Glucose Metabolism in Turner Syndrome

Abstract

Turner syndrome (TS) is one of the most common female chromosomal disorders. The condition is caused by complete or partial loss of a single X chromosome. Adult patients with TS have a high prevalence of diabetes mellitus (DM). Deranged glucose metabolism in this population seems to be genetically triggered. The traditional risk factors for DM in the general population may not play a major role in the pathogenesis of DM in patients with TS. This review focuses on the latest research studies pertaining to abnormalities of glucose metabolism in TS. We extensively review the available evidence pertaining to the influence of insulin secretion and sensitivity, obesity, autoimmunity, lifestyle, growth hormone, and sex hormone replacement therapy on the occurrence of DM in these patients.

Keywords: diabetes mellitus; estrogen; glucose metabolism; growth hormone; insulin resistance; turner syndrome.

Figure 1

Schematic illustration of the pathogenetic mechanisms of the development of diabetes mellitus (DM) in patients with Turner syndrome. The circle of impaired β-cell function is bigger than the one of insulin resistance, as we believe that insulin secretory response to glucose is a facet of TS that likely underlies the elevated risk of DM in these patients. It is the same for the circles of type 1 DM and type 2 DM. Among all the pathogenetic factors, X-chromosomal disorder may play the most important role. The arrows “→ ” indicate that more evidence may still be needed in this respect. GH: growth hormone; EP: Estradiol and progesterone; T1DM, type 1 diabetes mellitus; T2DM, type 2 diabetes mellitus.