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.     

Persistent increase in plasma and urinary leukotrienes after acute asthma.

Leukotrienes may mediate bronchoconstriction in asthma. Cysteinyl leukotriene production rises in vivo after allergen challenge, but few reports describe leukotriene concentrations in clinical asthma or in children. Using high performance liquid chromatography/radioimmunoassay, plasma and urinary leukotrienes in asthmatic children (aged 5-10 years) were measured during an acute exacerbation (peak expiratory flow (PEF) < 65%, n = 10) and one month later (PEF 74-169%, n = 9), and in non-atopic normal children (aged 1.3-13.2 years). In the asthmatics, geometric mean (95% confidence interval) plasma leukotriene B4 (LTB4) was 746 pg/ml (398 to 1403) acutely and 1026 pg/ml (662 to 1593) in remission, compared with 369 pg/ml (167 to 728) in the normal children (n = 14). Plasma cysteinyl leukotrienes were low or undetectable, but urinary leukotriene E4 (LTE4) was higher in the asthmatics during an acute episode (210 pmol/mmol creatinine, 101 to 454) and at follow up (179 pmol/mmol, 110 to 293), compared with the normal children (98 pmol/mmol, 81 to 118, n = 41). This persistent increase in plasma LTB4 and urinary LTE4 concentrations one month after a severe asthmatic episode suggests leukotriene production is related to chronic inflammation rather than to acute bronchoconstriction.     

Persistent increase in plasma and urinary leukotrienes after acute asthma

Leukotrienes may mediate bronchoconstriction in asthma. Cysteinyl leukotriene production rises in vivo after allergen challenge, but few reports describe leukotriene concentrations in clinical asthma or in children. Using high performance liquid chromatography/radioimmunoassay, plasma and urinary leukotrienes in asthmatic children (aged 5-10 years) were measured during an acute exacerbation (peak expiratory flow (PEF) < 65%, n = 10) and one month later (PEF 74-169%, n = 9), and in non-atopic normal children (aged 1.3-13.2 years). In the asthmatics, geometric mean (95% confidence interval) plasma leukotriene B4 (LTB4) was 746 pg/ml (398 to 1403) acutely and 1026 pg/ml (662 to 1593) in remission, compared with 369 pg/ml (167 to 728) in the normal children (n = 14). Plasma cysteinyl leukotrienes were low or undetectable, but urinary leukotriene E4 (LTE4) was higher in the asthmatics during an acute episode (210 pmol/mmol creatinine, 101 to 454) and at follow up (179 pmol/mmol, 110 to 293), compared with the normal children (98 pmol/mmol, 81 to 118, n = 41). This persistent increase in plasma LTB4 and urinary LTE4 concentrations one month after a severe asthmatic episode suggests leukotriene production is related to chronic inflammation rather than to acute bronchoconstriction.     

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.     

MTHFR基因多态性与儿童支气管哮喘易感性及糖皮质激素疗效的相关性

目的 探讨亚甲基四氢叶酸还原酶（methylenetetrahydrofolate reductase,MTHFR）基因多态性与儿童支气管哮喘易感性及糖皮质激素（glucocorticoid,GC）疗效的相关性。 方法 选取2018年6月至2020年12月住院治疗的儿童支气管哮喘患儿173例为观察组,均接受GC雾化吸入治疗,连续3个月。选取同期体检的健康儿童178例为对照组。采用PCR检测两组受试儿MTHFR基因C677T位点的基因型,分析两组基因型分布差异性;比较观察组不同基因型患儿治疗前后血清免疫球蛋白E、白细胞介素-8（interleukin-8,IL-8）、白三烯B4（leukotriene B4,LTB4）水平,肺功能指标差异及临床疗效差异。 结果 与对照组相比,观察组TT基因型及T等位基因频率均显著升高（P<0.001）;TT/CT基因型及T等位基因是支气管哮喘易感性的独立危险因素（OR分别为6.615、7.055,P<0.001）。GC治疗后3种基因型患儿免疫球蛋白E、IL-8和LTB4水平较治疗前显著降低,第1秒用力呼气容积（forced expiratory volume in 1 second,FEV1）、用力肺活量（forced vital capacity,FVC）、FEV1/FVC%较治疗前显著升高（P<0.001）;TT基因型患儿IL-8和LTB4水平显著低于CC基因型患儿,LTB4水平明显低于CT基因型患儿,TT基因型患儿FVC明显高于CT基因型患儿,FEV1/FVC%显著高于CC基因型患儿（P<0.05）;治疗后3种基因型患儿临床GC治疗疗效比较差异有统计学意义,其中TT基因型患儿GC疗效良好比例显著高于CC基因型患儿（P<0.05）,且TT基因型是GC疗效良好的独立影响因素（OR=2.111,P=0.018）。 结论 MTHFR基因多态性与儿童哮喘易感性及GC疗效相关,携带TT/CT基因型儿童支气管哮喘发病风险更高,TT基因型对GC治疗具有更高的敏感性。     

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.     

Leukotrienes in respiratory disease

Arachidonic acid metabolism via 5-lipoxygenase gives rise to a group of biologically active lipids known as leukotrienes: leukotriene B(4), which is a potent activator of leukocyte chemotaxis, and cysteinyl leukotrienes (leukotriene C(4), D(4)and E(4)) which account for the spasmogenic activity previously described as slow-reacting substance of anaphylaxis. The biological actions of leukotrienes and the observations that leukotrienes are synthesised in the lung following antigen provocation and are elevated in asthma, stimulated considerable activity in the pharmaceutical industry to find drugs that modulate the synthesis or actions of leukotrienes.Three cysteinyl leukotriene antagonists (zafirlukast [Accolate], montelukast [Singulair] and pranlukast) and one 5-lipoxygenase inhibitor (zileuton) have received regulatory approval for the treatment of asthma. The clinical data obtained from using these drugs are generally consistent and complimentary. As a class the leukotriene modulators produce a rapid improvement in lung function after the first oral dose. Lung function improvements are maintained on chronic administration and are associated with reductions in a variety of asthma symptom scores. All of the available data are consistent with the hypothesis that all the leukotriene modulators exert their clinical benefit primarily through interference with cysteinyl leukotrienes. There are no compelling clinical data for an additional contribution by leukotriene B(4)in human asthma. In other respiratory conditions such as COPD, which are characterised by pronounced neutrophil infiltration, it may be that the chemotactic properties of leukotriene B(4)are more important and therefore evaluation of 5-lipoxygenase inhibitors in this condition is warranted.The introduction of the leukotriene modulators into clinical practice is the culmination of over 60 years of research since the initial discovery of the slow-reacting substances. The leukotriene modulators, and in particular the cysteinyl leukotriene antagonists, provide respiratory physicians with an oral therapeutic option and have set an efficacy standard which new oral anti-inflammatory approaches will have to beat.     

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.     

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.     

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.     

Realities of expectorated sputum collection in the pediatric cystic fibrosis clinic

OBJECTIVES: To determine the proportion of pediatric patients with cystic fibrosis who do not produce expectorated sputum during routine ambulatory clinic visits and to identify clinical predictors of these patients. DESIGN: Cross-sectional study. SETTING: The cystic fibrosis clinic at The Hospital for Sick Children, Toronto, Ontario. PARTICIPANTS: One hundred eighty-three patients aged 6 to 18 years who attended the cystic fibrosis clinic between March 1, 2004, and November 30, 2004. INTERVENTION: Study patients were asked to expectorate sputum for bacterial culture. MAIN OUTCOME MEASURES: The proportion of patients unable to produce expectorated sputum was determined. Age, sex, forced expiratory volume in 1 second, pancreatic sufficiency, body mass index, and antibiotic use were compared between patients producing sputum and those not producing sputum. RESULTS: Eighty-three patients (45%) did not expectorate sputum. Patients not producing sputum as compared with those producing sputum were younger (mean age, 10.3 years vs 13.9 years, respectively; difference, 3.6 years; 95% confidence interval, 2.6-4.5) and had higher forced expiratory volume in 1 second (mean forced expiratory volumes in 1 second, 88% of predicted vs 72% of predicted, respectively; difference, 16%; 95% confidence interval, 10.1-22.2). Eighty-eight percent of patients not producing sputum had cough and 45% reported sputum production at home. CONCLUSIONS: Almost half of pediatric patients with cystic fibrosis aged 6 years and older do not expectorate sputum in the clinic, although nearly half of these patients do report producing sputum at home. The utility of home collection on the morning of a clinic visit and/or hypertonic saline induction should be evaluated to increase the number of useful specimens for microbiological culture.     

Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis

Fourteen patients aged 4.9 to 21.5 years with cystic fibrosis and moderate to severe lung disease, malnutrition, or growth failure were given nocturnal supplemental feeding by gastrostomy tube. Mean follow-up was for 1.1 years (range 0.8 to 2.78 years). Patients were studied to observe the effect of nutritional support on body composition, growth, pulmonary function, and quality of life. A contemporary group of patients with CF was retrospectively pair matched to the study group. The supplemental feeding resulted in positive changes in body composition and in growth velocity. Weight, as a percentage of standard in the control group, declined by 3% over 1 year, whereas it increased by 2% in the treatment group (P less than 0.05). Pulmonary function, assessed as a percent of predicted FVC and FEV1, did not change significantly in the treatment group over 1.1 years, whereas FVC declined by 12% (P less than 0.01) and FEV1 declined by 13% (P less than 0.01) in the control group. There was a marked increase in patient ability to participate in activities of daily living, even in those patients in whom pulmonary function deteriorated during the study.     

Prediction of mortality and timing of referral for lung transplantation in cystic fibrosis patients

Lung transplantation (Tx) is an optional treatment for cystic fibrosis (CF) patients with end-stage lung disease. The decision to place a patient on the Tx waiting list is frequently complex, difficult, and controversial. This study evaluated the current criteria for lung Tx and assessed additional parameters that may identify CF patients at high risk of death. Data were extracted from the medical records of 392 CF patients. Forty of these patients had a forced expiratory volume in 1 s (FEV(1)) less than 30% predicted, and nine of these 40 patients were transplanted. A comparison was performed between the survival of those transplanted (n = 9) and those not transplanted (n = 31), by means of Kaplan-Meier survival curves. The influence on survival of age, gender, nutritional status, sputum aspergillus, diabetes mellitus, recurrent hemoptysis, oxygen use, and the decline rate of FEV(1), were investigated by means of univariate and multivariate analyses. The rate of decline of FEV(1) was evaluated employing the linear regression model. CF patients with a FEV(1)< 30% and who did not receive a lung transplant had survived longer than CF patients who did receive a lung transplant (median survival 7.33 vs. 3.49 yr, 5-yr survival 73% vs. 29%). Two factors--rate of decline in FEV(1) values and age < 15 yr--were found to influence the mortality rate, while the other parameters examined did not. Our results indicate that the current criterion of FEV(1)< 30% predicted, alone is not sufficiently sensitive to predict the mortality rate in CF patients and time of referral for Tx, as many of these patients survive for long periods of time. Additional criteria to FEV(1)< 30%, should include rapidly declining FEV(1) values and age < 15 yr.     

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.     

Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa

To evaluate the relationship between Pseudomonas aeruginosa colonization and the development of lung disease, we studied 895 patients who attended our cystic fibrosis clinic between 1975 and 1988. The prevalence of P. aeruginosa colonization was 82%. Patients who acquired P. aeruginosa in the first year of life had a similar 10-year survival rate (85%) to that in patients who were colonized between the ages of 1 and 7 years (87%), and to that in patients colonized after the age of 7 years (78%). One year before colonization, mean age, forced expiratory volume in 1 second (FEV1), forced vital capacity, and forced expiratory flow in the mid-expiratory phase were similar to those in a group of patients who remained free of P. aeruginosa. No significant change in pulmonary function variables could be demonstrated 1 year and 2 years after the colonization. The rate and duration of hospitalization did not increase in the years after P. aeruginosa colonization compared with the years before colonization. By the age of 7 years, the mean percentage of predicted FEV1 was lower by 10% in patients who were already colonized by P. aeruginosa compared with those who were not colonized (p less than 0.01). A similar reduction in FEV1 was observed at all ages from 7 to 35 years, but no precipitate rate of decline in FEV1 could be associated with P. aeruginosa colonization. We conclude that although P. aeruginosa colonization is associated with 10% lower lung function, it does not cause an immediate and rapid reduction, as has been previously reported. The clinical course and the pulmonary deterioration in cystic fibrosis after P. aeruginosa colonization is a gradual and variable process.     

High-resolution CT pulmonary findings in children with severe asthma

ObjectiveTo compare quantitative CT parameters between children with severe asthma and healthy subjects, correlating to their clinical features.MethodsWe retrospectively analyzed CT data from 19 school-aged children (5–17 years) with severe asthma and 19 control school-aged children with pectus excavatum. The following CT parameters were evaluated: total lung volume (TLV), mean lung density (MLD), CT air trapping index (AT%) (attenuation ≤856 HU), airway wall thickness (AWT), and percentage of airway wall thickness (AWT%). Multi-detector computed tomography (MDCT) data were correlated to the following clinical parameters: forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), forced expiratory flow at 25–75% (FEF 25–75%), FEV1/FVC ratio, sputum and bronchoalveolar lavage analysis, serum IgE levels, and previous hospitalizations due to asthma.ResultsAsthma patients presented higher mean values of AT% (23.8 ± 6.7% vs. controls, 9.7 ± 3.2%), AWT (1.46 ± 0.22 mm vs. controls, 0.47 ± ?735 ± 28 HU vs. controls, ?666 ± 19 HU). Mean AT% was 29.0 ± 4.7% in subjects with previous hospitalization against 19.2 ± 5.0% in those with no prior hospitalization (p < 0.001). AT% presented very strong negative correlations with FVC (r = ?0.933, p < 0.001) and FEV1 (r = ?0.841, p < 0.001) and a moderate correlation with FEF 25–75% (r = ?0.608, p = 0.007). AT% correlation with FEV1/FVC ratio and serum IgE was weak (r = ?0.184, p = 0.452, and r = ?0.363, p = 0.202)ConclusionChildren with severe asthma present differences in quantitative chest CT scans compared to healthy controls with strong correlations with pulmonary function tests and previous hospitalizations due to asthma.     

昆明市5~14岁儿童肺通气功能参数实测值与Zap

目的 研究昆明市5~14岁健康儿童肺通气功能主要参数实测值占Zapletal方程式预计值的百分比,为临床准确判断肺通气功能提供依据。方法 纳入昆明市5~14岁健康儿童702名,其中男352名,女350名。采用Jaeger肺功能仪测定用力肺活量（FVC）、第1秒用力呼气容积（FEV1）、1秒率（FEV1/FVC）、最大中期呼气流量（MMEF）、用力呼气25%肺活量时瞬时流量（FEF25）、用力呼气50%肺活量时瞬时流量（FEF50）、用力呼气75%肺活量时瞬时流量（FEF75）、最高呼气流量（PEF）、每分钟最大通气量（MVV）,共9项指标,以肺功能仪中提供的Zalpetal预计值公式得出的数值作为所选择儿童的预计值,计算其实测值占预计值的百分比。结果 在702名儿童中,肺通气功能主要参数PEF、FVC、FEV1、FEV1/FVC、MVV实测值占预计值百分比的均值分别波动于102%~114%、94%~108%、98%~113%、98%~107%、141%~183%。气道流速指标功能参数FEF25、FEF50、FEF75、MMEF实测值占预计值百分比分别波动于98%~116%、85%~102%、71%~98%、83%~100%。各参数PEF、FVC、FEV1、FEV1/FVC、MVV、FEF25、FEF50、FEF75、MMEF实测值占Zapletal方程式预计值百分比的下限分别为88.2%、88.4%、92.0%、94.4%、118.5%、82.9%、70.0%、62.1%、70.1%。结论 昆明地区5~14岁健康儿童肺通气功能参数水平与Zapletal方程式提供的正常值存在一定差异;该地区此年龄段的健康儿童肺通气功能参数PEF、FVC、FEV、FEV1/FVC、MVV、FEF25、FEF50、FEF75、MMEF实测值占预计值百分比的正常参考值下限可考虑分别设为88.2%、88.4%、92.0%、94.4%、118.5%、82.9%、70.0%、62.1%、70.1%。     

昆明市5~14岁儿童肺通气功能参数实测值与Zap

目的 研究昆明市5~14岁健康儿童肺通气功能主要参数实测值占Zapletal方程式预计值的百分比,为临床准确判断肺通气功能提供依据。方法 纳入昆明市5~14岁健康儿童702名,其中男352名,女350名。采用Jaeger肺功能仪测定用力肺活量（FVC）、第1秒用力呼气容积（FEV1）、1秒率（FEV1/FVC）、最大中期呼气流量（MMEF）、用力呼气25%肺活量时瞬时流量（FEF25）、用力呼气50%肺活量时瞬时流量（FEF50）、用力呼气75%肺活量时瞬时流量（FEF75）、最高呼气流量（PEF）、每分钟最大通气量（MVV）,共9项指标,以肺功能仪中提供的Zalpetal预计值公式得出的数值作为所选择儿童的预计值,计算其实测值占预计值的百分比。结果 在702名儿童中,肺通气功能主要参数PEF、FVC、FEV1、FEV1/FVC、MVV实测值占预计值百分比的均值分别波动于102%~114%、94%~108%、98%~113%、98%~107%、141%~183%。气道流速指标功能参数FEF25、FEF50、FEF75、MMEF实测值占预计值百分比分别波动于98%~116%、85%~102%、71%~98%、83%~100%。各参数PEF、FVC、FEV1、FEV1/FVC、MVV、FEF25、FEF50、FEF75、MMEF实测值占Zapletal方程式预计值百分比的下限分别为88.2%、88.4%、92.0%、94.4%、118.5%、82.9%、70.0%、62.1%、70.1%。结论 昆明地区5~14岁健康儿童肺通气功能参数水平与Zapletal方程式提供的正常值存在一定差异;该地区此年龄段的健康儿童肺通气功能参数PEF、FVC、FEV、FEV1/FVC、MVV、FEF25、FEF50、FEF75、MMEF实测值占预计值百分比的正常参考值下限可考虑分别设为88.2%、88.4%、92.0%、94.4%、118.5%、82.9%、70.0%、62.1%、70.1%。     

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.     

Nitric oxide metabolites in cystic fibrosis lung disease

Although the activity of nitric oxide (NO) synthases are increased in lung tissue of patients with cystic fibrosis, the concentrations of nasal and exhaled NO have recently been found to be decreased in cystic fibrosis. This could either be due to reduced NO formation or metabolism of NO within airway fluids. In this study, the stable NO metabolites, nitrate and nitrite, were determined in the saliva and sputum of 18 stable cystic fibrosis patients, 21 cystic fibrosis patients during a pulmonary exacerbation, and in saliva and endotracheal secretions of normal controls. Median saliva concentrations of NO metabolites (nitrate plus nitrite) were 704 mumol/l (95% confidence interval (CI) 419 to 1477) in stable cystic fibrosis patients, 629 mumol/l (95% CI 382 to 1392) in cystic fibrosis patients presenting with pulmonary exacerbation, and 313 mumol/l (95% CI 312 to 454) in controls. Median sputum NO metabolite concentration in stable cystic fibrosis was 346 mumol/l (95% CI 311 to 504). This was not significantly different from cystic fibrosis patients presenting with pulmonary exacerbation (median 184 mumol/l, 95% CI 249 to 572), but significantly higher than in endotracheal secretions of controls (median 144 mumol/l, 95% CI 96 to 260). Sputum NO metabolite concentration in cystic fibrosis pulmonary exacerbation significantly increased during antibiotic treatment. A positive correlation was observed between sputum NO metabolites and lung function in stable cystic fibrosis, suggesting less airway NO formation in cystic fibrosis patients with more severe lung disease. These data indicate that decreased exhaled NO concentrations in cystic fibrosis patients may be due to retention and metabolism of NO within the airway secretions. However, sputum NO metabolites are not a useful marker of airway inflammation in cystic fibrosis lung disease.