We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Trends in Growth and Maturation in Children with Cystic Fibrosis Throughout Nine Decades.
- Authors
Mason, Kelly A.; Rogol, Alan D.
- Abstract
Since cystic fibrosis (CF) was first described in 1938, there have been many discoveries and innovations in the field, each having a profound impact on survival, growth and quality of life. For example, the introduction of enteric-coated pancreatic enzyme microspheres increased fat absorption and improved nutritional status. Early detection of CF through newborn screening facilitated prompt nutritional intervention for infants at high risk of malnutrition. Use of anti-pseudomonal therapy, such as inhaled tobramycin, increased weight gain and pulmonary function in addition to reducing pulmonary exacerbations. Similarly, DNAse and hypertonic saline improved pulmonary function and reduced exacerbations. The identification of the CFTR gene and its protein product were fundamental in understanding the pathophysiology of CF and paved the way for advances in both diagnosis and management. In fact, CFTR modulator therapies have revolutionized the care for individuals with CF. Here, we examine the impact of these interventions on the nutritional status, growth and pubertal maturation of children and adolescents with CF.
- Subjects
CYSTIC fibrosis; GROWTH of children; CYSTIC fibrosis transmembrane conductance regulator; HYPERTONIC saline solutions; PANCREATIC enzymes
- Publication
Frontiers in Endocrinology, 2022, Vol 13, p1
- ISSN
1664-2392
- Publication type
Article
- DOI
10.3389/fendo.2022.935354