Cystic fibrosis liver disease in adults: Limits of noninvasive tests of fibrosis

Hillaire, Sophie; Cazals–Hatem, Dominique; Erlinger, S; Paradis, Valérie

doi:10.1002/hep.29637

Cited by 3 publications

(2 citation statements)

References 3 publications

(7 reference statements)

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“…The complications were mostly cirrhosis and portal hypertension, as shown by others . As recently described, we also observed a subset of patients with portal hypertension but no cirrhosis . Differences in biochemical characteristics in patients with CFLD and severe CFLD were consistent with aggravation of the disease (Supporting Table ).…”

Section: Discussionmentioning

confidence: 98%

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

et al. 2018

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CFLD occurs not only during childhood but also later in the lifetime of patients with CF; male sex, CFTR F508del homozygosity, and history of meconium ileus are independent risk factors for CFLD development; earlier use of UDCA over the last 20 years has not changed the incidence of severe CFLD, leading to questions about the use of this treatment in young children given its possible adverse effects. This article is protected by copyright. All rights reserved.

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Section: Discussionmentioning

confidence: 98%

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

et al. 2018

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“…One of the most common cholangiopathies, cystic fibrosis (CF), is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) that encodes a chloride ion channel expressed by the cholangiocyte and responsible for the regulation of bile secretion. Mutations in CFTR result in the deposition of viscous, acidic bile that is toxic to the surrounding hepatocytes, leading to liver damage and organ failure 11, 12 . As the life expectancy of CF patients increases with the availability of new treatments that improve lung function, cystic fibrosis liver disease (CFLD) is emerging as a major cause of the morbidity and mortality in these patients 11 .…”

Section: Introductionmentioning

confidence: 99%

Generation of functional ciliated cholangiocytes from human pluripotent stem cells

Ogawa

Jiang

Xia

et al. 2021

Preprint

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The derivation of mature functional cholangiocytes from human pluripotent stem cells (hPSCs) would provide a model for studying the pathogenesis of cholangiopathies and for developing novel therapies to treat them. Current differentiation protocols are not efficient and give rise to cholangiocytes that are not fully mature, limiting their therapeutic applications. Here, we describe a new strategy to generate functional hPSC-derived cholangiocytes that display many characteristics of mature bile duct cells including high levels of CFTR and the presence of primary cilia capable of sensing flow. With this level of maturation, these cholangiocytes are amenable for testing the efficacy of new cystic fibrosis drugs and for studying the role of cilia in cholangiocyte development and function. Transplantation studies showed that the mature cholangiocytes generate ductal structures in the liver of immunocompromised mice providing the first indication that it may be possible to develop cell-based therapies to restore bile duct function in patients with biliary disease.

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Generation of functional ciliated cholangiocytes from human pluripotent stem cells

et al. 2021

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The derivation of mature functional cholangiocytes from human pluripotent stem cells (hPSCs) provides a model for studying the pathogenesis of cholangiopathies and for developing therapies to treat them. Current differentiation protocols are not efficient and give rise to cholangiocytes that are not fully mature, limiting their therapeutic applications. Here, we generate functional hPSC-derived cholangiocytes that display many characteristics of mature bile duct cells including high levels of cystic fibrosis transmembrane conductance regulator (CFTR) and the presence of primary cilia capable of sensing flow. With this level of maturation, these cholangiocytes are amenable for testing the efficacy of cystic fibrosis drugs and for studying the role of cilia in cholangiocyte development and function. Transplantation studies show that the mature cholangiocytes generate ductal structures in the liver of immunocompromised mice indicating that it may be possible to develop cell-based therapies to restore bile duct function in patients with biliary disease.

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Cystic fibrosis liver disease in adults: Limits of noninvasive tests of fibrosis

Cited by 3 publications

References 3 publications

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

Generation of functional ciliated cholangiocytes from human pluripotent stem cells

Generation of functional ciliated cholangiocytes from human pluripotent stem cells

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