A single centre experience of liver disease in adults with cystic fibrosis 1995-2006.
BACKGROUND
Liver disease is an important cause of death in adults with cystic fibrosis (CF). Ursodeoxycholic acid (UDCA) may slow progression. Managing varices and timely evaluation for liver transplantation are important.


METHODS
Adults with CF underwent annual review. Abnormalities of liver function tests or ultrasound prompted referral to the CF/liver clinic where UDCA was commenced. Endoscopic surveillance for varices was undertaken if ultrasound suggested portal hypertension.


RESULTS
154 patients were followed for a median 5 years. 43 had significant liver disease, 29 had cirrhosis with portal hypertension and 14 had ultrasound evidence of cirrhosis without portal hypertension. All started UDCA. Only one patient developed chronic liver failure and none required liver transplantation. 27 underwent endoscopy; 1 required variceal banding, the others had insignificant varices. Ultrasound was normal in 97 patients while five had steatosis; nine further patients had splenomegaly but no other evidence of portal hypertension. Neither spleen size nor platelet count correlated with portal hypertension.


CONCLUSIONS
Liver disease was common in adults with CF but disease progression was rare. Thus liver disease detected and closely monitored in adults appeared to have a milder course than childhood CF. Splenomegaly, unrelated to portal hypertension may be a consequence of CF.
Department of Medicine, University of Cambridge, Addenbrooke's Hospital, Hills Road, Cambridge, USA. kathryn.nash@addenbrookes.nhs.uk <kathryn.nash@addenbrookes.nhs.uk>
K L
KL
Nash
M E
ME
Allison
D
D
McKeon
D J
DJ
Lomas
C S
CS
Haworth
D
D
Bilton
Graeme J M
GJ
Alexander
D000328:Adult; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D015897:Comorbidity; D003550:Cystic Fibrosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000453:epidemiology; D008103:Liver Cirrhosis / Q000453:epidemiology; D008107:Liver Diseases / Q000453:epidemiology* / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D010976:Platelet Count; D011446:Prospective Studies; D013163:Splenomegaly; D013921:Thrombocytopenia / Q000453:epidemiology; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid