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Cited by 109Cystic Fibrosis Research AdvancesGenetic and Kidney Cyst DiseasesNeonatal Respiratory Health Research

Background and Epidemiology

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Sanders, Don B., Fink, Aliza K.·2016·Pediatric Clinics of North America
DOI 10.1016/j.pcl.2016.04.001PMC PMC4967225
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Abstract

Cystic fibrosis (CF) is the most common autosomal-recessive disease in white persons. Significant advances in therapies and outcomes have occurred for people with CF over the past 30 years. Many of these improvements have come about through the concerted efforts of the CF Foundation and international CF societies; networks of CF care centers; and the worldwide community of care providers, researchers, and patients and families. There are still hurdles to overcome to continue to improve the quality of life, reduce CF complications, prolong survival, and ultimately cure CF. This article reviews the epidemiology of CF, including trends in incidence and prevalence, clinical characteristics, common complications, and survival.

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  • Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein.

    “Cystic fibrosis is a lethal, inherited, autosomal recessive disorder that affects approximately 80,000 people worldwide and is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein that lead to reduced CFTR function.1-3”

    From Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele (2019) · cites this paper as [3]

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Authors
Sanders, D. B., & Fink, A. K.
Affiliations
  • Indiana University Health (US)
  • Cystic Fibrosis Foundation (US)
Corresponding author
Sanders, Don B.
ORCID
Sanders, Don B.
Venue
Pediatric Clinics of North America
Publisher
Elsevier BV
Published
2016-08
Volume
63
Issue
4
Pages
567-584
ISSN
0031-3955 (ISSN-L)
DOI
10.1016/j.pcl.2016.04.001
PMC
PMC4967225
OpenAlex
W2496887625
Cited by
109
References
6
Citations by year
2026: 4 · 2025: 10 · 2024: 12 · 2023: 10 · 2022: 17 · 2021: 23 · 2020: 16 · 2019: 10 · 2018: 3 · 2017: 4
FWCI
0.96
Type
journal article
Language
en
Open access
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Pediatric Clinics of North America (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • National Institutes of Health (KL2TR000428, UL1TR000427)
  • National Center for Advancing Translational Sciences
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
Keywords
cystic fibrosis, epidemiology, incidence, prevalence, clinical features, complications, survival, autosomal recessive disorder
MeSH terms
Adult, Child, Child, Preschool, Cystic Fibrosis, Humans, Infant, Infant, Newborn, Incidence, Prevalence, Survival Rate, Neonatal Screening

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