Cystic Fibrosis | American Academy of Pediatrics - AAP News

Cystic Fibrosis | American Academy of Pediatrics - AAP News

Cystic Fibrosis | American Academy of Pediatrics - AAP News

Posted: 01 Feb 2021 12:00 AM PST

  1. Kimberly M. Dickinson, MD, MPH*
  2. Joseph M. Collaco, MD, PhD*
  1. *Eudowood Division of Pediatric Respiratory Sciences, The Johns Hopkins University School of Medicine, Baltimore, MD

Abstract

Cystic fibrosis (CF) is one of the most commonly diagnosed genetic disorders. Clinical characteristics include progressive obstructive lung disease, sinusitis, exocrine pancreatic insufficiency leading to malabsorption and malnutrition, liver and pancreatic dysfunction, and male infertility. Although CF is a life-shortening disease, survival has continued to improve to a median age of 46.2 years due to earlier diagnosis through routine newborn screening, promulgation of evidence-based guidelines to optimize nutritional and pulmonary health, and the development of CF-specific interdisciplinary care centers. Future improvements in health and quality of life for individuals with CF are likely with the recent development of mutation-specific modulator therapies. In this review, we will cover the current understanding of the disease manifestations, diagnosis, and management as well as common complications seen in individuals with CF.
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