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CF-related liver disease

The problem

Ultrasound changes of the liver are common in children with CF - upto 60% will show show change in liver echogenicity. Most ultrasound abnormality will be a fatty liver (steatosis) - the cause of which is unknown and the consequence is uncertain. Upto 25% of patients will have more significant changes which can be identified as cirrhosis, which if it does occur will be present usually by the time the patient is 20 years old. Of those with changes of cirrhosis, significant health problems will arise in about 1 in 10 (around 2% of the entire CF population).

Diagnosis

We carry out annual liver ultrasound as well as annual LFTs and clotting. Ultrasound is the most sensitive test for CF-related liver disease.

Treatment

If the liver ultrasound is abnormal for 2 consequtive years, we start ursodeoxycholic acid 10mg/kg bd. The commonest side effect is diarrhoea. In established liver disease, the dose can be increased to 15mg/kg bd.

Complications

The most important complication of CF-related liver disease is the development of cirrhosis with portal hypertension and oesophageal varices. European best practice guidelines published in 2011 (Prof Deirdre Kelly from Birmingham liver unit was a co-author) state: "All patients with cirrhosis and splenomegaly and/or signs of hypersplenism should be screened for oesophageal varices at diagnosis by means of upper gastrointestinal endoscopy. If normal, it should be repeated every 2–3 years. Once oesophageal varices have developed, it is prudent to perform an upper GI endoscopy once a year and treat large varices (by banding). Any signs suggestive of possible GI bleeding (anemia, microcytosis, chronic iron depletion) should also prompt an upper GI endoscopy. Some patients may develop rectal varices and present with rectal bleeding".

In practice this means that children in the OCCFN identified as having cirrhotic (nodular) changes on ultrasound and evidence of portal hypertension (usually splenomegaly with or without hypersplenism) should be referred for review by Birmingham Liver Unit. Older teenagers (>16 years) can be referred to the adult hepatology team at the OUH - Dr Jane Collier

The rate of progression of CF-related liver disease is very variable.

The full guideline "Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease" can be downloaded here .