Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health.

RATIONALE: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal airways secretions, chronic endobronchial infection, and progressive airway obstruction. The use of medications to slow the progression of lung disease has led to significant improvement in survival. An evidence review of chronic medications for CF lung disease was performed in 2007 to provide guidance to clinicians in evaluating and selecting appropriate treatment for individuals with this disease. We have undertaken a new review of the literature to update the recommendations, including consideration of new medications and additional evidence on previously reviewed therapies. A multidisciplinary committee of experts in CF pulmonary care was established to review the evidence for use of chronic medications for CF lung disease and make treatment recommendations. Published evidence for chronic lung therapies was systematically reviewed and resulting treatment recommendations were graded based on the United States Preventive Services Task Force scheme. These guidelines provide up-to-date evidence of safety and efficacy of chronic treatments of CF lung disease, including the use of novel therapies that have not previously been included in CF pulmonary guidelines.

Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.

RATIONALE: Cystic fibrosis is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. Death is usually a result of respiratory failure. Newly introduced therapies and aggressive management of the lung disease have resulted in great improvements in length and quality of life, with the result that the median expected survival age has reached 36 years. However, as the number of treatments expands, the medical regimen becomes increasingly burdensome in time, money, and health resources. Hence, it is important that treatments should be recommended on the basis of available evidence of efficacy and safety. OBJECTIVES: The Cystic Fibrosis Foundation therefore established a committee to examine the clinical evidence for each therapy and to provide guidance for the prescription of these therapies. METHODS: The committee members developed and refined a series of questions related to drug therapies used in the maintenance of pulmonary function. We addressed the questions in one of three ways, based on available evidence: (1) commissioned systematic review, (2) modified systematic review, or (3) summary of existing Cochrane reviews. CONCLUSIONS: It is hoped that the guidelines provided in this article will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.

A multicenter comparison of intraaortic balloon pump utilization in isolated coronary artery bypass graft surgery.

BACKGROUND: Single-center studies suggest substantial variation in intraaortic balloon pump (IABP) utilization. Our purpose is to examine IABP utilization over time and across medical centers. METHODS: This was a prospective cohort of 29,961 consecutive patients undergoing isolated coronary artery bypass graft surgery, between 1995 and 2000, at 10 centers (eight in northern New England and two in Canada). RESULTS: A total of 2,678 (8.9%) patients received an IABP. The rate of preoperative IABP insertion was 6.3%, and that of intra- or postoperative insertion was 2.6%. During the 6 years, IABP use increased from 7.0% to 10.3% (p(trend) <0.001). Preoperative IABP insertion increased from 5.4% to 7.8% (p(trend) < 0.001). There was no significant increase in intra-/postoperative IABP insertion 1.7% to 3.4% (p(trend) = 0.34). Adjustment for changes in patient and disease characteristics did not substantially alter these results. The rate of IABP use varied substantially by center, from 5.9% to 16.4% (p < 0.001). Adjustment for patient and disease characteristics resulted in variation from 4.8% to 12.8% across the 10 centers (p < 0.001). The adjusted rates of preoperative IABP insertion varied from 3.6% to 13.7% (p < 0.001), and the rates of intra-/postoperative IABP insertion ranged from 1.0% to 5.2% (p < 0.001). There was no significant correlation between the rates of preoperative and intra-/postoperative IABP use (r(s) = 0.085, p = 0.815). CONCLUSIONS: During the 6 years, there was a 47% increase in the rate of IABP utilization. Even after adjustment, there was almost threefold variation in IABP use across centers. This variation likely reflects lack of consensus on the appropriate use of the IABP in CABG patients.

Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection.

DESCRIPTION: The Cystic Fibrosis (CF) Foundation developed clinical care guidelines for the prevention of Pseudomonas aeruginosa infection, the treatment of initial P. aeruginosa infection, and the use of bronchoscopy to obtain routine airway cultures in individuals with CF. METHODS: A multidisciplinary committee developed questions about the prevention and treatment of initial P. aeruginosa infection and the use of bronchoscopy to obtain routine airway cultures. The outcome measure of interest was cultures without P. aeruginosa growth. Systematic reviews of PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials were conducted in May 2012 and August 2013. Searches combined controlled vocabulary terms and text words for CF and terms relevant to each question. The entire committee reviewed the evidence, and final recommendation statements were graded using the U.S. Preventive Services Task Force system. Recommendation 1: The CF Foundation strongly recommends inhaled antibiotic therapy for the treatment of initial or new growth of P. aeruginosa from an airway culture (certainty of net benefit, high; estimate of net benefit, substantial; grade of recommendation, A). The favored antibiotic regimen is inhaled tobramycin (300 mg twice daily) for 28 days. Recommendation 2: The CF Foundation recommends against the use of prophylactic antipseudomonal antibiotics to prevent the acquisition P. aeruginosa (certainty of net benefit, moderate; estimate of net benefit, zero; grade of recommendation, D). Recommendation 3: The CF Foundation recommends routine oropharyngeal cultures rather than bronchoalveolar lavage cultures obtained by bronchoscopy in individuals with CF who cannot expectorate sputum to determine if they are infected with P. aeruginosa (certainty of net benefit, moderate; estimate of net benefit, moderate; grade of recommendation, B).