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Published in: Current Gastroenterology Reports 10/2021

01-10-2021 | Cystic Fibrosis | Small Intestine (D Sachar, Section Editor)

The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis

Authors: Daniel B. Karb, Linda C. Cummings

Published in: Current Gastroenterology Reports | Issue 10/2021

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Abstract

Purpose of Review

While commonly associated with pulmonary manifestations, cystic fibrosis (CF) is a systemic disease with wide-ranging effects on the gastrointestinal (GI) tract. This article reviews major recent updates in gastroenterological CF care and research.

Recent Findings

The high burden of GI symptoms in CF has led to recent studies assessing GI-specific symptom questionnaires and scoring systems. Intestinal dysbiosis potentially contributes to gastrointestinal symptoms in patients with CF and an increased risk of gastrointestinal cancers in CF. An increased incidence of colorectal cancer (CRC) has led to CF-specific CRC screening and surveillance recommendations. Pharmacologic therapies targeting specific cystic fibrosis transmembrane conductance regulator (CFTR) mutations have shown promise in treating GI manifestations of CF.

Summary

New research has highlighted the importance of intestinal dysbiosis in CF. Future studies should assess whether CFTR modulators affect the gut microbiome and whether altering the gut microbiome will impact GI symptoms and GI cancer risk.
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Metadata
Title
The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis
Authors
Daniel B. Karb
Linda C. Cummings
Publication date
01-10-2021
Publisher
Springer US
Published in
Current Gastroenterology Reports / Issue 10/2021
Print ISSN: 1522-8037
Electronic ISSN: 1534-312X
DOI
https://doi.org/10.1007/s11894-021-00817-2
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