Cystic fibrosis-associated liver disease: a population-based study.
OBJECTIVE
The aim of this study was to explore the clinical factors associated with the development of cystic fibrosis-associated liver disease (CFALD).


METHODS
This was a case-control study of all children (age 5-18 years) with established CFALD in the Republic of Ireland between January 1999 and June 2000. Each child was pair matched for age and sex with a patient with cystic fibrosis (CF) without evidence of liver disease. Only children with clinically overt liver disease were enrolled in the disease group.


RESULTS
Patients with established CFALD (n = 42; 26 boys) were enrolled. Children with CFALD had worse forced expiratory volume in 1 second values than those without CFALD. However, chest radiography and clinical scores did not differ between groups. Height (mean difference, -4.2 cm [95% confidence interval [CI], -7.41 to -0.90], P =.014), weight (mean difference, -3.21 kg [95% CI, -6.03 to -0.40], P =.026), and mid-upper arm circumference (mean difference, -1.23 cm [95% CI, -2.35 to -0.12], P =.031) were significantly lower among children with CFALD. Children with CFALD were given diagnoses of CF later than children without liver disease. There were more children with meconium ileus in the control group (14 vs 4) than among those with CFALD.


CONCLUSIONS
Children with established CFALD have impaired growth and nutrition, altered body composition, and worse forced expiratory volume in 1 second values. CFALD is associated with later age of diagnosis of CF.
Corbett
Katie
K
Department of Paediatrics and Children's Research Centre, Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Crumlin, Dublin 12, Ireland.
Kelleher
Suzanne
S
Rowland
Marion
M
Daly
Leslie
L
Drumm
Brendan
B
Canny
Gerard
G
Greally
Peter
P
Hayes
Roisin
R
Bourke
Billy
B
D000293:Adolescent; D000886:Anthropometry; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D006801:Humans; D007494:Ireland / Q000453:epidemiology; D008107:Liver Diseases / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D015995:Prevalence