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Review
. 2021 Mar 23;23(3):4.
doi: 10.1007/s11894-021-00806-5.

Luminal Gastrointestinal Manifestations of Cystic Fibrosis

Samuel J Burton  1 , Christine Hachem  1 , James M Abraham  2
Affiliations
Review

Luminal Gastrointestinal Manifestations of Cystic Fibrosis

Samuel J Burton et al. Curr Gastroenterol Rep. .

Abstract

Purpose of review: People with cystic fibrosis (CF) are living longer. General age-related and CF-specific gastrointestinal symptoms are increasingly recognized. In this article, we review the latest data on luminal gastrointestinal manifestations in CF.

Recent findings: People with CF have increased incidence of gastroesophageal reflux disease symptoms and often prescribed proton-pump inhibitors (PPI). PPI use may increase risk of pulmonary exacerbations. Evidence to support gastric fundoplication to improve pulmonary outcomes is limited. Features of intestinal dysmotility are common. There are distinct differences in the gut microbiome in the CF population which may have clinical implications. CF is a possible hereditary digestive cancer syndrome, particularly in regard to colorectal cancer (CRC) with earlier incidence of CRC and advanced colonic neoplasia. Early screening colonoscopy is warranted in the CF population. Gastrointestinal manifestations in CF are prevalent across all digestive organs. More study on the effect of interventions for symptomatic treatment and cancer screening is needed.

Keywords: Colorectal cancer; Cystic fibrosis; Dysmotility; GERD; Lung transplant.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006;173(5):475–82. - PubMed - DOI - PMC
    1. MacKenzie T, Gifford AH, Sabadosa KA, Quinton HB, Knapp EA, Goss CH, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry. Ann Intern Med. 2014;161(4):233–41. - PubMed - PMC - DOI
    1. Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D. Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: a longitudinal study using UK patient registry data. J Cyst Fibros. 2018;17(2):218–27. - PubMed - PMC - DOI
    1. Cystic Fibrosis Foundation patient registry annual data report. 2018.
    1. Balfour-Lynn IM, King JA. CFTR modulator therapies - effect on life expectancy in people with cystic fibrosis. Paediatr Respir Rev. 2020.

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