Comparison of Flutter device and chest physical therapy in the treatment of cystic fibrosis pulmonary exacerbation.

Chest physiotherapy (CPT) is recommended for the clearance of bronchial secretions in the management of patients with cystic fibrosis (CF). The Flutter valve (Scandipharm, Birmingham, AL) has been introduced as an alternative method to CPT for airway mucus clearance. The objective of this study was to compare the short-term effects of CPT and the Flutter valve on pulmonary function and exercise tolerance in patients with cystic fibrosis. Twenty-three patients, 5 to 21 years of age, were randomized to receive one of two interventions: CPT or the Flutter valve, upon admission to the hospital for a 2-week treatment of pulmonary exacerbation. Pulmonary function testing (PFTs) and the 6-min walk test were performed on admission, day 7, and day 14 of hospitalization. Data analysis indicated no significant differences between the two groups on admission. Both groups showed improvement in pulmonary function test results, but the Flutter group had a higher mean forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV(1)) compared to the CPT group after 1 week of intervention. Both groups continued to improve during the 2-week intervention, with no significant difference in FVC or FEV(1) between groups by the end of 2 weeks. Mean forced expiratory flow rate between 25-75% of vital capacity (FEF(25-75)), 6-min walk distance, and resting arterial oxyhemoglobin saturation (SaO(2)) showed little change by day 7, but improved significantly (P< 0.05) by day 14 of hospitalization in both groups, with no significant difference between groups. This study demonstrated that patients using the Flutter device had better pulmonary function after 1 week of therapy and similar improvement in pulmonary function and exercise tolerance compared to CPT after 2 weeks of therapy, suggesting that Flutter valve therapy is an acceptable alternative to standard CPT during in-hospital care of patients with CF.

Longitudinal follow-up of lung function from childhood to adolescence in prematurely born patients with neonatal chronic lung disease.

We investigated whether early lung function abnormalities in prematurely born children with a history of chronic lung disease improve in late childhood and adolescence. We performed a prospective, longitudinal evaluations of pulmonary function over an 8 year period. In seventeen patients from the age (mean +/- SD) of 8.2 +/- 1.2 years to the age of 15.1 +/- 1.6 years. They had been born at 29.1 +/- 1.9 weeks of gestation, with a birthweight of 1120 +/- 190 g, and they had received supplemental oxygen, with or without mechanical ventilation, for 40.4 +/- 23.8 days during the neonatal period. They all had radiographic evidence of chronic lung disease at 4 weeks of age. Annual measurements of lung volumes using the helium dilution technique, and of airway function with spirometry and maximal expiratory flow-volume curves over a 5 to 8 year period, were obtained. The results indicated that total lung capacity (TLC) and vital capacity (VC) were within the predicted normal range in all patients and increased over time. In contrast, the initially abnormal residual volume (RV) and RV/TLC ratio decreased over time, suggesting gradual resolution of air-trapping. The peak expiratory flow rate (PEFR), forced expiratory volume in 1 second (FEV1), and the ratio FEV1/FVC remained at or above the predicted normal range in all patients. FEF25-75, FEF50, and FEF75 were within normal limits in eight patients and abnormally low (more than 2 SD below the predicted normal value) in the remaining nine patients, indicating small airway obstruction. Eight of the nine patients with lower airway obstruction showed significant response to inhaled bronchodilator, and four responded to a histamine challenge. None of the eight patients with normal airway function responded to histamine, but four responded to bronchodilators. The perinatal history, family history of asthma, and exposure to smoking were similar in patients with and without airway obstruction. The height and weight were and remained within the normal range. We conclude that gradual normalization of air-trapping continues well into adolescence in virtually all patients with a history of prematurity and chronic lung disease. in contrast, airflow obstruction may persist but does not get worse later in life. Although chronic airflow obstruction probably is the consequence of injury to the small airways during the neonatal period, it is present in only some of the children, and it does not appear to be directly related to the perinatal history. Finally, there is evidence that airway hyperresponsiveness may be a contributing factor to the development and/or persistence of airflow obstruction in chronic lung disease of prematurity.

Contribution of airway hyperresponsiveness to lower airway obstruction after extracorporeal membrane oxygenation for meconium aspiration syndrome.

OBJECTIVE: To determine whether airway hyperresponsiveness contributes to the development of lower airway obstruction in infants recovering from severe meconium aspiration syndrome treated with extracorporeal membrane oxygenation (ECMO). DESIGN: Prospective comparison study of the response to bronchodilator during the acute and convalescent phase of severe meconium aspiration. SETTING: Pediatric/neonatal intensive care unit in a tertiary care hospital. PATIENTS: Seven neonates with severe meconium aspiration syndrome that was refractory to conventional mechanical ventilation, requiring ECMO treatment. INTERVENTIONS: Evaluation of the effect of bronchodilator treatment on the airway function at a postnatal age of 14 +/- 2.7 (SEM) days, after the patients had been off ECMO for 4.6 +/- 1.4 days, and comparison with the response the same patients had shown at a postnatal age of 2.7 +/- 0.6 days, when they had been on ECMO for 1.3 +/- 0.6 days. Lung mechanics and lower airway function were measured and compared before and after administration of aerosolized isoetharine early in the course of ECMO and again several days after ECMO. Maximum expiratory flow-volume curves produced by the deflation flow-volume curve technique were used for evaluating the lower airway function, and partial passive flow-volume curves were used for measuring respiratory system compliance and resistance. MEASUREMENTS AND MAIN RESULTS: During the first test, isoetharine produced a mild increase in maximum expiratory flows at 25% (MEF25) of forced vital capacity (FVC) (48 +/- 27% compared with baseline values), without significant change in the MEF25 to FVC ratio. During the second test approximately 2 wks later (post-ECMO), isoetharine increased MEF25 by 123 +/- 29% and increased the MEF25/FVC by 40 +/- 13% compared with baseline values. The percent change in both indices was significantly higher during the second test (p < .05) than in the first test. CONCLUSIONS: Airway obstruction in infants recovering from severe meconium aspiration syndrome is partially reversible with aerosolized isoetharine, indicating that airway hyperresponsiveness contributes to the pathogenesis of airway obstruction.

Lung mechanics during and after extracorporeal membrane oxygenation for meconium aspiration syndrome.

OBJECTIVE: To determine whether abnormalities in lung mechanics detected in infants during the acute phase of meconium aspiration syndrome persist after treatment with extracorporeal membrane oxygenation (EMCO). DESIGN: Prospective, descriptive study. Prospective evaluation of airway function and lung mechanics during and after ECMO by pulmonary function testing at 1.8 +/- 0.5 days of EMCO (period 1), follow-up at 1.4 +/- 0.2 days (period 2), and 7.0 +/- 0.9 days (period 3) after decannulation from ECMO. SETTING: Tertiary care neonatal/pediatric ICU. PATIENTS: Twelve neonates undergoing ECMO treatment for severe meconium aspiration syndrome that was refractory to conventional mechanical ventilation. INTERVENTIONS: Maximum expiratory flow-volume curves were studied with the deflation flow-volume curve technique, and compliance and resistance of the respiratory system were studied with partial passive flow-volume curves. MEASUREMENTS AND MAIN RESULTS: Respiratory system compliance was the only index of respiratory mechanics that was significantly (p less than .05) improved (0.96 +/- 0.1 vs. 0.61 +/- 0.1 mL/cm H2O/kg) immediately after decannulation from ECMO compared with period 1. Clinically important (p less than .05) improvement in forced vital capacity (28.0 +/- 5.5 vs. 16.1 +/- 1.9 mL/kg), respiratory system compliance (1.01 +/- 0.2 vs. 0.61 +/- 0.1 mL/cm H2O/kg), and maximum expiratory flow at 25%/forced vital capacity (1.0 +/- 0.3 vs. 2.2 +/- 0.3) was evident only during period 3 compared with period 1. CONCLUSIONS: We conclude that improvements in the clinical condition and oxygenation, permitting successful decannulation from ECMO, are achieved before clinically important improvements in lung mechanics.

Pulmonary dysfunction after primary closure of an abdominal wall defect and its improvement with bronchodilators.

To determine the extent of pulmonary dysfunction following primary closure of an abdominal wall defect, we obtained pulmonary function tests (PFT) in 11 newborn infants with gastroschisis and 6 with large omphaloceles admitted to a newborn ICU in a children's hospital. Patients were 1 to 30 days of age at the time of the PFT; all required endotracheal intubation and mechanical ventilation for operative procedures or for postoperative ventilatory support. Full-term infants (n = 21) undergoing minor surgical procedures provided comparative measurements. Flow-volume curves were obtained with manual inflation of the lungs followed by forced deflation using negative pressure, or by passive expiration, under sedation and pharmacologic paralysis. Deflation flow-volume curves gave measurements of forced vital capacity (FVC) and maximal expiratory flow at 25% of vital capacity from residual volume (MEF25). Modified passive mechanics technique gave passive expiratory curves that provided measurements of respiratory system compliance (Crs) and resistance (Rrs). Tests were done: within 48 h (period A), 3-7 days (period B), and 8-30 days after surgical repair (period C). Pulmonary function testing after nebulized 0.1% isoetharine (a bronchodilator), to test for bronchial reactivity, began midway during the study period in 15 patients. Preoperative and postoperative tests were obtained in 5 patients. Closure of an abdominal wall defect decreased FVC, Crs, and MEF25 by up to 50% of normal, reference values after surgery (P less than 0.05). FVC and MEF25 approached values of normal infants by 4 weeks, whereas Crs remained 50% lower.(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary dysfunction in surgical conditions of the newborn infant.

OBJECTIVE: To describe the pathophysiology of surgical conditions that are associated with respiratory insufficiency in the newborn infant. DESIGN: Survey. SETTING: Newborn ICU in a children's hospital. PATIENTS: Twenty-four newborn infants (1 to 28 days old) who required endotracheal intubation and mechanical ventilation for operative procedures or postoperative ventilatory support. INTERVENTIONS: Flow-volume curves obtained by manual inflation of the lungs, followed by forced deflation by negative pressure, and by passive expiration, under sedation and pharmacologic paralysis. MEASUREMENTS: Deflation flow-volume curves and passive expiratory curves were measured. Pulmonary function testing before and after bronchodilator administration (n = 11) began midway during the study period. Term and preterm groups served as controls. MAIN RESULTS: Forced vital capacity (FVC) was decreased in all groups with surgical disease as follows: abdominal wall defects and necrotizing enterocolitis groups to 48.3% and 62.1% that of preterm, respectively; pulmonary hypoplasia group to 55.5% that of term (p less than .05). Maximal expiratory flow at 25% of FVC decreased in all groups: abdominal wall defects and necrotizing enterocolitis group, to 36.8% and 37.9% that of preterm, respectively (p less than .05); pulmonary hypoplasia group, 20.0% that of term (p less than .05). The ratio of maximal expiratory flow at 25% of FVC divided by FVC was significantly decreased in necrotizing enterocolitis and pulmonary hypoplasia groups compared with that of preterm and term groups, respectively, but not in the abdominal wall defects group. Maximal expiratory flow at 25% of FVC, but not FVC, increased significantly (36%, p less than .05) after bronchodilator nebulization, indicating the presence of airway reactivity. Respiratory system compliance was decreased significantly (p less than .05) in all surgical disease groups compared with the term group. CONCLUSIONS: Bronchial reactivity contributes to decreased maximal expiratory flow at 25% of FVC, a feature also seen in premature infants with respiratory distress syndrome who later develop bronchopulmonary dysplasia. Babies who require chronic ventilatory support after operation and who have developed reactive airways may benefit from the administration of bronchodilators during postoperative ventilatory management.

Pulmonary function studies in a newborn with congenital laryngeal atresia.

Congenital laryngeal atresia is a rare cause of upper airway obstruction that leads to death unless a surgical airway is immediately established. We were able to resuscitate a baby boy with laryngeal atresia by the placement of an 18-gauge plastic intravenous cannula into the trachea, connected in turn to a 3-mL syringe without the plunger, and then to the connector to a 7.0-mm endotracheal tube. This arrangement allowed hand ventilation and sufficient gas exchange until a formal tracheotomy was established minutes later. The baby boy had deficient abdominal musculature (without cryptorchidism or obstructive uropathy), bilateral inguinal hernias, and idiopathic hypercalcemia (since spontaneously resolved), but no other major anomalies. His survival allowed measurements of pulmonary function in lungs distal to an obstructed upper airway, an arrangement that mimics experiments that examine the influence of lung fluid volume and pressure on developing lungs. The baby's lungs had a forced vital capacity (FVC) in the upper limits of normal (not grossly enlarged lungs seen in newborn animals undergoing ligation of the trachea in utero). Maximal expiratory flow at 25% of FVC from residual volume (MEF25) was decreased, indicating airway obstruction involving smaller airways. Although direct laryngoscopy failed to find a opening in the larynx, some communication probably existed during development to allow some drainage of lung fluid. This opening, in the form of a persistent pharyngoglottic duct, prevented gross distention of the developing lung, but provided an insufficient airway at birth.

Pulmonary function in newborns after repair of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia that limits survival, but the nature and extent of pulmonary dysfunction in neonates with CDH have not been studied. We performed pulmonary function tests (PFTs) in eight intubated infants who survived neonatal repair of CDH (wt, 3.33 +/- 0.15 kg; age, 20.1 +/- 2.7 d; mean +/- S.E.M.). PFTs obtained from six full-term infants (wt, 3.56 +/- 0.15 kg; age, 25.0 +/- 3.3 d) with no respiratory illness served as controls. The deflation flow-volume curve technique produced maximum expiratory flow-volume (MEFV) curves, giving reproducible measurements of forced vital capacity (FVC) and maximal expiratory flow at 25% of FVC (MEF25). Respiratory system compliance (Crs) and resistance (Rrs) were obtained with a modified passive mechanics technique. In seven of eight infants PFTs were repeated after nebulized bronchodilator (0.1% isoetharine). In neonates surviving CDH repair, as compared to those with normal lung function, FVC was significantly reduced (20.78 +/- 3.32 vs. 39.83 +/- 3.30 mL.kg-1, P less than 0.05). MEF25 was also markedly reduced (20.78 +/- 3.32 vs. 39.83 +/- 3.30 mL.kg-1.s-1, P less than 0.05), indicating lower airway obstruction. After administration of nebulized bronchodilator, PFTs showed significant increases from control values in both FVC (15.9%) and MEF25 (200%) without changes in Crs and Rrs. These findings indicate that neonates with CDH have restrictive lung defects, reflecting hypoplasia. After surgical repair and mechanical ventilation airway reactivity develops, primarily in smaller airways, and this may complicate the postoperative course.

Longitudinal changes in lung function during the first three years of premature infants with moderate to severe bronchopulmonary dysplasia.

Bronchopulmonary dysplasia (BPD) is a chronic obstructive pulmonary disease of prematurely born infants following prolonged mechanical ventilation and oxygen therapy. Developmental changes in pulmonary function of children with BPD during their early years have been difficult to study. We longitudinally studied maximal expiratory flow-volume curves by the forced deflation technique in 11 infants who had previous tracheostomy with moderate to severe BPD. Patients were classified into: those who were mechanically ventilated for less than 5 months (Group A), and those who were ventilated for 10 or more months (Group B). At 6 months of age, forced vital capacity (FVC) was 28.1 and 25.5 mL/kg in Group A and B, respectively, significantly less than normal (41.8 mL/kg). The maximum expiratory flow at 25% FVC (MEF25) at 6 months of age was 6.9 and 8.1 mL.kg-1.s-1 in Group A and B, respectively, (predicted value, 39.2 mL.kg-1.s-1). FVC reached the normal range by 12 months of age in Group A, but remained lower until 36 months of age in Group B. MEF25 gradually increased in Group A, reaching 18.0 mL.kg-1.s-1 at 36 months of age, whereas in Group B it was severely decreased at the same age (3.5 mL.kg-1.s-1). More than 75% of the patients had airway hyperreactivity at all ages. We have demonstrated that in patients with moderate to severe BPD, vital capacity is moderately decreased, but catches up to normal levels by 36 months of age. In contrast, severe lower airway obstruction persists in all infants, although in those with moderate BPD gradual improvement is seen. These findings suggest that in BPD neither obstruction of the smaller intrathoracic airways nor bronchial hyperreactivity resolves during the first 3 years of life.

Bronchial reactivity in infants in acute respiratory failure with viral bronchiolitis.

Airway reactivity and the effects of bronchodilators in infants are controversial. We studied the response to bronchodilator treatment in 14 mechanically ventilated infants (mean age, 2.74 months; range, 0.6-5.9) in respiratory failure caused by respiratory syncytial virus (RSV)-associated bronchiolitis. Sixteen infants without lung disease, undergoing elective surgery, provided normal values. Maximum expiratory deflation flow-volume (DFV) curves were produced by manual inflation of the lungs with an anesthesia bag to a predetermined static airway pressure followed by rapid deflation with a negative airway pressure before and after administration of bronchodilator. At baseline, the bronchiolitis group had a forced vital capacity (FVC) of 34.5 +/- 3.6 ml/kg compared with 41.8 +/- 1.5 ml/kg in the normal group; maximum expiratory flow rate at 25% of FVC (MEF25) was 10.2 +/- 2.0 ml/kg/s compared with 27.3 +/- 2.0 ml/kg/s in the normal group. The clinical and radiologic impression was severe lower airway obstruction and air trapping. After administration of bronchodilator, FVC did not increase significantly, but MEF25isov increased by over 30% in 13 of 14 infants. Mean MEF25 increased by 148 +/- 43.2% to 21.7 +/- 3.9 ml/kg/s (P less than 0.02). These findings indicate that during the acute phase of severe RSV-positive bronchiolitis most infants have airway reactivity that responds positively to bronchodilator treatment.

Early onset of airway reactivity in premature infants with bronchopulmonary dysplasia.

Pulmonary function during the early development of bronchopulmonary dysplasia (BPD) in premature infants is not well understood. Furthermore, it is not known how early airway reactivity appears in BPD. During a 14-month period we studied 32 infants (mean gestational age, 27.3 wk; mean birth weight, 1.02 kg) with respiratory distress syndrome in whom BPD eventually developed. We obtained maximal expiratory flow-volume (MEFV) curves by manual inflation of the lung followed by forced deflation with a negative pressure on 64 occasions (mean postnatal age, 43.1 days). At each test MEFV curves were obtained in 3 conditions: baseline; after normal saline aerosolization with manual ventilation as a control; and after bronchodilator. Maximal expiratory flow at 25% of FVC (Vmax25) was markedly decreased at baseline and remained decreased after saline control. The FVC also was decreased in both baseline and saline control studies. After bronchodilator there was a marked (p less than 0.001) increase in Vmax25 (+214% above saline control) together with a significant (p less than 0.001) increase in FVC (+21%). Of 23 infants studied after 3 wk of postnatal age, 21 exhibited a more than 30% increase in Vmax25 above control (defined as airway reactivity). The remaining 2 infants were already receiving bronchodilator therapy. The most premature infant with demonstrable airway reactivity was 26 wk postconception, and the youngest was 12 days old. In 13 infants who were studied initially before 3 wk of age, there was a highly significant correlation (r = 0.91 p less than 0.001) between the degree of airway reactivity and the severity of respiratory disease as determined by the duration of ventilator dependence. Airway reactivity may play an important role in the development and severity of BPD.

Tidal flow measurement in the decision to decannulate the pediatric patient.

Measurements of peak inspiratory flow obtained through the tracheostomy cannula (MIFT) during tidal breathing were compared to peak inspiratory flow measurements obtained through the mouth (MIFM) in 40 children to assess physiologic readiness to decannulate the tracheostomized pediatric patient. Ratio of peak flow MIFM/MIFT was 1.40 for 34 successfully decannulated children compared to 0.83 for 22 unsuccessful attempts (p less than 0.01). Tidal flow measurements are highly predictive (84%) in identifying children who are unlikely to be ready for decannulation. A schema is proposed to utilize tidal flow measurements as the first step in the decannulation process.