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Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes
  1. Lisette Leeuwen1,2,
  2. Annabel K Magoffin3,
  3. Dominic A Fitzgerald1,4,
  4. Marco Cipolli5,
  5. Kevin J Gaskin3,6
  1. 1Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, Australia
  2. 2Medical School, University of Groningen, Groningen, The Netherlands
  3. 3Department of Gastroenterology, The Children's Hospital at Westmead, Sydney, Australia
  4. 4Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Sydney, Australia
  5. 5Cystic Fibrosis Centre, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy
  6. 6James Fairfax Institute of Paediatric Nutrition, The Children's Hospital at Westmead, Sydney, Australia
  1. Correspondence to Professor K J Gaskin, Department of Gastroenterology, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia; kevin.gaskin{at}


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<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.

  • Cystic Fibrosis
  • Cholestasis
  • Liver disease
  • Meconium ileus
  • Newborn screening

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