Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus

J Cyst Fibros. 2019 Nov;18(6):857-862. doi: 10.1016/j.jcf.2019.09.008. Epub 2019 Oct 28.


Background: Meconium ileus (MI) affects up to 20% of newborns with cystic fibrosis (CF). We compared clinical outcomes between Australian paediatric CF patients with and without meconium ileus (non-MI).

Methods: This was a retrospective case-control study of MI and non-MI patients in New South Wales, Australia, from 1988 to 2010. MI patients were matched 1:1 with pancreatic insufficient non-MI patients for age, sex and CF clinic. Clinical measurements, nutrition and gastrointestinal outcomes over this period were compared between groups using linear mixed models for continuous variables to account for age.

Results: There were 162 matched pairs (N=324, 52% female) with mean (SD) age of 15.3 (8.2) and 14.9 (7.9) years for MI and non-MI patients respectively (P=0.6). MI patients aged 5-23 had poorer FEV1% compared to non-MI patients (estimate -0.070 SE [0.02], P=0.003). There were no significant differences in P. aeruginosa isolation rates; however S. aureus isolation rates were lower in MI patients (72%) compared to non-MI (82%) (OR 0.6 [0.3-1.0], P=0.03). Chronic colonisation rates for P. aeruginosa and S. aureus were not significantly different between groups. MI patients aged 2-20 had significantly lower BMI Z-scores over time (estimate -0.25 SE [0.1], P=0.02). MI patients were more likely to receive oral feed supplements (OR 2.8 [1.4-6.1], P=0.003) and gastrostomy formation (OR 4.4 [1.1-24.6], P=0.02).

Conclusions: CF patients with MI may have worse lung function, growth and nutrition than non-MI patients over time. Meconium ileus may be an early poor prognostic factor for CF.

Keywords: clinical outcomes; cystic fibrosis; growth; lung function; meconium ileus; nutrition.

MeSH terms

  • Adolescent
  • Australia / epidemiology
  • Case-Control Studies
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / epidemiology
  • Cystic Fibrosis* / microbiology
  • Cystic Fibrosis* / physiopathology
  • Early Diagnosis
  • Exocrine Pancreatic Insufficiency* / diagnosis
  • Exocrine Pancreatic Insufficiency* / etiology
  • Female
  • Growth Disorders* / diagnosis
  • Growth Disorders* / etiology
  • Humans
  • Infant, Newborn
  • Male
  • Malnutrition* / diagnosis
  • Malnutrition* / etiology
  • Meconium Ileus* / diagnosis
  • Meconium Ileus* / etiology
  • Nutritional Status
  • Prognosis
  • Pseudomonas aeruginosa / isolation & purification
  • Respiratory Function Tests / methods
  • Respiratory Function Tests / statistics & numerical data
  • Respiratory Tract Infections* / diagnosis
  • Respiratory Tract Infections* / microbiology
  • Retrospective Studies
  • Staphylococcus aureus / isolation & purification