Abstract
In children with childhood-onset growth hormone deficiency, replacement GH therapy is effective in normalizing height during childhood and achieving adult height within the genetic target range. GH has further beneficial effects on body composition and metabolism through adult life. The transition phase, defined as the period from mid to late teens until 6–7 years after the achievement of final height, represents a crucial time for reassessing children’s GH secretion and deciding whether GH therapy should be continued throughout life. Evidence-based guidelines for diagnosis and treatment of growth hormone deficient children during transition are lacking. The aim of this review is to critically review the up-to-date evidence on the best management of transition patients in order to ensure the correct definitive diagnosis and establish the appropriate therapeutic regimen.
Highlights
The main effect of GH therapy in childhood is to stimulate linear growth: the primary objective of GH replacement therapy in growth hormone deficient (GHD) children is to achieve adult height within the genetic target range
GH plays a key role in the regulation of body composition and metabolism and its deficiency in adulthood has been associated with reduced lean body mass and bone mineral density (BMD), increased visceral adiposity, abnormal lipid profile, decreased muscle strength, cardiovascular risk, and impaired quality of life (Rosén and Bengtsson, 1990; Cuneo et al, 1992, 1993; Rosén et al, 1993; de Boer et al, 1994, 1995; Weaver et al, 1995; Attanasio et al, 1997; Johansson et al, 2004)
It has been reported that in transition patients the use of a cut-off value of −2 SDS according to age and sex-related IGF-I levels would miss more than one third of GHD subjects, −2 SDS showing a sensitivity of 62%, −1.7 SDS of 77%, and −1.3 SDS of 87% (Maghnie et al, 2005; Corneli et al, 2007)
Summary
The main effect of GH therapy in childhood is to stimulate linear growth: the primary objective of GH replacement therapy in growth hormone deficient (GHD) children is to achieve adult height within the genetic target range. GH plays a key role in the regulation of body composition and metabolism and its deficiency in adulthood has been associated with reduced lean body mass and bone mineral density (BMD), increased visceral adiposity, abnormal lipid profile, decreased muscle strength, cardiovascular risk, and impaired quality of life (Rosén and Bengtsson, 1990; Cuneo et al, 1992, 1993; Rosén et al, 1993; de Boer et al, 1994, 1995; Weaver et al, 1995; Attanasio et al, 1997; Johansson et al, 2004) These non-growth promoting effects of GH are considered so important for body homeostasis to require lifelong GH administration in subjects with permanent GHD (Molitch et al, 2006).
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