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S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Modul 2: Diagnostik und Therapie bei der chronischen Infektion mit Pseudomonas aeruginosa


Schwarz, C; Schulte-Hubbert, B; Bend, J; et al; Jung, A; Möller, A (2018). S3-Leitlinie: Lungenerkrankung bei Mukoviszidose – Modul 2: Diagnostik und Therapie bei der chronischen Infektion mit Pseudomonas aeruginosa. Pneumologie, 72(5):347-392.

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 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. () 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 -infection. This is a S3-clinical guideline which implements a definition for chronic -infection and demonstrates evidence-based diagnostic methods and medical treatment for -infection in order to give guidance for individual treatment options.

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 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. () 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 -infection. This is a S3-clinical guideline which implements a definition for chronic -infection and demonstrates evidence-based diagnostic methods and medical treatment for -infection in order to give guidance for individual treatment options.

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Other titles:CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Pulmonary and Respiratory Medicine
Language:German
Date:May 2018
Deposited On:07 Dec 2018 08:53
Last Modified:29 Jul 2020 08:19
Publisher:Georg Thieme Verlag
ISSN:0934-8387
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1055/s-0044-100191
PubMed ID:29758578

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