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Severe insulin-like growth factor-1 deficiency (SPIGFD) is distinct from growth hormone deficiency (GHD)2. On this page you can read about the relationship between insulin-like growth factor (IGF)-1 and growth hormone (GH) in controlling growth in children and how SPIGFD develops.
IGF-1 is a 70-amino acid peptide hormone and growth factor considered one of the most important hormonal regulators of postnatal growth. 2,3 The major regulator of circulating IGF-1 is growth hormone (GH). 3
Growth
GH is secreted from the pituitary gland and binds to the GH receptor (GHR) in many tissues, including the growth plate and the liver. 2,4
Although GH has direct effects at the growth plate, its major growth-promoting action is mediated through the GHR in the liver, which stimulates the production of IGF-1. 4
IGF-1 is released from the liver into the peripheral tissues, where it has a direct impact on linear bone growth and bone remodeling.
GH, growth hormone; IGF-1; insulin-like growth factor. GH activates GHR in the liver to stimulate IGF-1 production and release; Circulating IGF-1 promotes normal growth.2,5
GH-IGF-1 Relationship
Deficiency in IGF-1 may lead to short stature in children and adolescents.6 Primary IGFD is characterized by abnormally low levels of IGF-1 in the presence of normal or elevated GH levels. 2,7
Normal growth becomes disrupted due to the inability of IGF-1 to mediate the growth-promoting activity of GH. 2, 7
GH, growth hormone; IGF-1; insulin-like growth factor-1; IGFD, IGF-1 deficiency. Primary IGFD is defined by low IGF-1 concentrations despite the presence of normal or elevated GH concentrations. GH is unable to activate the GHR in the liver or the GHR signaling is defective. IGF-1 is not stimulated and growth rate is affected. 1,2
IGF-1 Deficiency
After ruling out other diseases and inadequate nutrition, growth failure caused by SPIGFD is defined by:1
IGFD becomes SPIGFD
SPIGFD arises from a deficiency of either the production or peripheral action of IGF-1 on linear growth. 6
These defects may arise from a number of causes, including: 1,2,6
GH, growth hormone; GHR, growth hormone receptor; IGF-1; insulin-like growth factor-1; IGFD, IGF-1 deficiency.
What Causes SPIGFD?
Common adverse reactions include hypoglycemia, local and systemic hypersensitivity, and tonsillar hypertrophy.
INCRELEX® (mecasermin) is indicated for the treatment of growth failure in pediatric patients aged 2 years and older with severe primary IGF-1 deficiency* (IGFD), or with hormone (GH) gene deletion who have developed neutralizing antibodies to GH.1
Limitations of use: INCRELEX® is not a substitute to GH for approved GH indications. INCRELEX® is not indicated for use in patients with secondary forms of IGFD, such as GH deficiency, malnutrition, hypothyroidism, or chronic treatment with pharmacologic doses of anti-inflammatory steroids.1
*Severe primary IGF-1 deficiency (IGFD) is defined by height standard deviation score ≤ -3.0 and basal IGF-1 standard deviation score ≤ -3.0 and normal or elevated GH.