Cystic Fibrosis Pulmonary Guidelines

Abstract

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.

Pharmacokinetics and Pharmacodynamics of Aerosolized Antibacterial Agents in Chronically Infected Cystic Fibrosis Patients

“…The microbial ecology of CF is not only changing but also becoming increasingly complex, as demonstrated by a combination of culture-dependent and culture-independent approaches (52)(53)(54)(55)(56)(57)(58). Furthermore, CF-associated microbial communities are not only complex but also diverse in anatomically distinct regions of the CF lung (358).…”

A Role of Hypertonic Saline Solution in Treatment of Cystic Fibrosis

“…Именно поэтому изучению новых методов терапии бронхолегочной системы уделяется самое пристальное внимание. Помимо антибактериальных средств и кинезитера пии, важное место занимают муколитические пре параты, среди которых все большее значение прида ется гипертоническому раствору хлорида натрия (ГРХН), который было бы более корректно отнести к регидратантам мокроты [1,2].…”

“…Именно поэтому изучению новых методов терапии бронхолегочной системы уделяется самое пристальное внимание. Помимо антибактериальных средств и кинезитера пии, важное место занимают муколитические пре параты, среди которых все большее значение прида ется гипертоническому раствору хлорида натрия (ГРХН), который было бы более корректно отнести к регидратантам мокроты [1,2].Мукоцилиарный клиренс как в норме, так и при патологии является основным механизмом очище ния респираторной системы от вдыхаемых частиц и других веществ, содержащихся в бронхах. Основ ными составляющими этого механизма являются цилиарный эпителий и покрывающая его жидкость -бронхиальный секрет (БС).…”

Dyspnea Perception In Cystic Fibrosis Patients

“…For individuals with cystic fibrosis (CF), pulmonary disease is associated with increased morbidity, mortality, and poor prognosis (3). Dyspnea is a frequent symptom in CF patients with pulmonary impairment, and is often accompanied by physical inactivity, decreased exercise capacity, and quality of life (4)(5)(6)(7).…”

“…Few studies have evaluated dyspnea perception during exercise tests in CF patients (3,(8)(9)(10)(11). Chetta et al (9) showed a relationship between dyspnea perception and peripheral oxygen saturation during the 6-min walk test (6MWT) in CF adults with mild and moderate lung disease.…”