scholarly journals Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors

2013 ◽  
Vol 5 ◽  
pp. 290-294
Author(s):  
Sabina Więcek ◽  
Halina Woś ◽  
Urszula Grzybowska-Chlebowczyk
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Immunological Factors ◽  
Liver Changes

Evaluation of an oral ursodeoxycholic acid load in the assessment of bile acid malabsorption in cystic fibrosis

1983 ◽  
Vol 28 (4) ◽  
pp. 306-311 ◽  
Author(s):  
Carla Colombo ◽  
Aldo Roda ◽  
Enrico Roda ◽  
Lucia Piceni Sereni ◽  
Daniela Maspero ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Ursodeoxycholic Acid ◽  
Bile Acid Malabsorption ◽  
Acid Load

scholarly journals Bile acid homeostasis in gastrointestinal and metabolic complications of cystic fibrosis

2019 ◽  
Vol 18 (3) ◽  
pp. 313-320 ◽  
Author(s):  
Ivo P. van de Peppel ◽  
Frank A.J.A. Bodewes ◽  
Henkjan J. Verkade ◽  
Johan W. Jonker
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Metabolic Complications

scholarly journals Defective FXR-FGF15 signaling and bile acid homeostasis in cystic fibrosis mice can be restored by the laxative polyethylene glycol

2019 ◽  
Vol 316 (3) ◽  
pp. G404-G411 ◽  
Author(s):  
Anna Bertolini ◽  
Ivo P. van de Peppel ◽  
Marcela Doktorova-Demmin ◽  
Frank A. J. A. Bodewes ◽  
Hugo de Jonge ◽  
...  
Keyword(s):  
Gene Expression ◽  
Cystic Fibrosis ◽  
Polyethylene Glycol ◽  
Bile Acid ◽  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Farnesoid X Receptor ◽  
Fibroblast Growth ◽  
Gastrointestinal Complications ◽  
Osmotic Laxative

The gastrointestinal phenotype of cystic fibrosis (CF) features intestinal bile acid (BA) malabsorption, impaired intestinal farnesoid X receptor (FXR) activation, and consequently reduced fibroblast growth factor 19 (FGF19, FGF15 in mice) production. The osmotic laxative polyethylene glycol (PEG) has been shown to decrease intestinal mucus accumulation in CF mice and could, by doing so, improve BA reabsorption. Here we determined the effect of PEG on BA excretion and FXR-FGF15 signaling in CF mice. Male Cftr−/−tm1Unc (CF) and wild-type (WT) littermates were administered PEG 4000 in drinking water and fed either chow or a semisynthetic diet. PEG was withdrawn for 3 days before termination. Fecal BA excretion was measured at PEG dosages of 37 g/l (100%) and 0 g/l (0%). Ileal FXR activation was assessed by gene expression of its downstream targets Fgf15 and small heterodimer partner ( Shp). In CF mice, PEG withdrawal increased fecal BA excretion on either diet compared with full PEG dosage (chow, 2-fold, P = 0.06; semisynthetic, 4.4-fold, P = 0.007). PEG withdrawal did not affect fecal BA excretion in WT mice on either diet. After PEG withdrawal, gene expression levels of intestinal FXR target genes Fgf15 and Shp were decreased in CF mice but unaffected in WT littermates. PEG did not affect the gene expression of the main intestinal BA transporter apical sodium-dependent bile acid transporter (ASBT). PEG treatment ameliorates intestinal BA malabsorption in CF mice and restores intestinal FXR-FGF15 signaling, independent from Asbt gene expression. These findings highlight the potential of PEG in the prevention and treatment of the gastrointestinal phenotype of CF. NEW & NOTEWORTHY A gastrointestinal feature of cystic fibrosis is bile acid malabsorption and consequent impairment of farnesoid X receptor (FXR)-fibroblast growth factor 15 (FGF15) signaling. FXR-FGF15 signaling regulates various metabolic processes and could be implicated in metabolic and gastrointestinal complications of cystic fibrosis, such as diabetes and liver disease. In cystic fibrosis mice, treatment with the osmotic laxative polyethylene glycol is associated with decreased fecal bile acid loss and restoration of FXR-FGF15 signaling.

scholarly journals Bile Acid Malabsorption in Cystic Fibrosis; Membrane Vesicles, a Tool for Revealing the Role of the Ileal Brush Border Membrane

1985 ◽  
Vol 74 (s317) ◽  
pp. 28-30 ◽  
Author(s):  
F. W. M. ROOIJ ◽  
J. W. O. BERG ◽  
M. SINAASAPPEL ◽  
E. P. BOSMAN-JACOBS ◽  
A. C. TOUW-BLOMMESTEIJN
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Brush Border ◽  
Brush Border Membrane ◽  
Membrane Vesicles ◽  
Bile Acid Malabsorption

scholarly journals Aspects of bile acid metabolism in cystic fibrosis

1975 ◽  
Vol 50 (10) ◽  
pp. 769-778 ◽  
Author(s):  
M. C. Goodchild ◽  
G. M. Murphy ◽  
A. M. Howell ◽  
S. A. Nutter ◽  
C. M. Anderson
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Acid Metabolism ◽  
Bile Acid Metabolism

scholarly journals WS02.4 Metabolomic profiling suggests early bile acid changes in cystic fibrosis (CF)

2016 ◽  
Vol 15 ◽  
pp. S3-S4
Author(s):  
M.S. Muhlebach ◽  
C. O'Leary ◽  
S.M. Stick ◽  
W. Sha
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Metabolomic Profiling

scholarly journals BILE ACID KINETICS AND FECAL PATTERNS IN CYSTIC FIBROSIS

1974 ◽  
Vol 8 (4) ◽  
pp. 385-385
Author(s):  
C C Roy ◽  
A M Weber ◽  
L Chartrand ◽  
G Lepage ◽  
C L Morin ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid

scholarly journals Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption.

Gut ◽  
1986 ◽  
Vol 27 (9) ◽  
pp. 1043-1048 ◽  
Author(s):  
Z Weizman ◽  
P R Durie ◽  
H R Kopelman ◽  
S M Vesely ◽  
G G Forstner
Keyword(s):  
Cystic Fibrosis ◽  
Bile Acid ◽  
Acid Secretion ◽  
Fat Malabsorption ◽  
Bile Acid Secretion
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