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Konrad, J; Eber, E; Stadlbauer, V.
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
Paediatr Respir Rev. 2022; 42:9-16
Doi: 10.1016/j.prrv.2020.12.001
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PubMed
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- Führende Autor*innen der Med Uni Graz
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Konrad Julia
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Stadlbauer-Köllner Vanessa
- Co-Autor*innen der Med Uni Graz
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Eber Ernst
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- Abstract:
- 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.
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Cystic Fibrosis - therapy
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Cystic Fibrosis Transmembrane Conductance Regulator - genetics, metabolism
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Exocrine Pancreatic Insufficiency - drug therapy, etiology
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Gastrointestinal Diseases - complications, therapy
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Humans - administration & dosage
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Mutation - administration & dosage
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Quality of Life - administration & dosage
- Find related publications in this database (Keywords)
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Cystic fibrosis
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Gastrointestinal
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Pancreatic insufficiency
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Liver disease
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Gut microbiome
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CFTR modulator