Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes

Objective To identify the incidence and outcomes of cholestasis and meconium ileus (MI) in infants with cystic fibrosis (CF). Design Retrospective cohort study. Setting Single-centre study. Patients From January 1986 to December 2011, 401 infants with CF (69 with MI) presented to our centre. Main outcome measurements (1) incidence of cholestasis, (2) identification of risk factors for cholestasis, (3) association between the presence of cholestasis and MI and the development of clinically significant CF-associated liver disease (CFLD) defined as multilobular cirrhosis with portal hypertension. Results Cholestasis occurred in 23 of 401 infants (5.7%). There was a significantly higher incidence of cholestasis in infants with MI (27.1%) compared to those without MI (1.2%) (p