Cystic fibrosis (CF) – Related resources

Cochrane reviews

• Both short and long-acting beta-2 agonists may be beneficial both in the short and long term in individuals with cystic fibrosis who have bronchodilator responsiveness or bronchial hyperresponsiveness .
• There is insufficient evidence to support or oppose the long-term use of oxygen therapy for people with cystic fibrosis. Short-term oxygen therapy may improve oxygenation and exercise capacity..
• Non-invasive ventilation as an adjunct to other airway clearance techniques may be useful in improving respiratory function in people with cystic fibrosis, especially those who have difficulty expectorating sputum .
• There is insufficient evidence on the effects of positive expiratory pressure (PEP) devices for chest physiotherapy for people with cystic fibrosis .
• Omega-3 supplements may provide some benefits for people with cystic fibrosis with relatively few adverse effects .
• Oral calorie supplements appear not to provide any additional benefit in the nutritional management of moderately malnourished children with cystic fibrosis compared to dietary advice and monitoring alone .
• Oscillating devices may not provide any benefit over other methods of physiotherapeutic airway clearance in terms of lung function or sputum characteristics in people with cystic fibrosis .
• Oral steroids at prednisolone equivalent dose of 1–2 mg/kg alternate days may slow the progression of lung disease in cystic fibrosis but cause cataracts and impair growth .
• Topical administration of short-acting sodium channel blocker may not improve respiratory condition in people with cystic fibrosis and may even cause a decline in lung function .
• Giving anti-staphylococcal antibiotic prophylaxis to young children with cystic fibrosis may lead to fewer children having isolates of Staphylococcus aureus but the clinical importance of this finding is uncertain .
• Once and three times daily aminoglycoside antibiotics appear to be equally effective in the treatment of pulmonary exacerbations of cystic fibrosis .
• Drug therapies that reduce gastric acidity seem to improve fat absorption and gastro-intestinal symptoms such as abdominal pain in people with cystic fibrosis, although the evidence is insufficient .

Other Internet resources

• Cystic fibrosis. Orphanet ORPHA586

Literature

• Boyle MP. Adult cystic fibrosis. JAMA 2007 Oct 17;298(15):1787-93.
• Davies JC, Alton EW, Bush A. Cystic fibrosis. BMJ 2007 Dec 15;335(7632):1255-9.