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IGF-I and IGF Binding Protein-3 Generation Tests and Response to Growth Hormone in Children with Silver-Russell
Izabel C R Beserra1, Márcia G Ribeiro, Paulo F Collett-Solberg
1Divisão de Endocrinologia Pediátrica, Instituto de Puericultura e Pediatria Martagão Gesteira, Rua Bruno Lobo, 50, Rio de Janeiro, RJ 21941-912, Brazil.
Insights
Children with Silver-Russell Syndrome (SRS) show normal responses to the Insulin-like Growth Factor-I (IGF-I) generation test. This test did not correlate with growth velocity changes after 6 months of recombinant human Growth Hormone (rhGH) therapy.
Area of Science:
- Pediatric Endocrinology
- Growth Disorders
- Metabolic Research
Background:
- Silver-Russell Syndrome (SRS) is a rare genetic disorder characterized by intrauterine and postnatal growth retardation.
- Evaluating growth response in SRS patients is crucial for effective treatment strategies.
Purpose of the Study:
- To assess the IGF-I and IGFBP-3 generation test response in children with SRS.
- To compare this response with the actual growth velocity changes after 6 months of recombinant human Growth Hormone (rhGH) therapy.
Main Methods:
- Eight children diagnosed with SRS underwent a 6-month treatment with rhGH.
- IGF-I and IGFBP-3 levels were measured before and after four doses of rhGH.
- Growth velocity (GV) was monitored throughout the treatment period.
Main Results:
- The mean baseline growth velocity was 5.28 cm/year, increasing to 10.3 cm/year in five children after rhGH treatment.
- Most children exhibited normal baseline IGF-I levels, with levels normalizing in seven after rhGH administration.
- No significant correlation was found between the IGF-I generation test results and the observed growth response.
Conclusions:
- Children with SRS demonstrate a normal IGF-I generation test response.
- The IGF-I generation test is not a reliable predictor of growth velocity response to rhGH therapy in SRS patients.
Abstract:
Objectives. To evaluate, in children with Silver-Russell Syndrome, the response to the IGF-I and IGFBP-3 generation test and compare results to the growth response after 6 months of rhGH. Methods. Eight children (6 males), with a mean age of 5.71 ± 2.48 years and height SDS of -3.88 ± 1.28 received rhGH for 6 months. IGF-I and IGFBP-3 were analyzed before and after 4 doses of rhGH. Results. The mean growth velocity (GV) before treatment was 5.28 ± 1.9 cm/year. GV increased after rhGH in five children to a mean GV of 10.3 ± 3.64 cm/year. Six children had normal basal IGF-I levels and two low levels. After 4 doses of rhGH, the IGF-I levels were normal in seven. There was no correlation between the growth response and the IGF-I generation test. Conclusions. Children with SRS have normal IGF-I generation test. There is no correlation between the generation test and the growth velocity after 6 months of rhGH.
