Arch Dis Child 1999;81:129-132 ( August )
The evolution of liver disease in cystic fibrosis
S C Linga, J D Wilkinsonc, A S Hollmanc, J McCollb, T J Evansc, J Y Patona
a Department
of Child Health, University of Glasgow, Yorkhill NHS Trust, Glasgow G3
8SJ, UK, b Department of Statistics, University of Glasgow, c Royal Hospital for Sick
Children, Yorkhill NHS Trust, Glasgow G3 8SJ, UK
Correspondence to: Dr Ling.
email: lingsimon{at}hotmail.com
Accepted 30 April 1999
OBJECTIVES
To describe
prospectively the evolution of liver abnormalities in cystic fibrosis
(CF), and to assess their impact on nutritional status.
STUDY DESIGN124
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.
RESULTSAt 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.
CONCLUSIONSHepatic
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.
Keywords:
cystic fibrosis;
liver disease;
ultrasonography;
nutritional status
© 1999 by Archives of Disease in Childhood
