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Aim: To evaluate the long-term effect of montelukast on symptoms of cough and wheeze following RSV bronchiolitis. Methods: Fifty eight patients (aged ≤ 24 months) hospitalized with a first episode of RSV bronchiolitis were enrolled in this double blind prospective randomized trial comparing montelukast (n = 31) vs placebo (n = 27). Results: During the 3-month treatment period, there were no statistical significant differences between the two groups for symptom-free days and nights (48.5 [interquartile range 33.0.0–66.0] for montelukast vs 57.0 [29.0–71.0] for placebo p = 0.415) nor disease-free days and nights (44.5 days [26.0–54.0] vs 53.0 [22.3–71.0]; p = 0.266). During the 1 year follow-up, there were 41 exacerbations in the montelukast group vs 54 exacerbations in the placebo group (p = 0.57). Time to first exacerbation was not different. Number of unscheduled visits and need to start inhaled steroids were comparable in the two groups. Conclusion: Treatment with montelukast after hospital admission for RSV bronchiolitis in children younger than 2 years of age did not reduce symptoms of cough and wheeze. We cannot exclude that a subgroup of children may, however, benefit from this treatment.

Objective Difficult asthma implies persistent asthma symptoms despite therapy with high doses of inhaled corticosteroids. The objective was to evaluate children with difficult asthma in a setting that excludes aggravating factors such as poor treatment adherence and adverse environmental influences. Patients and Methods Sixty children (≥6 years) had been referred because of difficult asthma to the rehabilitation centre over a period of 10 years. The diagnosis of poor asthma symptom control was confirmed if exacerbations continued during stay in the centre or if symptoms interfered with daily activities at least 3 times a week. Results The median stay at the centre was 5 months. In four patients a diagnosis other than asthma was made. In five patients symptom control remained difficult. In the remaining 51 children, asthma symptoms became well controlled. Many factors contributed to poor asthma control in the home setting: poor treatment adherence (n = 32), parental smoking (n = 22), allergen exposure (n = 10). Psychosocial problems occurred in 36 children. Contributing factors often co-existed. During stay at the centre, lung function improved in the group with well controlled asthma symptoms (P

The source of acquisition of Pseudomonas aeruginosa in cystic fibrosis (CF) patients remains unknown. Patient-to-patient transmission has been well documented but the role of the environment as a source of initial infection is as yet unclear. In the present study, the origin of the first P. aeruginosa isolate in CF patients was investigated by comparing the P. aeruginosa genotype(s) from newly infected patients with genotypes of P. aeruginosa isolates from the home environment and from other patients from the same CF centre. A total of 50 newly infected patients were studied. P. aeruginosa could be cultured from 5.9% of the environmental samples, corresponding to 18 patients. For nine of these, the genotype of the environmental P. aeruginosa isolate was identical to the patient's isolate. In total, 72% of the environmental P. aeruginosa isolates were encountered in the bathroom. Patient-to-patient transmission within the CF centre could not be ruled out for three patients. In summary, a low prevalence of Pseudomonas aeruginosa was found in the home environment of the newly infected cystic fibrosis patients. The bathroom should be targeted in any preventive cleaning procedures. An environmental source of the new infection could not be ruled out in nine patients.

Emergencies always require rapid diagnosis and an urgent or semi-urgent medical, interventional, or surgical action. In most cases radiology plays an essential role in making an accurate diagnosis. Reviewing the causes of acute respiratory pathology in the different pediatric age groups, we thought it would be interesting to divide the pathologies into two main parts: one part concerning pathologies involving the air flow and the other part concerning pathology affecting the pulmonary parenchyma. We acknowledge, however, that both conditions can occur concomitantly. The esophagus is another anatomic structure in the thorax that can be responsible for acute pathology in children. Acute pathology predominantly involving the air flow can be intrinsic and/or extrinsic, affecting the upper airways, trachea, main and segmental bronchi, and the small airways. Acute lung pathology can be congenital, infectious, or less frequently, tumoral or traumatic in children. Pleural pathology and cardiogenic emergencies are discussed also. Acute esophageal pathology is discussed briefly.

The aim of this study was to evaluate the efficacy (expressed as effect on lung function) and tolerability of Symbicort (budesonide/formoterol in a single inhaler) in children with asthma. This was a double-blind, double-dummy, randomized, parallel-group, multicenter trial. After a 2-4-week run-in period, 286 asthmatic children (177 boys, 109 girls; mean age, 11 years; mean forced expiratory volume in 1 sec (FEV(1)), 75% predicted normal), previously treated with inhaled corticosteroids (average dose 548 microg/day), were randomized to 12 weeks' treatment with either budesonide/formoterol 80/4.5 microg, two inhalations twice daily (n = 148), or an equivalent dose of budesonide 100 microg, two inhalations twice daily (n = 138). Efficacy variables included morning and evening peak expiratory flow (PEF), spirometery, asthma symptoms, and use of rescue medication (beta(2)-agonists). Serial FEV(1) assessments were carried out on a subgroup of children (budesonide/formoterol, n = 41; budesonide, n = 40) at randomization and at week 12. Relative to baseline, morning PEF (primary variable) increased to a significantly greater extent with budesonide/formoterol than with budesonide alone (7.22% predicted normal vs 3.45% predicted normal; P < 0.001). Evening PEF also increased significantly with budesonide/formoterol (6.13% predicted normal vs. 2.73% predicted normal; P < 0.001), as did mean FEV(1) and serial FEV(1) measured over 12 hr (both P < 0.05). Similar improvements in asthma symptoms and rescue medication use were observed in both groups. The two treatment groups were similar in terms of their adverse-event profile and rates of discontinuation. Budesonide/formoterol in a single inhaler provided rapid improvements in PEF and FEV(1) compared to inhaled budesonide alone. These improvements were sustained throughout the study period. Budesonide/formoterol was well-tolerated in children with moderate persistent asthma.

In children, post-obstructive pulmonary edema is a rare condition, caused by a sudden change in upper airway patency. It causes dyspnea, tachypnea, hypoxemia, and at times hemoptysis and respiratory insufficiency. It occurs as a complication in the immediate post-operative period. Pediatricians should be aware of this clinical entity.

The terms consensus, guideline and position paper are sometimes employed as if they were interchangeable, but the purpose of such documents and the robustness of advice vary as the evidence base does not have the same depth in each. The Board of the European Cystic Fibrosis Society deemed it to be helpful to provide a short commentary on the definition of these terms, on their interconnections and on how ECFS considers them in documents endorsed by the society.

Soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) is demonstrating promise as an inflammatory biomarker of acute infection in various pulmonary conditions; including community acquired pneumonia, ventilator associated pneumonia and non-tuberculous mycobacterial infection.The expression of sTREM-1 has been poorly studied in all forms of bronchiectasis, both in the context of cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis.Induced sputum samples were collected for sTREM-1 determination in children with HIV-associated bronchiectasis and CF-bronchiectasis. The presence or absence of an exacerbation was noted at study entry. Lung function parameters (FEV1, FVC, FEV1 /FVC, FEF(25-75)) were measured using the Viasys SpiroPro Jaeger Spirometer (Hoechberg, Germany).A total of twenty-six children with HIV-associated bronchiectasis and seventeen with CF were included. With respect to sTREM-1, the levels were readily detected in both groups, but were significantly higher in children with HIV-associated bronchiectasis (1244.0 pg/ml (iqr 194.5; 3755.3 pg/ml) and 204.9 pg/ml (iqr 66.9; 653.6 pg/ml) P = 0.003. There was a positive correlation between sTREM-1 and IL-8 as well as sputum neutrophil elastase in the HIV-bronchiectasis group (r = 0.715 and r = 0.630), respectively both P 0.005. sTREM-1 was not further increased in subjects presenting with an acute pulmonary exacerbation in the HIV-associated bronchiectasis and in CF participants (P = 0.971 and P = 0.481), respectively. In the CF group sTREM-1 strongly correlated with FVC% predicted and FEV1 % predicted (r = 0.950 and r = 0.954), both P 0.005.The pulmonary innate immune functions are over-active in HIV-associated bronchiectasis, with readily detected sTREM-1 values, which were higher than those in CF. sTREM-1 does not correlate with markers of HIV-disease activity but does correlate with markers of neutrophilic inflammation. In CF sTREM-1 has a negative correlation with pulmonary function parameters.

More than 1900 different mutations in the CFTR gene have been reported. These are grouped into classes according to their effect on the synthesis and/or function of the CFTR protein. CFTR repair therapies that are mutation or mutation class specific are under development. To progress efficiently in the clinical phase of drug development, knowledge of the relative frequency of CFTR mutation classes in different populations is useful. Therefore, we describe the mutation class spectrum in 25,394 subjects with CF from 23 European countries.In 18/23 countries, 80% or more of the patients had at least one class II mutation, explained by F508del being by far the most frequent mutation. Overall 16.4% of European patients had at least one class I mutation but this varied from 3 countries with more than 30% to 4 countries with less than 10% of subjects. Overall only respectively 3.9, 3.3 and 3.0% of European subjects had at least one mutation of classes III, IV and V with again great variability: 14% of Irish patients had at least one class III mutation, 7% of Portuguese patients had at least one class IV mutation, and in 6 countries more than 5% of patients had at least one class V mutation.

Objective: Modern large single institutional reports on pulmonary sequestration (PS) are extremely rare. We were interested in comparing patients with PS referred by our pediatric versus adult pulmonologists. Methods: Hospital notes of all patients operated on between 1978 and 1997 for a congenital broncho-pulmonary malformation were reviewed. In 28 patients, the parenchymal lesion was vascularized by a systemic artery and was separated from the bronchial tree, thus matching the strict definition of PS. Patient characteristics and outcome were analyzed comparing the pediatric group (#16 years: na 13; mean age, 3 ^ 5 years) versus the adult group (.16 years: na 15; mean age, 33 ^ 13 years). Results: No significant differences between both groups were observed in sex, side, type of sequestration, pulmonary venous drainage, associated anomalies, hospital and late outcome, and patient’s overall score. Patients (na 21) with the intralobar type of sequestration presented significantly more often with an infection when compared with patients (na 7) with the extralobar type (91 versus 14%; Pa 0:0033). When compared with the pediatric group, patients in the adult group had significantly more respiratory infections (87 versus 38%; Pa 0:016), and also required a lobectomy more often (67 versus 31%; Pa 0:056). Conclusions: The extralobar type of sequestration often remains asymptomatic, and is usually an incidental finding during infancy. The intralobar type mostly presents with recurrent infections in adulthood resulting in more lobectomies. We believe these findings support our current policy to remove any pulmonary malformation whenever diagnosed in order to: (1), prevent infection and other potentially serious late complications which may compromise the surgical outcome; and (2), enhance the chance of a parenchymal-sparing resection. q 2001 Elsevier Science B.V. All rights reserved.

A culture from the lower airway secretions is the optimal sample to guide antibiotic therapy in cystic fibrosis (CF) lung disease. The authors therefore examined whether sputum induction is an efficient, safe and acceptable procedure in CF children without spontaneous expectorations. Nineteen patients were studied. Their mean age (range) was 8.6 yrs (4.3-15.2 yrs). Their mean forced expiratory volume in one second (FEV1) was 88% predicted (46-122%). NaCl solutions from 0.9-6% were inhaled, after baseline lung function tests before and after salbutamol. All patients did produce secretions. Alveolar macrophages were present in 16/19 induced samples. The procedure induced minor but significant bronchoconstriction: the mean change (range) in postsalbutamol FEV1 (% pred) was -7 (-24-16). Percutaneous oxygen saturation remained above 90% in all children. The test had to be discontinued in one child because of cough and wheeze. Acceptability of the procedure evaluated using a visual analogue scale from -7-7 showed a mean value (range) at the final concentration of -1.23 (-6.16-5.88). It is concluded that sputum induction is possible, safe and acceptable in cystic fibrosis children who do not expectorate spontaneously.

Background: Fat maldigestion occurs in most patients with cystic fibrosis. Conventional pancreatic enzyme replacement therapy partially corrects this defect. In this study, the mixed-triglyceride breath test was used to evaluate whether high-lipase enzymes are equivalent to conventional enzymes in improving fat maldigestion in children with cystic fibrosis. Methods: Fat digestion was studied in 11 patients with a mean age of 10.5 years. The mean intake of conventional enzyme capsules a day was 19. Four 13 C mixed-triglyceride tests were performed on separate days and in random order. One test was taken without enzyme substitution, one with three capsules of 8,000 FIP units Creon (pancreatinum. Kali-chemie Pharma, Hannover, Germany) and one with one capsule of 25.000 FIP units. The fourth test was made with 13 C octanoic acid to study gastric emptying time. Results: Without enzyme intake, the mean cumulative percentage of 13 C dose exhaled after 6 hours was 7.2 ± 3.7%. This increased to 14.4 ± 4% with intake of conventional pancreatinum and to 14.3 ± 5.1% with intake of high-lipase pancreatinum (p = 0.0008 for both; paired t-test). There was no difference between both treatments. Also, the time course of 13 C exhalation measured by percentage of 13 CO 2 exhaled per hour did not differ between enzyme treatments. Conclusions: The 13 C mixed-triglyceride test is noninvasive and documents improved lipid digestion with pancreatic enzyme replacement therapy. If the lipase dose is kept constant, results obtained with high-lipase preparations are equivalent to those obtained with conventional preparations.

Lung Clearance Index (LCI) is a promising endpoint for use in CF clinical trials. Since correlation with validated clinical endpoints has not yet been established, we investigated the association between baseline LCI and risk of respiratory tract exacerbations (RTE) in children with CF. Methods: During a prospective observational study, baseline LCI (N2 washout), FEV1 and CFQR respiratory domain (CFQRres) were measured. RTE, defined as an increase in respiratory symptoms treated with IV antibiotics, were recorded during one year. Whether basline LCI predicted RTE was assessed with a Poisson regression model and Kaplan–Meier plots. LCI z-scores were calculated from values in 57 healthy children. Results: In 63 children with CF (median age 12.4 years, range 5−19), mean LCI z-score was 5.3 (SD 4.6) and mean FEV1 z-score −0.9 (SD 1.3). CFQRres correlated with LCI (R = −0.43, p< 0.001), but not with FEV1 (R=0.24, p = 0.051). In the 53 patients with a normal FEV1, CFQRres and LCI were still correlated (R = −0.44, p = 0.002). During the 12 months follow up, 25 patients (40%) experienced 47 RTE. LCI and FEV1 were predictors of RTE. Time to first RTE decreased with worsening LCI quartiles (Log Rank test, p< 0.001). Similarly, compared to the quartile with the lowest LCI, yearly RTE rate ratio in increasing LCI quartiles was 2.8 (95%CI 0.6–13.9, p = 0.205), 4.7 (95%CI 1.0–21.4, p = 0.046) and 13.6 (95%CI 3.2–57.0, p< 0.001). In the group with normal FEV1, LCI but not FEV1 z-score was still a predictor of RTE. Conclusion: Baseline LCI predicts the risk of RTE in children with CF, even in the subgroup with normal FEV1. These data further support the use of LCI as surrogate outcome in CF clinical trials.

Worldwide almost no epidemiologic data are available on the prevalence or incidence of interstitial lung diseases (ILD) in the general population. Therefore, a registration programme of ILD-prevalence was organised by the VRGT (Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding), among about 100 Flemish pneumologists since 1990. Most categories of the classification by Crystal et al. (1) were included and the diagnostic criteria (histology, laboratory tests, clinic, radiology) were registered. The present paper presents the results of 1992-1994: twenty pneumologists had forwarded the summary files of 237 patients to the central office in 1992 (n = 68), 1993 (n = 90) and 1994 (n = 79). The diagnoses that were most frequently made were: sarcoidosis in 27%, idiopathic pulmonary fibrosis in 20%, hypersensitivity pneumonitis in 14% (of which 68% by birds) and collagen-vascular disease in 10% (of which 54% in rheumatoid arthritis). Less frequent causes were eosinophilic pneumonia (4%), inhalation of inorganic material (4%, anthracosilicosis being excluded), histiocytosis X (3%), drugs (3%), angiitis and granulomatosis (2%), pulmonary hemosiderosis (1%), lymphocytic infiltrative lung disease (1%) and lymphangioleiomyomatosis (1%). The order of relative frequencies of the different categories of diseases was the same in the 3 registration years. In 9% of the patients the diagnosis was confined to "undefined fibrosis". The diagnosis was confirmed by histology in 63% of the cases. The overall male-female ratio was nearly one, with, however, a male preponderance in hypersensitivity pneumonitis (22/12), UIP(8/3) and "undefined fibrosis" (14/7).(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary mechanics as well as chest wall width and depth were measured in 52 1-year-old survivors of newborn lung disease. Of the 52 patients examined, 22 had developed bronchopulmonary dysplasia (BPD). Chest wall depth was significantly less in the patients who survived with development of BPD compared with those who did not develop BPD. Pulmonary resistance and chest wall width-to-depth ratio were significantly increased in the patients with BPD. Because the chest wall of infants is highly compliant, we suggest that the flatter chest in patients with BPD could result from the abnormal pulmonary mechanics.Pediatr Pulmonol. 1994;18:104–107. © 1994 Wiley-Liss, Inc.