[HTML][HTML] The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery

V Scotet, C L'hostis, C Férec - Genes, 2020 - mdpi.com
Significant advances in the management of cystic fibrosis (CF) in recent decades have
dramatically changed the epidemiology and prognosis of this serious disease, which is no …

Increasing life expectancy in cystic fibrosis: Advances and challenges

KA McBennett, PB Davis, MW Konstan - Pediatric pulmonology, 2022 - Wiley Online Library
Since the first description of cystic fibrosis in 1938, there have been significant advances in
both quality of life and longevity for people living with this disease. In this article we describe …

Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report

PM Farrell, BJ Rosenstein, TB White, FJ Accurso… - The Journal of …, 2008 - Elsevier
Newborn screening (NBS) for cystic fibrosis (CF) is increasingly being implemented and is
soon likely to be in use throughout the United States, because early detection permits …

Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis

Z Li, MR Kosorok, PM Farrell, A Laxova, SEH West… - Jama, 2005 - jamanetwork.com
ContextAlthough Pseudomonas aeruginosa is the most common virulent respiratory
pathogen in cystic fibrosis (CF), the longitudinal development of P aeruginosa infection and …

Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life

M Cohen-Cymberknoh, D Shoseyov… - American journal of …, 2011 - atsjournals.org
The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and
cloning of the CFTR gene more than 21 years ago led to the identification of the structure …

Genotype and phenotype in cystic fibrosis

J Zielenski - Respiration, 2000 - karger.com
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) gene which encodes a protein expressed in the apical membrane of exocrine …

Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth

PM Farrell, MR Kosorok, MJ Rock, A Laxova… - …, 2001 - publications.aap.org
Objective. Despite its relative frequency among autosomal recessive diseases and the
availability of the sweat test, cystic fibrosis (CF) has been difficult to diagnose in early …

Infection, inflammation, and lung function decline in infants with cystic fibrosis

N Pillarisetti, E Williamson, B Linnane… - American journal of …, 2011 - atsjournals.org
Rationale: Better understanding of evolution of lung function in infants with cystic fibrosis
(CF) and its association with pulmonary inflammation and infection is crucial in informing …

Newborn screening for cystic fibrosis

C Castellani, J Massie, M Sontag… - The Lancet Respiratory …, 2016 - thelancet.com
Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early
identification of infants with cystic fibrosis was first recognised, the performance of newborn …

Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis

MW Konstan, SM Butler, MEB Wohl, M Stoddard… - The Journal of …, 2003 - Elsevier
Objective To determine the relation of growth and nutritional status to pulmonary function in
young children with cystic fibrosis (CF). Study design The relation of weight-for-age (WFA) …