Agreement of aspiration tests using barium videofluoroscopy, salivagram, and milk scan in children with cerebral palsy.

To study the agreement between three tests for aspiration, barium videofluoroscopy, salivagram, and milk scan we studied 63 children with severe non-ambulant spastic quadriplegic cerebral palsy (CP) aged 14 months to 16 years (32 males, 31 females). The salivagram was most frequently positive (56%, 95% confidence interval 43 to 68%); the next most frequently positive was barium videofluoroscopy when aspiration was defined as the presence of either laryngeal penetration of material or frank aspiration (39%, 95% confidence interval 26 to 53%). The milk scan was rarely positive (6%, 95% confidence interval 2 to 16%). Agreement between the tests of aspiration was poor. The maximum agreement (kappa=0.20) was between aspiration as diagnosed with the salivagram and by barium videofluoroscopy. Positive tests for aspiration are frequent in children with severe CP. Frequency varies widely depending on the investigation used. There is poor agreement between tests used for the diagnosis of aspiration. This information is of importance in assessing the significance of test results.

Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis.

BACKGROUND: Postural drainage chest physiotherapy in infants with cystic fibrosis (CF) exacerbates gastro-oesophageal reflux (GOR) and may contribute to a more rapid deterioration in lung function. AIMS: To compare standard postural drainage chest physiotherapy (SPT) and a modified physiotherapy regimen (MPT) without head-down tilt, with regard to GOR, arousal state, and cardiorespiratory function. METHODS: Twenty infants with CF underwent 30 hour oesophageal pH monitoring, during which four chest physiotherapy sessions were administered (day 1: MPT-SPT; day 2: SPT-MPT). Arousal state, heart rate, and oxygen saturation were documented for each of the physiotherapy positions (supine, prone, right lateral, and left lateral with (SPT) or without (MPT) 30 degrees head-down tilt). RESULTS: Significantly more reflux episodes occurred during SPT than during MPT, but there were no significant differences in median episode duration or fractional reflux time. During SPT, left lateral positioning was associated with fewer reflux episodes compared to other positions. During supine and prone positioning, more reflux episodes occurred during SPT than during MPT. Infants were significantly more likely to be awake or cry during SPT. There was a significant association between crying and reflux episodes for SPT. Non-nutritive sucking was associated with a significant reduction in reflux episodes during SPT. Oxygen saturation during SPT was significantly lower during crying and other waking, and non-nutritive sucking during SPT was associated with a significant increase in oxygen saturation. CONCLUSIONS: SPT is associated with GOR, distressed behaviour, and lower oxygen saturation.

Pseudomonas cross-infection from cystic fibrosis patients to non-cystic fibrosis patients: implications for inpatient care of respiratory patients.

A 14-year-old boy with bronchiectasis secondary to chronic aspiration developed multiresistant Pseudomonas aeruginosa lower respiratory disease following several inpatient periods where accommodation and physiotherapy services were shared with cystic fibrosis (CF) patients known to be infected with the genetically identical strain of P. aeruginosa. Cross-infection with P. aeruginosa between CF patients and non-CF patients has not previously been described, and this finding raises significant issues relevant to the treatment of patients with non-CF suppurative lung disease.

Early airway infection, inflammation, and lung function in cystic fibrosis.

AIMS: To determine the relation between lower airway infection and inflammation, respiratory symptoms, and lung function in infants and young children with cystic fibrosis (CF). METHODS: A prospective study of children with CF aged younger than 3 years, diagnosed by a newborn screening programme. All were clinically stable and had testing as outpatients. Subjects underwent bronchial lavage (BL) and lung function testing by the raised volume rapid thoracoabdominal compression technique under general anaesthesia. BL fluid was cultured and analysed for neutrophil count, interleukin 8, and neutrophil elastase. Lung function was assessed by forced expiratory volume in 0.5, 0.75, and 1 second. RESULTS: Thirty six children with CF were tested on 54 occasions. Lower airway infection shown by BL was associated with a 10% reduction in FEV(0.5) compared with subjects without infection. No relation was identified between airway inflammation and lung function. Daily moist cough within the week before testing was reported on 20/54 occasions, but in only seven (35%) was infection detected. Independent of either infection status or airway inflammation, those with daily cough had lower lung function than those without respiratory symptoms at the time of BL (mean adjusted FEV(0.5) 195 ml and 236 ml respectively). CONCLUSIONS: In young children with CF, both respiratory symptoms and airway infection have independent, additive effects on lung function, unrelated to airway inflammation. Further studies are needed to understand the mechanisms of airway obstruction in these young patients.

Multiple cavitating pulmonary nodules and clubbing in a 12-year-old girl.

We report on a 12-year-old girl with a prolonged history of cough and hemoptysis on three occasions. X-ray and computed tomography of the chest showed several cavitating lesions and mediastinal lymphadenopathy. Lung biopsy revealed nodular sclerosing Hodgkin's disease. Hodgkin's disease should be considered in the differential diagnosis of cavitating pulmonary lesions.

Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis.

OBJECTIVE: To determine the clinical consequences of acquiring Pseudomonas aeruginosa infection during early childhood in children with cystic fibrosis (CF). DESIGN: Prospective, observational cohort study of 56 children with CF identified by newborn screening during 1990-92. Each child underwent an annual bronchial lavage during the first 2 to 3 years of life. Clinical outcome was determined at 7 years of age. RESULTS: P aeruginosa infection was diagnosed in 24 (43%) cohort subjects. Four children died before 7 years of age, all of whom had been infected with a multi-resistant, mucoid strain of P aeruginosa (P =.04). In survivors, P aeruginosa infection was associated with significantly increased morbidity as measured by lower National Institutes of Health scores, greater variability in lung function, increased time in the hospital, and higher rates of recombinant human deoxyribonuclease therapy (P <.01). In this young CF cohort, best forced expiratory volume in 1 second was an insensitive measure of increased morbidity. CONCLUSIONS: Acquisition of P aeruginosa was common by 7 years of age in this CF birth cohort and was associated with increased morbidity and mortality. An improved disease severity score would improve the evaluation and study of early CF lung disease.

Interlobar differences in bronchoalveolar lavage fluid from children with cystic fibrosis.

Bronchoalveolar lavage (BAL) performed in specialist centres has improved the understanding of infant cystic fibrosis (CF) lung disease. As most researchers sample from a single lobe, it was determined whether BAL results could be generalized to other lung segments. Thirty-three CF children, aged 1.5-57 months, underwent in random order sequential BAL of their right middle and lingula lobes. Specimens from each lobe had separate quantitative bacteriology, cytology and cytokine analysis. Bacterial counts > or = 1 x 10(5) colony forming units (cfu) x mL(-1) were observed in nine (27%) subjects, including six involving only the right middle lobe. These six children had similar inflammatory indices in their right middle and lingula lobes, and interleukin (IL)-8 concentrations in the latter were significantly higher than that observed within the lingula lobes of the 24 CF children with bacterial counts < 1 x 10(5) cfu x mL(-1). Lingula neutrophil and IL-8 levels correlated best with right middle lobe bacteria numbers. This observational study in cystic fibrosis children suggests that while inflammation is detected in both lungs, bacterial distribution may be more inhomogeneous. Bronchoalveolar lavage microbiological findings from a single lobe may therefore, not be generalized to other lung segments. When performing bronchoalveolar lavage in cystic fibrosis children, it is important to sample from multiple sites.

Birth weight <1501 g and respiratory health at age 14.

AIMS: To determine the respiratory health in adolescence of children of birth weight <1501 g, and to compare the results with normal birthweight controls. METHODS: Prospective cohort study of children born in the Royal Women's Hospital, Melbourne. Two cohorts of preterm children (86 consecutive survivors 500-999 g birth weight, and 124 consecutive survivors 1000-1500 g birth weight) and a control group of 60 randomly selected children >2499 g birth weight were studied. Children were assessed at 14 years of age. A paediatrician determined the clinical respiratory status. Lung function was measured according to standard guidelines. RESULTS: Of 180 preterm children seen at age 14, 42 (23%) had bronchopulmonary dysplasia (BPD) in the newborn period. Readmission to hospital for respiratory ill health was infrequent in all groups and the rates of asthma were similar (15% in the 500-999 g birth weight group, 21% in the 1000-1500 g birth weight group, 21% in controls; 19% BPD, 18% no BPD). Overall, lung function was mostly within the normal range for all cohorts; few children had lung function abnormalities in clinically significant ranges. However, the preterm children had significantly lower values for variables reflecting flow. Lung function in children of 500-999 g birth weight was similar to children of 1000-1500 g birth weight. Preterm children with BPD had significantly lower values for variables reflecting flow than children without BPD. CONCLUSIONS: The respiratory health of children of birth weight <1501 g at 14 years of age is comparable to that of term controls.

Association between allergy and asthma from childhood to middle adulthood in an Australian cohort study.

A cohort of 378 asthmatic children was studied from 7 to 35 yr of age at 7-yr intervals. On selection for inclusion in the study sample, the children had a wide range of severity of wheezing. At each 7-yr review, asthma severity, the presence of eczema or hay fever, and skin test reactivity to house dust mite or rye grass were recorded by questionnaire or clinical interview. We report on the course of asthma and these atopic conditions over the study period and discuss associations between the two phenomena. The presence of an atopic condition in childhood was found to increase the odds of more severe asthma in later life (odds ratio [OR] = 1.66, 95% confidence interval [CI]: 1.17 to 2.36 in the case of eczema; OR = 1. 39, 95% CI: 1.00 to 1.92 for hay fever; and OR = 2.25, 95% CI: 1.49 to 3.39 for skin test reactivity). Additionally, the odds of eczema and hay fever in later life increased with severity of asthma in childhood. The findings of this study provide substantially new quantitative information on the extent of association between asthma and atopic conditions from childhood into middle adulthood.

Antenatal corticosteroids and outcome at 14 years of age in children with birth weight less than 1501 grams.

OBJECTIVE: To determine whether exposure to antenatal corticosteroid therapy was associated with adverse effects on growth, sensorineural outcome, or lung function of children of birth weight <1501 g at 14 years of age. DESIGN: Cohort study. SETTING: The Royal Women's Hospital, Melbourne, Australia. SUBJECTS: One hundred fifty-four consecutive survivors born from October 1, 1980 to March 31, 1982. INTERVENTIONS: The mothers of 78 survivors (51%) had been given corticosteroids antenatally to accelerate fetal lung maturation. Treatment with antenatal corticosteroids was nonrandom. No mother received >1 course of corticosteroids. OUTCOME MEASURES: The children were assessed at 14 years of age, corrected for prematurity. All assessors were unaware of the exposure of the child to antenatal corticosteroids. The assessments included measurements of growth and neurological, cognitive, and lung function. Growth measurements were converted into z scores (standard deviation) for the appropriate age and gender. RESULTS: Of the 154 survivors, 130 (84%) were assessed at 14 years of age. Overall, the children exposed to antenatal corticosteroids were significantly taller (height z score; mean difference:.39; 95% confidence interval:.001-. 79) and had better cognitive functioning (Wechsler Intelligence Scale for Children-Third Edition Full Scale; IQ mean difference: 6. 2; 95% confidence interval:.8-11.6) than those not exposed to corticosteroids. There were no other differences in sensorineural outcomes between the groups. Lung function was not significantly different between the groups. No conclusions were altered by adjustment for confounding variables. CONCLUSIONS: Exposure to 1 course of antenatal corticosteroid therapy was associated with some clinically and statistically improved outcomes at 14 years of age in children of birth weight <1501 g, with no obvious adverse effects on growth or on sensorineural, cognitive, or lung function. corticosteroids, growth, cognitive, IQ, lung function, adolescence.

Changes in lung function between age 8 and 14 years in children with birth weight of less than 1,501 g.

We set out to determine whether lung function of children with a birth weight of <1,501 g changed relative to expectations between the ages of 8 and 14 years. We hypothesized that changes in lung function may differ between those of birth weight above and below 1,000 g. The subjects of this study were born in the Royal Women's Hospital, Melbourne. There were 86 consecutive survivors with birth weights <1,000 g born between January 1, 1977 and March 31, 1982, and 124 consecutive survivors with birth weights 1,000-1,500 g born between October 1, 1980 and March 31, 1982. Lung function was measured at both age 8 and 14 years, corrected for prematurity in 78% (67/86) of those with birth weight <1,000 g, and in 69% (86/124) of those with birth weight 1,000-1,500 g. Overall, lung function was similar to predicted values at both 8 and 14 years of age [e.g., (forced expired volume in 1 s, FEV1% predicted) at age 8 years mean 88.5% (SD 14.7) and at age 14 years, mean 94.9% (SD 13.8)]. There were significant changes, mostly improvements, in lung function between age 8-14 years relative to predicted values: FEV1 (% predicted) increased between 8-14 years of age by a mean of 6.4 (95% confidence interval, 4.4-8.3). The improvements in some lung function variables were significantly greater in those of birth weight <1,000 g compared with those of birth weight 1,000-1,500 g: improvement in FEV1 (% predicted) between age 8-14 years in infants with birth weight <1,000 g had a mean of 10.3 (SD 13.1), and in those with birthweight 1,000-1,500 g a mean of 3.3 (SD 10.1). We conclude that lung function improved significantly relative to predicted values in children of birth weight <1,501 g between age 8-14 years. The improvements were greatest in those of birth weight <1,000 g.

Preventable factors in hospital admissions for asthma.

Asthma remains the second most common cause for admissions to a paediatric hospital bed. The aim of this study was to describe the characteristics of children admitted to hospital with an acute asthma attack and to identify factors that may prevent future hospital admissions. Parents of all children aged 3 to 15 years admitted to hospital for acute asthma were interviewed and the child's case record reviewed. Children were recruited consecutively in two groups: 141 summer/autumn and 125 winter/spring 1996. According to the pattern of symptoms in the previous 12 months, 61% of the children had infrequent episodic asthma, 26% had frequent episodic asthma, and 13% persistent asthma. Only 8% of children aged 8 years or less had persistent asthma, in contrast to 22% of those aged > 8 years. There was evidence of both inadequate prescription of preventive treatment and poor compliance in the frequent episodic and persistent asthma groups. Of the whole group, 44% had previously been given an acute asthma management plan, but only 9% of them used it before the current hospital admission. There was a delay in seeking medical advice (> 24 hours after the onset of symptoms) in 27% of all admissions. This study has identified potential areas where intervention may reduce the number of future admissions.

Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis.

AIM: To establish the incidence of pathological gastro-oesophageal reflux (GOR) in newly diagnosed infants with cystic fibrosis and to identify clinical predictors of increased reflux. METHODS: 26 infants with cystic fibrosis less than 6 months of age (14 male, 12 female; mean (SEM) age 2.1 (0.21) months, range 0.8 to 5.6 months) underwent prolonged oesophageal pH monitoring (mean duration 27.1 (0.49) hours; range 21.3 to 30.2 hours). Reflux symptoms, anthropometric variables, pancreatic status, meconium ileus, genotype, and chest x ray findings were correlated with pH monitoring data. RESULTS: Five infants (19.2%) had an abnormal fractional reflux time of greater than 10%, seven (26.9%) of 5-10%, and 14 (53.8%) of below 5%. Infants who presented with frequent vomiting had a significantly higher fractional reflux time than infants who had infrequent or no vomiting. There was no significant association between abnormal chest x rays and pathological GOR. Sex, genotype, nutritional status, meconium ileus, and pancreatic enzyme supplementation were not significantly associated with pathological GOR. CONCLUSIONS: About one in five newly diagnosed infants with cystic fibrosis had pathological GOR. Pathologically increased reflux was present before radiological lung disease was established. Apart from frequent vomiting, no useful clinical predictors of pathological reflux were found.

Severe viral respiratory infections in infants with cystic fibrosis.

Limited data in children with cystic fibrosis (CF) suggest that respiratory viral infections during infancy result in substantial morbidity. Eighty of 101 (79%) infants with CF diagnosed by neonatal screening during 1991-1996 were recruited into a prospective, multiple-birth cohort study. We aimed to perform an initial, then annual bronchoalveolar lavage (BAL) for bacterial and viral culture, cytology, IL-8, and elastolytic activity over the following 2 years. When possible, BAL was also performed during any hospitalization for a pulmonary exacerbation, and additional specimens for viral culture were collected by nasopharyngeal aspiration. Thirteen infants undergoing bronchoscopy for congenital stridor served as disease controls. During infancy, 31 children (39%) were hospitalized for respiratory disease and 20 (65%) cases had an etiologic agent identified. Respiratory viruses were detected in 16/31 (52%) cases, including four with simultaneous bacterial infection. Another four were infected with Staphylococcus aureus. Respiratory syncytial virus predominated and was found in seven infants. In the absence of bacteria, those with viral infections had acute onset of respiratory distress, were not treated with antibiotics, and had an uncomplicated hospital course. Compared to noninfected CF subjects and controls, infected infants had elevated BAL inflammatory indices (P < 0.01). Eleven of 31 (35%) hospitalized infants followed for 12-60 months acquired Pseudomonas aeruginosa, compared with only three of 49 (6%) subjects not hospitalized for respiratory symptoms during infancy (risk ratio 5.8, CI 1.9, 24). We conclude that respiratory viruses are important causes of hospitalization in CF infants. While viral infections were self-limited, they were accompanied by airway inflammatory changes, and admission to hospital was associated with early acquisition of Pseudomonas aeruginosa and persistent respiratory symptoms.

Lower airway inflammation in infants and young children with cystic fibrosis.

Airway inflammation is an important component of cystic fibrosis (CF) lung disease. To determine whether this begins early in the illness, before the onset of infection, we examined bronchoalveolar lavage (BAL) fluid from 46 newly diagnosed infants with CF under the age of 6 mo identified by a neonatal screening program. These infants were divided into three groups: 10 had not experienced respiratory symptoms or received antibiotics and pathogens were absent in their BAL fluid; 18 had clear evidence of lower respiratory viral or bacterial (> or = 10(5) CFU/ml) infection; and the remaining 18 had either respiratory symptoms, taken antibiotics, or had < 10(5) CFU/ml of respiratory pathogens. Their BAL cytology, interleukin-8, and elastolytic activity were compared with those from 13 control subjects. In a longitudinal study to assess if inflammation develops or persists in the absence of infection, the results of 56 paired annual BAL specimens from 44 CF infants were grouped according to whether they showed absence, development, clearance, or persistence of infection. In newly diagnosed infants with CF, those without infection had BAL profiles comparable with control subjects while those with a lower respiratory infection had evidence of airway inflammation. In older children, the development and persistence of infection was accompanied by increased inflammatory markers, whereas these were decreased in the absence, or with the clearance, of infection. We conclude that airway inflammation follows respiratory infection and, in young children, improves when pathogens are eradicated from the airways.

Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis.

UNLABELLED: Gastro-oesophageal reflux is increased in cystic fibrosis and it is possible that postural drainage techniques may exacerbate reflux, potentially resulting in aspiration and further impairment of pulmonary function. AIM: To evaluate the effects of physiotherapy with head down tilt (standard physiotherapy, SPT) on gastroesophageal reflux and to compare this with physiotherapy without head down tilt (modified physiotherapy, MPT). METHOD: Twenty (mean age 2.1 months) infants with cystic fibrosis underwent 30 hour oesophageal pH monitoring during which SPT and MPT were carried out for two sessions each on consecutive days. RESULTS: The number of reflux episodes per hour, but not their duration, was significantly increased during SPT compared with MPT (SPT 2.5 (0.4) v MPT 1.6 (0.3), p = 0.007) and to background (1.1 (0.)1, p = 0.0005). Fractional reflux time was also increased during SPT (11.7 (2.6)%) compared with background (6.9 (1.3)%) p = 0.03) but not compared with MPT (10.7 (2.7)%). There was no significant difference between MPT and background for number of reflux episodes, their duration, or fractional reflux time. CONCLUSIONS: SPT, but not MPT, was associated with a significant increase in gastro-oesophageal reflux in infants with cystic fibrosis.

Childhood asthma and lung function in mid-adult life.

The longitudinal lung function data in 286 subjects from a 28 year follow-up of childhood asthma is reported. Airway obstruction in mid-adult life was present mainly in those with moderately severe asthma. Subjects who had been wheeze free for at least 3 years, even if asthma had been persistent in childhood, had normal lung function and no increased bronchial reactivity. Only two subjects, both with persistent asthma from childhood, failed to show an improvement in FEV1 of greater than 10% following inhalation of a beta-adrenergic agonist. Subjects with relatively mild asthma who had not taken inhaled steroids did not appear to be disadvantaged with respect to lung function.

Bronchopulmonary dysplasia and very low birthweight: lung function at 11 years of age.

OBJECTIVE: To determine the relationship between lung function at 11 years of age and bronchopulmonary dysplasia (BPD) in very low birthweight (VLBW) children. METHODOLOGY: This study comprised 154 consecutive surviving VLBW children, divided into three groups with respect to their neonatal respiratory morbidity: group I developed BPD; group II required assisted ventilation but did not develop BPD; and group III required no assisted ventilation. Lung function tests were measured on 120/154 (77.9%) children at 11 years of age. The relationship between various lung function variables and neonatal lung disease was analysed by multiple linear regression. RESULTS: Several lung function variables reflecting airflow were significantly diminished in the BPD group (n = 15), and residual volume was significantly higher. Despite poorer lung function overall, few children in the BPD group had lung function abnormalities in the clinically significant range (n = 2[13.3%] with a forced expired volume in 1 $ < 75% predicted; n = 2[13.3%] with a forced vital capacity < 75% predicted; n = 1 [6.7%] with a residual volume/total lung capacity > 35%). There were no significant differences in lung function variables between group II (n = 41) and group III (n = 64). Changes in lung function tests between 8 and 11 years did not very significantly between the three groups. CONCLUSIONS: VLBW children with BPD in the newborn period have period have poorer lung function at 11 years of age than other surviving VLBW children without BPD, although few have lung function abnormalities in the clinically significant range.

Evaluation of bronchial responsiveness to exercise in children as an objective measure of asthma in epidemiological surveys.

BACKGROUND: Exercise has been proposed as a useful challenge test for the measurement of bronchial responsiveness in community surveys of the prevalence of childhood asthma. This study aimed to develop a standardised exercise challenge in which the sensitivity to detect asthma was increased by inhalation of dry air. METHODS: Sixty four children aged 12-13 years who had reported wheeze in the past 12 months and 70 control subjects were invited to participate in an exercise challenge at school. Subjects performed eight minutes of cycle exercise while breathing dry air at a workload calculated to produce a minute ventilation of 60% maximum voluntary ventilation during the final three minutes. A fall in forced expiratory volume in one second (FEV1) of 10% or more from baseline was considered a positive test. Data on recent asthma symptoms, asthma morbidity, and use of medication were collected by parent completed questionnaires in those subjects who reported wheeze in the past 12 months. Repeatability of the exercise test was determined in a further 13 children with known asthma. RESULTS: Fifty five children (88%) who reported wheeze in the previous 12 months and 54 control subjects (77%) were studied. Nine subjects in whom baseline FEV1 was less than 75% predicted did not perform the exercise test. Technically unsatisfactory tests were obtained in five subjects. Twenty six (57%) subjects who reported wheeze and three controls (6%) had a positive exercise test, giving a sensitivity of 57% (26 of 46) and specificity of 94% (47 of 50). Estimates of the repeatability of the exercise test showed a mean difference in percentage fall in FEV1 for patients with asthma of 3.08% (95% limits of agreement -7.76% to 13.92%). CONCLUSIONS: Despite attempts to maximise the stimulus to bronchoconstriction in this exercise challenge test, its sensitivity and specificity were not improved in comparison with previous epidemiological studies of the prevalence of asthma.