Insulin resistance depends on GH counter-regulation in two syndromes of short stature. (February 2018)

• Record Type: Journal Article
• Authors: Guevara-Aguirre, Jaime; Guevara, Alexandra; Bahamonde, Marisol
• Is Part Of: Growth hormone & IGF research. Volume 38(2018)
• Page Start: 44
• Page End: 48
• Publication Date: 2018-02
• Subjects: Laron syndrome (LS) -- New syndrome (NS) -- GH counter-regulation -- Insulin secretion -- Insulin resistance -- Cyclin-dependent kinase-1c gof mutation; Growth regulators -- Periodicals; Growth -- Regulation -- Periodicals; Somatomedin -- Periodicals; Somatomedins -- Periodicals; Growth Hormone -- Periodicals; Growth Substances -- Periodicals; Croissance -- Régulation -- Périodiques; Croissance -- Régulateurs -- Périodiques; Somatotrophine -- Périodiques; Somatomédine -- Périodiques; Growth -- Regulation; Growth regulators; Electronic journals; Periodicals; Electronic journals; 612.4
• DOI: 10.1016/j.ghir.2017.12.012
• Languages: English
• ISSNs: 1096-6374

Abstract

Abstract: Specific phenotypic features of subjects affected with genetic syndromes depend on peculiarities of expression of each discrete mutation and on extent of its divergence from normal physiology. In this context, and when studying the GH/IGF-I axis of subjects with two different syndromes that include severe short stature (SSS), we noticed different metabolic phenotypes in each cohort. Subjects with Laron syndrome (LS), who have GH insensitivity (GHI), display obesity, increased body fat, enhanced insulin sensitivity and diminished incidence of diabetes mellitus. Subjects with a new syndrome (NS), who have normal GH signaling, display intrauterine growth retardation (IUGR), normal to slightly elevated body fat content, insulin resistance and early onset type 2 diabetes mellitus (T2DM). In consequence, we were able to observe the clinical consequences of different GH counter-regulation status on carbohydrate metabolism, especially considering that subjects with either syndrome most likely have diminished pancreatic reserve. Highlights: Insulin resistance appears determined by GH counter-regulation in two syndromes of short stature in Ecuador Laron syndrome: Absent GH counter-regulation, normal birth size, adult obesity, low incidence of type 2 diabetes mellitus New syndrome: Normal GH counter-regulation, IUGR, normal BMI, insulin resistance, early onset Type 2 diabetes mellitus Both LS and NS most probably have diminished insulin secretion