Oesophageal atresia and tracheo-oesophageal fistula.

Oesophageal atresia-tracheo-oesophageal fistula has featured in paediatric surgery since its beginnings. The first successful primary repair was in 1941. With overall survival now exceeding 90% in dedicated centres, the emphasis has changed to reducing morbidity and achieving improvements in the quality of life. An overview of current and emerging strategies in managing patients with this condition is presented. Advances in developmental biology and molecular genetics reflecting improved understanding of the pathogenesis are highlighted.

Inhaled bronchodilators for cystic fibrosis.

BACKGROUND: Recurrent wheeze and breathlessness are common in people with cystic fibrosis, and bronchodilators are commonly prescribed. Despite their wide-scale and often long-term use, there is limited objective evidence about their efficacy in cystic fibrosis. OBJECTIVES: To evaluate the effectiveness of inhaled bronchodilators in children and adults with cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic databases searches, and handsearches of relevant journals and abstract books of conference proceedings. Latest search of the Group's Trials Register: August 2005 SELECTION CRITERIA: Randomised or quasi-randomised trials comparing inhaled bronchodilators to placebo or another inhaled bronchodilator in people with CF, diagnosed clinically and by sweat or genetic testing and at all stages and severity of lung disease. DATA COLLECTION AND ANALYSIS: The authors independently extracted data and assessed trial quality. If data were missing, the primary author was contacted where possible. The data were subgrouped into classes of bronchodilator and for each class into short-term effects (less than one week) and long-term effects (greater or equal to one week). MAIN RESULTS: The search identified 43 references. Fourteen trials, with a total of 257 participants, were suitable for inclusion. The trials were all cross-over in design; in this case a meta-analysis was not possible. There were varied conclusions from the different trials, reflecting their heterogeneity. Compared to placebo, short-acting beta-2 agonists increased forced expiratory volume at one second (FEV(1)) in the short term in three out of five trials, and in the long-term increased peak expiratory flow rate in individuals who had been shown to have bronchial hyperreactivity or bronchodilator responsiveness or both. Compared to placebo, long-acting beta-2 agonists increased FEV(1) and forced expiratory flow between 25% and 75% of expiratory flow (FEF 25-75%) in the short term in participants known to have bronchodilator responsiveness, but produced inconsistent results in long-term trials. Short acting-anticholinergics had no consistent effect on lung function tests in either the short or the long term. We found no published trials of fenoterol, formoterol or tiotropium and the use of these agents in cystic fibrosis cannot be supported. AUTHORS' CONCLUSIONS: It was not possible to determine fully the effectiveness of inhaled bronchodilators in cystic fibrosis as a meta-analysis was not possible. However, both short and long-acting beta-2 agonists can be beneficial both in the short and long term in individuals with demonstrable bronchodilator responsiveness or bronchial hyperrresponsiveness.

Trends in deaths from respiratory illness in children in England and Wales from 1968 to 2000.

BACKGROUND: Childhood mortality has decreased markedly over the last three decades. A study was undertaken to determine trends in deaths from respiratory illness in children in England and Wales. METHODS: Mortality data collected by the Office for National Statistics were analysed. The data included all deaths registered from all causes in children aged between 28 days and 16 years in England and Wales from 1 January 1968 to 31 December 2000. The main outcome measures were overall and age-specific mortality rates due to all respiratory disorders and specific rates for pneumonia, asthma, cystic fibrosis (CF), and bronchiolitis. RESULTS: In children aged 1-16 years the overall mortality rate (per 100,000 children) declined from 49.9 in 1968 to 16.3 in 2000, and rates due to respiratory illness fell from 8.6 to 1.3. The proportion of all deaths caused by respiratory illness in children aged 28 days to 16 years fell from 30.8% in 1968 to 9.9% in 2000. In post-neonatal infants (aged 28-364 days), the "all cause" mortality rate fell from 592.8 in 1968 to 176 in 2000 and the rates due to respiratory illness fell from 280 to 22.8. In 2000, pneumonia, asthma and CF together accounted for 73% of all respiratory deaths in 1-16 year olds. In this age group, mortality rates per 100,000 for pneumonia fell from 4.22 to 0.57, for asthma from 0.83 to 0.25, and for CF from 0.66 to 0.12 between 1968 and 2000. Over the same period mortality rates for pneumonia in post-neonatal infants fell from 165 to 6.78 per 100,000 and for CF from 4.88 to 0.33. Bronchiolitis mortality rates per 100,000 in post-neonatal infants fell from 21.47 in 1979 to 1.82 in 2000. CONCLUSIONS: Mortality rates due to all respiratory illnesses in children have fallen markedly in the last three decades. This decline has been more rapid than the overall decline in childhood mortality and respiratory diseases are now responsible for a smaller proportion of deaths in children. These data could provide a foundation for assessing the impact on mortality of future health initiatives such as the introduction of a universal pneumococcal vaccination programme in England and Wales.

Cardio-pulmonary involvement in juvenile systemic lupus erythematosus.

Cardio-pulmonary manifestations of systemic lupus erythematosus (SLE) are well recognized in adults. We report the occurrence of clinically significant cardio-pulmonary disease in a cohort of predominantly Caucasian children with SLE. All children with SLE attending the Royal Liverpool Children's NHS Trust between 1995 and 2003 were reviewed. Of 29 children with SLE, 27 (93%) were Caucasian. Nine (31%) had cardio-respiratory complications: cardiac only (n = 1); respiratory only (n = 4); both cardiac and respiratory manifestations (n = 4). Median (range) duration of follow-up of affected children: four years (six months to 11 years). Six out of eight (75%) presented with respiratory complications before SLE was diagnosed. Three children had pericardial effusions, one requiring pericardiocentesis for tamponade. One had cardiac conduction defects and another significant pulmonary hypertension. Respiratory complications comprised: interstitial lung disease (n = 4), with two showing evidence of pulmonary fibrosis; pleural effusions (n = 2), pulmonary haemorrhage (n = 1) and lupus pneumonitis (n = 1). Disease course was complicated by CMV infection in one child. Lung biopsy was performed in five cases. Seven were treated with cyclophosphamide with significant improvement in symptoms/lung function. Of this predominantly Caucasian paediatric cohort with SLE, 31% had significant cardio-pulmonary involvement. All children with SLE should have regular monitoring of their cardio-respiratory status.

The economic impact of preschool asthma and wheeze.

The aim of the present study was to determine the economic impact in the UK of wheezing disorders in preschool children. Health, societal and family-borne costs were calculated for a sample of 94 preschool children who attended hospital with a primary diagnosis of wheeze or asthma during 1998/1999. Sample costs were calculated using data from a structured interview schedule and from symptom diaries completed by trial parents, patients' general practice and hospital records, and hospital finance data. Health costs for 1-5-yr-olds in the UK were calculated using data from a postal population survey in the same region. It is estimated that 1-5-yr-old children with wheeze in the UK cost the health service a total of 53 million UK pounds (GBP). The greatest expenditure, 34 million GBP, was for primary care, representing 65.2% of total healthcare costs. Prescription costs represented 20.4% (11 million GBP) of total healthcare costs. Caring for preschool children with wheeze in the UK cost the health service 0.15% of its total budget in 1998/1999. The total costs to society of caring for the 0.88% of preschool children who attended hospital for asthma or wheeze in a year represented a further 2.6 million UK pounds. Primary prevention strategies at the population level promise more cost savings than any attempt at decreasing hospitalisations in those more severely ill.

Parental education and guided self-management of asthma and wheezing in the pre-school child: a randomised controlled trial.

BACKGROUND: The effects on morbidity were examined of providing an educational intervention and a written guided self-management plan to the parents of pre-school children following a recent attendance at hospital for asthma or wheeze. METHODS: A prospective, randomised, partially blinded, controlled trial was designed at two secondary care centres. Over a 13 month period 200 children aged 18 months to 5 years at the time of admission to a children's ward or attendance at an accident and emergency department or children's (emergency) assessment unit (A&E/CAU) with a primary diagnosis of acute severe asthma or wheezing were recruited. 101 children were randomised into the control group and received usual care and 99 were assigned to the intervention group and received: (1) a pre-school asthma booklet; (2) a written guided self-management plan; and (3) two 20 minute structured educational sessions between a specialist respiratory nurse and the parent(s) and child. Subjects were assessed at 3, 6, and 12 months. The main outcomes were GP consultation rates, hospital re-admissions, and attendances at A&E/CAU. Secondary outcomes included disability score, caregivers' quality of life, and parental knowledge of asthma. RESULTS: There were no statistically significant differences between the two groups during the 12 month follow up period for any of the main or secondary outcome measures. CONCLUSIONS: These results do not support the hypothesis that the introduction of an educational package and a written guided self-management plan to the parents of pre-school children with asthma who had recently attended hospital for troublesome asthma or wheeze reduces morbidity over the subsequent 12 months.

Symptomatic adrenal insufficiency during inhaled corticosteroid treatment.

Symptomatic adrenal insufficiency, presenting as hypoglycaemia or poor weight gain, may occur on withdrawal of corticosteroid treatment but has not previously been reported during inhaled corticosteroid treatment. This case series illustrates the occurrence of clinically significant adrenal insufficiency in asthmatic children while patients were on inhaled corticosteroid treatment and the unexpected modes of presentation. General practitioners and paediatricians need to be aware that this unusual but acute serious complication may occur in patients treated with inhaled corticosteroids.

Prolidase deficiency and systemic lupus erythematosus.

Two children with prolidase deficiency, an inborn error of proline metabolism, developed clinical and immunological abnormalities consistent with a diagnosis of systemic lupus erythematosus (SLE). The first child died from septicaemia, and SLE was only diagnosed during his terminal illness. As a result of this diagnosis his cousin, who was already known to have prolidase deficiency, was investigated further and a diagnosis of SLE confirmed. Following treatment with oral prednisolone her clinical condition has improved, although she has a persistently raised erythrocyte sedimentation rate (ESR) and florid facial rash. Both prolidase deficiency and SLE are associated with disturbances in immune function and have clinical features in common. It is likely that prolidase deficiency is a risk factor for the development of SLE. Additionally, patients with SLE should-where there is a family history or presentation in childhood-be specifically investigated for prolidase deficiency, since standard immunological or haematological investigations will not identify the characteristic biochemical abnormalities.

Comparative epidemiology of rotavirus, subgenus F (types 40 and 41) adenovirus and astrovirus gastroenteritis in children.

We report a 5 year prospective study of episodes of rotavirus, subgenus F adenovirus, and astrovirus gastroenteritis diagnosed by electron or immune electron microscopy in a single regional virology laboratory. Of 1426 total infections, the numbers in each category were 1117 (78.3%), 254 (17.8%), and 20 (7.9%), respectively. Using restriction endonuclease analysis or immune electron microscopy, all but 20 of the subgenus F adenovirus strains were classified as type 40 (n = 50) or type 41 (n = 184). Rotavirus and astrovirus infections were more prevalent in winter than summer, whereas subgenus F (either type 40 and 41) adenoviruses showed no seasonal variation in prevalence. The ratio of type 40 to all typable subgenus F adenoviruses declined between 1984 and 1986 and then increased again. Adenoviruses were relatively more important as causes of viral gastroenteritis in infants aged less than 6 months than in toddlers aged 12 months or more, but even in young infants more rotavirus than adenovirus infections were diagnosed. Our data confirmed the epidemiological differences between rotavirus, subgenus F adenovirus and rotavirus gastroenteritis and documented the shared epidemiological characteristics of type 40 or 41 adenovirus infections.