Format

Send to

Choose Destination
J Cyst Fibros. 2019 Oct 28. pii: S1569-1993(19)30892-6. doi: 10.1016/j.jcf.2019.09.008. [Epub ahead of print]

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

Author information

1
Department of Gastroenterology, Sydney Children's Hospital, High Street, Randwick NSW 2031, Australia.
2
Discipline of Paediatrics, School of Women's and Children's Health, University of New South Wales, Sydney NSW 2052, Australia.
3
Department of Gastroenterology, Sydney Children's Hospital, High Street, Randwick NSW 2031, Australia; Discipline of Paediatrics, School of Women's and Children's Health, University of New South Wales, Sydney NSW 2052, Australia; Molecular and Integrative Cystic Fibrosis (miCF) Research Centre, Sydney Children's Hospital, High Street, Randwick NSW 2031, Australia. Electronic address: keith.ooi@unsw.edu.au.

Abstract

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

PMID:
31672555
DOI:
10.1016/j.jcf.2019.09.008

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center