Update in cystic fibrosis 2018

BW Ramsey, GP Downey, CH Goss - American Journal of …, 2019 - atsjournals.org
In this review, we have selected several outstanding studies from the American Journal of
Respiratory and Critical Care Medicine (AJRCCM), the American Journal of Respiratory Cell …

Cystic fibrosis year in review 2018, part 2

AP Savant, SA McColley - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) research and case reports were robust in the year 2018. This report
summarizes publications related the multisystem effects of CF, pulmonary exacerbations …

Update in cystic fibrosis 2011

F Ratjen, SA McColley - American journal of respiratory and critical …, 2012 - atsjournals.org
Major advances in our understanding of cystic fibrosis (CF) lung disease and its treatment
have materialized over the last decades. Targeting therapies to the major pathophysiologic …

Update in cystic fibrosis 2014

T Ong, BW Ramsey - American journal of respiratory and critical …, 2015 - atsjournals.org
The clinical face of cystic fibrosis (CF) has significantly changed over the 25 years since its
basic genetic defect was discovered (1). Increasing numbers of patients are diagnosed at …

Unmet needs in cystic fibrosis: the next steps in improving outcomes

NE West, PA Flume - Expert review of respiratory medicine, 2018 - Taylor & Francis
Introduction: Cystic fibrosis (CF) outcomes and survival have improved over the last century
primarily due to advancements in antibiotics, nutritional, and pulmonary therapies …

Cystic fibrosis: the dawn of a new therapeutic era

SL Heltshe, J Cogen, KJ Ramos… - American Journal of …, 2017 - atsjournals.org
Clinical outcomes and management of cystic fibrosis (CF) have undergone startling and
dramatic advances in the last two decades. These improvements can be linked to the …

Outcome measures for development of new therapies in cystic fibrosis: are we making progress and what are the next steps?

BW Ramsey - Proceedings of the American Thoracic Society, 2007 - atsjournals.org
Since the discovery of the gene encoding the cystic fibrosis transmembrane regulator
protein (CFTR) in 1989, rapid expansion in our understanding of the basic defect has …

[HTML][HTML] Review of rapid advances in cystic fibrosis

SL Link, RP Nayak - Missouri medicine, 2020 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is an autosomal recessive disease that was previously a fatal pediatric
disease with no treatment; however, due to scientific advancements, the median age of …

Cystic fibrosis: a disease in transformation, yet more work to be done!

BW Ramsey, SC Bell - American Journal of Respiratory and Critical …, 2022 - atsjournals.org
What have we learned from real-world experience about highly effective CFTR modulation
and its impact on the lives of patients with cystic fibrosis? In 2019, the triple combination …

The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches

SY Graeber, MA Mall - The Lancet, 2023 - thelancet.com
With the 2019 breakthrough in the development of highly effective modulator therapy
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …