Retrospective review of cystic fibrosis presenting as infantile liver disease
Riki Shapiraa, Nedim Had
i
a, Ruggiero Francavillaa, George Koukulisb, John F Pricea, Giorgina Mieli-Vergania
a Department of Child
Health, King's College Hospital, Denmark Hill, London SE5 9RJ, UK, b Institute of Liver Studies, King's College
Hospital
Correspondence to: Dr Hadzic. email: nedim.hadzic{at}kcl.ac.uk
Accepted 6 January
1999
The mode of presentation, clinical course, and outcome of 12 infants with cystic fibrosis and liver disease referred over an 18 year
period were investigated retrospectively. Median age at presentation
was 6.5 weeks (range, 5-12). Two thirds were boys. Conjugated
hyperbilirubinaemia was the presenting symptom in 11 patients, and
hypoalbuminaemia in one. Jaundice was cleared over a median period of
7.36 months. Eight patients had bile duct proliferation on liver biopsy
and one required cholangiography to exclude biliary atresia. Classic
histological features of cystic fibrosis were only present in two
children biopsied at 8 and 18 months. Three patients had meconium
ileus, including one infant with concomitant 
1
antitrypsin deficiency, who required early liver transplantation. All
other patients had no signs of significant chronic liver disease during
a median follow up of 42 months (range, 10-205). Children with cystic
fibrosis and infantile liver disease have a good short and medium term prognosis.
Keywords: cystic fibrosis; conjugated hyperbilirubinaemia; chronic liver disease
© 1999 by Archives of Disease in Childhood
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