Overt liver disease in cystic fibrosis is a rare condition. Only 1–5 % of all patients show a severe disturbance of the liver cell function or portal hypertension. In contrast, liver architecture is much more often disturbed at post mortem examination. The experience is that liver
pathology increases with age which will result in rising numbers of patients in the future parallel to the increasing life expectancy of the patients. Bile plugs are commonly found in the portal tract and probably represent the essential abnormality of the liver in CF. Recently new methods
have been developed for the investigation of the bile synthesis which will be helpful in the understanding of the CF defect in the liver.
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Document Type: Research Article
From the Department of Paediatrics, Subdivision Gastroenterology, University Hospital/Sophia Children's Hospital and Erasmus University Rotterdam, Rotterdam, The Netherlands
November 1, 1989