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Biomedical subjects

S Godfrey

Publications and source records attributed to S Godfrey.

At least 19 recordsLinked to original sources

Nissen fundoplication in the treatment of children with familial dysautonomia.

Thirty-four children with familial dysautonomia (FD) underwent Nissen fundoplication and gastrostomy. The indications for operation were persistent cyclic vomiting that resulted in repeated aspiration pneumonia (94% of the patients), chronic dehydration (82%), failure to thrive (97%), and frequent hospitalizations (76%). There was no operative or early postoperative mortality. Long-term follow-up for up to 12 years was available. Eight patients died during this period from 7 months to 7.5 years postoperatively. In 5 patients (15% of the operated patients), the fundoplication ceased to function 16 months to 5 years postoperatively, which was attributed mainly to repeated severe dysautonomic crises with vigorous retching. Vomiting ceased in 85% of the symptomatic patients; pulmonary deterioration was halted, and the frequency of aspiration pneumonia was reduced in 68%; nutritional improvement was seen in 44%; the hydration status improved in 88%; and the frequency of hospital admissions decreased in 74%. These long-term findings resulted in a significant improvement in the quality of life for the majority of the patients. The absence of operative mortality and the low postoperative morbidity, together with the long-term beneficial results of this surgical procedure, should encourage early surgical intervention in selected FD patients.

Adolescent

Role of infection in the middle lobe syndrome in asthma.

Twenty one children with asthma aged 1.0-10.5 years (mean (SD) 3.3 (2.5) years) were admitted to the hospital to evaluate pulmonary right middle lobe or lingular collapse lasting one to 12 months (mean (SD) 4.4 (3.8) months). Seven children had mild asthma and were treated with inhaled beta 2 agonists as needed. Nine had moderate asthma treated with either sodium cromoglycate or slow release theophylline. Five had severe asthma treated with inhaled steroids. Each child underwent fibreoptic bronchoscopy under local anaesthesia and a bronchoalveolar lavage. Differential cell counts of the lavage fluid revealed predominance of neutrophils in 12 patients (57%). In nine of these patients cultures grew pathogenic bacteria, mainly Haemophilus influenzae and Streptococcus pneumoniae. There was no correlation between the severity of asthma and a positive bacterial culture. There was also no correlation between the duration of the right middle lobe collapse and a positive culture. We conclude that longstanding right middle lobe collapse in asthmatic children is often associated with bacterial infection.

Asthma

Hollow bone in the bronchus or the danger of chicken soup.

A 6-month-old infant arrived at the emergency room with severe respiratory distress after being spoon-fed chicken soup. Air entry was normal, chest X-rays were normal, and bronchoscopy revealed a hollow chicken bone lining the whole left main bronchus.

Animals

Enhancement of theophylline clearance by oral albuterol.

The possible effect of albuterol on theophylline clearance was studied in ten adult volunteers. Subjects received intravenous aminophylline loading dose (5.6 mg/kg), with oral albuterol (4 mg every 6 h), or inhaled albuterol (200 micrograms every 6 h), or alone (control). Theophylline levels were determined for 12-h periods. Theophylline clearance and elimination t 1/2 were calculated. Theophylline clearance was significantly higher when given with oral albuterol, in comparison with control (0.83 +/- 0.05 vs 0.73 +/- 0.06 ml/kg/min, p less than 0.02). Theophylline elimination t 1/2 was shorter with the coadministration of oral albuterol, compared with control (7.1 +/- 0.3 vs. 8.1 +/- 0.6 h, p less than 0.02). These alterations in theophylline clearance and elimination were greater in subjects who had lower control theophylline clearance. Theophylline clearance and elimination t 1/2 recorded with inhaled albuterol were not significantly different from control values. Coadministration of oral albuterol and theophylline resulted in enhancement of theophylline clearance, particularly in subjects with initially slow theophylline elimination. Such patients may require theophylline dosage adjustment as a result of this interaction.

Administration, Inhalation

Acquired tracheoesophageal fistula in infancy and childhood.

Acquired tracheoesophageal fistula (TEF) is a rare entity in the pediatric age group. We report two pediatric patients with acquired TEF caused by shells of pistachio nuts. In both patients the primary operation did not resolve the problem and a second intervention for recurrent fistula was needed. The special nature of acquired TEF, particularly the one described herein, requires delayed surgical intervention and meticulous separation of the respiratory and alimentary tracts by an intercostal muscle flap.

Child, Preschool

Assessment and treatment of childhood asthma.

In June 1990 a meeting of Paediatric Respiratory Physicians was held near Adelaide. The guidelines for assessing and treating asthma as prepared by the Thoracic Society of Australia and New Zealand for the management of patients with asthma was considered. Although not explicitly stated, these guidelines were designed for adult asthmatics. There was complete agreement that a companion statement was needed to take into account differences between the management of children and adults. This document fulfils that role. Further recommendations are found in a statement prepared by Australian and New Zealand respiratory paediatricians following a workshop in June 1989. This present statement should be read in association with the previous documents.

Asthma

Bronchial provocation determined by breath sounds compared with lung function.

Bronchial provocation testing with methacholine was undertaken in 15 children aged 5 to 8 years with obstructive lung disease, mostly asthma (13/15). The methacholine was inhaled during two minutes of tidal breathing in increasing concentrations. After each inhalation, lung function was measured and clinical signs recorded independently by two observers unaware of each other's results. The logarithm of the concentration of methacholine which caused wheezing over the trachea correlated closely with the logarithm of the concentration of methacholine causing a 20% fall in the forced expiratory volume in one second (FEV1) but was 52% greater on average. At the end of the test there was a mean (SD) fall in FEV1 of 33.3 (7.4)% and a fall in oxygen saturation of 5.2 (3.1)%. Bronchial provocation testing by listening for wheeze over the trachea is a safe technique, which correlates with objective measures of lung function in young children.

Bronchi

Exercise but not methacholine differentiates asthma from chronic lung disease in children.

Bronchial provocation challenges with exercise and methacholine were performed on the same day or within a short interval in 52 children with asthma, 22 with other types of chronic lung disease (including cystic fibrosis), and 19 control subjects with no evidence of chronic lung disease. There were no significant differences in the baseline lung function before the two types of challenge in the individual groups and differences between the patients with asthma and with chronic lung disease were minor. When the mean -2 SD of the methacholine response of the control group was taken as the lower limit of normal, 49/52 (94%) patients with asthma and 18/22 (82%) with chronic lung disease responded abnormally. In contrast, with the mean +2 SD of the exercise response of the control group as the upper limit of normal, 41/52 (79%) asthmatic patients responded but none of those with chronic lung disease. Thus the response to the two types of challenge helps to distinguish asthma from other types of chronic lung disease in children.

Adolescent

Nonspecific bronchial reactivity in asthmatic children depends on severity but not on age.

Bronchial reactivity to inhaled methacholine was measured by the steady-state tidal breathing method in asthmatic children aged 1 to 17 yr. The children were divided into three clinical groups according to their minimal therapeutic requirements: mild asthma, children requiring infrequent treatment with inhaled beta-agonists (81 patients); moderate asthma, children requiring daily preventive treatment with either cromolyn sodium or slow-release theophylline (67 patients); and severe asthma, children requiring daily preventive treatment with oral or inhaled steroids (34 patients). They were also divided into three age groups: from 1 to 6 yr, tested by using bronchial provocation with tracheal auscultation (BPTA) to determine the methacholine concentration causing wheezing (PCW); and from 7 to 11 yr and 12 to 17 yr, using lung function testing to determine the concentration causing a 20% fall in FEV1 (PC20). For the whole group the mean level of bronchial reactivity to methacholine correlated inversely with the severity of bronchial asthma according to the minimal drug requirements (p less than 0.0001) and was similar over the whole age range (p less than 0.9965) for each severity grouping. In the older children the difference between moderate and severe asthma was not significant, but this may have been a result of the effect of corticosteroids in the severe group. We concluded that age has no significant effect on the methacholine response in asthmatic children over a wide age range.

Adolescent

Corticosteroids do not affect the clinical or physiological status of infants with bronchiolitis.

The treatment of infants aged 1.5-11.0 months suffering from acute bronchiolitis with a combination of inhaled albuterol and systemic corticosteroids or inhaled albuterol and placebo was compared in 50 infants in a double blind study. The mean initial clinical score and the rate of improvement was similar in the two groups. The mean +/- SD hospital stay was 5.0 +/- 1.2 days for the steroid group and 5.2 +/- 1.7 days for the placebo group. Lung function was measured in 14 infants (7 from each group) and showed evidence of increased lung volumes and severe airway obstruction in the acute stage (the mean values for the steroid group were: TGV, 31 mL/kg; SGaw, 0.104 L/s.cmH2O; VmaxFRC, 12.9 mL/s/kg; for the placebo group: TGV, 35 mL/kg; SGaw, 0.104 L/s.cmH2O; VmaxFRC, 8.5 mL/s/kg) which had improved 2-4 weeks later (steroid group: TGV, 25 mL/kg; SGaw, 0.168 L/s.cmH2O; VmaxFRC, 21.6 mL/s/kg; -placebo group: TGV, 24 mL/kg, SGaw, 0.198 L/s.cmH2O, VmaxFRC, 17.5 mL/s/kg). There were no significant differences of thoracic gas volume, specific airway conductance, and forced expiratory flow at resting lung volume between the two groups, either in the acute or convalescent stages. We conclude that corticosteroids do not change the rate of clinical improvement in acute bronchiolitis, nor do they effect lung function 2-4 weeks later.

Administration, Inhalation