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ABSTRACT: Pulmonary function and nutritional status are important determinants of exercise capacity in patients with cystic fibrosis (CF). Studies investigating the effects of determinants, such as genotype or infection and inflammation, are scarce and have never been analysed in a multivariate longitudinal model. A prospective longitudinal cohort study was performed to evaluate whether genotype, chronic inflammation and infection were associated with changes in exercise capacity. Furthermore, we investigated whether exercise capacity can predict clinical outcome. 504 exercise tests of 149 adolescents with CF were evaluated. Maximal oxygen uptake corrected for body mass % predicted declined 20% during adolescence, and was associated with immunoglobulin (Ig)G levels and chronic Pseudomonas aeruginosa infection. A lower exercise capacity was associated with a higher mortality, steeper decline in pulmonary function and greater increase in IgG levels. Since a decline in exercise capacity during adolescence was negatively associated with IgG levels and chronic P. aeruginosa infection, these data emphasise the importance of prevention and treatment of chronic inflammation and infections in patients with CF. Furthermore, a lower exercise capacity was associated with a higher mortality rate, steeper decline in pulmonary function and higher increase in IgG levels with increasing age in adolescents with CF. This stresses the value of regular exercise testing for assessing prognosis in adolescents with CF.
European Respiratory Journal 09/2011; 39(4):893-8. · 5.89 Impact Factor
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ABSTRACT: Despite vaccination, pertussis is still endemic in the Netherlands. A literature search was performed to verify what is known about the role of Bordetella species in children with cystic fibrosis, with regard to the incidence of Bordetella infections, the involvement in pulmonary exacerbations and the influence on chronic course. Little is known about the frequency of Bordetella infections and the involvement of Bordetella species both in relation to the chronic course of cystic fibrosis and to pulmonary exacerbations. Since it is difficult to detect Bordetella species in cultures and few sputum cultures investigated have been obtained during an exacerbation, it is likely that the frequency of Bordetella species in CF patients is underestimated. Identification of Bordetella species in these patients may have serious consequences for the treatment of exacerbations in CF. Future research investigating the role of Bordetella species in cystic fibrosis should use specific techniques to detect Bordetella in cultures.
Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 06/2011; 10(5):307-12. · 3.19 Impact Factor
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ABSTRACT: Increased work of breathing is considered to be a limiting factor in patients with cystic fibrosis (CF) performing aerobic exercise. We hypothesized that adolescents with CF and with static hyperinflation are more prone to a ventilatorily limited exercise capacity than non-static hyperinflated adolescents with CF. Exercise data of 119 adolescents with CF [range 12-18 years], stratified for static hyperinflation, defined as ratio of residual volume to total lung capacity (RV/TLC) > 30%, were obtained during a progressive bicycle ergometer test with gas analysis and analyzed for ventilatory limitation. Static hyperinflation showed a significant, though weak association (Φ 0.38; P < 0.001) with a ventilatorily limited exercise capacity (breathing reserve index at maximal effort >0.70; FEV(1) < 80% predicted and reduced exercise capacity, defined as VO(2peak) < 85% predicted). Analysis of association for increasing degrees of hyperinflation showed an increase to Φ 0.49 (P < 0.001) for RV/TLC > 50%. In adolescents with static hyperinflation, peak work rate (W(peak) ; 3.1 ± 0.7 W/kg (75.1 ± 17.3% of predicted), peak oxygen uptake (VO(2peak) /kg (ml/min/kg); 39.2 ± 9.2 ml/min/kg (91.0 ± 20.3% of predicted), peak heart rate (HR(peak) ; 176 ± 19 beats/min) were significantly (P < 0.05) decreased when compared with non-static hyperinflated adolescents (W(peak) 3.5 ± 0.5 W/kg (81.4 ± 10.0% of predicted)); VO(2peak) /kg (ml/min/kg); 43.1 ± 7.5 ml/min/kg (98.0 ± 15.1% of predicted); and HR(peak) 185 ± 14 beats/min). Additionally, no difference was found in the degree of association of FEV(1) (%) and RV/TLC (%) with VO(2peak) /kg(pred) and W(peak) /kg(Pred) , but we found the RV/TLC (%) to be a slightly stronger predictor of VO(2peak) /kg(pred) and W(peak) /kg(Pred) than FEV(1) (%). These results indicate that the presence of static hyperinflation in adolescents with CF by itself does not strongly influence ventilatory constraints during exercise and that static hyperinflation is only a slightly stronger predictor of W(peak) /kg(Pred) and VO(2peak) /kg(Pred) than airflow obstruction (FEV(1) (%)).
Pediatric Pulmonology 02/2011; 46(2):119-24. · 2.53 Impact Factor
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ABSTRACT: Long-term clinical trials have shown that daily treatment with recombinant human deoxyribonuclease (rhDNAse) in patients with mild to moderate cystic fibrosis (CF) improves lung function and decreases the number of respiratory exacerbations. The aim of this study was to analyze the effect of rhDNAse on the bacterial colonization of the airways in children with CF.
This was a retrospective cohort study. From the database of the CF Center Utrecht, we selected two groups, an rhDNAse group (daily 2.5 mg rhDNAse) and a control group (no rhDNAse). Primary outcome parameter was the difference in change in bacterial colonization between the treatment and control group during 1.5-year. Secondary outcome parameters were changes in lung function (FEV1) and pulmonary exacerbations.
Children treated with rhDNAse showed no significant changes in bacterial colonization during the treatment period, apart from an increase of P. aeruginosa positive cultures, both compared to baseline (53.1% versus 25%, p < 0.05) and control group (no change during study period, 37% versus 37%). The change in FEV1 after one year of treatment was +4.0% in the treatment group versus -0.3% in the control group (p = 0.22). There were no significant changes in number of pulmonary exacerbations.
This study showed no significant beneficial decrease in bacterial airway colonization during 1.5-year of treatment with rhDNAse. The positive effects of rhDNAse on the lung function can therefore not be explained by a change in airway colonization.
Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace / Fondazione clinica del lavoro, IRCCS [and] Istituto di clinica tisiologica e malattie apparato respiratorio, Università di Napoli, Secondo ateneo 03/2010; 73(1):12-7.
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ABSTRACT: Here we report two children with a pulmonary inflammatory pseudotumour; a rare entity in children, that often initially presents as a pneumonia, but with the possibility of serious consequences if unrecognised and untreated. One of the children presented is 6 months which is extremely young for this tumour. Difficulties in presentation, management strategies and prognosis are described. Certainly, this is a condition that should be considered even in a very young child with an inflammatory condition presenting as a solid lesion in the lung which does not resolve or even progresses.
Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace / Fondazione clinica del lavoro, IRCCS [and] Istituto di clinica tisiologica e malattie apparato respiratorio, Università di Napoli, Secondo ateneo 07/2009; 71(2):71-5.
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ABSTRACT: Standard exhalation time for Fractional exhaled Nitric Oxide (FeNO) measurements is 10 sec but this is difficult for young children. Recommended exhalation time for children is 6 sec, but this was never substantiated in literature. We aimed to investigate the agreement between FeNO values measured with exhalation times of 6 and 10 sec and the preference of children for either method. The study population comprised children aged 5-17 years visiting the Pediatric Pulmonology outpatient clinic. FeNO values, measured during 6 (FeNO-6) and 10 (FeNO-10) sec (random order) using the single-breath online (SBOL) technique, were compared. Preferences for exhalation times were related to FVC values. Ninety-eight children (mean age 10.6 years) were included. Median FeNO-6 (15.2 ppb) and FeNO-10 (13.6 ppb) did not differ significantly (P = 0.259). Mean difference between FeNO-6 and FeNO-10 was -0.3 ppb, limits of agreement ranging from -5.8 ppb to +5.3 ppb. Sixty percent of children with a Forced Vital Capacity (FVC) less than 3 L preferred the FeNO-6 method. We found good agreement between FeNO-6 and FeNO-10, so they can be used interchangeably. An exhalation time of 6 sec was preferred by the majority of subjects with a FVC below 3 L.
Pediatric Pulmonology 05/2009; 44(4):340-4. · 2.53 Impact Factor
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ABSTRACT: The aim of the present study was to evaluate airway disease progression assessed by chest radiology, expiratory interrupter resistance (Rint,exp) and spirometry in young children with cystic fibrosis (CF) over a 3-yr period. Two chest radiographs combined with two R(int,exp) measurements were performed with a 3-yr interval in 21 preschool children (age (mean+/-sd) 3.2+/-0.9 yrs) and 30 schoolchildren with CF (age 7.2+/-1.9 yrs). Chest radiographs were scored using five different CF scoring systems and Rint,exp measurements were expressed as height-adjusted Z-scores. Spirometry was assessed in schoolchildren and the results were expressed as a percentage of predicted values. Chest radiograph scores worsened significantly over the 3-yr period and a tendency towards more pronounced changes was observed, especially for the Wisconsin score, in preschool children. Most preschool and schoolchildren had Rint,exp Z-scores within the normal range at start and follow-up, and the annual change in Rint,exp Z-score was not significant. In schoolchildren, only the forced expiratory volume in one second as a percentage of forced vital capacity declined significantly during the study period. In summary, in young children with cystic fibrosis, chest radiograph scores worsen significantly over time even while lung function remains stable.
European Respiratory Journal 09/2007; 30(2):279-85. · 5.89 Impact Factor
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ABSTRACT: Measurement of thoracic excursion (TEM) during maximal ventilation is part of the physiotherapy assessment in children with cystic fibrosis (CF).
The purpose of our study was to examine the reliability of TEM and its relation with pulmonary function.
Thoracic excursions were measured using a measuring tape. Thirty children participated in an intra-observer and inter-observer reliability study. Reliability was determined by calculating the typical error in repeated measurement, limits of agreement and correlation coefficients. Cross-sectional data from the annual check-ups were used to measure the relation between TEM and pulmonary function.
In the intra-observer reliability study the typical error was 0.31 cm, the limits of agreement were +/-0.86 cm. Pearson's r and ICC were 0.96 and 0.95, respectively. In the inter-observer reliability study these values were 0.56 cm, +/-1.55 cm, 0.85 and 0.85, respectively. TEM correlated significantly with height (0.31, P<0.001), FVC (0.44, P<0.001), FEV1 (0.41, P<0.001) and TLC (0.19, P<0.05), and there was a significant inverse correlation with RV%TLC (-0.45, P<0.001).
TEM is a reliable assessment tool. Thoracic excursion is significantly, although moderately correlated with pulmonary function.
Journal of Cystic Fibrosis 06/2005; 4(2):129-33. · 3.19 Impact Factor
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ABSTRACT: This study was performed to evaluate the applicability of a simple device (MicroRint) for measuring airway resistance, to derive normal values and to compare values with maximal expiratory flow volume (MEFV) parameters in asthmatic and healthy children. Repetitive R(int) measurements were performed in 125 healthy children and 107 asthmatic children (age range 0.8-16.8 years). In 42 asthmatic patients R(int) and MEFV values were compared and in 29 asthmatic children bronchodilation testing was performed. Successful R(int) measurements were possible in 91% of the children. The mean coefficient of variation of repeated measurements was 7.1 (+/-6.1)%. R(int) values of healthy children showed a significant curvilinear correlation with age (r=-0.80, P < 0.001) and height (r=-0.81, P < 0.001). In asthmatic and healthy children R(int) values were comparable. A significant inverse correlation was found between R(int) and MEFV values (for FEV1 and R(int) r=-0.80, P < 0.001). After bronchodilation there was a significant increase in FEV1 and decrease in R(int), but changes between the two parameters did not correlate. In conclusion, the interrupter technique is feasible and repeatable in children and has a significant correlation with other parameters of airway caliber. Baseline values do not discriminate healthy from asthmatic children.
Respiratory Medicine 04/2003; 97(4):366-74. · 2.47 Impact Factor
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ABSTRACT: In children with mild asthma, who show hardly any abnormalities in pulmonary function, objective measurement of the effect of inhaled corticosteroids is difficult. The short term effect of fluticasone propionate (FP) in these children was evaluated, using both subjective and objective parameters. A total of 68 children (5-10 yrs old) were randomly assigned to either FP 250 microg or placebo twice daily as metered-dose inhaler via spacer during 12 weeks. Symptom scores, use of rescue medication, wheezing, parent global evaluation and pulmonary function tests including forced expiratory volume in one second (FEV1), peak expiratory flow (PEF) and bronchial responsiveness (provocation dose of methacholine causing a 20% fall in FEV1 (PD20)) were evaluated. FP-treated versus placebo-treated children showed significant changes in percentage symptom-free days, use of beta2-mimetics, morning and evening PEF, FEV1 % pred and wheezing. No significant improvements were found in parent global evaluation, absolute values of FEV1 nor PD20. These findings show that inhaled corticosteroids are effective in children with mild asthma. This effect can be assessed by both objective and subjective parameters. Early start of inhaled corticosteroids should be considered even when pulmonary function is normal.
European Respiratory Journal 01/2003; 20(6):1470-5. · 5.89 Impact Factor
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ABSTRACT: The interrupter technique is a convenient and sensitive technique for studying airway function in subjects who cannot actively participate in (forced) ventilatory function tests. Reference values for preschool children exist but are lacking for children >7 yrs. Reference values were obtained for expiratory interrupter resistance (R(int,e)) in 208 healthy Dutch Caucasian children 3-13 yrs of age. A curvilinear relationship between R(int,e) and height was observed, similar to published airways resistance data measured by plethysmography. No significant differences in cross-sectional trend or level of R(int,e) were observed according to sex. It was found that Z-scores could be used to express individual R(int,e) values and to describe intra- and interindividual differences based on the reference equation: 10logR(int,e)=0.645-0.00668x standing height (cm) kPa x L(-1) x s(-1) and residual SD (0.093 kPa x L(-1) x s(-1)). Expiratory interrupter resistance provides a tool for clinical and epidemiological assessment of airway function in a large age range.
European Respiratory Journal 10/2002; 20(4):907-11. · 5.89 Impact Factor
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