About this Research Topic
Cystic Fibrosis (CF) has always been considered a disease with prevalent lung involvement. However, the absence or dysfunction of CFTR protein in biliary, intestinal, and pancreatic epithelium in patients with CF causes extrapulmonary manifestations such as upper gastrointestinal symptoms, pancreatic disease, liver disease, meconium ileus in newborn infants or distal intestinal obstruction syndrome along with an increased risk of gastrointestinal cancers. Furthermore, the advent of highly effective modulator therapy that has improved the life expectancy of patients, has also modified the management of gastrointestinal and hepatic complications. For example, little is known about the efficacy and safety of the new CFTR modulators in patients with CF-related liver disease (CFLD) or those who have undergone liver transplantation. Consequently, it is now imperative that subjects with variable patterns of hepatobiliary involvement are assessed carefully during treatment, it is also important to identify and evaluate non-invasive biomarkers that could enable an early evaluation of the response to treatment and detect any progression of hepatic fibrosis.
This Research Topic aims to provide an update on the current diagnostic and therapeutic management of gastrointestinal and liver disease in patients with CF in the era of CFTR modulator therapies.
Topics will include, but are not limited to, the following:
1. Pathophysiology of gastrointestinal and hepatic disease in patients with CF
2. Non-invasive diagnostic strategies for CFLD
3. Surgical management of gastrointestinal and hepatic complications of CF
4. Impact of CFTR modulator therapies on gastrointestinal and CFLD progression
5. Hepatotoxicity secondary to CFTR modulators
6. New insights into the gut microbiome in CF
7. Metabolomics perspectives in CF research
Original articles, short communications, systematic reviews, meta-analyses, case reports, and mini-reviews focusing on these topics are welcome.
This Research Topic aims to provide an update on the current diagnostic and therapeutic management of gastrointestinal and liver disease in patients with CF in the era of CFTR modulator therapies.
Topics will include, but are not limited to, the following:
1. Pathophysiology of gastrointestinal and hepatic disease in patients with CF
2. Non-invasive diagnostic strategies for CFLD
3. Surgical management of gastrointestinal and hepatic complications of CF
4. Impact of CFTR modulator therapies on gastrointestinal and CFLD progression
5. Hepatotoxicity secondary to CFTR modulators
6. New insights into the gut microbiome in CF
7. Metabolomics perspectives in CF research
Original articles, short communications, systematic reviews, meta-analyses, case reports, and mini-reviews focusing on these topics are welcome.
Keywords: Cystic fibrosis-associated liver disease (CFLD), Highly effective modulator therapy, CFTR Modulators, Ursodeoxycholic Acid, Cirrhosis, Distal intestinal obstructive syndrome, Portal hypertension, Gastrointestinal disorders
Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.
