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Predictors of severe chest infections in pediatric neuromuscular disorders.

Chest infections are serious complications in neuromuscular disorders. The predictive values of lung and respiratory muscle function including peak cough flow still remain unclear. We performed retrospective analysis of 46 children and adolescents (12.7+/-3.7 years) in whom lung function, respiratory muscle function and peak cough flows had been obtained. Data were related to: (1). number of chest infections and days of antibiotic treatment the year prior to the study and (2). history of severe chest infection requiring hospital admission. The number of chest infections and the number of days treated with antibiotics correlated with Inspiratory Vital Capacity IVC, peak cough flow PCF and Peak Expiratory Pressure PEP. Twenty-two patients were hospitalized at least once due to severe chest infection. IVC (0.65 vs. 1.44 l; P<0.0001) and PCF (116 vs. 211 l/min; P<0.0005) in these patients were significantly lower than in the non-hospitalized group. IVC<1.1l and PCF<160 l/min were specific and sensitive thresholds to discriminate between patients who had already suffered severe chest infections and those who had not. Therefore, spirometry and peak cough flow are reliable tests to identify patients at high risk for severe chest infections. Patients with IVC below 1.1l and/or PCF below 160 l/min should be well monitored and introduced to assisted coughing techniques.

Adolescent↗

Reduced airway responsiveness in nonelite runners.

PURPOSE: The effects of endurance training on airway responsiveness in nonasthmatic subjects are poorly defined. We hypothesized that airway responsiveness may differ between none-lite endurance athletes and sedentary subjects, and studied healthy, nonelite runners and sedentary controls by single-dose methacholine challenges carried out in the absence of deep inspirations, in that deep inspirations are known to oppose airway narrowing in nonasthmatic subjects. METHODS: A total of 20 nonasthmatic none-lite runners (mean age+/- SD: 43.0+/- 8.5 yr; training volume: 68 km.wk; range: 40-100; racing experience: 11+/- 8 yr) and 20 sedentary controls (age: 44.0+/- 20.6 yr) were studied, all of them being normo-reactive to standard methacholine challenge up to 25 mg.mL concentration. All subjects were studied at rest; six runners were also studied about 1 h after completing the Palermo marathon (December 8, 2001). The primary outcome of the study was the inspiratory vital capacity (IVC) obtained after single-dose methacholine inhalation at the end of 20 min of deep inspiration prohibition. RESULTS: At rest, IVC decreased by 10.5+/-8.1% after challenge with methacholine at 75 mg.mL in athletes, and by 24.3+/-16.1% after a methacholine concentration of 52+/-5.7 mg.mL in sedentary controls (P=0.002). The decreased response to methacholine in runners did not correlate with static lung volumes, amount of weekly training, or running experience. CONCLUSION: Methacholine challenge under deep inspiration prohibition revealed that endurance training attenuates airway responsiveness in nonasthmatic, none-lite runners. Airway hyporesponsiveness was potentiated after the marathon, suggesting involvement of humoral (i.e., catecholamine levels), airway factors (i.e., nitric oxide), or both in modulating airway tone after exercise.

Adult↗

Lumbar traction: its effect on respiration.

The purpose of this study was to examine the effects of lumbar traction on inspiratory vital capacity (IVC), tidal volume (TV), and respiratory rate (fb). Using an 8-L recording spirometer, we measured the above values on 30 healthy volunteers during three conditions--lying supine, lying supine with a traction garment applied, and lying supine with the traction garment and a distractive force applied. The differences in IVC, TV, and fb among the three conditions were examined by using an analysis of variance for repeated measures followed by a Tukey test. Statistically significant differences were found for IVC, TV, and fb among the three conditions (p less than .001). The results indicated that IVC and TV were lowest and fb was highest when the distractive force was applied to the traction garment. We suggest that when lumbar traction is used for patients who have respiratory disease, the patients should be observed closely for signs of respiratory distress during the first few treatments.

Adult↗

Effect of inspiratory resistance and theophylline on respiratory muscle strength in patients with amyotrophic lateral sclerosis.

The effect of breathing through inspiratory flow resistive loads ranging between 4.5 and 27.0 cm H2O/L/s was assessed in eight patients with amyotrophic lateral sclerosis (ALS) and in eight control subjects. ALS patients developed respiratory muscle fatigue manifested by significant declines in negative inspiratory pressure (18.3%), vital capacity (7.2%), and peak inspiratory flow rate (5.5%). Control subjects did not fatigue with these resistances. In ALS patients, theophylline increased respiratory muscle strength after resistive breathing as manifested by an increase in negative inspiratory pressure (28.2%), vital capacity (10%), and peak inspiratory flow rate (11.8%). It is concluded that in patients with ALS, the already weakened respiratory muscles are easily fatigued. Furthermore, theophylline can strengthen loaded respiratory muscles in patients with ALS.

Airway Resistance↗

Inspiratory muscle training in patients with prior polio who use part-time assisted ventilation.

OBJECTIVE: To evaluate whether inspiratory muscle training in patients with prior poliomyelitis (and with symptoms and signs of hypoventilation) who use part-time assisted ventilation could improve symptoms and respiratory muscle function without adverse effects. DESIGN: Intervention study with before-after trial. SETTING: Training was performed in the patients' homes; assessments were performed at the hospital. PATIENTS: Ten prior-polio patients were included. Three of them did not complete the trial, and their data were not included in results of training. INTERVENTION: Ten weeks of daily inspiratory muscle training. MAIN OUTCOME MEASURES: Spirometry, maximal inspiratory and expiratory pressures, inspiratory muscle endurance, and questions regarding activities of daily living were performed. RESULTS: Inspiratory endurance capacity over 5 minutes improved from 10.7 to 16.7cm H2O (p < .05) assessed at 15 on the Borg scale, and most patients improved in activities of daily living. The whole-body endurance capacity remained stable over the studied period. CONCLUSION: Inspiratory muscle training and close supervision can increase respiratory muscle endurance and improve well-being in patients with prior polio who use part-time assisted ventilation.

Activities of Daily Living↗

Effect of fibreoptic bronchoscopy on pulmonary function.

Several studies have shown that after fibreoptic bronchoscopy there may be a deterioration in lung function but it is not known whether this is due to the premedication, the topical anaesthetic, or the obstruction produced by the bronchoscope. The effects of each part of the procedure on spirometric measurements were studied in patients with lung disease and in normal non-smokers. Measurements were made after premedication (papaveretum and atropine) in seven patients and after topical anaesthesia of the bronchial tree (340 mg lignocaine) with and without the bronchoscope in the trachea in 21 patients and 10 control subjects. Premedication had no effect. In the normal subjects lignocaine produced significant falls in FEV1, forced vital capacity (FVC), peak expiratory flow (PEF), and peak inspiratory flow (PIF), and insertion of the bronchoscope caused further falls that were also significant. In the patients, however, although anaesthesia produced significant falls in FEV1, FVC, PEF, and PIF of similar magnitude to those found in the normal subjects, there was no further important decrease when the bronchoscope was inserted. It is concluded that the major effect of bronchoscopy on lung function is due to topical lignocaine in the airways, and in patients with lung disease (excluding asthma or a central obstructing carcinoma) the insertion of the bronchoscope causes little additional obstruction.

Administration, Topical↗

The effect of inspiratory resistive training on exercise capacity in optimally treated patients with severe chronic airflow limitation.

This study was designed to determine whether inspiratory resistive training could improve the exercise performance of patients with severe chronic airflow limitation who had already received optimum conventional therapy with bronchodilators and physiotherapy. Eighteen patients were studied. Ten patients were trained with an inspiratory resistance device for six weeks and eight patients used a placebo device. Psychological factors, likely to influence exercise capacity, were taken into account. Although there was no significant increase in maximum inspiratory pressure in the ten trained subjects, inspiratory muscle endurance was improved. Exercise performance, as assessed by progressive cycle exercise, stair climbing, 12 minute walking distance and treadmill walking did not change significantly in either group. It was concluded that inspiratory muscle training, using a currently available technique, produced no additional improvement in exercise capacity beyond that achieved by conventional bronchodilator and rehabilitation therapy.

Aged↗

[Obstructive syndrome in laryngotracheal stenosis].

Pulmonary function studies were carried out in 13 patients in whom the diagnosis of a central (laryngeal or tracheal) stenosis was confirmed by endoscopy. Following lung function tests were performed: static lung volumes (VC, RV, TLC), FEV1.0 and FIV1.0, flow-volume loops of forced expiratory and forced inspiratory vital capacity efforts from which PEFR and PIFR, maximal expiratory (VEmx50) and maximal inspiratory flow at 50 per cent of vital capacity (VLmx50) were measured, Raw, helium mixing time and pharmacological test with acethylcholine. FIV1.0 values and flow-volume loops permitted the classification of the patients into 3 groups: 1. fixed obstruction (6 cases): decrease in about equal proportions of maximal expiratory and inspiratory flows (FEV1.0 and FIV1.0); normal FEV1.0/FIV1.0 ratio; 2. extrathoracic variable obstruction (1 case): predominant decrease in maximal inspiratory flows (FIV1.0). FEV1.0/FIV1.0 ratio is twice as normal; 3. intrathoracic variable obstruction (5 cases): exclusive or predominant reduction in maximal expiratory flows, as in COPD but with the difference that the expiratory limb of the V:V loop showed an excessive reduction in PEFR, a plateau in the midzone of the VC and a terminal portion with normal configuration. Raw was almost always increased, RV sometimes, helium mixing time was prolonged only in presence of COPD or lobar stenosis; pharmacological tests were negative in 3 out of 4 cases. Pulmonary function abnormalities did not allow a prognosis of the nature of the underlying lesion. The severity of the obstruction as evaluated by endoscopic examination was not related to the functional disturbances. In one case no functional alterations were found despite a severe tracheal stenosis.

Adult↗

Vital capacity in trained and untrained healthy young adults in the Netherlands.

Slow inspiratory vital capacity was measured in 226 healthy young adults, aged from 17 to 35 years. The group included 119 men and 107 women, 87 trained subjects, 71 untrained subjects who intended to take part in a training program for competitive rowing, and 68 untrained subjects who never took part in any competitive sport. The vital capacity increased with height, weight, fat-free mass, height X fat-free mass, and height-independent fat-free mass, with men having significantly higher vital capacities than women of the same height or weight. In both males and females vital capacity showed the best relation with height X fat-free mass (correlation coefficients are 0.78 and 0.57 respectively). Multiple regression on vital capacity with height, weight, fat-free mass, height X fat-free mass, height-independent fat-free mass, percentage body fat, and age increased the correlation coefficient only slightly (0.80 and 0.59 respectively). The subjects had vital capacities that were much higher than those predicted for them by equations originating from the USA. There was no difference between the observed vital capacities and those predicted by equations originating from Europe. There is a difference in vital capacity between the European subjects studied and subjects of similar height studied in the USA. This implies that equations derived from subjects in the USA cannot be applied to European subjects. From our results we conclude that vital capacity is not increased by physical activity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adipose Tissue↗

Changes in pulmonary hyperinflation and bronchial hyperresponsiveness following treatment with lansoprazole in children with cystic fibrosis.

SUMMARY. In this prospective open study of 14 children with cystic fibrosis (CF), we evaluated the effect of 1 year adjuvant therapy with lansoprazole, a proton pump inhibitor (PPI), on growth, fecal fat loss, body composition and lung function. Only stable patients with pancreatic insufficiency were included, and their data were compared to those of a large Dutch pediatric normal reference population. During the use of the PPI, mean weight and height did not change significantly, while body mass index improved (P < 0.05). An immediate significant and persistent reduction of fecal acid steatocrit (P < 0.05) was demonstrated. Compared to normal Dutch children, the CF patients showed significantly decreased standard deviation scores (SDS) for total body fat (TBF, -0.966) and fat-free mass (FFM, -1.826). Under lansoprazole, TBF improved significantly (P < 0.05), while mean FFM remained unchanged. A significant improvement in total lung capacity (P < 0.05), residual volume (P = 0.055), and maximal inspiratory mouth pressure (P = 0.002) was also demonstrated. Hyperinflation tended to decrease during the use of a PPI. Daily recordings of peak expiratory flow (PEF) showed a maximal diurnal variability of 28% of recent best PEF and minimal morning PEF of 72% of recent best PEF, confirming that bronchial hyperresponsiveness is increased in CF. We conclude that adjuvant therapy with lansoprazole in young CF patients with persistent fat malabsorption, decreased fat losses and improved total body fat. Lung hyperinflation decreased, which may partly explain the improvement in inspiratory muscle performance. The simultaneous improvements in body composition and lung hyperinflation suggest a relationship between these two parameters. Further research is necessary to confirm such a relationship and to elucidate the mechanisms involved.

2-Pyridinylmethylsulfinylbenzimidazoles↗

Respiratory and limb muscle function in lung allograft recipients.

Lung transplantation recipients have reduced exercise capacity despite normal resting pulmonary and hemodynamic function. The limiting factor may be contractile dysfunction of skeletal muscle. To test this postulate, we measured limb and respiratory muscle function in nine clinically stable lung allograft recipients (six men and three women, aged 30 to 65 yr, at 5 to 102 mo after transplantation) with reduced exercise capacity. Respiratory muscle strength was tested by measuring maximal inspiratory and expiratory pressure (MIP and MEP, respectively). Ankle dorsiflexor muscle strength was measured during maximal voluntary contraction (MVC). In a subset of six recipients, we also measured contractile properties and fatigue characteristics of the tibialis anterior muscle, using electrical stimulation of the motor point. Data were compared with values from age- and sex-matched control subjects. MIP values of transplant recipients did not differ from control values; however, MEP was blunted by 30% relative to control (p < 0.05), and MVC was decreased by 39% (p < 0.05). The force-frequency relationships and fatigue characteristics of the tibialis anterior were not different between the patient and control groups. We conclude that stable lung allograft recipients experience expiratory and lower limb weakness that may contribute to exercise intolerance.

Adult↗

P 0.1/PIMax: an index for assessing respiratory capacity in acute respiratory failure.

We studied airway occlusion pressure (P 0.1) and maximal inspiratory pressure (PIMax) in 10 healthy volunteers (Group A), 10 early postsurgical cardiac patients on spontaneous breathing (Group B), 10 patients mechanically ventilated for ARF (Group C), 10 patients weaning from mechanical ventilation after ARF (Group D) and 10 patients extubated after post-ARF (Group E). We calculated the index P 0.1/PIMax in an attempt to link the ventilatory demands and muscle ventilatory reserve. We found that the sensitivity and specificity in diagnosing the need for either full (C), partial (D) or no ventilatory support (A, B, E) by means of the P 0.1 were C = (50%, 95%), D = (70%, 72%) and A + B + E = (83%, 90%) respectively. When the index P 0.1/PIMax was used they were C = (90%, 100%), D = (80%, 87%) and A + B + E = (86%, 90%). We conclude that the index P 0.1/PIMax increases the reliability of P 0.1 alone to correctly classify the patients that will need either full, partial or no ventilatory support in ARF.

Adult↗

Practical interest in the detection of functional abnormalities in infants and children with lung disease.

There are new techniques which have been developed in order to assess objective functional data concerning the severity and type of abnormalities in infants and children with lung disease. In the present review some applications of the various techniques are given in connection with deductions, which can be based on such functional findings. Insight into the mechanisms leading to lung disease and the institution of appropriate therapeutic guidelines largely depend on whether the degree of pulmonary hyperinflation or restriction, of bronchial obstruction, of bronchial hyperreactivity, of ventilation inequalities and the function of gas exchange can be objectively evaluated. Recent advances in the non-invasive assessment of lung function in infants and children promises progress in this task.

Adolescent↗

Respiratory retraining of refractory cough and laryngopharyngeal reflux in patients with paradoxical vocal fold movement disorder.

OBJECTIVES/HYPOTHESIS: The objective was to describe a case series of patients with refractory cough and paradoxical vocal fold movement disorder treated with respiratory retraining therapy. STUDY DESIGN: Retrospective review of a case series in a tertiary medical care center. METHODS: Five patients with laryngopharyngeal reflux were identified with refractory cough and paradoxical fold movement disorder on transnasal fiberoptic laryngoscopy by a greater than 50% reduction in airway during inspiration. The were four women and one man (age range, 42-67 y). All patients had normal forced vital capacity and forced expiratory flow but decreased ratio of forced inspiratory volume at 0.5 seconds (FIV(0.5)) to forced inspiratory vital capacity (FIVC) before starting therapy. All patients were treated with more than 6 months of twice-daily proton pump inhibitor therapy with improvement in reflux symptoms but persistent and severe daytime cough. They were subsequently treated with respiratory retraining therapy. Patients were asked to rate subjectively the severity of cough at the onset and conclusion of therapy. All patients underwent pulmonary function testing before and after therapy. Long-term follow-up ranged from 5 to 17 months. RESULTS: Patients received two to seven sessions of respiratory retraining therapy. The mean severity score changed from 9.2 before therapy to 1.3 after therapy. All patients subjectively described an improvement in the severity of their cough. Transnasal flexible laryngoscopy demonstrated improvement in paradoxical vocal fold movement, and pulmonary function testing showed improvement in the FIV(0.5)/FIVC ratio. CONCLUSION: Patients with laryngopharyngeal reflux and refractory cough in the absence of pulmonary disease should be evaluated for paradoxical vocal fold movement disorder. Respiratory retraining therapy may represent an effective therapy for cough in the absence of relief from standard management of laryngopharyngeal reflux.

Adult↗

Reference values and prediction equations for normal lung function in a non-smoking white urban population.

Prediction equations for normal lung function have been derived from tests on 179 healthy, non-smoking, white urban dwellers. The subjects, 96 women (height 1.46-1.77 m) and 83 men (height 1.61-1.96 m) aged 18-86 years, underwent measurements of spirometric flow and volume, multi-breath helium dilution lung volumes, and single breath carbon monoxide transfer factor and the single breath nitrogen washout test. Regression analysis using height, age, and weight as independent variables was used to provide predicted values for both sexes. Correlation coefficients were similar to those found in previous studies but normal ranges for spirometic measurements were narrower than in many previous studies, and spirometric flow and volume measurements were higher than those obtained in studies that included cigarette smokers, reflecting our more stringent criteria for selecting subjects and the newer standardised technical methods adopted. Multi-breath helium dilution values for total lung capacity were similar to those found in previous studies but the inspiratory vital capacity was larger and the residual volume reduced. Values for carbon monoxide transfer factor and the single breath nitrogen washout did not differ significantly from existing values. A complete set of lung function reference values and prediction equations for both sexes has been derived from a single population. The exclusion of cigarette smokers and subjects with respiratory symptoms has produced values that should have a greater sensitivity in the detection of mild lung disease.

Adolescent↗