title: Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes.
abstract: OBJECTIVE
  To identify the incidence and outcomes of cholestasis and meconium ileus (MI) in infants with cystic fibrosis (CF).
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Single-centre study.
  
  
  METHODS
  From January 1986 to December 2011, 401 infants with CF (69 with MI) presented to our centre.
  
  
  METHODS
  (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<0.001). Infants with MI had a 30.36-fold increased risk of developing cholestasis compared to those without MI (p<0.001). Cholestasis resolved in all children, at a median (range) age of 9.2 (0.8-53.2) months in the MI group and 10.2 (2.0-19.4) months in the non-MI group. The majority of cholestatic infants (87.0%) and infants with MI (92.8%) did not develop clinically significant CFLD, not significantly different than either the 93.9% of non-cholestatic infants nor the 93.7% infants without MI.
  
  
  CONCLUSIONS
  Cholestasis is an uncommon condition in CF affecting only 5.7% of the screened newborn CF population. The greatest risk factor for developing cholestasis is the presence of MI. However, the presence of MI appears not to be associated with the development of CFLD. An effect of neonatal cholestasis on the development of CFLD cannot be excluded by this study.
authors.affiliation: Department of Respiratory Medicine, The Children's Hospital at Westmead, , Sydney, Australia.
authors.forename: Lisette
authors.initials: L
authors.lastname: Leeuwen
authors.forename: Annabel K
authors.initials: AK
authors.lastname: Magoffin
authors.forename: Dominic A
authors.initials: DA
authors.lastname: Fitzgerald
authors.forename: Marco
authors.initials: M
authors.lastname: Cipolli
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Gaskin
mesh_terms: D002779:Cholestasis / Q000150:complications* / Q000453:epidemiology; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D045823:Ileus / Q000150:complications* / Q000453:epidemiology; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008470:Meconium; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors
keywords: Cholestasis; Cystic Fibrosis; Liver disease; Meconium ileus; Newborn screening