Heritability of lung disease severity in cystic fibrosis.

ABSTRACT:

Rationale

Obstructive lung disease, the major cause of mortality in cystic fibrosis (CF), is poorly correlated with mutations in the disease-causing gene, indicating that other factors determine severity of lung disease.

Objectives

To quantify the contribution of modifier genes to variation in CF lung disease severity.

Methods

Pulmonary function data from patients with CF living with their affected twin or sibling were converted into reference values based on both healthy and CF populations. The best measure of FEV(1) within the last year was used for cross-sectional analysis. FEV(1) measures collected over at least 4 years were used for longitudinal analysis. Genetic contribution to disease variation (i.e., heritability) was estimated in two ways: by comparing simila

SUBMITTER: Vanscoy LL 

PROVIDER: S-EPMC1899267 | biostudies-literature | 2007 May

REPOSITORIES: biostudies-literature