Hepatobiliary Disease in Cystic Fibrosis

Objective To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA). Case Summary Two infants with cystic fibrosis (CF)-associated hepatobiliary disease, manifesting as cholestatic jaundice and elevated liver enzymes within the first 6 weeks of life, had improved biochemical indices of liver function following treatment with UDCA 20–40 mg/kg/d. Discussion To our knowledge, this is the first report of UDCA treatment in infants with CF-associated cholestatic jaundice. Infants and children require treatment with increased doses of UDCA to compensate for reduced intestinal absorption of bile acid and immaturity of the enterohepatic circulation. Conclusions UDCA appears to be a cost-effective treatment for CF-associated hepatobiliary disease in infants and children.

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Hepatobiliary disease in children and adolescents with cystic fibrosis

Jornal de Pediatria (Versão em Português) ◽

10.1016/j.jpedp.2017.09.019 ◽

2018 ◽

Vol 94 (5) ◽

pp. 504-510

Author(s):

Fernanda de S. Nascimento ◽

Nelson A. Sena ◽

Tatiane da A. Ferreira ◽

Cibele D.F. Marques ◽

Luciana R. Silva ◽

...

Keyword(s):

Cystic Fibrosis ◽

Children And Adolescents ◽

Hepatobiliary Disease

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Hepatobiliary disease in children and adolescents with cystic fibrosis

Jornal de Pediatria ◽

10.1016/j.jped.2017.07.006 ◽

2018 ◽

Vol 94 (5) ◽

pp. 504-510 ◽

Cited By ~ 2

Author(s):

Fernanda de S. Nascimento ◽

Nelson A. Sena ◽

Tatiane da A. Ferreira ◽

Cibele D.F. Marques ◽

Luciana R. Silva ◽

...

Keyword(s):

Cystic Fibrosis ◽

Children And Adolescents ◽

Hepatobiliary Disease

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Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors

The Journal of Pediatrics ◽

10.1067/s0022-3476(03)00329-9 ◽

2003 ◽

Vol 143 (3) ◽

pp. 343-350 ◽

Cited By ~ 44

Author(s):

Catherine Lenaerts ◽

Chantale Lapierre ◽

Heidi Patriquin ◽

Nathalie Bureau ◽

Guy Lepage ◽

...

Keyword(s):

Cystic Fibrosis ◽

Predisposing Factors ◽

Hepatobiliary Disease

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Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology

AJP Gastrointestinal and Liver Physiology ◽

10.1152/ajpgi.00146.2014 ◽

2015 ◽

Vol 308 (6) ◽

pp. G459-G471 ◽

Cited By ~ 26

Author(s):

Alicia K. Olivier ◽

Katherine N. Gibson-Corley ◽

David K. Meyerholz

Keyword(s):

Cystic Fibrosis ◽

Gastrointestinal Tract ◽

Animal Models ◽

Liver Diseases ◽

Multiple Organ ◽

Hepatobiliary Disease ◽

Future Directions ◽

Organ Systems

Multiple organ systems, including the gastrointestinal tract, pancreas, and hepatobiliary systems, are affected by cystic fibrosis (CF). Many of these changes begin early in life and are difficult to study in young CF patients. Recent development of novel CF animal models has expanded opportunities in the field to better understand CF pathogenesis and evaluate traditional and innovative therapeutics. In this review, we discuss manifestations of CF disease in gastrointestinal, pancreatic, and hepatobiliary systems of humans and animal models. We also compare the similarities and limitations of animal models and discuss future directions for modeling CF.

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