Section 10 - Cystic Fibrosis Clinical Care Pathway

7.2 Liver Disease
Introduction
Pathological changes of liver disease such as bile plugging of the smaller
biliary ducts and scattered areas of fibrosis, pericholangitis and dilatation of
the ductules occurs in the liver of many young children with CF and may
even be present to a minor degree at birth. These changes progress slowly,
variably and unpredictably towards focal biliary cirrhosis. Eventually portal
hypertension often with oesophageal varices and hepatic failure intervenes.
Ascites, hypersplenism and gall stones are additional manifestations of hepatic
disease. Symptomatic liver disease is seen in 4% of patients only, it peaks at
16-20 years of age (9%) and is twice as common in males.
Hepatic failure is only occasionally seen in the paediatric clinic but post
mortem studies have found that over 50% of patients have liver cirrhosis. CFrelated
liver disease is an increasingly important cause of portal hypertension
and multilobular cirrhosis in the young adult.
Screening for liver disease
• Clinical examination:
Hepatomegaly - is likely to represent fibrosis/cirrhosis if hard
and irregular especially if the left lobe is
prominent in the epigastrium.
- is likely to be due to fatty infiltration if the
liver enlargement is smooth, regular and
both lobes are equally involved, especially
if present in a malnourished child.
- beware of apparent liver enlargement due
to hyperinflated chest, especially in
younger children.
Splenomegaly - almost all patient with varices will have
splenomegaly and documentation of its
presence is essential in screening for portal
hypertension.
• Liver function tests: Mild biochemical abnormalities are often
detected early in life but are of little
clinical significance and bear no relation to
portal pressure especially with normal
clinical findings. Elongation of the
prothrombin time is more likely to
represent vitamin K malabsorption than
hepatic malfunction. There may be some
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