The Cystic Fibrosis Intestine

Overview

Journal Cold Spring Harb Perspect Med

Specialty General Medicine

Date 2013 Jun 22

PMID 23788646

Citations 92

Authors

Robert C De Lisle

Drucy Borowitz

Affiliations

Soon will be listed here.

Abstract

The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans.

Citing Articles

Sensing Mucus Physiological Property In Situ by Wireless Millimeter-Scale Soft Robots.

Xiao B, Xu Y, Edwards S, Balakumar L, Dong X Adv Funct Mater. 2025; 34(8).

PMID: 39990597 PMC: 11845219. DOI: 10.1002/adfm.202307751.

Persistent delay in maturation of the developing gut microbiota in infants with cystic fibrosis.

Verster A, Salerno P, Valls R, Barrack K, Price C, McClure E mBio. 2025; 16(3):e0342024.

PMID: 39945545 PMC: 11898760. DOI: 10.1128/mbio.03420-24.

Fatty acid abnormalities in cystic fibrosis-the missing link for a cure?.

Drzymala-Czyz S, Walkowiak J, Colombo C, Alicandro G, Storrosten O, Kolsgaard M iScience. 2024; 27(11):111153.

PMID: 39620135 PMC: 11607544. DOI: 10.1016/j.isci.2024.111153.

Normalization of Cystic Fibrosis Immune System Reverses Intestinal Neutrophilic Inflammation and Significantly Improves the Survival of Cystic Fibrosis Mice.

Scull C, Hu Y, Jennings S, Wang G Cell Mol Gastroenterol Hepatol. 2024; 19(2):101424.

PMID: 39510500 PMC: 11720009. DOI: 10.1016/j.jcmgh.2024.101424.

Fecal Microbiota Transplantation for Recurrent Infections in a Cystic Fibrosis Child Previously Screen Positive, Inconclusive Diagnosis (CFSPID): A Case Report.

Marsiglia R, Pane S, Del Chierico F, Russo A, Vernocchi P, Romani L Microorganisms. 2024; 12(10).

PMID: 39458368 PMC: 11509880. DOI: 10.3390/microorganisms12102059.

References

Stallings V, Stark L, Robinson K, Feranchak A, Quinton H . Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008; 108(5):832-9. DOI: 10.1016/j.jada.2008.02.020. View

Borowitz D, Durie P, Clarke L, Werlin S, Taylor C, Semler J . Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2005; 41(3):273-85. DOI: 10.1097/01.mpg.0000178439.64675.8d. View

Park H, Lee M . Transepithelial bicarbonate secretion: lessons from the pancreas. Cold Spring Harb Perspect Med. 2012; 2(10). PMC: 3475396. DOI: 10.1101/cshperspect.a009571. View

Lindblad A, Glaumann H, Strandvik B . Natural history of liver disease in cystic fibrosis. Hepatology. 1999; 30(5):1151-8. DOI: 10.1002/hep.510300527. View

Smyth R, Croft N, OHea U, Marshall T, Ferguson A . Intestinal inflammation in cystic fibrosis. Arch Dis Child. 2000; 82(5):394-9. PMC: 1718322. DOI: 10.1136/adc.82.5.394. View

Guilbault C, Saeed Z, Downey G, Radzioch D . Cystic fibrosis mouse models. Am J Respir Cell Mol Biol. 2006; 36(1):1-7. DOI: 10.1165/rcmb.2006-0184TR. View

Stylianou C, Galli-Tsinopoulou A, Koliakos G, Fotoulaki M, Nousia-Arvanitakis S . Ghrelin and leptin levels in young adults with cystic fibrosis: relationship with body fat. J Cyst Fibros. 2007; 6(4):293-6. DOI: 10.1016/j.jcf.2006.10.011. View

Steinkamp G, Wiedemann B . Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax. 2002; 57(7):596-601. PMC: 1746376. DOI: 10.1136/thorax.57.7.596. View

Carey M . Bile acids and bile salts: ionization and solubility properties. Hepatology. 1984; 4(5 Suppl):66S-71S. DOI: 10.1002/hep.1840040812. View

10.

Werlin S, Benuri-Silbiger I, Kerem E, Adler S, Goldin E, Zimmerman J . Evidence of intestinal inflammation in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010; 51(3):304-8. DOI: 10.1097/MPG.0b013e3181d1b013. View

11.

Braden B . Methods and functions: Breath tests. Best Pract Res Clin Gastroenterol. 2009; 23(3):337-52. DOI: 10.1016/j.bpg.2009.02.014. View

12.

Clarke L, Gawenis L, Bradford E, Judd L, Boyle K, Simpson J . Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice. Am J Physiol Gastrointest Liver Physiol. 2004; 286(6):G1050-8. DOI: 10.1152/ajpgi.00393.2003. View

13.

Sun X, Sui H, Fisher J, Yan Z, Liu X, Cho H . Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest. 2010; 120(9):3149-60. PMC: 2929732. DOI: 10.1172/JCI43052. View

14.

Bradford E, Sartor M, Gawenis L, Clarke L, Shull G . Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice. Am J Physiol Gastrointest Liver Physiol. 2009; 296(4):G886-98. PMC: 2670667. DOI: 10.1152/ajpgi.90520.2008. View

15.

Norkina O, Burnett T, De Lisle R . Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine. Infect Immun. 2004; 72(10):6040-9. PMC: 517588. DOI: 10.1128/IAI.72.10.6040-6049.2004. View

16.

Canale-Zambrano J, Auger M, Haston C . Toll-like receptor-4 genotype influences the survival of cystic fibrosis mice. Am J Physiol Gastrointest Liver Physiol. 2010; 299(2):G381-90. DOI: 10.1152/ajpgi.00003.2010. View

17.

Edelman S, Kasper D . Symbiotic commensal bacteria direct maturation of the host immune system. Curr Opin Gastroenterol. 2009; 24(6):720-4. DOI: 10.1097/MOG.0b013e32830c4355. View

18.

Montalto M, Gallo A, Ianiro G, Santoro L, DOnofrio F, Ricci R . Can chronic gastritis cause an increase in fecal calprotectin concentrations?. World J Gastroenterol. 2010; 16(27):3406-10. PMC: 2904887. DOI: 10.3748/wjg.v16.i27.3406. View

19.

Bali A, Stableforth D, Asquith P . Prolonged small-intestinal transit time in cystic fibrosis. Br Med J (Clin Res Ed). 1983; 287(6398):1011-3. PMC: 1549570. DOI: 10.1136/bmj.287.6398.1011. View

20.

Dalzell A, Heaf D . Oro-caecal transit time and intra-luminal pH in cystic fibrosis patients with distal intestinal obstruction syndrome. Acta Univ Carol Med (Praha). 1990; 36(1-4):159-60. View