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Observational Study
. 2021;94(7-8):297-306.
doi: 10.1159/000520233. Epub 2021 Oct 15.

Increased Prevalence of Beta-Cell Dysfunction despite Normal HbA1c in Youth and Young Adults with Turner Syndrome

Nicole Sheanon  1 , Deborah Elder  1 , Jane Khoury  2 , Lori Casnellie  3 , Iris Gutmark-Little  1 , Joseph Cernich  4 , Phillipe F Backeljauw  1
Affiliations
Observational Study

Increased Prevalence of Beta-Cell Dysfunction despite Normal HbA1c in Youth and Young Adults with Turner Syndrome

Nicole Sheanon et al. Horm Res Paediatr. 2021.

Abstract

Introduction: Adult women with Turner syndrome (TS) have a high prevalence of diabetes and β-cell dysfunction that increases morbidity and mortality, but it is unknown if there is β-cell dysfunction present in youth with TS. This study aimed to determine the prevalence of β-cell dysfunction in youth with TS and the impact of traditional therapies on insulin sensitivity (SI) and insulin secretion.

Methods: Cross-sectional, observational study recruited 60 girls with TS and 60 healthy controls (HC) matched on pubertal status. Each subject had a history, physical exam, and oral glucose tolerance test (OGTT). Oral glucose and c-peptide minimal modeling was used to determine β-cell function.

Results: Twenty-one TS girls (35%) met criteria for prediabetes. Impaired fasting glucose was present in 18% of girls with TS and 3% HC (p value = 0.02). Impaired glucose tolerance was present in 23% of TS girls and 0% HC (p value <0.001). The hemoglobin A1c was not different between TS and HC (median 5%, p = 0.42). Youth with TS had significant reductions in SI, β-cell responsivity (Φ), and disposition index (DI) compared to HC. These differences remained significant when controlling for body mass index z-score (p values: 0.0006, 0.002, <0.0001 for SI, Φ total, DI, respectively).

Conclusions: β-Cell dysfunction is present in youth with TS compared to controls. The presence of both reduced insulin secretion and SI suggest a unique TS-related glycemic phenotype. Based on the data from this study, we strongly suggest that providers employ serial OGTT to screen for glucose abnormalities in TS youth.

Keywords: Diabetes; Glucose; Turner syndrome; Youth; β-Cell function.

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Conflict of interest statement

Conflict of Interest Statement

Joseph Cernich has a consulting agreement with Novo-Nordisk. The remaining authors have no disclosures.

Figures

Figure 1:
Figure 1:
a- Glucose vs time during OGTT, b- Insulin vs time during OGTT, c- C-peptide vs time during OGTT
Figure 1:
Figure 1:
a- Glucose vs time during OGTT, b- Insulin vs time during OGTT, c- C-peptide vs time during OGTT
Figure 1:
Figure 1:
a- Glucose vs time during OGTT, b- Insulin vs time during OGTT, c- C-peptide vs time during OGTT
Figure 2.
Figure 2.
Minimal model Indices in HC vs TS
Figure 3.
Figure 3.
Minimal model Indices based on Pubertal Status a. Insulin sensitivity index, b. β cell Responsivity, and c. Disposition Index
Figure 3.
Figure 3.
Minimal model Indices based on Pubertal Status a. Insulin sensitivity index, b. β cell Responsivity, and c. Disposition Index
Figure 3.
Figure 3.
Minimal model Indices based on Pubertal Status a. Insulin sensitivity index, b. β cell Responsivity, and c. Disposition Index
See this image and copyright information in PMC

References

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