Outcome in cystic fibrosis liver disease.
OBJECTIVE
Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.


METHODS
This is a 7-year follow-up of 42 children with CFLD and their age- and sex-matched controls. Participants were reviewed clinically, biochemically, and radiologically at follow-up.


RESULTS
Overall, 85% (72 of 84) of the original cohort were included, 36 CFLD participants and 36 CF controls. There was no significant difference in the number of deaths/transplants between groups (7 of 36 (19.4%) CFLD participants, 3 of 36 (8.3%) CF controls). There was a tendency for participants with CFLD to die younger than their respective CF controls. There was no difference in height, weight, body mass index, or pulmonary function between the groups. Nutritional parameters (sum skinfold thickness 31.6 vs. 42.3, P=0.03; mean upper arm fat area 15.08 vs. 10.59, P=0.001; Shwachman score 43.7 vs. 32.1, P=0.001) were worse among CFLD participants than among CF controls. Cystic fibrosis-related diabetes was more common in CFLD participants (11 of 27 (40.7%) vs. 5 of 33 (15.2%), P=0.02). Eight children (22.2%) with evidence of CFLD at baseline had no clinical evidence of liver disease as adults.


CONCLUSIONS
Patients with CFLD have a more severe CF phenotype than do CF patients without liver disease. However, a subgroup of children with CFLD will not manifest clinically significant liver disease as adults.
Rowland
Marion
M
The Children's Research Centre, Dublin, Ireland. marion.rowland@ucd.ie
Gallagher
Charles G
CG
O'Laoide
Risteard
R
Canny
Gerard
G
Broderick
Annemarie
A
Hayes
Roisin
R
Greally
Peter
P
Slattery
Dubhfeassa
D
Daly
Leslie
L
Durie
Peter
P
Bourke
Billy
B
D000293:Adolescent; D017677:Age Distribution; D016022:Case-Control Studies; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D016001:Confidence Intervals; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008107:Liver Diseases / Q000209:etiology* / Q000401:mortality* / Q000628:therapy; D008111:Liver Function Tests; D008297:Male; D012016:Reference Values; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D016019:Survival Analysis; D013997:Time Factors; D055815:Young Adult