Long-term pulmonary outcome after meconium ileus in cystic fibrosis.

OBJECTIVE

To identify cystic fibrosis (CF) patients with a history of meconium ileus (MI) and to compare long-term outcome of these patients with CF patients without MI.

PATIENTS

370 CF patients who were treated at the Munich CF centre in 2006, mean age 17.1 years (range: 0-48), were classified with regard to a history of MI.

METHODS

Retrospective chart analysis regarding history of MI, age, and lung function and first detection of Pseudomonas aeruginosa as the primary outcome variables.

RESULTS

We identified 60 patients with MI (16.2% of all patients with a mean age of 16.2 +/- 9 years (0-41) who did not differ in age from patients without MI (17.3 +/- 10; 0-48), whereas the mean age of CF diagnosis was different (0.4 vs. 21.8 months, P < 0.001). Cross sectional analysis of best Forced expiratory volume (FEV% predicted) in 2006 was not different between patients with MI (89 +/- 23%, 17-134) and those without (88 +/- 27%, 21-148, P = 0.73). Longitudinal analysis using FEV1 values of more than 9,000 lung function tests also showed no difference in FEV1 although first detection of P. aeruginosa in patients with MI was significantly earlier.

CONCLUSION

MI was associated with hospitalization, surgery, and early infection with P. aeruginosa. However, the long-term outcome of CF patients with or without MI was the same. This finding might be explained by the considerably earlier CF diagnosis in CF patients with MI including the benefit of an early initiation of appropriate CF therapy.