Epidemiology of liver disease in cystic fibrosis: a longitudinal study

Lamireau, T.; Monnereau, Sylvie; Martin, Steven R.; Marcotte, Jacques-Édouard; Winnock, María; Álvarez, Fernando

doi:10.1016/j.jhep.2004.08.006

Cited by 181 publications

(169 citation statements)

References 36 publications

Supporting

Mentioning

141

Contrasting

Unclassified

Order By: Relevance

“…Intriguingly, several epidemiological studies (11,12,35,38), if not all (58), indicate that an association exists between CF-related liver disease and the incidence of intestinal obstructions, suggesting an interrelated pathogenesis. In CF, intestinal obstructions occur predominantly in the distal intestine, both in newborns (MI) and in later life (DIOS).…”

Section: Discussionmentioning

confidence: 99%

Activation of CFTR by ASBT-mediated bile salt absorption

Bijvelds¹,

Jorna²,

Verkade³

et al. 2005

American Journal of Physiology-Gastrointestinal and Liver Physi

View full text Add to dashboard Cite

In cholangiocytes, bile salt (BS) uptake via the apical sodium-dependent bile acid transporter (ASBT) may evoke ductular flow by enhancing cAMP-mediated signaling to the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. We considered that ASBT-mediated BS uptake in the distal ileum might also modulate intestinal fluid secretion. Taurocholate (TC) induced a biphasic rise in the short circuit current across ileal tissue, reflecting transepithelial electrogenic ion transport. This response was sensitive to bumetanide and largely abrogated in Cftr-null mice, indicating that it predominantly reflects CFTR-mediated Cl−secretion. The residual response in Cftr-null mice could be attributed to electrogenic ASBT activity, as it matched the TC-coupled absorptive Na+flux. TC-evoked Cl−secretion required ASBT-mediated TC uptake, because it was blocked by a selective ASBT inhibitor and was restricted to the distal ileum. Suppression of neurotransmitter or prostaglandin release, blocking of the histamine H1receptor, or pretreatment with 5-hydroxytryptamine did not abrogate the TC response, suggesting that neurocrine or immune mediators of Cl−secretion are not involved. Responses to TC were retained after carbachol treatment and after permeabilization of the basolateral membrane with nystatin, indicating that BS modulate CFTR channel gating rather than the driving force for Cl−exit. TC-induced Cl−secretion was maintained in cGMP-dependent protein kinase II-deficient mice and only partially inhibited by the cAMP-dependent protein kinase inhibitor H89, suggesting a mechanism of CFTR activation different from cAMP or cGMP signaling. We conclude that active BS absorption in the ileum triggers CFTR activation and, consequently, local salt and water secretion, which may serve to prevent intestinal obstruction in the postprandial state.

show abstract

Section: Discussionmentioning

confidence: 99%

Activation of CFTR by ASBT-mediated bile salt absorption

Bijvelds¹,

Jorna²,

Verkade³

et al. 2005

American Journal of Physiology-Gastrointestinal and Liver Physi

View full text Add to dashboard Cite

show abstract

“…neonatal intestinal obstruction), and more than 80% develop pancreatic insufficiency (2). While both intestinal and pancreatic dysfunctions contribute to malnutrition, there is evidence to indicate that the nutritional status may influence liver and lung injury in these patients (3)(4)(5). CF mice with a complete exon-10 knockout of the CFTR gene (Cftr tm1Unc ) (6) develop severe CF-like intestinal obstruction.…”

mentioning

confidence: 99%

Impact of Nutrition on Phenotype in CFTR-deficient Mice

et al. 2007

View full text Add to dashboard Cite

“…In this aspect, an epidemiologic cohort study has illustrated a prevalence of 41% of liver disease in CF patients which mainly occurs in the first decade of life (23). Besides, Ling et al have reported a prevalence of 42% abnormality in biochemical and 35% in US findings of liver in 124 CF children of the UK at the initial assessment, in which by longitudinal follow up; abnormal biochemistry preceded or coincided with clinical or US abnormality in 74% developed new abnormalities (22).…”

Section: Discussionmentioning

confidence: 99%

Liver Involvement in Iranian Children With Cystic Fibrosis: Ultrasonography and Biochemical Findings

et al. 2013

View full text Add to dashboard Cite

Excellence in Tehran, Iran. In all, 114 patients with cystic fibrosis (70 boys, 44 girls) were enrolled. Sample blood test including AST, ALT, GGT and abdominal Sonography was obtained from all patients. Abnormal liver function test was defined by two consecutive occasions; ALT and/ or AST levels were ≥ 2 times the upper limit of normal values. GGT normal values were defined by patient age. Data were analyzed using χ2 test and independent T test. Statistical significance was defined as P values of < 0.05 by SPSS ver.19 software. Results: Abnormal liver function test was detected in CF patients. As well, liver sonogram was abnormal in approximately one-third of the patients. This study showed a higher prevalence of biochemical abnormality in patients with abnormal livers ultrasonography. Conclusions: Noninvasive paraclinical evaluation methods could be recommended in the patients with CF for early detection of silent liver abnormalities before progression to end stage liver disease.

show abstract

Epidemiology of liver disease in cystic fibrosis: a longitudinal study

Cited by 181 publications

References 36 publications

Activation of CFTR by ASBT-mediated bile salt absorption

Activation of CFTR by ASBT-mediated bile salt absorption

Impact of Nutrition on Phenotype in CFTR-deficient Mice

Liver Involvement in Iranian Children With Cystic Fibrosis: Ultrasonography and Biochemical Findings

Contact Info

Product

Resources

About