Abstract

BackgroundTo investigate glucose homeostasis in detail in Turner syndrome (TS), where impaired glucose tolerance (IGT) and type 2 diabetes are frequent.MethodsCross sectional study of women with Turner syndrome (TS)(n = 13) and age and body mass index matched controls (C) (n = 13), evaluated by glucose tolerance (oral and intravenous glucose tolerance test (OGTT and IVGTT)), insulin sensitivity (hyperinsulinemic, euglycemic clamp), beta-cell function (hyperglycaemic clamp, arginine and GLP-1 stimulation) and insulin pulsatility.ResultsFasting glucose and insulin levels were similar. Higher glucose responses was seen in TS during OGTT and IVGTT, persisting after correction for body weight or muscle mass, while insulin responses were similar in TS and C, despite the higher glucose level in TS, leading to an insufficient increase in insulin response during dynamic testing. Insulin sensitivity was comparable in the two groups (TS vs. control: 8.6 ± 1.8 vs. 8.9 ± 1.8 mg/kg*30 min; p = 0.6), and the insulin responses to dynamic β-cell function tests were similar. Insulin secretion patterns examined by deconvolution analysis, approximate entropy, spectral analysis and autocorrelation analysis were similar. In addition we found low IGF-I, higher levels of cortisol and norepinephrine and an increased waist-hip ratio in TS.ConclusionsYoung normal weight TS women show significant glucose intolerance in spite of normal insulin secretion during hyperglycaemic clamping and normal insulin sensitivity. We recommend regularly testing for diabetes in TS.Trial RegistrationRegistered with http://clinicaltrials.com, ID nr: NCT00419107

Highlights

  • To investigate glucose homeostasis in detail in Turner syndrome (TS), where impaired glucose tolerance (IGT) and type 2 diabetes are frequent

  • IGT is present in 25-78% of adult TS populations evaluated by oral glucose tolerance testing (OGTT) [4,5], and seems to be more prevalent in TS compared to both healthy controls and women with premature ovarian failure and reduced oestrogen exposure [5]

  • Anthropometry and energy expenditure The close matching of TS and controls resulted in comparable age, BMI, fat mass (FM) (%) and lean body mass (LBM) (%) (Table 1)

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Summary

Introduction

To investigate glucose homeostasis in detail in Turner syndrome (TS), where impaired glucose tolerance (IGT) and type 2 diabetes are frequent. Turner syndrome (TS) is usually associated with reduced adult height and gonadal dysgenesis, premature ovarian failure and infertility. Reports of impaired glucose tolerance (IGT) in TS [1,2] have been followed by studies finding several abnormalities of the glucose metabolism in both girls [3] and women [4,5] with TS. IGT is present in 25-78% of adult TS populations evaluated by oral glucose tolerance testing (OGTT) [4,5], and seems to be more prevalent in TS compared to both healthy controls and women with premature ovarian failure and reduced oestrogen exposure [5]. The exact mechanism behind the increased occurrence of type 2 diabetes is not clear

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