Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.

BACKGROUND: Cystic fibrosis is the most common, life-threatening, recessively inherited disease of Caucasian populations. It is a multisystem disorder caused by a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator protein which is important in producing sweat, digestive juices and mucus.The impaired or absent function of this protein results in the production of viscous mucus within the lungs and an environment that is susceptible to chronic airway obstruction and pulmonary colonization by a range of pathogenic bacteria. Morbidity and mortality of cystic fibrosis is related to chronic pulmonary sepsis and its complications by these bacteria.Influenza can worsen the course of the disease in cystic fibrosis by increasing the risk of pneumonia and secondary respiratory complications. Antiviral agents form an important part of influenza management and include the neuraminidase inhibitors zanamivir and oseltamivir. These inhibitors can limit the infection and prevent the spread of the virus. OBJECTIVES: To assess the effects of neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 12 August 2009. SELECTION CRITERIA: Randomised controlled trials and quasi-randomised controlled trials comparing neuraminidase inhibitors with placebo or other antiviral drugs. DATA COLLECTION AND ANALYSIS: Two review authors had planned to independently screen studies, extract data and assess risk of bias using standard Cochrane Collaboration methodologies. No studies were identified for inclusion. MAIN RESULTS: No relevant studies were retrieved after a comprehensive search of the literature. AUTHORS' CONCLUSIONS: We were unable to identify any randomised controlled trials or quasi-randomised controlled trials on the efficacy of neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis. The absence of high level evidence for the effectiveness of these interventions emphasises the need for well-designed, adequately powered, randomised controlled clinical trials.

Prevention of chronic Pseudomonas aeruginosa infection in people with cystic fibrosis.

Cystic fibrosis is the most common genetically inherited disease in the Caucasian population, with approximately 30,000 patients in the USA and more than 50,000 patients worldwide. The primary defect in the cystic fibrosis transmembrane regulator gene affects the production and/or function of the cystic fibrosis transmembrane regulator protein. Depending on the severity of the genetic defect, patients may have minimal disease expression (e.g., male infertility) or multisystem involvement, including recurrent respiratory infection progressing to respiratory failure, hepatobiliary disease, exocrine pancreatic insufficiency, diabetes mellitus and gastrointestinal tract motility problems. Pseudomonas aeruginosa is commonly isolated from the lower respiratory tract in early childhood. Chronic infection is associated with increased morbidity and mortality. P. aeruginosa infection may be acquired from the environment or by person-to-person contact. Clinicians should adopt a proactive protocol to prevent chronic infection. The cornerstones of such a policy are microbiological surveillance, infection control and antibiotic-based eradication regimens.