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Spinal braces such as the Soft Boston Orthosis (SBO) help stabilize scoliosis and improve sitting, positioning, and head control in individuals with cerebral palsy. However, their impact on pulmonary mechanics in this population has not been studied. We examined the effect of a Soft Boston Orthosis on the pulmonary mechanics and gas exchange in 12 children and young adults (5-23 years of age) with severe cerebral palsy. Pulmonary resistance, compliance, tidal volume, minute ventilation, work of breathing, oxygen saturation, and end-tidal CO2 tension were measured with the subjects seated both with and without the orthosis and in the supine position without the orthosis. There were no significant differences in the measured parameters when comparing subjects with and without their orthoses in the sitting or in the supine position. As would be expected in individuals with severe cerebral palsy, pulmonary resistance was increased (7.33 cm H2O/L/s) and compliance was decreased (0.12 L/cm H2O) compared to reported normal values. Work of breathing was greatest in the sitting position without the orthosis (1.2 dynes/cm), suggesting that the improved positioning achieved with the orthosis may decrease the work of breathing. We conclude that the application of a Soft Boston Orthosis does not impact negatively on pulmonary mechanics and gas exchange in young people with severe cerebral palsy.

Wheeze as an indicator of airway obstruction during bronchoprovocation lacks sensitivity. We therefore studied whether induced airway narrowing is revealed by changes in normal (vesicular) lung sounds. Fifteen subjects with asthma and nine healthy controls, aged 8-16 years, performed a standardized methacholine challenge. Respiratory sounds were recorded with eight contact sensors, placed posteriorly over the right and left superior and basal lower lobes, and anteriorly over both upper lobes, the right middle lobe, and the trachea. Average spectra of normal inspiratory and expiratory sounds, excluding wheeze, were characterized in 12 asthmatics and 9 controls at flows of 1 +/- 0.2 L/sec. Airway narrowing was accompanied by significant changes in lung sounds, but not in tracheal sounds. Lung sounds showed a decrease in power at low frequencies during inspiration and an increase in power at high frequencies during expiration. These changes already occurred at a decrease in forced expiratory volume in 1 sec of less than 10% from baseline and were fully reversed after inhalation of salbutamol. Thus, lung sounds were sensitive to changes in airway caliber, but were not specific indicators of bronchial hyperresponsiveness.

The effect of large doses of salbutamol (S) and ipratropium bromide (IB) were tested in a double-blind, randomized, crossover study. Nine patients with cystic fibrosis (CF), aged 12.8 +/- 2 years (mean +/- SE), were studied for 8 h on 2 separate days. Pulmonary function tests (PFTs) included spirometry (FEV1), lung volumes (FRC), and airway resistance (Raw) measured by body plethysmography. Heart rate (HR) and oxygen saturation (SaO2) were measured before each test. On 1 day patients received S 200 micrograms, S 400 micrograms, and IB 80 micrograms, by inhalation at 45-min interval (sequence A). On the other day, the sequence was IB 80 micrograms, S 200 micrograms, and S 400 micrograms (sequence B). The PFTs were obtained at baseline, 45 min after each inhalation, and 4 and 8 h after baseline measurements. Baseline PFTs (mean +/- SE) were not significantly different on the 2 study days (FEV1, 1.48 +/- 0.1 vs 1.42 +/- 0.1 L; FRC, 2.77 +/- 0.6 vs 2.87 +/- 0.6 L; Raw, 4.04 +/- 0.2 vs 4.00 +/- 0.3 cm H2O/L/s). The FEV1 and Raw improved from baseline after each inhalation, and at 4 and 8 h during both days (p < 0.05). Forty-five minutes after S 200 micrograms, plus S 400 micrograms, FEV1, FRC, and Raw were not significantly different compared with the values 45 min after IB 80 micrograms, plus S 200 micrograms (1.67 +/- 0.1 vs 1.63 +/- 0.1 L; 2.81 +/- 0.6 vs 2.65 +/- 0.5 L; and 2.98 +/- 0.2 vs 2.66 +/- 0.1 cm H2O/L/s, respectively). The PFTs were not significantly different after maximal doses of IB (80 micrograms) compared with S (600 micrograms). The HR and SaO2 were not significantly different from baseline throughout the study period. These results indicate that both single and sequential therapy have a similar acute bronchodilator effect provided that large doses are used. We speculate that adrenergic and muscarinic pathways are equally important in airflow obstruction in patients with CF.

Patients with cystic fibrosis had a better acute bronchodilator response to albuterol and ipratropium bromide than to either drug alone. Further studies of long-term efficacy and safety seem justified.

The effect of theophylline (T) was studied in 12 children with cystic fibrosis (CF) (seven males and five females) aged 7 to 17 yr (mean, 11.8 +/- 2.8). T or placebo (P) was taken for 10 days in a double-blind, crossover controlled trial. During Nights 9 and 10 of each study period, the subjects slept in a sleep laboratory, where complete polysomnographs were recorded. Patients were subdivided according to T blood levels above or below 10 micrograms/ml: five children had a mean serum T of 11.7 +/- 1.6 micrograms/ml (Group 1), and seven patients had serum T levels of 6.7 +/- 1.5 micrograms/ml (Group 2). There was no difference in pulmonary function between the two periods, but Group 2 had significantly better baseline lung function (p less than 0.01). During sleep while on T, Group 1 had a higher mean SaO2 (93.1 +/- 0.3% T versus 90.7 +/- 0.3% P; p less than 0.0001), less time with a decrease greater than 5% in SaO2 from baseline (43.7 +/- 7.9 min T versus 85.8 +/- 7.9 min P; p less than 0.01), and a lower heart rate (HR), (77.9 +/- 5.1 bpm T versus 86.1 +/- 4.2 bpm P; p less than 0.01). Group 2 did not show differences in these parameters. T significantly disrupted sleep as measured by sleep efficiency and total wake time independently of serum level (p less than 0.01). There was no significant effect of T on the incidence of apnea, hypopnea, or body movements during sleep.(ABSTRACT TRUNCATED AT 250 WORDS)