Puberty and Growth in Cystic Fibrosis: A Review.

Cystic fibrosis (CF) is a chronic disease process that has historically been associated with delayed puberty and growth. Mechanisms for delayed growth and puberty in chronic disease include malnutrition, inflammation, alterations of the growth hormone axis, chronic corticosteroid use, and changes to gonadotropin signaling or release. Early evidence suggested that people with CF have delayed puberty, decreased peak height velocity, and decreased final adult height compared to the general population. Management of delayed puberty in CF typically involves addressing the underlying cause of delay, and evidence for the utility of growth hormone to optimize growth in people with CF is inconsistent. In the era of highly effective modulator therapies for CF, early studies are examining changes in the growth and puberty trajectory in youth with CF. The full impact of highly effective modulator therapy on height outcomes remains unclear.