BACKGROUND Respiratory viral infections lead to bronchial inflammation in patients with cystic fibrosis, especially during pulmonary exacerbations. The aim of this study was to determine the impact of viral-associated pulmonary exacerbations in children with cystic fibrosis and failure to improve forced expiratory volume in 1 s (FEV1 ) after an appropriate treatment. METHODS We lead a pilot s...

Reports of simple pulmonary function tests in cystic fibrosis first appeared in 1954, when West, Levin, and Di Sant' Agnese showed a characteristic finding in 6 patients, aged 12-16 years, of difficulty in moving air rapidly in or out of the lungs. Cook et al. (1957) studied the lung volume in 53 patients, aged 1-31 years, and found that 810% of those diagnosed as having cystic fibrosis were fo...

BACKGROUND It has been suggested that oxidative stress contributes to lung injury in cystic fibrosis. There is, however, no direct evidence of increased pulmonary oxidative stress in cystic fibrosis nor of the effects of inflammation on the major pulmonary antioxidant, glutathione. A study was undertaken to measure these parameters in infants and young children in the presence or absence of pul...

BACKGROUND With increasing life expectancy, issues of fertility and pregnancy are pertinent to patients with cystic fibrosis. For this reason, the four Scottish Cystic Fibrosis centres asked men and women with cystic fibrosis about their attitudes to fertility and to information given to them by health professionals. METHODS A postal questionnaire was sent to 116 men and 79 women aged 16 year...

Respir Rev 2005;6:227–35. 41. Equi A, Balfour-Lynn IM, Bush A, et al. Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial. Lancet 2002;360:978–84. 42. Wolter J, Seeney S, Bell S, et al. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax 2002;57:212–16. 43. Saiman L, Marshall B...

13C breath tests are a safe, non-invasive way of assessing nutrient digestion and absorption that can be used repeatedly in infancy and childhood. The aim of this study was to assess their value for measuring fat digestion in infants and young children with cystic fibrosis, and healthy controls whose pancreatic exocrine function is immature, and to monitor pancreatic enzyme supplementation. Six...

Airway nitric oxide production is decreased in cystic fibrosis. As growth hormone therapy has been shown to increase nitric oxide production in growth hormone-deficient patients, it may also affect nitric oxide production in patients with cystic fibrosis. The objective of the present study was to investigate the effect of growth hormone therapy on systemic and airway nitric oxide formation in p...

Congenital absence of the vas occurs in up to 1% of men. Congenital unilateral absence of the vas deferens can be related to cystic fibrosis transmembrane conductance regulator mutations or in 79% of cases, renal agenesis. We present a case of each, diagnosed in children at operation for elective inguinal hernia repairs. One patient had associated ipsilateral renal agenesis with a normal cystic...

BACKGROUND Pulmonary changes that occur in cystic fibrosis may influence inspiratory muscle strength and endurance. We evaluated inspiratory muscle strength and endurance in children and adolescents with cystic fibrosis in comparison with healthy subjects. METHODS This is a cross-sectional observational study with subjects with cystic fibrosis and paired healthy individuals, age 6-18 y. Spiro...

shwaehman syndrome, next to cystic fibrosis, is the second cause of congenita! exocrine pancreatic insufficiency in children. it appears as steatorrhea, recurrent infections and hematologic abnormalities such as neutropenia, skeletal dysplasia and short stature. in this study, we reviewed 3 patients' histories. all of them showed cellular chemotaxic defect. one of them had been affected by neph...