Early Diagnosis of Cystic Fibrosis in the Newborn Period and Risk of Pseudomonas aeruginosa Acquisition in the First 10 Years of Life: A Registry-Based …

SS Wang, SC FitzSimmons, LA O'Leary, MJ Rock… - …, 2001 - publications.aap.org
… Using data from CF patients in the national Cystic Fibrosis Foundation Patient Registry,
we attempted to determine whether early asymptomatic diagnosis of CF is associated with …

Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease

MO Wielpütz, M Puderbach… - American journal of …, 2014 - atsjournals.org
… structural lung disease starts early in infants and preschool children with cystic fibrosis (CF), …
, and noninvasive detection and monitoring of early CF lung disease remain challenging. …

Prognosis in cystic fibrosis

BJ Rosenstein, PL Zeitlin - Current opinion in pulmonary medicine, 1995 - journals.lww.com
… data registry of the National Cystic Fibrosis Foundation, 33.8% are … cystic fibrosis
transmembrane conductance regulator (CFTR) mutation and clinical status. Although there were …

Early childhood wheezing is associated with lower lung function in cystic fibrosis

, … of the Epidemiologic Study of Cystic Fibrosis - Pediatric …, 2014 - Wiley Online Library
… in young children with and without cystic fibrosis (CF). Half of … , but the relationship between
early childhood wheezing and … The Epidemiologic Study of Cystic Fibrosis (ESCF) is a large …

Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index

Y Belessis, B Dixon, G Hawkins, J Pereira… - American journal of …, 2012 - atsjournals.org
… be a useful marker of early CF lung disease. Furthermore, … Cystic fibrosis (CF) lung disease
is characterized by unremitting cycles of airway infection and inflammation that begin early in …

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter

G Davies, S Stanojevic, E Raywood… - European …, 2020 - Eur Respiratory Soc
… Lung clearance index (LCI) in the early years was associated with LCI during adolescence
in children with cystic fibrosis. Pre-school LCI may help to identify children in whom treatment …

Progression of lung disease in preschool patients with cystic fibrosis

S Stanojevic, SD Davis, G Retsch-Bogart… - American journal of …, 2017 - atsjournals.org
… be a useful marker to track early disease progression and guide therapies in young patients
with cystic fibrosis. Cystic fibrosis (CF) lung disease begins early in life and often progresses …

Update in cystic fibrosis 2018

BW Ramsey, GP Downey, CH Goss - American Journal of …, 2019 - atsjournals.org
Early lung disease in infants and preschool children with cystic fibrosis: what have we …
neutrophils is associated with early lung damage in children with cystic fibrosis. Am J Respir …

Strategies in early clinical development for the treatment of basic defects of cystic fibrosis

B Dhooghe, JB Haaf, S Noel, T Leal - Expert Opinion on …, 2016 - Taylor & Francis
… of the gene responsible for cystic fibrosis (CF), controversies … of fundamental therapies in
early stages of development for … compounds being currently tested are in very early (I-II) phases …

Roadmap for an early gene therapy for cystic fibrosis airway disease

MS Carlon, D Vidović, S Birket - Prenatal Diagnosis, 2017 - Wiley Online Library
cystic fibrosis (CF). CF is a chronic autosomal recessive genetic disorder caused by mutations
in the cystic fibrosis … the median age at death remains early thirties.7 The pathophysiology …