Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators
| Autor: | Julia Konrad, Ernst Eber, Vanessa Stadlbauer |
|---|---|
| Rok vydání: | 2022 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty Cystic Fibrosis Gastrointestinal Diseases Population Cystic Fibrosis Transmembrane Conductance Regulator Disease Cystic fibrosis Gastroenterology 03 medical and health sciences Liver disease 0302 clinical medicine Internal medicine medicine Humans Large intestine 030212 general & internal medicine education education.field_of_study Gastrointestinal tract biology business.industry medicine.disease Gut microbiome Cystic fibrosis transmembrane conductance regulator medicine.anatomical_structure 030228 respiratory system Mutation Pediatrics Perinatology and Child Health Quality of Life biology.protein Exocrine Pancreatic Insufficiency business |
| Zdroj: | Paediatric Respiratory Reviews. 42:9-16 |
| ISSN: | 1526-0542 |
| Popis: | Cystic fibrosis (CF) - although primarily a lung disease - also causes a variety of gastrointestinal manifestations which are important for diagnosis, prognosis and quality of life. All parts of the gastrointestinal tract can be affected by CF. Besides the well-known pancreatic insufficiency, gastroesophageal reflux disease, liver disease and diseases of the large intestine are important pathologies that impact on prognosis and also impair quality of life. Diagnosis and management of gastrointestinal manifestations will be discussed in this review. Since optimisation of CF therapy is associated with a significantly longer life-span of CF patients nowadays, also gastrointestinal malignancies, which are more common in CF than in the non-CF population need to be considered. Furthermore, novel evidence on the role of the gut microbiome in CF is emerging. The introduction of cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators gives hope for symptom alleviation and even cure of gastrointestinal manifestations of CF. |
| Databáze: | OpenAIRE |
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