0
Original Research: CYSTIC FIBROSIS |

Classic Respiratory Disease but Atypical Diagnostic Testing Distinguishes Adult Presentation of Cystic Fibrosis

Claire L. Keating, MD; Xinhua Liu, PhD; Emily A. DiMango, MD
Author and Funding Information

From the Gunnar Esiason Adult Cystic Fibrosis and Lung Program (Drs Keating and DiMango), Division of Pulmonary, Allergy and Critical Care Medicine, Columbia University Medical Center; and the Mailman School of Public Health (Dr Liu), Columbia University, New York, NY.

Correspondence to: Claire L. Keating, MD, Columbia University Medical Center, 622 W 168th St, PH 8, New York, NY 10032; e-mail: ck2132@columbia.edu


Funding/Support: This work was supported in part by the Cystic Fibrosis Foundation Clinical Research Facilitation Award [C079-CRF06A] and the National Institute of Environmental Health Sciences Center [Grant P30ES09089].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestpubs.org/site/misc/reprints.xhtml).


© 2010 American College of Chest Physicians


Chest. 2010;137(5):1157-1163. doi:10.1378/chest.09-1352
Text Size: A A A
Published online

Background:  The majority of new cases of cystic fibrosis (CF) are diagnosed before age 2 years. Diagnoses in older individuals have increased because of improved genetic testing and increased awareness of the disease. A comprehensive description of clinical, genetic, and microbiologic characteristics of adult-age presentation of CF does not exist. We compare newly diagnosed CF in adults with newly diagnosed CF in children and adolescents in the United States.

Methods:  This is a cross-sectional study of new CF diagnoses from the Cystic Fibrosis Foundation Patient Registry between 1995 and 2005. Diagnostic, microbiologic, and clinical features during year of diagnosis were analyzed for subjects by age group. Descriptive statistics were calculated for variables on characteristics by age group.

Results:  A total of 9,766 new diagnoses of CF were reported to the Registry between 1995 and 2005. The proportion of adult diagnoses increased significantly in the years 2001 to 2005 as compared with 1995 to 2000 (9.0% vs 7.7%, P = .012). FEV1% predicted decreased with increasing age at diagnosis (P < .001). Infection with Pseudomonas aeruginosa was most common in adults (P < .001). Both the number of positive sweat chloride tests and prevalence of ΔF508 mutation, the most common mutation in the United States, decreased significantly with older age at diagnosis (P < .001).

Conclusions:  Between 1995 and 2005, the proportion of new diagnoses of CF in adults in the United States increased significantly. Adults present with commonly described CF respiratory disease (Pseudomonas aeruginosa infection and reduced lung function), but have lower sweat chloride values and lower frequency of ΔF508 mutation. Knowledge of clinical characteristics and diagnostic limitations of adult patients presenting with CF will hopefully lead to earlier recognition and intervention.

Figures in this Article

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543