Variceal hemorrhage and cystic fibrosis: Outcomes and implications for liver transplantation

Citations Over TimeTop 12% of 2005 papers

Abstract

Autopsy and imaging studies show that liver involvement is common in cystic fibrosis. However, complications of chronic liver disease including portal hypertension and variceal bleeding are infrequently encountered, and the degree to which variceal hemorrhage affects prognosis in cystic fibrosis is unclear. This uncertainty has lead to debate as to whether liver transplantation is indicated in these patients. We describe a case series of 18 patients and compare their survival with a control group of cystic fibrosis patients without liver disease. The median age at first bleed was 20.0 years (range 9.7-30.9). The median survival after first bleed was 8.4 years, compared to 13.0 years in the control group (P = 0.15). A total of 14 patients have died, 9 from respiratory disease with no discernable contribution from their liver disease. Liver disease contributed to 4 deaths. Only 1 patient suffered a fatal hemorrhage, which may have been either variceal or bronchial in origin. Long-term survival is a frequent occurrence in patients with cystic fibrosis who suffer variceal hemorrhage, and age at death is comparable to the general cystic fibrosis population. In conclusion, this suggests that liver transplantation is not indicated in these patients without additional features of liver decompensation.

Related Papers

• → Is severe progressive liver disease caused by alpha‐1‐antitrypsin deficiency more common in children or adults?(2016)45 cited
• → Indicators and outcome of liver transplantation in acute liver decompensation after flares of hepatitis B(2010)19 cited
• → Liver disease and anesthesia(1993)1 cited
• → Comparison between MELD and Child- Pugh Scores for liver allocation(2007)
• → Advances in the application of model for end-stage liver disease and MELD-Na scores in liver transplantation(2019)