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Review
. 2013 Sep 1;3(9):a009753.
doi: 10.1101/cshperspect.a009753.

The cystic fibrosis intestine

Robert C De Lisle  1 , Drucy Borowitz
Affiliations
Review

The cystic fibrosis intestine

Robert C De Lisle et al. Cold Spring Harb Perspect Med. .

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.

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Figures

Figure 1.
Figure 1.
Obstruction of the intestines in CF. (A) Microcolon of disuse with prestenotic dilatation, and (B) meconium casts taken from an infant with cystic fibrosis and meconium ileus.
Figure 2.
Figure 2.
Bubbly granular mass in the right lower quadrant of a patient with distal intestinal obstruction syndrome (DIOS).
See this image and copyright information in PMC

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