Susceptibility to corticosteroid-induced adrenal suppression: a genome-wide association study.

Background: A serious adverse effect of corticosteroid therapy is adrenal suppression. Our aim was to identify genetic variants affecting susceptibility to corticosteroid-induced adrenal suppression.

Methods: We enrolled children with asthma who used inhaled corticosteroids as part of their treatment from 25 sites across the UK (discovery cohort), as part of the Pharmacogenetics of Adrenal Suppression with Inhaled Steroids (PASS) study.

WITHDRAWN: Inhaled bronchodilators for cystic fibrosis.

This review has been withdrawn because it has been split into the following reviews: 'Long‐acting inhaled bronchodilators for cystic fibrosis' and 'Short‐acting inhaled bronchodilators for cystic fibrosis'. The editorial group responsible for this previously published document have withdrawn it from publication.

'Less may be best'-Pediatric parapneumonic effusion and empyema management: Lessons from a UK center.

Background: Children with empyema are managed at our center using a protocol-driven clinical care pathway. Chemical fibrinolysis is deployed as first-line management for significant pleural disease. We therefore examined clinical outcome(s) to benchmark standards of care while analyzing disease severity with introduction of the pneumococcal conjugate vaccine.

Adrenal responses to a low-dose short synacthen test in children with asthma.

Objectives: Corticosteroids are known to cause adrenal suppression. The aim of this study was to assess clinical factors affecting responses to a low dose short synacthen test (LDSST) in asthmatic children using corticosteroids.

Design: Patients were recruited from secondary care paediatric asthma populations within the UK.

Large ABCA3 and SFTPC deletions resulting in lung disease.

Rationale: Mutations in genes encoding proteins important in the function and metabolism of pulmonary surfactant are recognized causes of lung disease. Clinical genetic testing is available for these disorders, but children with phenotypes consistent with surfactant dysfunction and no identifiable mutations in the known causative genes have been reported.

Objectives: To identify the mechanism(s) for lung disease in two children with the phenotype of surfactant dysfunction who had negative testing in clinical laboratories for gene mutations causing surfactant dysfunction.

Early morning salivary cortisol and cortisone, and adrenal responses to a simplified low-dose short Synacthen test in children with asthma.

Objective: To examine serum cortisol responses to a simplified low-dose short Synacthen test (LDSST) in children treated with inhaled corticosteroids (ICS) for asthma and to compare these to early morning salivary cortisol (EMSC) and cortisone (EMSCn) levels.

Design: Early morning salivary cortisol and EMSCn samples were collected for three consecutive days. On day three, Synacthen 500 ng/1·73 m(2) was administered intravenously.

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.

Secondary pulmonary hypertension.

Recent research has greatly improved our understanding of the pathophysiology of pulmonary hypertension. There is increasing recognition that pulmonary hypertension is an important complication of many childhood respiratory diseases including cystic fibrosis, interstitial lung diseases, upper airways obstruction and disorders of the respiratory muscles and chest wall. Chronic hypoxaemia and, in some cases, destruction of the vascular bed are the key factors.

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.

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.

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.

Qualitative study of young people's and parents' beliefs about childhood asthma.

Background: Asthma continues to be a common childhood chronic illness managed principally in primary care. Self-management requires co-ordinated efforts of young people, carers and health professionals. Non-compliance occurs even when parents are supervising care, suggesting that decisions are made on the basis of beliefs that contrast with professional advice.

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.

Asthma in adolescence.

The care of adolescents with asthma has been largely neglected, yet adolescents have particular needs that differ from those of children or adults with asthma. There are over 800,000 teenagers in the UK who suffer from asthma and underdiagnosis and poor treatment are common. The prevalence and level of morbidity from asthma in adolescents are as high as or higher than the rates in younger schoolchildren.

Assessment of the child with recurrent chest infections.

The child with recurrent chest infections presents the clinician with a difficult diagnostic challenge. Does the child have a simply-managed cause for their symptoms, such as recurrent viral respiratory infections or asthma, or is there evidence of a more serious underlying pathology, such as bronchiectasis? Many different disorders present in this way, including cystic fibrosis, a range of immunodeficiency syndromes, and congenital abnormalities of the respiratory tract. In some affected children, lung damage follows a single severe pneumonia: in others it is the result of inhalation of food or a foreign body.

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.

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.

Patient access to the Internet.

Aim: To discern the availability of the Internet to patients attending clinics and surgeries in North Manchester General Hospital, UK.

Method: A multidisciplinary group designed a questionnaire to be distributed to patients attending the authors' clinics between each October and November for five years.

Results: The data from 1998 and 1999 are presented here.

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.