Intestinal Permeability in Cystic Fibrosis in Relation to Genotype

Hallberg, K.; Grzegorczyk, Adam; Larson, Göran; Strandvik, Birgitta

doi:10.1097/00005176-199709000-00008

Cited by 33 publications

(26 citation statements)

References 32 publications

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“…Actual fat absorption is less than intake because, even with enzyme supplementation, 5-20% of dietary fat is not absorbed in persons with CF (25). The ratio of absorbed fat to carbohydrate in CF may be further altered by abnormalities in carbohydrate absorption, because enhanced jejunal absorption of glucose (26) and other sugars (27) has been shown. Thus, the combination of decreased fat and increased glucose absorption in CF may lead to a postprandial metabolic milieu similar to that found in individuals consuming a highcarbohydrate, low-fat diet, and might be an explanation for isolated hypertriglyceridemia.…”

Section: Discussionmentioning

confidence: 99%

Abnormal lipid concentrations in cystic fibrosis

Figueroa¹,

Milla²,

Parks³

et al. 2002

The American Journal of Clinical Nutrition

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Section: Discussionmentioning

confidence: 99%

Abnormal lipid concentrations in cystic fibrosis

Figueroa¹,

Milla²,

Parks³

et al. 2002

The American Journal of Clinical Nutrition

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“…Recently, studies have suggested that pathological modifications in EFAD may be the consequence of cell adhesion disorders [200]. …”

Section: Intraluminal Abnormalitiesmentioning

confidence: 99%

Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency

et al. 2005

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Transport mechanisms, whereby alimentary lipids are digested and packaged into small emulsion particles that enter intestinal cells to be translocated to the plasma in the form of chylomicrons, are impaired in cystic fibrosis. The purpose of this paper is to focus on defects that are related to intraluminal and intracellular events in this life-limiting genetic disorder. Specific evidence is presented to highlight the relationship between fat malabsorption and essential fatty acid deficiency commonly found in patients with cystic fibrosis that are often related to the genotype. Given the interdependency of pulmonary disease, pancreatic insufficiency and nutritional status, greater attention should be paid to the optimal correction of fat malabsorption and essential fatty acid deficiency in order to improve the quality of life and extend the life span of patients with cystic fibrosis.

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“…Patients with CF exhibit intestinal inflammation (De Lisle and Borowitz 2013;Werlin et al 2010), and intestinal permeability is also increased (Hallberg et al 1997;van Elburg et al 1996). My lab has shown that increased intestinal permeability also occurs in the widely used mouse model of CF, the Cftr knockout mouse (CF mouse) (De Lisle et al 2011).…”

Section: Introductionmentioning

confidence: 99%

Disrupted tight junctions in the small intestine of cystic fibrosis mice

Lisle

2013

Cell Tissue Res

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The tight junction (TJ) is the major determinant of paracellular permeability, which in the gut protects the body from entry of harmful substances such as microbial components. In cystic fibrosis (CF) there is increased permeability of the small intestine both in humans and in CF mice. To gain insight into the mechanisms of increased intestinal permeability in CF, I analyzed the composition of the TJ in the cystic fibrosis transmembrane conductance regulator (Cftr) knockout mouse model. Significant changes in TJ gene expression in the CF intestine were found for Cldn1, Cldn7, Cldn8, and Pmp22 which were expressed at lower levels; and Cldn2 which was expressed at a higher level. Protein levels of claudin-2 were increased in the CF intestine as compared to wild type, while other TJ proteins were not significantly different. In the villus epithelium of the CF intestine, all TJ components analyzed were mislocalized to the basal cytoplasm and showed varying degrees of loss from the TJ and apico-lateral surfaces. The pore-forming claudin-2 in the CF intestine showed more intense staining but was correctly localized to the TJ, principally in the crypts which are enlarged in CF. The cytokine TNFα, known to affect TJ, was elevated to 160% of wild type in the CF intestine. In summary, there is a dramatic redistribution of claudin proteins from the TJ/lateral membrane to the basal cytoplasm of the villus epithelium in the CF intestine. These changes in TJ protein localization in CF are likely to be involved in the increased permeability of the CF small intestine to macromolecules, and TNFα may be a causative factor.

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Intestinal Permeability in Cystic Fibrosis in Relation to Genotype

Abstract: These data show that the IP in CF was related to patient genotype; those homozygozous or heterozygous for delta F508 having a significantly increased IP compared with patients with unidentified genotypes, who had IP values within the normal range.

Cited by 33 publications

References 32 publications

Abnormal lipid concentrations in cystic fibrosis

Abnormal lipid concentrations in cystic fibrosis

Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency

Disrupted tight junctions in the small intestine of cystic fibrosis mice

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