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Pneumologie ; 78(6): 367-399, 2024 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-38350639

RESUMO

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. 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 airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung 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 in order to give guidance for individual treatment options.


Assuntos
Antibacterianos , Fibrose Cística , Guias de Prática Clínica como Assunto , Infecções por Pseudomonas , Pseudomonas aeruginosa , Humanos , Infecções por Pseudomonas/diagnóstico , Infecções por Pseudomonas/tratamento farmacológico , Fibrose Cística/microbiologia , Fibrose Cística/terapia , Alemanha , Antibacterianos/uso terapêutico , Pneumologia/normas , Medicina Baseada em Evidências
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