Hepatobiliary Disease in Cystic Fibrosis

Strandvik, Birgitta

doi:10.1002/9780470987049.ch11

Cited by 2 publications

(2 citation statements)

References 141 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The pathophysiological mechanisms that lead to liver disease are yet to be fully elucidated. Proposed mechanisms include altered bile acid metabolism, hepatotoxic drugs, elevated levels of cytokines, biliary obstruction, vitamin deficiencies, essential fatty acid deficiency, and bacterial toxins 21, 22. Studies have shown that the lack of CFTR alters ductular chloride secretion, which results in viscous biliary secretions with subsequent biliary obstruction that leads to focal biliary fibrosis and ultimately cirrhosis 23, 24.…”

Section: Cfmentioning

confidence: 99%

Metabolic liver disease in children

Hansen

Horslen

2008

Liver Transpl

View full text Add to dashboard Cite

show abstract

Section: Cfmentioning

confidence: 99%

Metabolic liver disease in children

Hansen

Horslen

2008

Liver Transpl

View full text Add to dashboard Cite

show abstract

mentioning

confidence: 99%

Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database

Arnon

Annunziato

Miloh

et al. 2011

Pediatric Transplantation

View full text Add to dashboard Cite

A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.

show abstract

Hepatobiliary Disease in Cystic Fibrosis

Cited by 2 publications

References 141 publications

Metabolic liver disease in children

Metabolic liver disease in children

Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database

Contact Info

Product

Resources

About