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Laron syndrome (LS), also known as growth hormone insensitivity or growth hormone receptor deficiency (GHRD), is an autosomal recessive disorder characterized by a lack of insulin-like growth factor 1 (IGF-1; somatomedin-C) production in response to growth hormone (GH; hGH; somatotropin). [6]
The diagnosis of growth hormone deficiency is a multi-step procedure that involves pituitary MRI, biochemical testing (growth hormone stimulation tests and measurement of IGF-1/IGFBP3), clinical and auxological examination, and genetic test results. [8]
Low IGF-1 levels are associated with cardiovascular disease, while high IGF-1 levels are associated with cancer. Mid-range IGF-1 levels are associated with the lowest mortality. A synthetic analog of IGF-1, mecasermin, is used for the treatment of growth failure in children with severe IGF-1 deficiency. [15]
Treatment is by growth hormone replacement using synthetic human growth hormone. [1] The frequency of the condition is unclear. [2] Most cases are initially noticed in children. [1] The genetic forms of this disease are estimated to affect about 1 in 7,000 people. [3] Most types occur equally in males and females though males are more often ...
Mecasermin, sold under the brand name Increlex, also known as recombinant human insulin-like growth factor-1 (rhIGF-1), is a recombinant form of human insulin-like growth factor 1 (IGF-I) which is used in the long-term treatment of growth failure and short stature in children with severe primary IGF-I deficiency, for instance due to growth hormone deficiency or Laron syndrome (growth hormone ...
The IGF-1 receptor is the "physiological" receptor. IGF-1 binds to it at significantly higher affinity than it binds the insulin receptor. Like the insulin receptor, the IGF-1 receptor is a receptor tyrosine kinase—meaning the receptor signals by causing the addition of a phosphate molecule on particular tyrosines. The IGF-2 receptor only ...
Tercica applied to the Food and Drug Administration (FDA) for approval of Increlex as a long-term therapy for growth failure in children with severe primary IGF-1 deficiency (Primary IGFD), which is characterized by growth failure, and as a treatment for children with growth hormone (GH) gene deletion who have developed neutralizing antibodies ...
Mecasermin rinfabate (INN, USAN) (brand name Iplex), also known as rhIGF-1/rhIGFBP-3, is a drug consisting of recombinant human insulin-like growth factor 1 (IGF-1) and recombinant human insulin-like growth factor binding protein-3 (IGFBP-3) which is used for the treatment of amyotrophic lateral sclerosis (Lou Gehrig's disease).