Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF.

ABSTRACT:

Background

Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM.

Purpose

To examine the prevalence and clinical consequences of TM in children with CF.

Methods

We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV1 , CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset.

Results

Eighty-nine percent of children with CF had at least one bronchoscopy (n = 97/109). Fifteen percent of these

SUBMITTER: Fischer AJ 

PROVIDER: S-EPMC4711356 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature