Chest ACCP Member Benefits
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Guest Access | Sign In via User Name/Password
This Article
Right arrow Full Text Free
Right arrow Full Text (PDF) Free
Right arrow Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Article Archive
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via ISI Web of Science (3)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Slieker, M. G.
Right arrow Articles by van der Ent, C. K.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Slieker, M. G.
Right arrow Articles by van der Ent, C. K.
(Chest. 2005;128:2309-2315.)
© 2005 American College of Chest Physicians

Birth Prevalence and Survival in Cystic Fibrosis*

A National Cohort Study in the Netherlands

Martijn G. Slieker, MD; Cuno S. P. M. Uiterwaal, MD, PhD; Maarten Sinaasappel, MD, PhD; Harry G. M. Heijerman, MD, PhD; Johan van der Laag, MD and Cornelis K. van der Ent, MD, PhD

* From the Cystic Fibrosis Center Utrecht (Drs. Slieker, van der Laag, and van der Ent), University Medical Center Utrecht, Utrecht; the Julius Center for Health Sciences and Primary Care (Dr. Uiterwaal), University Medical Center Utrecht, Utrecht; the Department of Pediatric Gastro-Enterology (Dr. Sinaasappel), Erasmus Medical Center, Rotterdam; and the Adult Cystic Fibrosis Center (Dr. Heijerman), Haga Teaching Hospital, The Hague, the Netherlands.

Correspondence to: Martijn G. Slieker, MD, Cystic Fibrosis Center Utrecht, University Medical Center Utrecht, PO Box 85090, 3508 AB Utrecht, the Netherlands; e-mail: m.g.slieker{at}umcutrecht.nl

Background: Birth prevalence and survival in patients with cystic fibrosis (CF) in the Netherlands were last investigated > 30 years ago. However, since then the birth prevalence may have decreased because of genetic counseling and an increased number of newborns of non-European descent. Although survival of CF patients has increased worldwide, a significantly lower median age at death was recently reported in the Netherlands compared with data from the United States.

Objectives: To analyze birth prevalence and survival in CF patients in the Netherlands, and to compare this survival data with US CF data.

Design: Survey of all CF patients living in the Netherlands, and analysis of Dutch CF mortality statistics using data from the Dutch central statistics office, Statistics Netherlands (Voorburg, the Netherlands), and a comparison with Cystic Fibrosis Foundation (Bethesda, MD) patient registry data.

Setting: All CF centers in the Netherlands and the United States.

Participants: All CF patients treated in the Netherlands on January 1, 2001, and all persons who died of CF between 1974 and 2000, and an equivalent US population.

Measurements: Birth prevalence and birth cohort-specific survival.

Results: The overall birth prevalence of CF for 1974 to 1994 was 1 in 4,750 live births, which is a considerable decrease compared with 1961 to 1965 (1 in 3,600 live births). Estimated survival to 30 years increased from 6% in the 1950-to-1954 cohort, to 36% in the 1970-to-1973 cohort. Exact survival could be calculated from 1974 onwards. Survival to 15 years increased from 72% from the 1974-to-1979 cohort, to 91% in the 1985-to-1989 cohort. Survival in the United States in the 1980-to-1984 cohort was better compared to the Netherlands, but this difference has disappeared over subsequent cohorts.

Conclusions: The actual birth prevalence of CF in the Netherlands is clearly lower than it was 30 years ago. Survival in CF has dramatically improved. The difference in survival between the Netherlands and the United States, as observed in the cohorts born > 20 years ago, has disappeared.

Key Words: birth prevalence • cystic fibrosis • survival






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2005 by the American College of Chest Physicians.