Insulin-Like Growth Factor-I and Insulin-Like Growth Factor Binding Protein-3 Cotreatment versus Insulin-Like Growth Factor-I Alone in Two Brothers with Growth Hormone Insensitivity Syndrome: Effects on Insulin Sensitivity, Body Composition and Linear Growth
2011 (English)In: HORMONE RESEARCH IN PAEDIATRICS, ISSN 1663-2818, Vol. 76, no 5, 355-366 p.Article in journal (Refereed) Published
Background/Aims: Growth hormone insensitivity syndrome (GHIS) is caused by a defective growth hormone receptor (GHR) and is associated with insulin-like growth factor-I (IGF-I) deficiency, severely short stature and, from adolescence, fasting hyperglycemia and obesity. We studied the effects of treatment with IGF-I in either a 1:1 molar complex with IGFBP-3 (IGF-I/BP-3-Tx) or with IGF-I alone (IGF-I-Tx) on metabolism and linear growth. Methods: Two brothers, compound heterozygous for a GHR gene defect, were studied. After 8 months without treatment, we examined the short- and long-term effects of IGF-I/BP-3-Tx and, subsequently, IGF-I-Tx on 12-hour overnight levels of IGF-I, GH, insulin, IGFBP-1, insulin sensitivity by hyperinsulinemic euglycemic clamp, body composition by dual-energy X-ray absorptiometry and linear growth. Results: Mean overnight levels of insulin decreased and IGFBP-1, a measure of hepatic insulin sensitivity, increased on both regimens, but was more pronounced on IGF-I-Tx. Insulin sensitivity by clamp showed no consistent changes. Lean body mass increased and abdominal fat mass decreased in both subjects on IGF-I-Tx. However, the changes were inconsistent during IGF-I/BP-3-Tx. Height velocity was low without treatment, increased slightly on IGF-I/BP-3-Tx and doubled on IGF-I-Tx. Conclusion: Both modalities of IGF-I improved determinants of hepatic insulin sensitivity, body composition and linear growth rate; however, IGF-I alone seemed to be more efficient.
Place, publisher, year, edition, pages
Karger , 2011. Vol. 76, no 5, 355-366 p.
Growth hormone insensitivity syndrome; Insulin-like growth factor-I; Body composition; Growth; Children
Medical and Health Sciences
IdentifiersURN: urn:nbn:se:liu:diva-73360DOI: 10.1159/000330410ISI: 000297576800010OAI: oai:DiVA.org:liu-73360DiVA: diva2:471329