The evolution of liver disease in cystic fibrosis.
OBJECTIVE
To describe prospectively the evolution of liver abnormalities in cystic fibrosis (CF), and to assess their impact on nutritional status.


METHODS
124 children (61 boys) with CF (median age, 5.4 years; range, 0.1-13.9) were followed longitudinally for a median of four years. Annual clinical examination, biochemistry, and ultrasound assessment were performed. Chrispin-Norman score, anthropometry, and bacterial colonisation of airway secretions were measured at each assessment.


RESULTS
At initial assessment, 45% of the patients had no liver abnormalities, 42% had biochemical abnormality, 35% ultrasound abnormality, and 6% had clinical abnormality of the liver. In this cross sectional analysis, abnormal biochemistry was present in 40% of children with ultrasound or clinical abnormalities, but when longitudinal follow up data were analysed, abnormal biochemistry preceded or coincided with abnormal ultrasound or clinical hepatosplenomegaly in three quarters of 53 children developing new abnormalities. Eighty four of 124 children (68%) showed ultrasound or clinical evidence of liver abnormality at some point during the four years of follow up. No association was found between liver disease and nutritional status.


CONCLUSIONS
Hepatic abnormality was common in this group of children with CF, was often predicted by intermittent biochemical abnormalities, and was not associated with deterioration in nutritional status.
Ling
S C
SC
Department of Child Health, University of Glasgow, Yorkhill NHS Trust, Glasgow G3 8SJ, UK. lingsimon@hotmail.com
Wilkinson
J D
JD
Hollman
A S
AS
McColl
J
J
Evans
T J
TJ
Paton
J Y
JY
D000293:Adolescent; D001424:Bacterial Infections / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000201:enzymology / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000201:enzymology / Q000209:etiology* / Q000473:pathology; D008137:Longitudinal Studies; D008297:Male; D000637:Transaminases / Q000378:metabolism
D000637:Transaminases