Understanding the pathogensis of liver disease in patients with intestinal failure

Project Details

The most common cause of death in patients with short bowel syndrome is sepsis in the setting of severe intestinal failure-associated liver disease.Liver disease occurs in ~65% of infants requiring long-term parenteral nutrition therapy following small bowel resection and is the cause of death in 3-19% of SBS infants. However, despite this, the cause and the complex mechanisms underlying the development of IFALD are poorly understood. Patients with SBS are a complex, heterogeneous group and access to tissue is limited, therefore the development of a relevant preclinical model is needed if we are to advance knowledge of the pathogenesis of liver disease in patients with short bowel syndrome. The primary aim of this work is to improve the clinical outcome of patients with short bowel syndrome.

Collaborators

  • Hudson Institute of Medical Rseaerch
  • University College Cork

Funding

  • National Health and Medical Research Council

Research Publications

  • PERIERA-FANTINI P, BINES JE, LAPTHORNE S, FOUHY F, SCURR M, COTTER P, GAHAN C, JOYCE S.
    Short bowel syndrome (SBS)- associated alterations within the gut-liver axis evolve early and persist long-term in the  piglet model of short bowel syndrome. Journal of Gastroenterology and Hepatology 2016: 31 (12): 1946-1955. (PMID:27037739).  
  • PEREIRA-FANTINI PM, LAPTHORNE S, JOYCE SA, GAHAN C, FULLER PJ, BINES JE.
    FXR agonist treatment alters bile acid metabolism but exacerbates liver damage in a piglet model of short bowel syndrome. Cellular and Molecular Gastroenterology and Hepatology. 2016 (in press)
  • PEREIRA-FANTINI PM, BYARS SG, PITT J, LAPTHORNE S, FOUHY F, COTTER PD, BINES JE.
    Defining the metabolic impact of microbial dysbiosis in a piglet model of short-bowel syndrome-associated liver disease (SBS-ALD)Scientific Reports (in press)
  • LAPTHORNE S, BINES JE, FOUHY F, DELLIOS NL, WILSON G, THOMAS SL, SCURR M, STANTON C, COTTER PD, PEREIRA-FANTINI PM
    Changes in the colon microbiota and intestinal cytokine gene expression following minimal intestinal surgery.
    World Journal of Gastroenterology, 2014; 21(14): 4150-8.
  • PEREIRA-FANTINI P, LAPTHORNE S, JOYCE SA, DELLIOS N, WILSON G, FOUHY F, THOMAS SL, SCURR M, HILL C, GAHAN CG, COTTER PD, FULLER PJ, HARDIKAR W, BINES JE
    Altered FXR signalling is associated with bile acid dysmetabolism in short bowel syndrome-associated liver disease.
    Journal of Hepatology, 2014; 61:1115-1125.

Research Group

Intestinal Failure and Clinical Nutrition Group



Faculty Research Themes

Infection and Immunology



Key Contact

For further information about this research, please contact the research group leader.

Department / Centre

Paediatrics

Unit / Centre

Intestinal Failure and Clinical Nutrition Group