conclusions MDP challenge test is feasible in children and is a suitable alternative for bronchial challenge testing in childhood. Pediatr. Pulmonol. 2011; 46:842–848. © 2011 Wiley-Liss, Inc.

Background: Wheezing disorders in childhood vary widely in clinical presentation and disease course. During the last years, several ways to classify wheezing children into different disease phenotypes have been proposed and are increasingly used for clinical guidance, but validation of these hypothetical entities is difficult.Methodology/Principal Findings: The aim of this study was to develop a testable disease model which reflects the full spectrum of wheezing illness in preschool children. We performed a qualitative study among a panel of 7 experienced clinicians from 4 European countries working in primary, secondary and tertiary paediatric care. In a series of questionnaire surveys and structured discussions, we found a general consensus that preschool wheezing disorders consist of several phenotypes, with a great heterogeneity of specific disease concepts between clinicians. Initially, 24 disease entities were described among the 7 physicians. In structured discussions, these could be narrowed down to three entities which were linked to proposed mechanisms: a) allergic wheeze, b) non-allergic wheeze due to structural airway narrowing and c) non-allergic wheeze due to increased immune response to viral infections. This disease model will serve to create an artificial dataset that allows the validation of data-driven multidimensional methods, such as cluster analysis, which have been proposed for identification of wheezing phenotypes in children.Conclusions/Significance: While there appears to be wide agreement among clinicians that wheezing disorders consist of several diseases, there is less agreement regarding their number and nature. A great diversity of disease concepts exist but a unified phenotype classification reflecting underlying disease mechanisms is lacking. We propose a disease model which may help guide future research so that proposed mechanisms are measured at the right time and their role in disease heterogeneity can be studied.

Practicability is crucial for successful implementation of fractional exhaled nitric oxide (FeNO) measurement into asthma management. The study aimed at comparing a conventional chemiluminescence NO analyser (EcoMedics) with a hand-held device (NIOX MINO) and offline FeNO measurement using a commercially available system in an unselected cohort of children aged 6-16 yr. A secondary objective was to confirm FeNO stability over time in 15 samples from adult volunteers obtained using the offline system. Sixty-six children (mean +/- s.d. age 11.8 +/- 3.0 yr) underwent single breath FeNO measurement in triplets with each device. Offline collected FeNO was measured after offline breath collection into a Mylar balloon and subsequent analysis using the chemiluminescence NO analyser. Variability and between-method agreement were assessed, and stability over time within the Mylar balloons was tested by repeated hourly measurements. FeNO levels ranged from 2 to 113 p.p.b. Intra-class correlation was excellent (r = 0.98, p < 0.001 for each pair). Bland-Altman plots and back-transformation of logarithmic mean differences revealed fair agreement between methods. Stability over time was confirmed over 10 h both at room temperature and when stored under cooling conditions. FeNO values obtained using the chemiluminescence NO analyser, the portable NIOX MINO system and the offline collection technique show between-method agreement within clinically acceptable range.

Objectives The aim of the study was to determine if a new conductivity measuring sweat test system (Nanoduct) could reliably identify patients with cystic fibrosis (CF) and differentiate them from healthy subjects. Study design On the same day and in the same patient, the new system was tested in comparison with the Macroduct sweat collection system measuring chloride concentration and osmolality. Results Subjects (n = 111) 3 weeks to 60 years of age were investigated. Three children had no sweat production, and in 14 children, only conductivity could be measured. In the remaining 94 subjects, the new system identified all patients with classic CF (mean conductivity, 115 mmol/L; range, 92 to 137) and differentiated them from healthy subjects (mean conductivity, 36 mmol/L; range, 17 to 59) within a mean time of 20 minutes. Conclusions Measuring sweat conductivity using the new test system reliably differentiated between patients with and those without CF. This suggests that the new system could be used as a diagnostic test in addition to its suggested screening value.

Our objective was to determine the repeatability of bronchial responsiveness to mannitol dry powder (MDP) as an objective marker of asthma in children. MDP challenge was performed in children with stable asthma at the same time of the day on two separate occasions within a week. The test was terminated after a 15% fall of forced expiratory volume in 1 sec (FEV1) and the provocative dose to produce a 15% fall in FEV1 (PD15) were calculated. Seventeen children (aged 9–16 years) on inhaled corticosteroids (200–1,500 mcg) were studied. Mean baseline FEV1 before the challenges were 95% (81–119) and 96% (74–121), respectively, with a standard deviation of differences of 5.2%. PD15 values ranged from 7–387 mg, with a geometric mean of 38 mg for the first and 49 mg for the second test. Of the 17, all but two pairs of tests achieved a PD15 within one dose of capsules. Four children had a negative challenge on two occasions. A high relative reliability was reflected by a concordance coefficient of 0.86. In conclusion, MDP is a convenient challenge which is easy to administer and is well-tolerated by children. It is a highly reproducible test of airway responsiveness in children with moderate to severe persistent asthma on inhaled corticosteroids within 7 days under laboratory conditions. Pediatr Pulmonol. 2003; 36:490–494. © 2003 Wiley-Liss, Inc.

Objective: Previous studies have demonstrated that coating spacers with ionic detergents minimizes the static charge and thereby improves in vivo drug deposition. The present study aims to examine the effect of coated spacers versus non-coated spacers in the clinical situation. Methods: A randomized, double-blind study in children with asthma and a ratio of forced expiratory volume in 1 s to forced vital capacity (FEV1/FVC) of ≤72% predicted was carried out. Spirometry was performed at baseline and at 10 min and 20 min after inhalation of two puffs of salbutamol (100 µg/puff) through either a detergent-coated or a non-coated spacer. Results: Fifty children were studied (mean age 11.6 years, range 7−18 years): 26 in the group using coated spacers (CG); and 24 in the group using non-coated spacers (NCG). The mean percentage change in FEV1 from baseline 10 min after inhalation was 18.8% (range 5−50%) in the CG versus 18.5% (range 3−35%) in the NCG. At 20 min after inhalation, the per cent increase in FEV1 was 19.8% (range 0−50%) in the CG versus 19.5% (range 9−35%) in the NCG. There was no significant difference between groups in the percentage change in FEV1 after 10 min (P = 0.91), or after 20 min (P = 0.93). Conclusions: There was no improvement in bronchodilatation from detergent-coated spacers in the present study, possibly because a maximal bronchodilator response was achieved with the lower output.

Objective: Implementation of clinical guidelines is frequently delayed well beyond their dissemination and the publication of clinical evidence. The recently published Australian guidelines for the management of acute viral bronchiolitis (AVB) have been evaluated by assessing the current practice of Australian paediatricians. Methods: Questionnaire survey of all Australian paediatricians and a review of the literature. Results: Of a total of 891 questionnaires, 555 (62%) were returned. Of the respondents, 373 (67%) treated children with AVB and, of these, 232 (67%) treated 10–50 children per year. A wide variation in management practice for both outpatient and inpatient treatment of AVB was identified. Up to 70% of paediatricians who treated AVB indicated using pharmaceutical agents in their outpatient management (88% in inpatient management), most using these agents ‘sometimes’ or in high-risk children. Paediatric respiratory physicians tended to use bronchodilators less frequently than general paediatricians. Compared with many countries in Europe, few Australian paediatricians routinely use supplementary drugs in the inpatient management of AVB; in particular, bronchodilators (61 vs 7%) and corticosteroids (11 vs 1%) are used far less often. A review of the literature demonstrated that pharmaceutical agents do not influence the course of AVB. Conclusions: Despite a lack of evidence for their efficacy and the recommendation of the Australian guidelines, pharmaceutical agents are frequently used in the management of AVB by paediatricians in Australia, although far less than reported in a recently published European survey. Guidelines alone are not sufficient to implement change and there is a need for more specific strategies to ensure that children receive appropriate management for this common condition.

Background Newborn screening (NBS) for Cystic Fibrosis (CF) has been introduced in many countries, but there is no ideal protocol suitable for all countries. This retrospective study was conducted to evaluate whether the planned two step CF NBS with immunoreactive trypsinogen (IRT) and 7 CFTR mutations would have detected all clinically diagnosed children with CF in Switzerland. Methods IRT was measured using AutoDELFIA Neonatal IRT-Kit in stored NBS cards. Results Between 2006 and 2009, 66 children with CF were reported, 4 of which were excluded for various reasons (born in another country, NBS at 6months, no informed consent). 98% (61/62) had significantly higher IRT compared to matched control group. There was one false negative IRT result in an asymptomatic child with atypical CF (normal pancreatic function and sweat test). Conclusions All children but one with atypical CF would have been detected with the planned two step protocol.