Arch Dis Child 1991 Jun;66(6):698-701

Prevalence of liver disease in cystic fibrosis

Scott-Jupp R, Lama M, Tanner MS
Department of Child Health, University of Leicester.

Abstract
To assess the prevalence, demography, and clinical features of liver disease
among patients with cystic fibrosis the case notes of 524 patients of all ages
who were attending the cystic fibrosis clinic were studied. Computer databases
were used to establish the condition of the liver in a further 576 such
patients. The overall prevalence of overt liver disease indicated by the
presence of an enlarged liver or spleen (or both) was 4.2%. The age related
prevalence rose to a peak in adolescence, and then fell in patients over 20
years old. The implied increase in mortality among those with liver disease was
not explained by deaths from liver disease, which were rare. Male patients were
significantly more affected than female, the ratio being 3:1 among adolescents.
Increasing prevalence of liver disease in patients with cystic fibrosis is,
therefore, not just a result of longevity