Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis.

ABSTRACT:

Rationale

Long-term survivors of cystic fibrosis (CF) (age > 40 yr) are a growing population comprising both patients diagnosed with classic manifestations in childhood, and nonclassic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts.

Objectives

Examine effects of age at diagnosis and gender on disease progression, setting of care, response to treatment, and mortality in long-term survivors of CF.

Methods

Retrospective analysis of the Colorado CF Database (1992-2008), CF Foundation Registry (1992-2007), and Multiple Cause of Death Index (1992-2005).

Measurements and main results

Patients with CF diagnosed in childhood and who survive to age 40 years have more severe CFTR genotypes and phenoty

SUBMITTER: Nick JA 

PROVIDER: S-EPMC2937235 | biostudies-literature | 2010 Sep

REPOSITORIES: biostudies-literature