Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial.

OBJECTIVE--To assess the benefit of nebulised amiloride added to the standard inpatient treatment of a respiratory exacerbation in cystic fibrosis. DESIGN--Prospective, randomised, double blind, placebo controlled trial. SUBJECTS--27 cystic fibrosis patients (mean age 12.8 years). SETTING--Two hospitals in Leeds, UK. RESULTS--Both forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) showed improvements over the course of treatment, although there was no difference in respiratory function between the two groups at any of three time periods during the study. The time to reach peak FVC was significantly reduced in the amiloride group (4.2 v 7.6 days; 95% CI 0.4 to 6.4 days), but not in the time to reach peak FEV1 (5.7 v 7.9 days; 95% CI -1.2 to 5.6 days). CONCLUSIONS--Amiloride did not result in a greater overall improvement in respiratory function. There was a suggestion that it may have an effect on the rate of improvement, and thus may possibly influence the duration of treatment. This hypothesis deserves further evaluation.     

Clinical outcomes of newborn screening for cystic fibrosis.

AIM: To determine how early diagnosis of cystic fibrosis, using neonatal screening, affects long term clinical outcome. METHODS: Fifty seven children with cystic fibrosis born before neonatal screening was introduced (1978 to mid 1981) and a further 60 children born during the first three years of the programme (mid 1981 to 1984), were followed up to the age of 10. The cohorts were compared on measures of clinical outcome, including height, weight, lung function tests, chest x-ray picture and Shwachman score. RESULTS: Age and sex adjusted standard deviation scores (SDS) for height and weight were consistently higher in children screened for cystic fibrosis than in those born before screening. At 10 years of age, average differences in SDS between groups were 0.4 (95% CI -0.1, 0.8) for weight and 0.3 (95% CI -0.1, 0.7) for height. This translates to an average difference of about 2.7 cm in height and 1.7 kg in weight. Mean FEV1 and FVC (as percentage predicted) were significantly higher in the screened cohort at 5 and 10 years of age, with an average difference of 9.4% FEV1 (95% CI 0.8, 17.9) and 8.4% FVC (95% CI 1.8, 15.0) at 10 years. Chest x-ray scores were not different between the groups at any age, but by 10 years screened patients scored an average 5.3 (95% CI 1.2, 9.4) points higher on the Shwachman score. CONCLUSION: Although not a randomised trial, this long term observational study indicates that early treatment made possible by neonatal screening may be important in determining subsequent clinical outcomes for children with cystic fibrosis. For countries contemplating the introduction of neonatal screening for cystic fibrosis, its introduction to some areas in a cluster randomised design will permit validation of studies performed to date.     

Montelukast versus Budesonide as a First Line Preventive Therapy in Mild Persistent Asthma in 2 to 18 y

Objectives

To compare the efficacy of oral Montelukast and inhaled Budesonide as a first line preventive therapy in mild persistent asthma in age group 2–18 y.

Methods

This prospective randomized controlled clinical study was conducted for 12 wk. Sixty patients of mild persistent asthma aged 2 to 18 y were randomly allocated to either oral Montelukast (n?=?60) or inhaled Budesonide (n?=?60) group. Outcomes measured were improvement in peak expiratory flow rate (PEFR), forced expiratory volume 1 s/forced vital capacity (FEV1/FVC), day time and night time symptoms and frequency of exacerbations and need to change medications.

Results

There was significant improvement in PEFR, FEV1/FVC, day time and night time symptoms and frequency of exacerbations in both groups. However, more significant improvement in FEV1/FVC (CI 95 %, p?=?0.029) and day time symptoms (CI 95 %, p?=?0.002) was seen in Budesonide group compared to Montelukast group.

Conclusions

The present study suggests that oral Montelukast is not inferior to Budesonide in treatment of mild persistent asthma in 2 to 18 y children in terms of control of symptoms and improvement in pulmonary function tests over a 12 wk period. However, there was more significant improvement in day time symptoms, more significant increase in FEV1/FVC ratio and less exacerbation in patients receiving Budesonide compared to those receiving Montelukast. However, side effects due to long term use of steroids such as growth stunting and bone osteopenia should also be considered before recommending. Trial registered at CTRI no. REF/2012/09/004035     

Risk factors for reduced lung function in Australian Aboriginal children

AIM: To determine the influence of perinatal and childhood exposures on lung function in a cohort of Australian Aboriginal children. METHODS: This was a cross-sectional study of 547 Northern Territory Aboriginal children, aged 8-14 years, belonging to a birth cohort. Assessment included physical examination and spirometry as well as retrospective review of centralised hospital records. The effect of select perinatal and childhood exposures on lung function outcomes (forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and forced expiratory flow between 25 and 75 s (FEF25-75)) adjusted for age, sex, height and other measures of size was examined using multiple regression. RESULTS: Non-urban residence (FEV1 -5% (95% confidence interval, CI 0.91-0.99), FVC -9% (95% CI 0.87-0.95)), current cough (FEV1 -6% (95% CI 0.91-0.97), FVC -4% (95% CI 0.93-0.97), FEF25-75 -8% (95% CI 0.86-0.98)) and hospitalisations for respiratory disease (FEV1 -10% (95% CI 0.86-0.95), FEF25-75 -12% (95% CI 0.70-0.87)) all had significant negative effects on adjusted lung function measures. Children with a non-Aboriginal ancestor had significantly better lung function. No perinatal exposure other than neonatal lung disease had any significant effect on adjusted lung function. CONCLUSIONS: For Northern Territory Aboriginal children factors related to the childhood environment are more important than perinatal factors in determining childhood lung function.     

Effect of respiratory virus infections including rhinovirus on clinical status in cystic fibrosis

One hundred and eight patients with cystic fibrosis were investigated over one year to determine whether an association existed between rhinovirus or other respiratory virus infection and clinical status. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC), Shwachman score, Chrispin-Norman chest radiograph score, and percentage weight for height were recorded at the beginning and end of the study; days of intravenous antibiotics were noted. Nasopharyngeal aspirates were taken for viral studies during respiratory exacerbations. Serum was collected at enrollment and 2-6 weeks after each respiratory exacerbation. One hundred and fifty seven exacerbations occurred in 76 patients. Respiratory virus infection was detected in 44 exacerbations and rhinovirus was present in 16% (25/157) of exacerbations. Patients with one or more respiratory virus infections were compared with those who had none. When all respiratory virus infections were considered, patients had a significantly greater deterioration in Shwachman score and received significantly more days of intravenous antibiotics. When rhinovirus was considered separately, patients received significantly more days of intravenous antibiotics, but showed no deterioration in clinical status. However, patients infected with another respiratory virus had a significant decline in FEV1, with trends towards significance for decline in FVC and Shwachman score.     

Neonatal cerebral venous thrombosis

It is postulated that a vigorous host inflammatory response in the cystic fibrosis lung contributes to lung injury. Tumour necrosis factor-alpha (TNF-alpha) may play a part in that process and in the generation of leukotrienes. Therefore, the relationships between sputum TNF-alpha, leukotriene concentration, and lung function abnormalities in 16 children with cystic fibrosis were investigated. Each subject provided sputum samples and performed spirometry. TNF-alpha was measured by enzyme linked immunosorbent assay; individual leukotrienes were separated using high performance liquid chromatography and quantified by radioimmunoassay. The geometric mean concentration of TNF-alpha was 129.7 pg/ml and 95% confidence interval 48.2 to 348.3. Mean (SEM) leukotriene B4 (LTB4) was 97.8 (22.9) pmol/g and total cysteinyl leukotrienes were 60.9 (14.8) pmol/g. Mean (SD) forced expiratory volume in one second (FEV1) of the group was 53 (15)% of predicted and forced vital capacity (FVC) was 65 (14)% of predicted. There was a significant positive correlation between TNF-alpha and both LTB4 and the total cysteinyl leukotriene sputum content. An inverse relationship existed between TNF-alpha and FEV1 and FVC. Moreover, a negative correlation was observed between sputum LTB4 and FEV1 and FVC. These results suggest that TNF-alpha and the leukotrienes may participate in the airways inflammation and airflow obstruction observed in cystic fibrosis subjects and support the hypothesis that TNF-alpha upregulates the 5-lipoxygenase pathway in vivo.     

Effect of respiratory virus infections including rhinovirus on clinical status in cystic fibrosis.

One hundred and eight patients with cystic fibrosis were investigated over one year to determine whether an association existed between rhinovirus or other respiratory virus infection and clinical status. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC), Shwachman score, Chrispin-Norman chest radiograph score, and percentage weight for height were recorded at the beginning and end of the study; days of intravenous antibiotics were noted. Nasopharyngeal aspirates were taken for viral studies during respiratory exacerbations. Serum was collected at enrollment and 2-6 weeks after each respiratory exacerbation. One hundred and fifty seven exacerbations occurred in 76 patients. Respiratory virus infection was detected in 44 exacerbations and rhinovirus was present in 16% (25/157) of exacerbations. Patients with one or more respiratory virus infections were compared with those who had none. When all respiratory virus infections were considered, patients had a significantly greater deterioration in Shwachman score and received significantly more days of intravenous antibiotics. When rhinovirus was considered separately, patients received significantly more days of intravenous antibiotics, but showed no deterioration in clinical status. However, patients infected with another respiratory virus had a significant decline in FEV1, with trends towards significance for decline in FVC and Shwachman score.     

Sputum tumour necrosis factor-alpha and leukotriene concentrations in cystic fibrosis

It is postulated that a vigorous host inflammatory response in the cystic fibrosis lung contributes to lung injury. Tumour necrosis factor-alpha (TNF-alpha) may play a part in that process and in the generation of leukotrienes. Therefore, the relationships between sputum TNF-alpha, leukotriene concentration, and lung function abnormalities in 16 children with cystic fibrosis were investigated. Each subject provided sputum samples and performed spirometry. TNF-alpha was measured by enzyme linked immunosorbent assay; individual leukotrienes were separated using high performance liquid chromatography and quantified by radioimmunoassay. The geometric mean concentration of TNF-alpha was 129.7 pg/ml and 95% confidence interval 48.2 to 348.3. Mean (SEM) leukotriene B4 (LTB4) was 97.8 (22.9) pmol/g and total cysteinyl leukotrienes were 60.9 (14.8) pmol/g. Mean (SD) forced expiratory volume in one second (FEV1) of the group was 53 (15)% of predicted and forced vital capacity (FVC) was 65 (14)% of predicted. There was a significant positive correlation between TNF-alpha and both LTB4 and the total cysteinyl leukotriene sputum content. An inverse relationship existed between TNF-alpha and FEV1 and FVC. Moreover, a negative correlation was observed between sputum LTB4 and FEV1 and FVC. These results suggest that TNF-alpha and the leukotrienes may participate in the airways inflammation and airflow obstruction observed in cystic fibrosis subjects and support the hypothesis that TNF-alpha upregulates the 5-lipoxygenase pathway in vivo.     

Randomised controlled trial of inhaled corticosteroids (fluticasone propionate) in cystic fibrosis

BACKGROUND: Controlling lung inflammation may be the key to improving morbidity and mortality in cystic fibrosis. OBJECTIVE: To assess the effects of inhaled corticosteroids on lung inflammation in cystic fibrosis. DESIGN: Double blind placebo controlled randomised sequence crossover trial. Fluticasone propionate (400 micrograms/day) was given as a dry powder inhaler for six weeks with a four week washout period before crossover. OUTCOME MEASURES: Sputum inflammatory markers (interleukin-8, tumour necrosis factor-alpha (TNF-alpha) and neutrophil elastase-both free and bound to alpha 1-antiprotease), sputum interleukin-10, lung function, and symptomatology. SUBJECTS: Twenty three children from a regional cystic fibrosis centre were enrolled into the study, with mean age 10.3 years (range 7 to 17 years) and mean baseline forced expiratory volume in one second (FEV1) of 64% (range 21% to 102%) predicted for sex and height. One patient was excluded for non-compliance to the study protocol. RESULTS: No significant benefit was shown for the use of fluticasone propionate in any of the outcomes. For sputum interleukin-8 there was an estimated true treatment median difference of 142 pg/ml (95% confidence interval (CI) 8 to 2866 pg/ml) in favour of placebo; while for maximal expiratory flow at 25% (MEF25%) remaining forced vital capacity predicted for sex and height there was a 15 percentage points (pp) (95% CI 4 to 26 pp) mean treatment difference in favour of placebo. Sputum interleukin-10 was undetected in any samples and unaffected by fluticasone propionate. Neither atopic status, baseline FEV1, nor concomitant DNase therapy had any effect on response to treatment. CONCLUSIONS: Lack of benefit from fluticasone propionate was most likely due to failure of the drug to penetrate the viscid mucus lining the airways. It is suggested a large multicentre trial with higher doses given for a longer time by a different delivery system is required to assess efficacy.     

Respiratory health in a total very low birthweight cohort and their classroom controls

AIMS: To compare the respiratory health and function at 8 to 9 years of age of a total population based cohort of 300 very low birthweight (VLBW) children with that of two classroom controls (n = 590) matched for age and sex. STUDY DESIGN: Cohort study with controls. SETTING: Schools throughout Scotland. RESULTS: The VLBW children were more likely than their peers to use an inhaler, to be absent from school, and to be admitted to hospital because of respiratory illness. They were significantly shorter than their classroom controls, but even after adjusting for differences in height, the VLBW children had reduced forced vital capacity (FVC); this was associated with a history of prolonged ventilation (> 28 days) and pneumothorax in the neonatal period. There were no significant differences between the groups in forced expiratory volume in one second (FEV1)/FVC but twice as many (7.9% v 3.7%) of the VLBW children had ratios < 70%, denoting obstructive airways disease. Poor expiratory function was associated with neonatal respiratory distress syndrome, prolonged ventilation, and the need for > 40% oxygen. Exercise induced airway narrowing was increased in VLBW children (odds ratio = 2.0; 95% confidence interval 1.2 to 3.4) and was very little changed by adjustment for inhaler use and exposure to cigarette smoke. CONCLUSIONS: As in other low birthweight cohorts, respiratory morbidity was increased. Unlike previous studies, FVC was more affected than expiratory function in this VLBW population. Our findings support the hypothesis that poorer lung function is associated with very low birth weight, but not with intrauterine growth retardation.     

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目的探讨学龄前儿童用力肺活量测定的质量控制标准。方法2004年4～9月,对深圳地区3～7岁正常儿童343例(男184例,女159例),采用意大利COSMED公司生产的COSMED流量传感仪,参考美国胸科协会可接受曲线标准,通过测定用力肺活量(FVC)、0.5s用力呼气容积(FEV0.5)、0.75s用力呼气容积(FEV0.75)、1s用力呼气容积(FEV1)以及0.5s用力呼气容积占用力肺活量比值(FEV0.5/FVC)、0.75s用力呼气容积占用力肺活量比值(FEV0.75/FVC)、1s用力呼气容积占用力肺活量比值(FEV1/FVC)、外推容量(VBE)、外推容量占用力肺活量比值(VBE/FVC)、呼气时间(FET100%)及最佳2次的FVC、FEV0.75、FEV0.5、FEV1变异等指标,分析学龄前儿童用力肺活量测定的质量控制标准。结果279名(81.3%)儿童能够成功完成测试。平均VBE为(42.71±13.61)mL,95百分位数为64mL,最大为72mL;VBE/FVC为(3.93±1.34)%,95百分位数为6.36%,最大为9.26%;52例(18.6%)VBE/FVC>5%;年龄越小的儿童其VBE/FVC越高;VBE/FVC与身高呈负相关(P<0.05)。儿童平均呼气时间为(1.61±0.52)s,5百分位数为0.9s,18例(6.5%)呼气时间<1s。儿童最佳2次的FVC、FEV1、FEV0.75、FEV0.5变异均<0.2L;约63.1%儿童最佳2次的FEV0.75的变异<5%;约66.2%最佳2次的FEV1变异<5%,各变异<0.1L的百分比为90%～93%。结论建议对于中国学龄前儿童用力肺活量的质控标准为:曲线起始以VBE为标准,VBE/FVC<6.5%或VBE<65mL,取最大值;曲线终止以呼气时间≥0.9s,且呼气相时间容积曲线显示呼气容量出现平台,持续时间≥1s为标准;FEV0.5及FEV0.75需在报告中报告;曲线的重复性标准为最佳2次FVC及FEV0.75的变异<10%或<0.1L(取最大值)。     

Efficacy, tolerance, and pharmacokinetics of once daily tobramycin for pseudomonas exacerbations in cystic fibrosis

OBJECTIVE: To compare once daily with thrice daily tobramycin for treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis. DESIGN: 22 patients with cystic fibrosis, mean (SD) age 11 (3.4) years (range 5.6-19.3), with pulmonary pseudomonas exacerbations were randomly assigned to receive a 14 day course of tobramycin (15 mg/kg/day) either in three infusions (group A) (n = 10) or a single daily infusion (group B) (n = 12), combined with ceftazidime (200 mg/kg/day as three intravenous injections). Efficacy was assessed by comparison of pulmonary, nutritional, and inflammatory indices on days 1 and 14. Cochlear and renal tolerance were assessed on days 1 and 14. Tobramycin concentration was measured in serum and sputum 1, 2, 3, 4, 8, and 24 hours after the start of the infusion. Analysis was by non-parametric Wilcoxon test. RESULTS: Variables improving (p < 0.05) in both groups A and B were, respectively: weight/height (+4% and +3.1%), plasma prealbumin (+66 and +63 mg/l), forced vital capacity (FVC) (+14% and +11%), forced expiratory volume in one second (+15% and +14%), and forced expiratory flow between 25% and 75% of FVC (+13% and +21%). Improvement was not significantly different between groups. Renal and cochlear indices remained within the normal range. Serum peak concentration of tobramycin on day 1 was 13.2 (7.1) mg/l in group A and 42.5 (11.2) mg/l in group B (p < 0.001); serum trough was 1.1 (0.8) mg/l in group A and 0.3 (0.2) mg/l in group B (p < 0.01). Tobramycin concentrations in sputum were two to three times higher in group B than group A. CONCLUSIONS: Once daily tobramycin combined with three injections of ceftazidime is safe and effective for the treatment of pseudomonas exacerbations in cystic fibrosis patients.     

Respiratory health in a total very low birthweight cohort and their classroom controls.

AIMS: To compare the respiratory health and function at 8 to 9 years of age of a total population based cohort of 300 very low birthweight (VLBW) children with that of two classroom controls (n = 590) matched for age and sex. STUDY DESIGN: Cohort study with controls. SETTING: Schools throughout Scotland. RESULTS: The VLBW children were more likely than their peers to use an inhaler, to be absent from school, and to be admitted to hospital because of respiratory illness. They were significantly shorter than their classroom controls, but even after adjusting for differences in height, the VLBW children had reduced forced vital capacity (FVC); this was associated with a history of prolonged ventilation (> 28 days) and pneumothorax in the neonatal period. There were no significant differences between the groups in forced expiratory volume in one second (FEV1)/FVC but twice as many (7.9% v 3.7%) of the VLBW children had ratios < 70%, denoting obstructive airways disease. Poor expiratory function was associated with neonatal respiratory distress syndrome, prolonged ventilation, and the need for > 40% oxygen. Exercise induced airway narrowing was increased in VLBW children (odds ratio = 2.0; 95% confidence interval 1.2 to 3.4) and was very little changed by adjustment for inhaler use and exposure to cigarette smoke. CONCLUSIONS: As in other low birthweight cohorts, respiratory morbidity was increased. Unlike previous studies, FVC was more affected than expiratory function in this VLBW population. Our findings support the hypothesis that poorer lung function is associated with very low birth weight, but not with intrauterine growth retardation.     

Systematic review of N-acetylcysteine in cystic fibrosis

A systematic review was carried out to evaluate whether the use of N-acetylcysteine to improve lung function in patients with cystic fibrosis is supported by published evidence. Medline and the Cochrane Library were searched and the reference lists of all retrieved papers and of relevant chapters of three major textbooks were scanned. Data on lung function (forced expiratory volume in one second (FEV1)) were extracted from controlled clinical trials and pooled as weighted mean differences for analysis. Twenty-three studies, mostly uncontrolled clinical observations, were retrieved. Only three randomized controlled clinical trials on nebulized N-acetylcysteine in cystic fibrosis were found, not showing any beneficial effect on lung function. Six randomized controlled clinical trials on oral N-acetylcysteine in cystic fibrosis were found, with a total number of 181 patients. There was a tendency towards a beneficial effect on lung function of oral N-acetylcysteine therapy on FEV1 but it was small (2.3%, 95% CI from (-0.3 to 4.9% of predicted) and of doubtful clinical relevance. In all studies, follow-up was 3 months or shorter. In conclusion, at present there is no evidence supporting the use of N-acetylcysteine in cystic fibrosis, although­ a beneficial effect with long-term use of N-acetylcysteine in cystic fibrosis cannot be excluded.     

Influence of genetic variants on childhood lung function – The Generation R Study

Genetic variants associated with adult lung function could already exert the effects on childhood lung function. We aimed to examine the associations of adult lung function‐related genetic variants with childhood lung function and asthma, and whether these associations were modified by atopic predisposition, tobacco smoke exposure, or early growth characteristics. In a population‐based prospective cohort study among 3347 children, we selected 7 and 20 single nucleotide polymorphisms (SNPs) associated with adult forced expiratory volume in 1 second (FEV ₁) and FEV ₁/forced vital capacity (FEV ₁/FVC ), respectively. Weighted genetic risk scores (GRS s) for FEV ₁ and FEV ₁/FVC were constructed. At age 10, FEV ₁, FVC , FEV ₁/FVC , forced expiratory flow between 25% and 75% (FEF _25‐75), and forced expiratory flow at 75% (FEF ₇₅) of FVC were measured, and information on asthma was obtained by parental‐reported questionnaires. The FEV ₁‐GRS was associated with lower childhood FEV ₁, FEV ₁/FVC , and FEF ₇₅ (Z ‐score (95% CI ): ?0.03 (?0.05, ?0.01), ?0.03 (?0.05, ?0.01), and ?0.04 (?0.05, ?0.01), respectively, per additional risk allele). The FEV ₁/FVC ‐GRS was associated with lower childhood FEV ₁/FVC and FEF ₇₅ (Z ‐score (95% CI ): ?0.04 (?0.05, ?0.03) and ?0.03 (?0.05, ?0.02), respectively, per additional risk allele). Effect estimates of FEV ₁‐GRS with FEF _25‐75, FEV ₁, FEF ₇₅, and FVC , and of FEV ₁/FVC ‐GRS with FEV ₁/FVC and FEF _25‐75 were stronger among children exposed to non‐atopic mothers, smoking during pregnancy or in childhood, or those born with a lower birthweight, respectively (P ‐values for interaction < .05). Genetic risk scores were not associated with asthma. Adult lung function‐related genetic variants were associated with childhood lung function. Maternal atopy, smoking during pregnancy or in childhood, and birthweight modified the observed effects.     

Normal growth in cystic fibrosis associated with a specialised centre.

OBJECTIVE: To assess the impact of lifetime continuous care within the John Hunter Hospital cystic fibrosis (CF) clinics on growth and lung function. DESIGN: A cross sectional survey of variables affecting nutritional status in CF was undertaken for 1993 and 1997. Data were retrieved from medical records and grouped into 5 year age bands. MAIN OUTCOME MEASURES: Change in height z-score, weight centile, and forced expiratory volume in one second (FEV(1)) between patient cohorts receiving specialised care for different lengths of time. RESULTS: Improved mean height z-score (-0.880 v -0.047) and weight centile (28.3% v 48.1%) for the 10-15 year age group in 1997, who had received continuous lifetime care within the clinic, compared with the same age group in 1993, for whom continuous medical care started at an older age. There was no corresponding improvement in FEV(1), as an indicator of lung function, in this group (81.6% predicted v 89.5% predicted). CONCLUSIONS: This study suggests that lifetime continuous care within a specialised CF centre is associated with improved growth but not improved lung function.     

Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis

OBJECTIVE: To evaluate a high-resolution computed tomography (HRCT) scoring system, clinical parameters, and pulmonary function measurements in patients with cystic fibrosis (CF) before and after therapy for a pulmonary exacerbation. STUDY DESIGN: Patients (n = 17) were evaluated by spirometer-triggered HRCT imaging, clinical parameters, and pulmonary function tests (PFTs) before and after treatment. HRCT scans were reviewed by 3 radiologists using a modified Bhalla scoring system. RESULTS: Bronchiectasis, bronchial wall thickening, and air trapping were identified in all subjects on initial evaluation. The initial total HRCT score correlated significantly with the Brasfield score (r = -.91, P <.001) and several PFT measures. After treatment, there were improvements in the acute change clinical score (ACCS) (P <.001), most pulmonary function measurements, and total HRCT score (P <.05). Bronchiectasis, bronchial wall thickening, and air trapping did not significantly change. Mucus plugging subcomponent HRCT score, slow vital capacity (SVC), forced expiratory volume in 1 second (FEV(1)), and forced vital capacity (FVC) (percent predicted) and reversible and total HRCT scores were most sensitive to change by effect size analysis. CONCLUSIONS: Improvements occurred with treatment in total and reversible HRCT scores, PFTs, and ACCS. Total and reversible HRCT scores and percent predicted SVC, FEV1, and FVC were the most sensitive to change. The greatest change was seen in the mucus plugging subcomponent HRCT score.     

Changes in dynamic respiratory volumes in obese children and adolescents related to weight loss and sex]

Obesity is associated with various alterations in lung function in adults. These alterations appear to be proportional to the degree of EP and the beneficial effect of weight loss on respiratory function has been reported. Therefore, in 35 children and adolescents affected by essential obesity of medium-severe degree, we have evaluated the following parameters: FVC (forced vital capacity), PEF (peak expiratory flow), FEV1 (forced expiratory volume), FEV75, FEV50, FEV25, before and after six months of dieting. Twelve subjects (34%) showed at least a pathologic value of PEF and/or FEV50 before dieting. All the female patients normalized their parameters after six months of dieting, whilst 5 out of 7 males still showed pathologic respiratory indexes, although a similar weight loss was obtained in the two groups of patients. Our study enhances the presence of respiratory functions derangements in a significant percentage of children with medium-severe degree of obesity. A careful monitoring of these subjects is therefore necessary, in order to prevent further progression of the lung function damage. After dieting the pulmonary function improved in female patients only, suggesting that factors other than the EP are involved in the pathogenesis of the respiratory alterations.     

Respiratory virus infections in cystic fibrosis

Respiratory virus infections have pronounced and long-lasting effects on patients with cystic fibrosis (CF), resulting in significant declines in FVC, FEV(1) and Shwachman score, significantly increasing both the frequency and duration of hospitalisation. Deleterious effects on patients with CF have been reported for most viruses studied but the effects of respiratory syncytial virus and influenza appear the greatest. There is circumstantial evidence that respiratory virus infections may facilitate bacterial infections, particularly Pseudomonas aeruginosa.     

Comparative trial of manual and mechanical percussion technique with gravity-assisted bronchial drainage in patients with cystic fibrosis.

Chest physiotherapy still remains one of the most important aspects in the treatment of chest complications of cystic fibrosis. A mechanical device that allows the patient with cystic fibrosis to do his own chest physiotherapy will be of great benefit if it is as effective as manual percussion. 14 patients with cystic fibrosis using mechanical and manual percussion physiotherapy were studied by measuring sputum volumes, and FEV and FVC. Results with mechanical percussor were as good as with the manual percussor and, therefore, it would be reasonable for the older patient to use the former on his own.