Search tips
Search criteria 


Logo of gutGutView this articleSubmit a manuscriptReceive email alertsContact usBMJ
Gut. 1978 June; 19(6): 514–520.
PMCID: PMC1412022

Congenital hepatic fibrosis: the long-term prognosis.


The long-term prognosis in congenital hepatic fibrosis has been assessed in 30 patients, 13 treated at Newcastle and 17 from other British centres. Twenty-four patients had been followed-up for more than five years from diagnosis. Shunt operations, performed in 18 patients, were successful in controlling haemorrhage with a low mortality (1/18), low incidence of recurrent haemorrhage (3/18) and portal-systemic encephalopathy (3/18) but with a higher incidence of postoperative jaundice (7/18). About a third of the survivors had some evidence of hepatic dysfunction but none had chronic portal systemic encephalopathy. Intelligence quotient was close to normal but educational achievement and job status were low. Serum albumin fell after shunt surgery and a few patients developed ascites: anaemia and hypersplenism however, were corrected by surgery. Blood ammonia is raised in survivors with congenital hepatic fibrosis and rises further after a normal meal; further observations are needed on cerebral function after several decades of survival.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (962K), or click on a page image below to browse page by page.

Articles from Gut are provided here courtesy of BMJ Group