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Current Problems in Pediatrics, 1993 The past few years have seen amazing progress in our understanding of the underlying cellular and molecular bases of CF. This new understanding has raised hopes for improved treatments in the very near future. These new treatments are expected to improve even further the prognosis for survival in this disorder, which has improved nearly 1 year for ...
Savant A +3 more
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Nature Reviews Disease PrimersCystic fibrosis is a rare genetic disease caused by mutations in CFTR, the gene encoding cystic fibrosis transmembrane conductance regulator (CFTR). The discovery of CFTR in 1989 has enabled the unravelling of disease mechanisms and, more recently, the development of CFTR-directed therapeutics that target the underlying molecular defect.
Yu E, Sankari A, Sharma S.
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BMJ, 2007 As the Good Witch told Dorothy in the Wizard of Oz: it is always best to start at the beginning. Growing up, I always knew that I had cystic fibrosis, the same way I knew I had blue eyes and my cousins could all run faster than me. I was lucky enough—though my parents did not think so at the time—to be diagnosed at two days old.
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Deutsches Ärzteblatt international, 2017 Universal screening of newborn babies for cystic fibrosis was launched in Germany on 1 September 2016. Here we present up-to-date information on the diagnosis, treatment, and prognosis of this disease.This article is based on relevant publications retrieved by a selective search in PubMed, along with guidelines from Germany and abroad and systematic ...
Radtke, Thomas +2 more
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The Lancet, 1998 OBJECTIVE: Due to the great advances recently achieved in the treatment of Cystic Fibrosis as well as to the fact that pediatricians need to have a better understanding of this disease, the authors propose an extensive review of the subject. METHODS: We selected the most outstanding publications on Cystic Fibrosis in the international literature of the
B J, Rosenstein, P L, Zeitlin
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Pneumothorax in cystic fibrosis [PDF]
Current Opinion in Pulmonary Medicine, 2003 Spontaneous pneumothorax is a common complication in patients with cystic fibrosis (CF). It is thought to occur more frequently in patients with more advanced disease. Recommendations on the management of pneumothorax in CF are based on retrospective analyses and reports from CF centers.
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The Journal of the Royal College of Physicians of Edinburgh, 2013 There are now more adult than paediatric cystic fibrosis (CF) patients and their life expectancy continues to improve. This means that CF patients will be more commonly encountered in a variety of hospital settings including fertility services, gastrointestinal (GI) clinics, diabetes clinics, surgical wards, and acute admissions.
A E, Leitch, H C, Rodgers
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Journal of Pediatric Gastroenterology and Nutrition, 1989 SANTAMARIA, FRANCESCA +5 more
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