ABSTRACT
Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90â% of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection. This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.
Subject(s)
Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Practice Guidelines as Topic , Pseudomonas aeruginosa/isolation & purification , Cystic Fibrosis/complications , Cystic Fibrosis/microbiology , Cystic Fibrosis Transmembrane Conductance Regulator/metabolism , Germany , Humans , Pseudomonas Infections/diagnosisABSTRACT
Damage to the airways and lung parenchyma from aspiration is a common clinical problem with diagnostic and therapeutic challenges. The insult may be acute or chronic. The amount of concentration, chemical composition, and size of particles vary, and the lesion produced may be reversible or irreversible. Death may be immediate or late. Recovery may be complete, or crippling pulmonary disease may follow. In this review is reported on the aspiration of foreign bodies or liquids into the respiratory tract. Specialty: in infancy, esophageal foreign bodies may cause mainly respiratory symptoms. Adequate diagnosis and therapy are discussed.