BACKGROUND We evaluated the exercise capacity of children with cystic fibrosis to determine whether ventilatory limitation associated with static hyperinflation is related with decreased exercise capacity, thus predisposing these children to arterial hypoxemia during progressive exercise. METHODS Thirty-seven children, ages 8-17 years, underwent spirometry, body plethysmography, and cardiopul...

BACKGROUND Published data on the frequency and types of flexible bronchoscopic airway appearances in children with non-cystic fibrosis bronchiectasis and chronic suppurative lung disease are unavailable. The aims of this study were to describe airway appearances and frequency of airway abnormalities and to relate these airway abnormalities to chest high resolution computed tomography (cHRCT) fi...

Nitric oxide (NO) is present in exhaled air of humans. This NO is mostly produced in the upper airways, whereas basal NO excretion in the lower airways is low. Children with Kartagener's syndrome have an almost total lack of NO in nasally derived air, whereas adult asthmatics have increased NO in orally exhaled air. NO excretion was measured in the nasal cavity and in orally exhaled air in 19 h...

BACKGROUND Provision of medical care for adult patients with cystic fibrosis is an increasing problem as the number of patients surviving into adulthood increases. Recent reports have suggested that care is best provided in specialist centres because of longer survival. Recent changes in the National Health Service funding and delivery of service may adversely affect the provision of such a spe...

Although H influenzae may be found in the sputum of children with cystic fibrosis, we were surprised that Staphylococcus aureus was not also isolated. Furthermore, C albicans is an unusual finding in children's sputum unless they have been receiving long term antibiotic treatment. Diarrhoea and failure to thrive are common presenting features in children with symptomatic HIV infection.2 In our ...

In childhood, pancreatic exocrine insufficiency is rarely encountered except as one of the features of cystic fibrosis, which is in contrast to the pattern of pancreatic disease in adults, where pancreatitis is common. However, paediatricians recognize that some children with symptoms of pancreatic insufficiency do not have the chronic chest infection and raised levels of electrolytes in the sw...

Abstract Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case M. pediatric patient primary ciliary dyskinesia he was successfully treated parenteral amikacin, linezolid oral clarithromycin combined inhaled amikacin. Clinical improvem...

Bronchodilators syndrome occurring in association with ipratropium. Nebulised bronchodilators are used extensively in children with cystic fibrosis, but in many Optimal dosage regimens for b agonists have not been investigated, and doses given to chilinstances this use is not yet based on objective evidence of clinical benefit. dren with cystic fibrosis are usually similar to those used in asth...

The diets of 20 children with cystic fibrosis were analysed for energy and nutrient content with simultaneous measurement of energy losses in stools. Median energy intakes were in excess of the WHO estimated daily requirements (118.2%) when expressed as MJ/kg/24 hours, the excess almost accounted for by energy losses in the stools. When expressed as MJ/24 hours, however, median energy intakes w...