Completely revised and updated, the second edition of this authoritative guide provides the latest information on the diagnosis, treatment, and ongoing management of pulmonary issues in children. Available for purchase at https://www.aap.org/Pediatric-Pulmonology-2nd-Edition-Paperback
45: Cystic Fibrosis
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Published:November 2023
Michael S. Schechter, MD, MPH, FAAP, "Cystic Fibrosis", Pediatric Pulmonology, American Academy of Pediatrics Section on Pediatric Pulmonology and Sleep Medicine, Michael J. Light, MD, FAAP, Kristin Van Hook, MD, MPH, FAAP
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Cystic fibrosis (CF) was, in the past, typically described as the most common lethal genetic disease in the white population, but the outlook for people with a CF diagnosis has improved dramatically with advancements in treatment, and new therapies are expected to lead to even greater changes. The US Cystic Fibrosis Foundation (CFF) median projected survival age in 2019 (before generalized availability of highly effective modulator therapies, described later, in Management) was 48.4 years (95% CI, 45.9–51.5 years) (Figure 45-1).1 Birth prevalence varies with racial or ethnic background; CF occurs in approximately 1 in 3,000 white Americans...
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