Peter D. Phelan

The natural history of childhood asthma to adult life.

A randomly selected group of 331 children who had started to wheeze in childhood and a control group of 77 children were prospectively studied clinically and physiologically from 7 to 21 years of age. Most subjects improved during adolescence and about 55% of those whose wheezing had started before 7 years and stopped before adolescence remained wheeze free. Forty-five per cent of subjects who had apparently ceased to wheeze at 14 years had minor recurrences of wheezing between 14 and 21 years of age. Fewer than 20% of those with persistent symptoms in childhood had become totally wheeze free during adolescence, although there was amelioration in symptoms. Girls did less well during adolescence than boys, so that there was no longer an increased preponderance of boys with increasing severity of asthma. Normal growth was achieved in all grades despite the persistence of symptoms in many cases. At 21 years of age features of airways obstruction were often found during an interval phase, especially in those who had more persistent symptoms.

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. Pediatr Pulmonol. 1997; 23:14–20.

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.

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.

A randomised, placebo controlled trial of inhaled salbutamol and beclomethasone for recurrent cough

AIMS To test the hypothesis that inhaled salbutamol or beclomethasone will reduce the frequency of cough in children with recurrent cough. A secondary aim was to determine if the presence of airway hyperresponsiveness (AHR) can predict the response. DESIGN Randomised, double blind, placebo controlled trial. METHODS During a coughing phase, 43 children (age 6–17 years) with recurrent cough were randomised to receive inhaled salbutamol or placebo (phase I) for 5–7 days and then beclomethasone or placebo (phase II) for 4–5 weeks, and in a subgroup of children for 8–9 weeks. The children used an ambulatory cough meter, kept cough diaries, and performed the capsaicin cough sensitivity, hypertonic saline bronchoprovocation, and skin prick tests. RESULTS Salbutamol or beclomethasone had no effect on cough frequency or score, irrespective of the presence of AHR. CONCLUSIONS Most children with recurrent cough without other evidence of airway obstruction, do not have asthma and neither inhaled salbutamol nor beclomethasone is beneficial.

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.

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

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 gastro-oesophageal 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. CONCLUSION SPT, but not MPT, was associated with a significant increase in gastro-oesophageal reflux in infants with cystic fibrosis.

Reproductive health in young women with cystic fibrosis: Knowledge, behavior and attitudes

PURPOSE The improved life-expectancy in cystic fibrosis (CF) results in the fact that the majority of affected young women now survive to face the same reproductive health decisions as other women, in addition to those that specifically relate to CF. The aim of this study was to assess the reproductive health knowledge of women with CF, to investigate the range of their reproductive health problems, and to review their reproductive health attitudes and behaviors. METHODS Women aged 18 years and over attending CF services in the state of Victoria, Australia were invited to complete a reproductive health questionnaire. Comparison subjects (n = 76) were enrolled from 2 primary care practices. RESULTS Fifty-five women participated (89%), with a median age of 22 years (range 18-50). There was no significant difference in marital status between the two groups and a similar proportion were sexually active, yet women with CF were less likely to use contraception. A majority of women with CF believed that fertility was reduced, and there was poor knowledge of the potentially deleterious effect of pregnancy. A relatively high proportion were planning to become pregnant in the near future. Twenty-two percent had tried to conceive, with a success rate of 67%. CONCLUSIONS Women with CF are currently lacking important information about reproductive health that potentially has a major impact on their health and their lives.

The self-image of adolescents with cystic fibrosis

PURPOSE The changing expectations, therapies and outcomes for young people with cystic fibrosis (CF) necessitate a re-evaluation of the impact of this chronic illness on adolescent development. The aim of this study was to assess the psychological well-being and adjustment of contemporary adolescents with CF. METHODS Forty-nine adolescents with CF (24 males, 25 females) aged 14-18 years were enrolled from the Royal Childrens Hospital CF Clinic, Melbourne, Australia. The Offer Self-Image Questionnaire was administered to participating subjects whose growth and pubertal developmental and pulmonary function was assessed. RESULTS The mean (SD) FEV1% was 82 (21), and mean (SD) National Institute of Health clinical score was 81 (12). There were no significant differences between males and females in pulmonary function or clinical scores, but growth and pubertal development were delayed in both sexes. Females, but not males, with CF were less well-adjusted than their healthy peers. Two-way analysis of variance was performed and showed significant sex differences in sub-scales of the Offer questionnaire numbers I, II, III, VI, VIII, IX, as well as in the total score. Age was significant only for scales II and VIII, and only scale II displayed an interaction between sex and age. CONCLUSIONS This study suggests that adjustment and self-esteem are less than ideal in young people with CF, especially females.

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.