Digital clubbing and pulmonary function abnormalities in children with lung disease

Digital clubbing and pulmonary function tests were measured in children, adolescents, and adults with chronic lung diseases to determine pulmonary function correlates with a quantitative measure of clubbing. The group had a mean age of 13.8 +/- 6.0 (SD) years, mean PaO2 of 81 +/- 21 mm Hg, and mean FEV1 of 60% +/- 26% predicted. Digital clubbing was diagnosed in 43 cases when the distal phalangeal depth to interphalangeal depth (DPD/IPD) ratio, measured on a finger cast, was greater than or equal to 1 (greater than 3 SD above mean from 85 controls; no history of pulmonary disease; mean age, 14.8 +/- 7.6). The PaO2 of patients with digital clubbing was 69.4 +/- 2.1 (SEM) mm Hg compared with 88.3 +/- 1.3 mm Hg in those without digital clubbing (P less than 0.0001). Digital clubbing was present in 39 of the 84 (46%) hypoxic patients (PaO2 less than or equal to 88) but only four of the 78 (5%) normoxic patients (P less than 0.0001). The DPD/IPD ratio was negatively correlated with PaO2 in subjects with cystic fibrosis and interstitial fibrosis. Weak negative correlations were seen for all other subjects except asthmatics. Overall, the DPD/IPD ratio was significantly correlated with PaO2 (r = -0.53; P less than 0.0001). The DPD/IPD ratio was correlated with other lung function abnormalities (increased RV, decreased FEV1, and FEF25%-75%) only for the subjects with cystic fibrosis. We conclude that digital clubbing is associated with hypoxemia and airway obstruction. The relation is seen most clearly in subjects with cystic fibrosis, possibly reflecting the prolonged duration of hypoxemia. Digital clubbing is rarely seen in normoxic subjects.     

Assessing the usefulness of outcomes measured in a cystic fibrosis treatment trial

Forced expiratory volume in 1s (FEV(1)) is the usual primary outcome variable in clinical trials in cystic fibrosis (CF). Usually, several secondary outcomes are also measured. We assessed which secondary outcomes are likely to give additional clinically useful information about treatment effects, in order to inform the design of future studies. The study was performed as part of a trial comparing daily rhDNase with alternate day rhDNase and hypertonic saline in CF. The primary outcome was FEV(1). Secondary outcomes were forced vital capacity (FVC), forced expiratory flow at 25-75% of forced vital capacity (FEF(25-75)), number of pulmonary exacerbations, weight gain, quality of life (QOL), and exercise tolerance. The usefulness of each secondary outcome was investigated by assessing if the change in that outcome over the treatment period could be predicted from the primary outcome. Change in FEV(1) correlated with changes in FVC (r(2)=0.76, P=0.001), FEF(25-75) (r(2)=0.64, P=0.001), weight (r(2)=0.08, P=0.001), and change in oxygen saturation with exercise (r(2)=0.08, P=0.001). However, it did not correlate with changes in visual analogue score (VAS) with exercise, QOL, nor with the occurrence of pulmonary exacerbations. Only the outcomes QOL and VAS with exercise actually provided additional information to FEV(1) in this study.     

Assessing correlations between spirometry and Shwachman-Kulczycki score in children and adolescents

A retrospective observational study was carried out to analyze the correlations between formal pulmonary function tests and the Shwachman-Kulczycki (SK) score. Forty-six Brazilian cystic fibrosis (CF) patients, clinically stable, aged 7-19 years, were included. Clinical and radiological findings of the SK score system and spirometry variables were assessed by independent observers in a blinded manner. The strongest correlation (r = 0.75; 95% CI, 0.59-0.85; P < 0.001) was found for forced expiratory volume in 1 sec (FEV1). Despite some peculiarities of our patients, the results are in agreement with studies undertaken in industrialized countries, showing good correlation between FEV1 and SK score and its usefulness in managing CF. Accordingly, in countries where pulmonary function testing is not available, the SK score remains a valuable parameter on which to base treatment results.     

Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia.

STUDY OBJECTIVES: To determine (1) the prevalence of pulmonary hypertension and cardiac dysfunction in adult cystic fibrosis (CF) patients with severe lung disease, (2) the relationship between these cardiovascular abnormalities and hypoxemia, and (3) the impact of subclinical pulmonary hypertension on survival. DESIGN: Single-blind, cross-sectional study. SETTING: Ambulatory clinic of the Adult CF program at a tertiary-level hospital. PATIENTS: Clinically stable patients with severe lung disease (FEV1 < 40% of predicted normal value) who were not receiving supplemental oxygen. A second cohort of patients in stable condition with less severe lung disease (FEV1 40 to 65% predicted) was also recruited to enable multivariate analysis for the determinants of pulmonary hypertension. MEASUREMENTS AND RESULTS: Eighteen patients with severe lung disease (FEV1 28 +/- 7% of predicted normal value) were initially studied. Each patient had overnight polysomnography, pulmonary function tests, and Doppler echocardiography. Arterial oxygen saturation (SaO2) was reduced during wakefulness (87.1 +/- 6.1%) and fell during sleep (84.0 +/- 6.6%) while transcutaneous PCO2 was normal during wakefulness (41.1 +/- 6.9 mm Hg) and increased during sleep (46.6 +/- 4.7 mm Hg). Left ventricular size, systolic function, and diastolic function were normal except in one patient who had had a previous silent myocardial infarction due to coronary artery disease. Qualitative assessment of right ventricular function was normal in all patients. Pulmonary artery systolic pressure (PASP) was increased (> 35 mm Hg) in seven patients without clinical evidence of cor pulmonale. Regression analysis was performed by combining these data with data from an additional 15 CF patients with moderately severe lung disease (FEV1 56.3 +/- 8.9% predicted normal) who were recruited to a modified study protocol that included overnight oximetry, pulmonary function tests, and Doppler echocardiography. None of these patients had evidence of hypoxemia and only three had mild elevation of PASP (36, 37, and 39 mm Hg). Linear regression analysis revealed that PASP was significantly correlated with FEV1 (r = -0.44; p = 0.013), and SaO2 during wakefulness (r =-0.60; p = 0.0003), during sleep (r = -0.56; p = 0.0008), and after 6 min of exercise (r = -0.75; p < 0.0001). Multivariate analysis revealed that awake SaO2 was a significantly better predictor of PASP than FEV1 (p = 0.0104). Clinical follow-up of the original cohort for up to 5 years revealed that mortality was significantly higher in those with pulmonary hypertension than those without pulmonary hypertension (p = 0.0129). CONCLUSIONS: In adult CF patients with severe stable lung disease, left and right ventricular function is well maintained in the absence of significant coronary artery disease; pulmonary hypertension develops in a significant proportion of patients and is strongly correlated with oxygen status, independent of lung function; and subclinical pulmonary hypertension is associated with an increased mortality.     

Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis.

BACKGROUND: In patients with cystic fibrosis (CF), it has been proposed that hypoxemia and hypercapnia occur during episodes of stress, such as exercise and sleep, and that respiratory muscle weakness because of malnutrition may be responsible. METHODS: Pulmonary function, respiratory muscle strength, and nutrition were assessed and correlated with the degree of hypoxemia and hypercapnia during exercise and sleep in 14 patients with CF and 8 control subjects. RESULTS: Despite no differences in maximum static inspiratory pressure (PImax) between the two groups, the CF group developed more severe hypoxemia (minimum oxyhemoglobin saturation [SpO2], 89 +/- 5% vs 96 +/- 2%; p < 0.001) and hypercapnia (maximum transcutaneous CO2 tension [PtcCO2], 43 +/- 6 vs 33 +/- 7 mm Hg; p < 0.01) during exercise. Similarly, during sleep, the CF group developed greater hypoxemia (minimum SpO2, 82 +/- 8% vs 91 +/- 2%; p < 0.005), although CO2 levels were not significantly different (maximum PtcCO2, 48 +/- 7 vs 50 +/- 2 mm Hg). Within the CF group, exercise-related hypoxemia and hypercapnia did not correlate with FEV1, residual volume/total lung capacity ratio (RV/TLC), PImax, or body mass index (BMI). Hypoxemia and hypercapnia during sleep correlated with markers of gas trapping (RV vs minimum arterial oxygen saturation [r = -0.654; p < 0.05]), RV vs maximum PtcCO2 (r = 0.878; p < 0.001), and RV/TLC vs maximum PtcCO2 (r = 0.790; p < 0.01) but not with PImax or BMI. CONCLUSION: Patients with moderately severe CF develop hypoxemia and hypercapnia during exercise and sleep to a greater extent than healthy subjects with similar respiratory muscle strength and nutritional status. Neither respiratory muscle weakness nor malnutrition are necessary to develop hypoxemia or hypercapnia during exercise or sleep.     

Oxygen saturation in adult cystic fibrosis patients during exercise at high altitude.

The objective of this study was to determine the frequency and severity of decreased arterial oxy-hemoglobin saturation during exercise in adults with cystic fibrosis at 1,500 m above sea level. A convenience sample of 50 adults with cystic fibrosis who did not have hypoxemia (oxygen saturation, < 90%) at rest were evaluated. Spirometry was performed according to American Thoracic Society standards, and maximal exercise tests were performed on an electronically braked cycle ergometer using a ramp protocol individualized for each patient. Pulse oximetry was measured every 2 min. When exercising at high altitude, 45 of 50 patients had a decrease in arterial oxy-hemoglobin saturation from baseline to some degree. In 29 patients, oxy-hemoglobin saturation fell below 90%; in 14 patients, it fell below 85%; and in 4 patients, it fell below 80%. Oxy-hemoglobin saturation decreased to < 90% in 12 of 14 patients with severe pulmonary disease (FEV(1) < 40% predicted), in 15 of 26 patients with moderate disease (40% less than or equal to FEV(1) < 70% predicted), in 2 of 6 patients with mild disease (70% less than or equal to FEV(1) < 90% predicted), and in 0 of 4 with normal pulmonary function (FEV(1) greater than or equal to 90%). Percent predicted FEV(1) (r = 0.57; P < 0.0001) and FEV(1)/FVC ratio (r = 0.52; P < 0.0001) most highly correlated with arterial oxy-hemoglobin saturation at peak exercise. We conclude that at 1,500 m above sea level, adult CF patients with obstructive airways disease are at significant risk for decreased arterial oxy-hemoglobin saturation during exercise. A supervised exercise test should be considered prior to recommending an exercise program for such patients.     

Oxidative stress and lipid-derived inflammatory mediators during acute exacerbations of cystic fibrosis

BACKGROUND AND OBJECTIVE: In cystic fibrosis (CF) very few studies have assessed sputum 8-iso-PGF2alpha levels during pulmonary exacerbations as a direct measure of airway oxidative stress. The role of other lipid-derived inflammatory mediators, such as the cysteinyl leukotrienes (cys-LTs) and prostaglandin (PG)-E2, during exacerbations is also poorly defined and the effect of conventional antibiotic therapy on these components of the inflammatory process is unclear. METHODS: Sputum 8-iso-PGF2alpha, total cys-LT and PGE2 levels were measured in 17 CF patients experiencing a pulmonary exacerbation and repeated analysis were performed in 15 of these patients after antibiotic treatment. Eight stable CF and nine healthy subjects provided control data. RESULTS: Sputum 8-iso-PGF2alpha was significantly elevated in acute, but not stable CF patients versus healthy controls (P < 0.001). Similarly, sputum cys-LT and PGE2 levels were increased in acute compared with stable CF patients and healthy controls (P 相似文献   

The Relationship Between Severity of Liver Cirrhosis and Pulmonary Function Tests

Pulmonary complications, mainly hepatopulmonary syndrome (HPS), are frequently observed in liver cirrhosis. In this study, the aim was to investigate the frequency of hypoxemia and impairment of pulmonary function tests (PFT) in patients with liver cirrhosis and to examine the relationships of these impairments with liver failure. A total of 39 patients with cirrhosis, 24 males and 15 females, were included in our study. The mean age of the patients was 47.5 +/- 17.2 years. Arterial blood gases, PFT, and carbon monoxide diffusion tests (DLCO) were performed in all patients. Out of 39 cirrhotic patients, 21 (53.8%) had ascites, whereas 18 (46.2%) did not. Seven patients were in the Child-Pugh A group, 21 in the Child-Pugh B group, and 11 patients were in the Child-Pugh C group. Hypoxia was found in 33.3% of the patients. Although the PaO2 and SaO2 values of patients with ascites were lower compared to those without ascites (P < 0.05), no statistically significant difference was determined in the comparison of hypoxia between the groups (P > 0.05). Among the PFT parameters, FEV1/FVC and FEF25-75% values were found to be lower in patients with ascites than those without (P < 0.05). No differences were established between these two groups of patients in terms of DLCO (P > 0.05). While no differences were found in comparison of the DLCO values in between the groups (P > 0.05), there was a statistically significant difference in the ratio of DLCO to the alveolar ventilation (DLCO/VA) in between the groups (P < 0.05). On the other hand, a negative correlation was found between the DLCO/VA and Child points when the relationship between the Child-Pugh score and PFT parameters were investigated (r = -0.371, P < 0.05). Consequently, a relationship was established between the severity of liver failure and diffusion tests showing pulmonary complications invasively. We believe diffusions tests should be performed in addition to the PFT in order to determine pulmonary involvements particularly in patients who are candidates for liver transplantation.     

Anaerobic exercise in pediatric cystic fibrosis

Anaerobic fitness is important for daily functioning of children with cystic fibrosis (CF). The aim of this study was to assess the determinants of anaerobic performance in CF. Anaerobic performance was measured in 39 children with CF (mean age, 13.2 +/- 1.8 (SD) years, forced expired volume in 1 sec (FEV(1)) 81.6 +/- 22.1% predicted), using a Wingate anaerobic test. Significant associations were found for peak power (PP) and mean power (MP) with fat-free mass (FFM) body weight, body mass index, maximal isometric muscle force, and aerobic capacity. Pulmonary function was correlated with anaerobic indices when controlled for FFM. Multiple regression analysis indicated that FFM and FEV(1) accounted for 82% and 86% of the variability in PP and MP, respectively. Patients with moderate CF (FEV(1) < 80%), as compared to mild CF (FEV(1) >/= 80%), had higher PP (difference = 85 W, 95% CI = 27-144 W) and MP (difference = 53 W, 95% CI = 42-63 W) at equivalent FFM. Our results indicate that FFM and pulmonary function are important determinants of anaerobic exercise performance in children with CF. With progression of pulmonary disease, anaerobic performance may be enhanced.     

Timing and driving components of the breathing strategy in children with cystic fibrosis during exercise

The aim of this study was twofold: first, to determine the breathing strategies of children with cystic fibrosis (CF) during exercise, and secondly, to see if there was a correlation with lung function parameters. We determined the tension-time index of the inspiratory muscles (T(T0.1)) during exercise in nine children with CF, who were compared with nine healthy children with a similar age distribution. T(T0.1) was determined as followed T(T0.1) = P0.1/PImax . T(I)/T(TOT), where P0.1 is mouth occlusion pressure, PImax is maximal inspiratory pressure, and T(I)/T(TOT) is the duty cycle. CF children showed a significant decrease of their forced expiratory volume in 1 sec (FEV1), forced vital capacity (FCV), and FEV1/FVC, whereas the residual volume to total lung capacity ratio (RV/TLC) ratio and functional residual capacity (FRC) were significantly increased (P < 0.001). Children with CF showed mild malnutrition assessed by actual weight expressed by percentage of ideal weight for height, age, and gender (weight/height ratio; 82.3 +/- 3.6%). Children with CF showed a significant reduction in their PImax (69.3 +/- 4.2 vs. 93.8 +/- 7 cmH2O). We found a negative linear correlation between PImax and weight/height only in children with CF (r = 0.9, P < 0.001). During exercise, P(0.1), P0.1/PImax, and T(T0.1) were significantly higher, for a same percent maximal oxygen uptake in children with CF. On the contrary, T(I)/T(TOT) ratio was significantly lower in children with CF compared with healthy children. At maximal exercise, children with CF showed a T(T0.1) = 0.16 vs. 0.14 in healthy children (P < 0.001). We observed at maximal exercise that P0.1/PImax increased as FEV1/FVC decreased (r = -0.90, P < 0.001), and increased as RV/TLC increased (r = 0.92, P < 0.001) only in children with CF. Inversely, T(I)/T(TOT) decreased as FEV1/FVC decreased (r = 0.89, P < 0.001), and T(I)/T(TOT) decreased as RV/TLC increased (r = -0.94, P < 0.001). These results suggest that children with CF adopted a breathing strategy during exercise in limiting the increase of the duty cycle. Two determinants of this strategy were degrees of airway obstruction and hyperinflation.     

Correlation between inhalant allergen-specific IgE and pulmonary function in children with asthma

Sensitization to aeroallergens is associated with diminished lung function in adults. Little has been studied on the relationship between the inhalant allergen-specific IgE and pulmonary function in asthmatic children. This study was focused on four major inhalant allergens found in Korea, including Dermatophagoides pteronyssinus (Der p.), Dermatophagoides farinae (Der f.), and Alternaria- and German cockroach-specific IgEs, with evaluation of pulmonary function in relation to the amount of allergens. The parents or legal guardians of participants enrolled in this study gave informed consent. Fifty-five asthmatic patients and 48 nonasthmatic children were included. The amounts of specific IgE for the four specified inhalant allergens were determined by employing the CAP system FEIA. Forced expiratory volume in 1 sec (FEV(1))/forced vital capacity (FVC), FEV(1), and forced expiratory flow between 25% and 75% of FVC (FEF(25-75)) of subjects were evaluated through pulmonary function tests. In the asthmatic group, FEV(1), FEV(1)/FVC, and FEF(25-75) were significantly reduced (P < 0.05): reduction in FEV(1) (r = -0.44) and FEF(25-75) (r = -0.33) in association with the Der f.-specific allergen, and reduction in FEV(1) (r = -0.37) and FEF(25-75) (r = -0.34) in association with the Der p.-specific allergen, were observed. However, there was no significant correlation with German cockroach and Alternaria allergen. In the control group, no significant correlation was detectable between the allergen-specific IgE titers and the results of pulmonary function tests. In asthmatic patients, Der p.- and Der f.-specific IgEs, and not German cockroach and Alternaria, seem to play a considerable role in reduced pulmonary function among asthmatic children.     

Utility of the breathing reserve index at the anaerobic threshold in determining ventilatory-limited exercise in adult cystic fibrosis patients

OBJECTIVES: Cardiopulmonary exercise testing in cystic fibrosis (CF) patients is useful to assess functional status and prognosis. Using the current interpretation guidelines, the utility of this testing will be limited in those patients who cannot exercise to a near-maximal level. This study investigates the utility of the breathing reserve index at the anaerobic threshold (BRIAT), which is defined as minute ventilation at the anaerobic threshold (AT)/maximum voluntary ventilation (MVV), to distinguish ventilatory-limited (VL) CF patients from nonventilatory-limited (NVL) CF patients. DESIGN: Exercise studies on 53 adult CF patients at baseline clinical status performed from 1993 to 1999 were reviewed, of which 40 met the inclusion criteria. The studies were performed via ramp protocol to the symptom-limited maximum on a cycle ergometer with breath-by-breath expired gas analysis. AT was determined noninvasively via the V-Slope method. The patients were classified as VL if they had abnormal spirometry findings, reduced exercise capacity, and a breathing reserve index at maximum exercise (BRImax) of > or = 0.7. NVL patients had a normal BRImax and met the criteria for a maximal study. RESULTS: VL patients (21 patients) had significantly lower FVC, FEV(1), MVV, and body mass index than NVL patients (19 patients). The BRIAT for the VL group was significantly higher than that for the NVL group (p < 0.001). Logistic regression analysis revealed that BRIAT discriminated VL patients from NVL patients better than a variety of nonexercise variables tested. The BRIAT correlated extremely well with BRImax (r = 0.89; p < 0.01), FVC (r = -0.67; p < 0.001), FEV(1) (r = -0.76; p < 0.001), and FEV(1)/FVC ratio (r = -0.683; p < 0.001). A BRIAT value of 0.29 distinguished VL CF patients from NVL CF patients with 95.2% sensitivity and 84.2% specificity. CONCLUSIONS: The BRIAT assessed noninvasively correlates well with commonly used measurements of pulmonary function and accurately distinguishes CF patients with and without a ventilatory limitation to exercise. The BRIAT may have utility in the interpretation of exercise studies in CF patients who are unable to exercise to a maximal level.     

Relationship between sputum inflammatory markers,lung function,and lung pathology on high-resolution computed tomography in children with cystic fibrosis

High-resolution computed tomography (HRCT) is a sensitive technique for early visualisation and location of cystic fibrosis (CF) bronchopathology, and has been shown to detect acute reversible and chronic changes. It would be expected to correlate with markers of the underlying pathological processes, such as sputum cytokines and cytology, as well as with pulmonary function tests (PFTs). Our aim was to study the relationship between PFTs, sputum cytology, and sputum cytokine interleukin-8 (IL-8) and HRCT in CF patients. Prospective standardized collection of sputum samples was performed at the time of routine annual high-resolution CT scans. Forced expired volume in 1 sec (FEV(1)) and forced vital capacity (FVC) were recorded. Sputum processing was selective, with dispersal by the three-enzyme technique. IL-8 measurements were by kit assay. HRCT scans were scored by a pediatric radiologist, blinded to clinical condition, using a modified Bhalla score.Forty-three CT scans were performed on 34 children with CF between March 1998 and April 2000. Mean age was 12.3 years (range, 6-21 years), FEV(1) (% predicted) was 67% (range, 23-120%), and mean modified Bhalla score was 11.2 (range, 0-22). Sputum IL-8 concentration (mean, 86; range, 4-150 ng/mL) and total cell count (mean, 31.9 x 10(6)/mL; range, 21.8-42.0 x 10(6)/mL) were high. FEV(1) and FVC correlated with modified Bhalla score (r = -0.66, P < 0.0001 for both), and most individual components of the score, especially mosaic perfusion (r = -0.64, r = -0.61 respectively, P < 0.0001) and extent of bronchiectasis (r = -0.61, P < 0.0001 for both). The combination of these two predicted 58% of the variability in FEV(1) on analysis of variance (P < 0.0001). Sputum total cell count correlated weakly with modified Bhalla score (r = 0.38, P < 0.05) and with FEV(1) and FVC (r = -0.36, P < 0.05; and r = -0.46, P < 0.01). Differential cell counts, cell viability, and IL-8 did not correlate with modified Bhalla scores, or with reversible components such as mucus plugging, centrilobular nodules, or peribronchial thickening.In conclusion, pathological changes on HRCT correlated with lung function but not with sputum markers of inflammation.     

Spirometry in 3- to 6-year-old children with cystic fibrosis

Spirometry is routinely used to assess pulmonary function of older children and adults with cystic fibrosis (CF); however, few data exist concerning the preschool age group. We have reported normative spirometric data for 3- to 6-year-old children. The current study was designed to assess a similarly aged group of clinically stable patients with CF. Thirty-three of 38 children with CF were able to perform 2 or 3 technically acceptable maneuvers. These patients had significantly decreased FVC, FEV(1), FEV(1)/FVC, and FEF(25-75) when expressed as z scores (number of SD from predicted): -0.75 +/- 1.63, -1.23 +/- 1.97, -0.87 +/- 1.33, and -0.74 +/- 1.63, respectively. There were significant positive correlations of the Brasfield radiological score with FVC and FEV(1) z scores (r(2) = 0.26, p < 0.01 and r(2) = 0.24, p < 0.01). In addition, homozygous patients for the DeltaF508 mutation had lower z scores for FVC (-1.21 versus 0.47, p < 0.01) and FEV(1) (-1.38 versus 0.21, p < 0.05) than heterozygous patients. Of the 14 patients who had full flow-volume spirometric measurements during infancy, 10 had FEF(25-75) z scores greater than -2 at both evaluations. Our findings suggest that spirometry can successfully be used to assess lung function in preschool children with CF and has the potential for longitudinal assessment from infancy through adulthood.     

Home versus hospital intravenous antibiotic therapy for acute pulmonary exacerbations in children with cystic fibrosis

To compare the effectiveness of home versus hospital intravenous (IV) antibiotic therapy for acute pulmonary exacerbations in children with cystic fibrosis (CF). A retrospective chart review was performed of 143 encounters for pulmonary exacerbations in 50 patients with CF. All encounters were categorized into two groups based on location of completion of antibiotic therapy: hospital group completed treatment in hospital (n = 64), home group completed treatment at home (n = 79). Percent change was calculated for forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1), forced expiratory flow rate between 25 percent and 75 percent of vital capacity (FEF(25-75%)), maximum forced expiratory flow (FEF(max)), oxygen saturation (O2 SAT), and weight. Means of percent change (PC) from the beginning to the end of IV antibiotic treatment in outcome variables were compared. Total duration of treatment was compared between the two groups. The two groups had no significant differences at baseline in all outcome variables. Treatment of exacerbations in both groups resulted in significant improvement of lung function, O2 SATS, and weight (P 相似文献   

Non-invasive tension time index in relation to severity of disease in children with cystic fibrosis

The non-invasive tension-time index of the inspiratory muscles at rest (TTMUS) can be used for assessing respiratory muscle function in children with cystic fibrosis (CF). This study aimed to investigate how TTMUS becomes altered with increasing pulmonary impairment, and which factors determine TTMUS changes in CF. We assessed TTMUS in 47 patients with stable CF ranging in age from 9 to 26 years and in 47 controls of same age and gender. Pulmonary impairment was assessed by the pulmonary function score (PFS) according to Cropp (PFS 0-2 = no, 3-7 = mild, 8-12 = moderate, and 13-18 = severe dysfunction). Median TTMUS was significantly higher in the entire CF-group than in controls ((0.112 (0.079-0.174) vs. 0.07 (0.052-0.094), P < 0.001)). It was nearly identical in CF-patients without (0.079 (0.056-0.114)) and mild (0.080 (0.059-0.128)) pulmonary dysfunction. It was non-significantly higher in subjects with moderate (0.118 (0.103-0.173)) and grossly elevated in individuals with severe (0.232 (0.211-0.31), P < 0.001)) respiratory impairment when compared to the other PFS-groups. TTMUS was significantly related to percent predicted airway resistance (Raw%pred) (r = 0.60, P < 0.001), percent predicted Forced Expiratory Volume in 1 sec (r = -0.49, P < 0.001), percent predicted Vital Capacity (-0.57, P < 0.001), Functional Residual Capacity in percent Total Lung Capacity (r = 0.42, P = 0.003), and transcutaneous oxygen saturation (r = -0.49, P < 0.001). By contrast, Raw%pred was the only variable that had a significant effect on TTMUS (P = 0.01), when a multivariate logistic regression was applied, using the median of the entire CF-cohort to dichotomise TTMUS. These findings suggest that subjects with stable CF and severe pulmonary dysfunction are prone to respiratory muscle fatigue, and that airway obstruction is an important factor contributing to the increase of TTMUS in CF. Regular determination of TTMUS may be clinically useful during course of disease, and may aid the decision to institute therapies like respiratory muscle training or non-invasive intermittent ventilation.     

Increased arginase activity in cystic fibrosis airways

RATIONALE: Airway nitric oxide concentrations are reduced in cystic fibrosis (CF). Arginases compete for L-arginine, the substrate of nitric oxide synthesis. OBJECTIVES: We hypothesized that increased arginase activity may be one factor contributing to nitric oxide deficiency in CF. MEASUREMENTS: We therefore studied sputum arginase activity, exhaled nitric oxide, and pulmonary function in patients with cystic fibrosis. RESULTS: Mean (+/- SEM) sputum arginase activity was significantly higher in patients admitted for pulmonary exacerbation compared with patients with stable disease (1.032 +/- 0.148 vs. 0.370 +/- 0.091 U/mg protein, p = 0.004). Fourteen days of intravenous antibiotic treatment resulted in significantly decreased sputum arginase activity in all patients (p = 0.0002). However, arginase activity was still significantly (p = 0.0001) higher in CF sputum after treatment for exacerbation compared with induced sputum from healthy control subjects (0.026 +/- 0.006 U/mg protein). Negative correlations were found for sputum arginase activity at admission with FEV1 (r = -0.41, p = 0.01), as well as changes in arginase activity with percent change in FEV1 during antibiotic therapy (r = -0.4, p < 0.01) in CF. Exhaled nitric oxide in CF was positively correlated to FEV1 (r = 0.34, p = 0.007), and in patients admitted for pulmonary exacerbation negatively correlated to sputum arginase activity (r = -0.45, p = 0.03). CONCLUSIONS: These data suggest that increased sputum arginase activity contributes to nitric oxide deficiency in CF lung disease and may be relevant in the pathogenesis of CF airway disease.     

Hypoxemia during air travel in patients with chronic obstructive pulmonary disease

STUDY OBJECTIVE: To quantitate and identify determinants of the severity of hypoxemia during air travel in patients with chronic obstructive pulmonary disease. DESIGN: Prospective study of physiologic variables before and during intervention. SETTING: Referral-based pulmonary disease clinic at a U.S. Army medical center. PATIENTS: Eighteen ambulatory retired servicemen (age 68 +/- 6 [SD] years) with severe chronic obstructive pulmonary disease (forced expiratory volume in the first second [FEV1] 31% +/- 10% of predicted). INTERVENTION: Altitude simulation equivalent to 2438 meters (8000 feet) above sea level in a hypobaric chamber. MEASUREMENTS AND MAIN RESULTS: Radial artery catheter blood oxygen tension in the patients declined from a ground value (PaO2G) at sea level of 72.4 +/- 9 mm Hg to an altitude value (PaO2Alt) of 47.4 +/- 6 mm Hg after 45 minutes of steady state hypobaric exposure. The PaO2G correlated with PaO2Alt (r = 0.587; P less than 0.01). Multiple regression analysis revealed that the preflight FEV1 reduced the variability in PaO2Alt not explained by PaO2G in the equation: PaO2Alt = 0.453 [PaO2G] + 0.386 [FEV1% predicted] + 2.440 (r = 0.847; P less than 0.001). Residuals from two previously published formulas using PaO2G also correlated with FEV1 (r greater than or equal to 0.765; P less than 0.001). CONCLUSIONS: Arterial blood oxygen tension declined to clinically significant levels in most patients during hypobaric exposure. When combined with the preflight arterial PO2 at ground level (PaO2G), the measurement of the preflight FEV1 improved prediction of PaO2 at altitude (PaO2Alt) in patients with severe chronic obstructive pulmonary disease.     

Relationship between arterial blood gas values, pulmonary function tests and treadmill exercise testing parameters in patients with COPD

OBJECTIVE: There have been controversial reports regarding the relationship between exercise tolerance and resting pulmonary function in patients with COPD. The aim of this study was to examine the relationship between resting pulmonary function tests (rPFT) and cardiopulmonary exercise testing parameters (CETP) and their value in estimating exercise tolerance of patients. METHODOLOGY: In total, 45 patients with COPD (nine females, 36 males; mean age 61.2 +/- 11.2) and 21 healthy subjects (four females, 17 males; mean age 60.3 +/- 9.7) as a control group were studied. COPD patients (group I) were divided into three subgroups according to their FEV(1) (mild/group II: FEV(1) 60-79% of predicted; moderate/group III: FEV(1) 40-59%; severe/group IV: FEV(1) < 40%). In controls FEV(1) was >/= 80%. RESULTS: There were significant correlations between FEV(1) and CETP in group III (maximal O(2) consumption (mVO(2)), r= 0.35, P < 0.005; total treadmill time (TTT), r= 0.31, P < 0.01; total metabolic equivalent values (TMET), r= 0.29, P < 0.01)) and in group IV (mVO(2), r= 0.49, P < 0.001; TTT, r= 0.45, P < 0.005; TMET, r= 0.31, P < 0.01; peak heart rate (pHR), r= 0.29, P < 0.02; frequency of ventricular extrasystole (fVES), r=-0.27, P < 0.05). Additionally, in group IV there were significant correlations between PaO(2) and CETP (mVO(2), r= 0.41, P < 0.02; TTT, r= 0.38, P < 0.03; TMET, r= 0.31, P < 0.05; pHR, r= 0.29, P < 0.05; fVES, r=-0.28, P < 0.05). CONCLUSION: There are significant correlations of resting FEV(1)% predicted and PaO(2) values with CETP in patients with moderate and severe COPD and these parameters may also have a role as indicators of exercise tolerance in these COPD patients.     

Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation

Objective measures of lung function are critical for the treatment and study of lung diseases such as cystic fibrosis (CF). Spirometry is the most widely used and accepted method of pulmonary function testing in CF, but not all patients can perform the maneuvers required to obtain valid results from spirometry. The forced oscillation technique (FOT) requires less cooperation than spirometry. The goals of this study were to determine if FOT could detect changes in lung function in CF patients receiving inpatient treatment of respiratory tract exacerbations (RTEs), and to gather preliminary data on the magnitude of these changes and the variability of FOT data in such patients. We performed a retrospective chart review of CF patients admitted to the hospital for RTEs. We identified 14 patients who had both spirometry and FOT performed at the beginning and end of their treatment course. Their mean age was 15.9 years (range, 8-18). The mean forced expiratory volume in 1 sec (FEV1) on admission was 62.57% predicted. FEV1 increased by 27.1 +/- 33.15% (mean +/- SD, P = 0.008). The absolute value of reactance at 5 Hz (X5) decreased by 22.3 +/- 25.1% (P = 0.005), while resistance at 5 Hz decreased by 11.6 +/- 17.3% (P = 0.025). There was a significant relationship between changes in FEV1 and X5 (P = 0.003, r2 = 0.54). Our study demonstrates that FOT can detect significant changes in lung function in CF patients receiving treatment for RTEs. We speculate that FOT can serve as an alternative method to measure lung function in CF patients unable to perform spirometry, such as young children.