Part 1 : Factors influencing outcomes in cystic fibrosis

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Chest

                                                                January

1, 2003

HEADLINE: Factors influencing outcomes in cystic fibrosis *: a

center-based

 analysis; clinical investigations.

BYLINE: , ; , M.; Konstan, W.;

, Wayne;

 Wohl, Ellen B.

    Context: Guidelines for managing cystic fibrosis (CF)

patients have been

 widely circulated, but little is known about the variations in

practice between

 sites and their association with outcomes.

    Objective: To determine whether differences in lung health

existed between

 groups of patients attending different CF care sites and to determine

whether

 these differences are associated with differences in monitoring and

 intervention.

    Design: The analysis was conducted using data from the

Epidemiologic Study

 of Cystic Fibrosis from 1995 through 1996.

    Setting: This was an observational database collecting

prospective

 information from a large number of CF patients undergoing routine

care in North

 America.

    Participants: Participating sites that had at least 50 CF

patients who had

 each made at least one visit to a center during the 2-year study

period were

 ranked on the basis of median values for FE[V.sub.1] within each of

three age

 groups (6 to 12 years, 13 to 17 years, and [greater than or equal to]

18 years).

    Interventions: There were no prespecified interventions in

this

 observational study.

    Main outcome measures: The frequency of patient monitoring

and the use of

 therapeutic interventions were compared between sites in the upper

and lower

 quartiles after stratification within the site for disease severity.

    Results: Within-site rankings tended to be consistent across

the three age

 groups. Patients who were treated at higher ranking sites had more

frequent

 monitoring of their clinical status, measurements of lung function,

and cultures

 for respiratory pathogens. These patients also received more

interventions,

 particularly IV antibiotics for pulmonary exacerbations.

    Conclusion: We found substantial differences in lung health

across different

 CF care sites. We found that frequent monitoring and increased use of

 appropriate medications in the management of CF are associated with

improved

 outcomes.

Becki

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