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This is a prospective study of wheezing children, who were followed up from the ages of seven to 21 years. Allergy factors were documented in all subjects. Hay fever and positive reactions to skin allergy tests were more prevalent in 21-year-old subjects with severe asthma. However, 50% of subjects who had ceased wheezing has hay fever, and 65% had at least one positive skin test reaction. The number of asthmatic subjects with hay fever and positive skin test reactions progressively increased between the ages of seven and 21 years. Eczema was significantly associated only with severe asthma. More 21-year-old subjects with severe asthma wee breast-fed in infancy for longer periods than in the control group and in groups with mild to moderate asthma. Further investigation is needed before the hypothesis that breast feeding protects against the development of allergic phenomena can be accepted.

A single-blind crossover trial comparing beclomethasone dipropionate by inhalation with prednisolone by mouth in the treatment of asthmatic children over a period of 10 months is reported. Fourteen children aged between nine and 16 years entered the trial. All had severe chronic asthma requiring maintenance oral prednisolone therapy in doses ranging from 2 to 7-5 mg/day. Beclomethasone dipropionate given by inhalation in a dose of 400 mug/day was found to be a satisfactory alternative to prednisolone by mouth for controlling the symptoms of asthma. During the course of this trial, three of the children died suddenly during acute exacerbations of asthma. All three had evidence of extensive inflammation of the tracheobronchial tree.

Twenty children, aged 8 to 17 years, with bronchial asthma were each given 0.02ml/kg, 0.03ml/kg and 0.04ml/kg terbutaline respirator solution (10mg/ml), one dose at a time on three separate occasions. Terbutaline by nebuliser produced a bronchodilator response within five minutes which reached near peak levels by 15 minutes. There was a wide variation in response. A dose of 0.02ml/kg produce a mean increase in FEV1 of 55 percent and an increase in MMEFR of 121 percent. Doses of 0.03ml/kg and 0.04ml/kg given to the same children resulted in slightly better, but not statistically significant different responses. The effect lasted for four hours, although the MMEFR was falling at this time. The baseline FEV1 did not affect the bronchodilator response. An inhalation of 0.02ml/kg of terbutaline respirator solution (10mg/ml) will produce an adequate bronchodilator response in most children.

Lung function was studied in 20 children, aged 8-17 years, who had successfully repaired oesophageal atresia with tracheo-oesophageal fistula. Spirometry and plethysmography showed mild restrictive lung disease. Airways obstruction was assessed using maximal expiratory and inspiratory flow-volume loops in air and after breathing a helium-oxygen mixture. Analysis of the flow-volume data demonstrated significant obstruction at the level of the trachea and little evidence of small airways disease. Bronchial hyperreactivity, as assessed by histamine challenge, was present in 22% of subjects. Respiratory and oesophageal symptoms were common, but decreased in frequency with increasing age. The mechanisms involved in the frequent respiratory symptoms these children suffer are discussed in the light of the physiological findings.

Measurements of maximum expiratory flows and lung volumes were made on 6 occasions at weekly intervals in 14 children who were known over several years to have severe chronic asthma. 11 of the 14 had persistent lung hyperinflation and marked reduction in maximum expiratory flow rates although there was considerable variability in individual measurements. The other 3 usually had hyperinflation and reduced expiratory flow rates. 3 times daily measurements at home of peak expiratory flow rate did not contribute further to assessment. The results of the study indicated that one or two measurements of maximum expiratory flow calculated from a maximum expiratory flow volume curve and of lung volumes recorded in a body plethysmograph are of value in identifying the child with severe chronic asthma.

Data on 622 patients with cystic fibrosis born in Victoria, Australia from 1955 to 1980 and on 344 surviving patients in the care of a specialist clinic on June 30, 1983 were analyzed for factors associated with better survival and a less rapid progress of lung disease. Presentation with predominantly gastrointestinal symptoms, other than meconium ileus, was associated with an improved prognosis, whereas presentation with predominantly respiratory symptoms was associated with a worse prognosis. While infants diagnosed before the age of 6 months as a result of routine testing because of a family history of the disease seemed to have less rapid progress of lung disease, their ultimate survival did not seem to be better than that of patients presenting symptomatically after the newborn period. The sex of the patients did not appear to have prognostic significance. Age at diagnosis did not affect rate of progress of lung disease or survival when infants dying within 6 months of birth were excluded. There was a close association between the extent of lung disease at diagnosis and current lung disease. Failure to reverse extensive disease at diagnosis or deterioration of lung disease in the first year after diagnosis was associated with a less favourable course.

The effect of nebulised fenoterol was compared with that of a similar dose administered by metered aerosol in 14 children, aged 7 to 17 years with moderately severe asthma. The initial response to fenoterol delivered by metered aerosol or nebuliser was the same, but a second dose by nebuliser after a dose by metered aerosol produced maximum potential bronchodilatation which was not seen when a second dose by metered aerosol was given after that by nebuliser. Administration of a bronchodilator by nebuliser does seem advantageous in the treatment of some children.

Respiratory syncytial virus and parainfluenza viruses are the major pathogens in acute lower respiratory infection in infants and younger children. They show distinct seasonal patterns. An annual epidemic of respiratory syncytial virus infection is seen in Melbourne and this coincides with the coldest months of the year. Parainfluenza virus Type 1, the most frequent cause of laryngotracheobronchitis, occurs as an autumn epidemic every second year. Parainfluenza virus Types 2 and 3 are present most years and do not show a clear seasonal pattern.

The survival of 320 patients, who were born in and after 1958 with cystic fibrosis and managed by the Departments of Thoracic Medicine and Gastroenterology, Royal Children's Hospital, Melbourne, is reviewed. Eighty per cent of patients survived to 11 years of age, and 64% to 18 years. Of patients managed between 1973 and 1977, 91% survived to 12 years of age, and 80% to 17 years of age. In the same period, 79% of patients survived for 16 years after diagnosis. Forty-four per cent of the 240 patients currently being managed have no significant permanent lung disease, and only 9% have advanced disease.

A case is reported of Rendu-Osler-Weber syndrome presenting in a 9 year old girl as recurrent haemoptysis. Despite a normal chest X-ray, the patient was found to have multiple areas of angiodysplasia in the major airways of her right lung.

Mycoplasma pneumonia usually follows a benign course and the patient does not require hospitalization. The present report summarizes the feature of eight children admitted for a moderately severe pneumonia during an epidemic of Mycoplasma pneumoniae in Victoria. All children were previously healthy. The usual presenting symptoms included cough, fever, lethargy, and weight loss. All children had moderately severe respiratory distress and physical signs in the chest consistent with extensive parenchymal involvement. Half of this group had radiological evidence of a small pleural effusion. Complement fixation titres for Mycoplasma pneumoniae in paired samples confirmed the diagnosis. Clinical and radiological resolution was complete after one to three months. It is suggested that severe mycoplasma pneumonia may be more common than previously appreciated.

The importance of bronchial hyper-reactivity and allergy, and treatment with bronchodilators and corticosteroids, in the management of patients with cystic fibrosis is poorly understood. Three tests generally regarded as useful in the diagnosis of asthma were evaluated in 25 children with cystic fibrosis. The constancy of a child's response was assessed by histamine bronchial provocation, exercise challenge, and tests of skin allergy during a 6-month period. Although a positive response to these tests was related to impaired pulmonary function, 44% of children had variable responses to histamine, 56% to exercise, and 24% to skin tests which were unrelated to exacerbations of chest infection or to changes in pulmonary function. These results show the complex nature of airways hyper-reactivity and allergy in cystic fibrosis, and suggest that 'anti-asthma' therapy is not justified solely on the basis of one positive response to these tests.

The clinical and pathological features of five children who died of asthma over a recent 12-month period are reported. All had severe, chronic asthma requiring maintenance corticosteroid therapy. Three had been receiving beclomethasone dipropionate by inhalation and these had acute inflammation of the tracheobronchial tree at necropsy. Adrenal atrophy was found in all four cases examined histologically, despite normal short tetracosactrin tests in three of these shortly before they died. The need for high-dose corticosteroid by mouth for exacerbations of asthma in those weaned from oral steroids is emphasized by these deaths. The introduction of beclomethasone dipropionate by inhalation has led to an increase in the number of children in this high-risk group.

During the last decade, many new methods of assessing pulmonary function in infants have been introduced. However, considerable controversy has arisen when these different techniques are used to assess the efficacy of various inhaled medications. The currently employed measures of pulmonary function have been reported to have coefficients of variation of less than 10%. However, these figures refer to repeated measurements made in individual babies over a short period of time, with the infant in a constant position. They may not be applicable to studies on the effects of drugs when a significant time interval elapses between measurements, particularly if the infant is repositioned during this time. In the present study, we found that the variability of measurements of thoracic gas volume (TGV), passive respiratory mechanics, and forced expiration approximately doubled if the infant was taken out of the plethysmograph, or repositioned within the plethysmograph, between sets of measurements. The between-test coefficients of variation ranged from 4.8% to 18.9% for TGV, 7.8% to 23.5% for respiratory system resistance, from 5.0% to 24.1% for respiratory system compliance, and from 10.8% to 36.1% for maximal flow at functional residual capacity. These results demonstrate that the commonly employed infant pulmonary function tests have wide variability in infants with acute wheezing illnesses. Before studying the efficacy of therapeutic interventions in such infants, it is necessary to establish the individual variability of the tests in the infants under the conditions of the study. Failure to do so may result in erroneous conclusions and recommendations.

A recurrence rate for meconium ileus of 39% was found among C.F. affected siblings in a study of 488 families with at least one C.F. child born over a 24 year period. The recurrence rate was highly significant and indicated a familial trend for the occurrence of meconium ileus. The findings of the study support the existence of genetic heterogeneity in cystic fibrosis.

From a cohort of 543 healthy children and adolescents, subjects were studied annually to obtain longitudinal data so that precise predictive values for lung volumes and flows from ages 8 to 19 years could be obtained. Strict guidelines for subject selection and pulmonary function testing were used. These data are presented in a readily usable form as equations, graphs, and tables.

A group of 323 subjects who had wheezed in childhood and 48 control subjects of the same age were studied prospectively from 7 to 28 years of age. A classification system based on wheezing frequency was found to correlate well with clinical and spirometric features of airway obstruction. The amount of wheezing in early adolescence seemed to be a guide for severity in later life with 73% of those with few symptoms at 14 continuing to have little or no asthma at 28 years. Similarly 68% of those with frequent wheezing at 14 still suffered from recurrent asthma at 28 years. Most subjects with frequent wheezing at 21 continued to have comparable asthma at 28 years. Of those with infrequent wheezing at 21, 44% had worsened at 28 years. Women fared better than men between 21 and 28 with 19% having worse symptoms compared with 28% of men. Treatment at all ages was generally inadequate. The number of smokers among those with asthma was of concern.

Height and armspan were measured in healthy 8-18 year old boys and girls to assess the appropriate correction factor to be applied when height cannot be measured easily. No correction factor was found necessary, height being directly estimated from armspan.

The use of bracing in the treatment of mild idiopathic scoliosis is controversial. A study of 33 adolescents showed that bracing significantly decreased lung volumes. Functional residual capacity was reduced by a mean of 26%, 18% of children showing a reduction of greater than 40%. The mean reduction in total lung capacity was 16% and in forced vital capacity 18%. This restriction of lung function by bracing might have a deleterious effect on lung growth or might impose an additional risk factor in the presence of other disorders, such as asthma and diaphragmatic weakness. The use of bracing in individuals with mild scoliosis should be judiciously reassessed.

The effect of nebulized ipratropium bromide in 14 infants (mean age: 20 weeks, range: 4-41) with acute respiratory syncytial virus bronchiolitis was examined. A modified rapid chest compression technique was used to obtain partial expiratory flow-volume curves and maximum flow at functional residual capacity. Passive respiratory mechanics were assessed by brief occlusion at end inspiration. Thoracic gas volume was measured in a body plethysmograph. No significant difference was found in forced and passive respiratory mechanics pre- and post-ipratropium bromide. No subgroups could be identified. These results do not support the use of ipratropium bromide in acute viral bronchiolitis.

Chest wall deformities developed after thoracotomy for esophageal atresia, in 77 of 232 patients (33%) who did not have a congenital vertebral anomaly. Anterior chest wall asymmetry was present in 47, scoliosis in 18 and a combination of both in 12 patients. Scoliosis was convex away from the incision in two thirds of those affected. Anterior chest wall deformity was more common in patients greater than 25 years of age, and scoliosis was more common in patients who had had multiple thoracotomies. Breast surgery to minimize inequality was required in three female patients, and spinal surgery in one patient. Twenty-two of 53 patients with a congenital vertebral anomaly developed scoliosis, eight of whom required surgery. The scoliosis was probably the result of the vertebral anomaly in these patients, who are particularly at risk for progressive deformity.

In a material of 22 Danish, 26 Canadian, 10 Australian, 5 English and 5 American families with at least 2 children affected with cystic fibrosis (CF) a combined positive LOD score of 3.46 was found for the relationship cystic fibrosis-paraoxonase (PON) at recombination fraction theta = 0.07 in males and theta = 0.13 in females. Assuming a three allele model for PON the LOD score was 4.50 at the same recombination fractions. This confirms our earlier finding of an indication of CF-PON synteny.

An age-matched group of 247 subjects who had had asthma as children and 39 control subjects were studied with lung function and bronchial reactivity testing at the age of 28 years. Lung function of those who had ceased wheezing was essentially normal, but lung function was increasingly abnormal in those who continued to wheeze. Those with frequent wheezing at 28 demonstrated a greater decline in their respiratory function since the age of 7 years than the other groups. Methacholine provocation testing was performed in 91% of the group and was associated with frequency of wheezing and evidence of small airways obstruction. No association was found between reactivity and loss of ventilatory capacity since 21 years of age.

Respiratory function studies were carried out in 22 infants who had successful repair of diaphragmatic herniae of the Bochdalek type. Thoracic gas volume was initially reduced in only 3 of these, but subsequent studies showed that improvement occurred. There were no consistent abnormalities in either dynamic compliance or mean pulmonary conductance. This is evidence that there is rapid adaptation which compensates for any alteration in the parenchymatous tissue in the lungs or abnormalities in the bronchial tree in infants soon after the repair of congenital diaphragmetic herniae. Further studies are necessary to determine the changes in these lungs with growth.

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.

During a 12-month period, 527 consecutive admissions for laryngotracheobronchitis (LTB) were reviewed to determine the epidemiology of hospitalized LTB patients and to better define patients who may benefit from therapy other than close observation. Viral cultures were obtained in 442 patients and were positive in 70%. Disease severity at the time of admission was unrelated to patient age or sex. Duration of hospitalization, however, was inversely related to age (p less than 0.001). Laboratory investigations were rarely abnormal or of therapeutic value. Patients who on admission had stridor without sternal and chest wall retractions recovered rapidly and spontaneously; they were frequently discharged within 48 hours and never required artificial airways. Children who had sternal and chest wall retractions on admission experienced longer hospitalizations, frequently received medical intervention such as aqueous mist therapy or racemic epinephrine, and had a 6% risk of requiring artificial airway support. This group of children should be studied selectively for the benefits of specific medical therapies and diagnostic evaluations.

Twenty children who had endoscopically confirmed laryngomalacia were reviewed at 8 to 16 years of age. All had developed stridor in the first 6 weeks of life; stridor had stopped or largely resolved by age 4 years. Proximal airway function was assessed by expiratory and inspiratory flow volume loops and compared with control values. These children as a group had variable extrathoracic obstruction to inspiratory airflow, as indicated by a significantly low maximal inspiratory flow at 50% of vital capacity and a high mean ratio of maximal expiratory to maximal inspiratory flow at 50% vital capacity. Although limitation to inspiratory airflow was detectable in later childhood, it was not associated with troublesome symptoms. Significant stridor always resolved by 4 years of age, but some children experienced minor stridor under stress in later childhood.

We attempted to measure the distribution of ventilation in 24 children who had cystic fibrosis (CF) and 58 control subjects (ages 3.9-6.8 years) using the multiple breath nitrogen washout (MBNW) test. Moment analysis was performed on the MBNW curve, and the first and second moment ratios were derived. Only 40 (49%) of the children were able to complete the test satisfactorily and success was related to age (P less than 0.01). Children who had CF had higher moment ratios, both first and second, than did controls (P less than 0.001); also, CF subjects who had more severe symptoms had higher moment ratios than did those who had minimal symptoms (P less than 0.005). The first moment ratio had a lower coefficient of variation than did the second (14% vs 31%) and, in 8 children who performed the test in duplicate, it also had a lower index of intrasubject variability (6.4% vs 15.1%). Both ratios were lower than those described in older children and in adults, which is consistent with the more rapid washout seen in this age group. Although moment analysis of the MBNW curve can be performed in approximately 50% of children 4 to 6-years-old, the complexity of the test, the apparatus, and the analysis and the high failure rate in children under the age of 6 are likely to restrict its use to research studies in specialized pediatric respiratory laboratories.