Liver Disease in Cystic Fibrosis

@article{Colombo2006LiverDI,
  title={Liver Disease in Cystic Fibrosis},
  author={Carla Colombo and Maria Chiara Russo and Laura Zazzeron and Giovanna Romano},
  journal={Journal of Pediatric Gastroenterology and Nutrition},
  year={2006},
  volume={43},
  pages={S49-S55}
}
Liver involvement in Cystic Fibrosis (CF) is much less frequent than both pulmonary and pancreatic diseases that are present in 80-90% of CF patients; liver disease (LD) affects only one third of CF patients, however, because of the decreasing mortality from extrahepatic causes, its recognition and management is becoming a relevant clinical issue. Recent observations suggest that clinical expression of LD in CF may be influenced by genetic modifiers; their identification is an important issue… 
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Cystic fibrosis-associated liver disease.
TLDR
Molecular and clinical genetics of CF, including genetic modifiers of CF-associated liver disease, are reviewed, and practical recommendations for genetic testing, diagnosis and treatment of hepatobiliary manifestations in CF are provided.
Nutrition and Cystic Fibrosis Related Liver Disease
TLDR
This chapter provides an overview of liver involvement in CF, address the disorders that occur in the liver as a result of nutritional deficiencies, and address the nutritional evaluation and management of the two liver diseases in CF that directly affect nutritional status: cholestasis and cirrhosis.
PERSPECTIVES IN CLINICAL GASTROENTEROLOGY AND HEPATOLOGY Gastrointestinal Complications of Cystic Fibrosis
TLDR
This review outlines pathophysiology and common gastrointestinal ailments in the CF population along with current medical and surgical management and proposes a therapeutic plan to address specific clinical entities.
Liver Changes in the Course of Cystic Fibrosis
TLDR
The aim of the chapter was to review the risk factors, clinical symptoms, diagnostic methods, and treatment of liver changes in the course of cystic fibrosis.
Unexpected diagnosis of cystic fibrosis at liver biopsy: a report of four pediatric cases
TLDR
In four cases of pediatric patients in whom the diagnosis of cystic fibrosis was made only after the histological examination of a liver specimen obtained by biopsy or at autopsy, the diagnosis was confirmed by the existence of known pathogenic mutations in the CFTR gene.
Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands.
  • F. Pals, H. Verkade, +8 authors F. Bodewes
  • Medicine
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
  • 2019
TLDR
In the Netherlands, the presence of CFC is associated with a higher risk for early mortality and an approximately 10-year lower median age at death and this substantial poorer outcome was not reflected in a lower baseline lung function or a diminished nutritional status.
TIMP-1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease.
  • T. Rath, K. Menendez, +7 authors M. Roderfeld
  • Medicine
    Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
  • 2012
TLDR
Transient elastography was increased in adults and children with CF hepatopathy compared to those without and exhibited a high diagnostic accuracy for CF liver disease and TIMP-2 represent powerful biomarkers for CF associated Liver disease and portal hypertension.
The cystic fibrosis intestine.
TLDR
The effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment are described and mouse models of CF have greatly advanced understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans.
EASL Clinical Practice Guidelines: Autoimmune hepatitis.
Autoimmune hepatitis (AIH) was the first liver disease for which an effective therapeutic intervention, corticosteroid treatment, was convincingly demonstrated in controlled clinical trials. However,…
Indications for pediatric liver transplantation. Data from the Heidelberg pediatric liver transplantation program.
  • G. Engelmann, J. Schmidt, +6 authors J. Meyburg
  • Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2007
TLDR
Indications for liver transplantation in infants and children, including acute liver failure, chronic liver failure with pruritus, complications of cholestasis and failure to thrive, and the occurrence of complications of liver disease are reviewed.
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