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Review
. 2019 Oct;18 Suppl 2(Suppl 2):S82-S87.
doi: 10.1016/j.jcf.2019.08.010.

Growth failure and treatment in cystic fibrosis

Trang N Le  1 , Abeer Anabtawi  2 , Melissa S Putman  3 , Vin Tangpricha  4 , Michael S Stalvey  5
Affiliations
Review

Growth failure and treatment in cystic fibrosis

Trang N Le et al. J Cyst Fibros. 2019 Oct.

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.

Keywords: Cystic fibrosis; Growth; Growth delay; Short stature.

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Figures

Figure 1.
Figure 1.. Contributors to Poor Growth in CF.
Multiple factors are implicated in poor growth in CF affect the GH-IGF-1-growth plate axis and can impair growth through actions on GH-IGF-1 signaling and through direct effects on the growth plate.
See this image and copyright information in PMC

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