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The future of cystic fibrosis care: a global perspective
Executive summary The past six decades have seen remarkable improvements in health
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …
Cystic fibrosis in the year 2020: A disease with a new face
K De Boeck - Acta paediatrica, 2020 - Wiley Online Library
The autosomal recessive disease cystic fibrosis (CF) was once untreatable and deadly in
childhood, but now most patients survive to adulthood. Many countries have instituted CF …
childhood, but now most patients survive to adulthood. Many countries have instituted CF …
Ivacaftor treatment of cystic fibrosis in children aged 12 to< 24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study
M Rosenfeld, CE Wainwright, M Higgins… - The lancet respiratory …, 2018 - thelancet.com
Background Ivacaftor is generally safe and effective in patients aged 2 years and older who
have cystic fibrosis and specific CFTR mutations. We assessed its use in children aged 12 …
have cystic fibrosis and specific CFTR mutations. We assessed its use in children aged 12 …
Risk factors for bronchiectasis in children with cystic fibrosis
Background Bronchiectasis develops early in the course of cystic fibrosis, being detectable
in infants as young as 10 weeks of age, and is persistent and progressive. We sought to …
in infants as young as 10 weeks of age, and is persistent and progressive. We sought to …
Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis
Although destructive airway disease is evident in young children with cystic fibrosis (CF),
little is known about the nature of the early CF lung environment triggering the disease. To …
little is known about the nature of the early CF lung environment triggering the disease. To …
Key mechanisms governing resolution of lung inflammation
CT Robb, KH Regan, DA Dorward, AG Rossi - Seminars in …, 2016 - Springer
Innate immunity normally provides excellent defence against invading microorganisms.
Acute inflammation is a form of innate immune defence and represents one of the primary …
Acute inflammation is a form of innate immune defence and represents one of the primary …
[HTML][HTML] Impact of Pseudomonas aeruginosa Infection on Patients with Chronic Inflammatory Airway Diseases
M Garcia-Clemente, D de la Rosa, L Máiz… - Journal of clinical …, 2020 - mdpi.com
Pseudomonas aeruginosa (P. aeruginosa) is a ubiquitous and opportunistic microorganism
and is considered one of the most significant pathogens that produce chronic colonization …
and is considered one of the most significant pathogens that produce chronic colonization …
Clinical practice guidelines from the cystic fibrosis foundation for preschoolers with cystic fibrosis
T Lahiri, SE Hempstead, C Brady, CL Cannon… - …, 2016 - publications.aap.org
Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With
Cystic Fibrosis | Pediatrics | American Academy of Pediatrics Skip to Main Content …
Cystic Fibrosis | Pediatrics | American Academy of Pediatrics Skip to Main Content …
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
Rationale: Studies demonstrating early structural lung damage in infants and preschool
children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to …
children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to …
Correction of sensor crosstalk error in Exhalyzer D multiple-breath washout device significantly impacts outcomes in children with cystic fibrosis
F Wyler, MA Oestreich, BS Frauchiger… - Journal of applied …, 2021 - journals.physiology.org
Nitrogen multiple-breath washout is an established technique to assess functional residual
capacity and ventilation inhomogeneity in the lung. Accurate measurement of gas …
capacity and ventilation inhomogeneity in the lung. Accurate measurement of gas …