Format

Send to

Choose Destination
J Pediatr Surg. 2006 Sep;41(9):1556-60.

Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study.

Author information

1
Cystic Fibrosis Centre, Hospital Robert Debré 48, bd Serurier, 75019 Paris, France. anne.munck@rdb.ap-hop-paris.fr

Abstract

OBJECTIVE:

This matched case-control study compared the nutritional and the pulmonary long-term outcomes of cystic fibrosis (CF) patients presenting a history of meconium ileus (MI) with early-diagnosed symptomatic CF without MI (non-MI).

MATERIAL AND METHOD:

Twenty-six patients with CF treated for MI between 1980 and 1997 have been matched for sex, birth date, and earliest CF symptomatic diagnosis for the children with non-MI CF. Clinical characteristics, genotype and complications were evaluated as well as the progression of the CF disease from infancy to 15 years old by nutritional status (z score weight, z score height), pulmonary function tests (PFTs) (FVC and FEV1), and Pseudomonas aeruginosa acquisition.

RESULTS:

Median duration of the follow-up was 12.5 years (range, 10-17 years). Genotype identification showed no significant difference. Further on, the rate of complications and the occurrence of chronic P. aeruginosa colonization did not differ. At age of 15 years (n = 13), nutritional status and PFTs did not demonstrate any significant difference.

CONCLUSION:

These results suggest that adequate initial nutritional and medical management of MI allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, MI did not represent an additional risk factor for the patient's life.

PMID:
16952591
DOI:
10.1016/j.jpedsurg.2006.05.014
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center