Growth failure and treatment in cystic fibrosis

• Authors: Trang N. Le, Virginia Commonwealth University; Abeer Anabtawi, University of Kansas; Melissa S. Putman, Boston Children's Hospital; Vin Tangpricha, Emory University; Michael S. Stalvey, Atlanta VA Medical Center
• Journal Title: JOURNAL OF CYSTIC FIBROSIS
• Volume: Volume 18
• Publisher: ELSEVIER | 2019-10-01, Pages S82-S87
• Type of Work: Article | Post-print: After Peer Review
• Publisher DOI: http://doi.org/10.1016/j.jcf.2019.08.010
• Subjects: Health Sciences, Medicine and Surgery; Health Sciences, Human Development
• Keywords: Science & Technology; Life Sciences & Biomedicine; Respiratory System; Growth; Short stature; Cystic fibrosis; Growth delay

Abstract

Poor growth has long been a characteristic feature of cystic fibrosis (CF) and is significantly linked to lung function and overall health status. Improvements in pulmonary and nutrition care for patients with cystic fibrosis (CF) have resulted in better growth outcomes; however, height gains have not paralleled the improvements in weight in children with CF, and patients with more severe CF mutations remain significantly more affected. Many factors affect the growth hormone-IGF-1 axis and the growth plate of the long bones, including the chronic inflammatory state associated with CF. There are also increasing data on the direct effects of CFTR on bone and implications for CFTR modulators in attaining optimal growth. Treatments aimed at improving growth in CF are also reviewed here.