The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.
Impaired Insulin Secretion in the Turner Metabolic Syndrome
Abstract
An increased prevalence of impaired glucose homeostasis (IGH) and diabetes mellitus is reported in monosomy X, or Turner syndrome (TS). To determine whether IGH is an intrinsic feature of this syndrome, independent of obesity or hypogonadism, we compared results of a standard oral glucose challenge in age- and body mass index-matched women with TS and with karyotypically normal premature ovarian failure (POF). Fasting glucose levels were normal in both groups, but glucose values after oral glucose challenge were higher in TS [2-h glucose, 135 +/- 36 mg/dl (7.5 +/- 2.0 mmol/liter) in TS and 97 +/- 18 mg/dl (5.4 +/- 1.0 mmol/liter) in POF; P < 0.0001]. Glucose-stimulated insulin secretion was lower in TS; e.g. the initial insulin response (DeltaI/DeltaG(30)) was decreased by 60% compared with POF (P < 0.0001). We also compared responses to a standard iv glucose tolerance test in women with TS and in age- and body mass index-matched normal women and found that the insulin area under the curve was 50% lower in women with TS (P = 0.003). Insulin sensitivity measured by the quantitative insulin sensitivity check index was higher in women with TS compared with both control groups. Thus, IGH is not secondary to obesity or hypogonadism in TS, but it is a distinct entity characterized by decreased insulin secretion, suggesting that haploinsufficiency for X-chromosome gene(s) impairs beta-cell function and predisposes to diabetes mellitus in TS.
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“Patients with TS are inherently at increased risk of disorders of carbohydrate metabolism226,227 and have a specific defect in glucose-stimulated insulin secretion.227,228”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [227]
In the citing paper
“Individuals with TS are at an increased risk for both type 1 and type 2 diabetes.29,222,227,357,384,706”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [227]
In the citing paper
“Furthermore, multiple studies demonstrate impaired beta cell function as well as reduced insulin sensitivity are involved in the development of diabetes in TS ,227,229,384,705,707,708 while one study found glucose intolerance despite apparently normal beta cell function.709”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [227]
Citations
Cites
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“Insulin sensitivity measured by the quantitative insulin sensitivity check index (15) was higher in women with TS (0.”
MethodSelf-citationWildin, R. S., Ramsdell, F., Peake, J., Faravelli, F., Casanova, J., Buist, N. R. M., Levy‐Lahad, E., Mazzella, M., Goulet, O., Perroni, L., et al. (2001) · Nature Genetics · doi:10.1038/83707 · Cited by 1,916
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“fold increase in insulin-dependent diabetes mellitus (defined by the use of insulin) and an approximately 4-fold increase in noninsulin-dependent diabetes mellitus in TS (3).”
BackgroundResultInsulin release and peripheral sensitivity at the oral glucose tolerance test
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Items 101–136 of 136
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Moreno, C. N., Reche, F. C., Pintor, A. V., Enríquez, F. V., & Román, M. D. R. B. (2014) · Endocrinología y Nutrición (English Edition) · doi:10.1016/j.endoen.2014.05.003 · Cited by 1
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Binder, G., Frank, L., Ziegler, J., Blumenstock, G., & Schweizer, R. (2017) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem-2016-0295 · Cited by 1
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Other Specific Types of Diabetes
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Self-citationGonadal dysgenesis: ovarian function and reproductive health in Turner syndrome
Saenger, P. H., & Rodriguez‐Buritica, D. (2013) · Cambridge University Press eBooks · doi:10.1017/cbo9781139003254.004
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Nagaeva, E. V. (2011) · Problems of Endocrinology · doi:10.14341/probl201157171-79
Kim, J. H., Kang, M. J., Shin, C. H., & Yang, S. W. (2009) · Korean Journal of Pediatrics · doi:10.3345/kjp.2009.52.3.370
atualização Epidemiological, Endocrine and Metabolic Features in Turner Syndrome
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Obara-Moszyńska, M., Banaszak-Ziemska, M., & Niedziela, M. (2015)
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Backeljauw, P. F., & Gutmark‐Little, I. (2018) · Springer eBooks · doi:10.1007/978-3-319-73782-9_6
Kahlert, E. (2019) · eDiss (Georg-August-Universität Göttingen) · doi:10.53846/goediss-7332
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Siqueira, R. D. A., Carlos, A. S., d’Avila, J. C., Moreno, A. M., Alves, E. L., Farias, M. L. F., de Mendonça, L. M. C., & Guimarães, M. M. (2020) · Archives of Endocrinology and Metabolism · doi:10.20945/2359-3997000000287
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Gutmark‐Little, I., Muthuvel, G., & Backeljauw, P. F. (2024) · Springer eBooks · doi:10.1007/978-3-031-66296-6_6
Bartel, W. P., Accordino, R. E., Awidi, B., & Prager, L. M. (2024) · Springer eBooks · doi:10.1007/978-3-031-76269-7_11
Metadata
- Authors
- Bakalov, V. K., Cooley, M. M., Quon, M. J., Luo, M. L., Yanovski, J. A., Nelson, L. M., Sullivan, G., & Bondy, C. A.
- Affiliations
- Developmental Endocrinology Branch (V.K.B., M.M.C., M.L.L., J.A.Y., L.M.N., C.A.B.), Bethesda, Maryland 20892
- National Institutes of Health (US)
- Corresponding author
- Bondy, Carolyn A.
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2004-07-01
- Volume
- 89
- Issue
- 7
- Pages
- 3516-3520
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2149926350
- Cited by
- 137
- References
- 24
- Citations by year
- 2025: 3 · 2024: 6 · 2023: 6 · 2022: 3 · 2021: 5 · 2020: 6 · 2019: 6 · 2018: 6 · 2017: 7 · 2016: 2 · 2015: 4 · 2014: 6 · 2013: 7 · 2012: 8
- FWCI
- 5.18
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (Z99HD999999)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (Z01HD000641)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes, Cardiovascular Risks, and Lipoproteins
- Keywords
- Turner syndrome, monosomy X, impaired glucose homeostasis, impaired insulin secretion, beta-cell function, premature ovarian failure, insulin sensitivity, QUICKI, diabetes risk, oral glucose tolerance test
- MeSH terms
- Insulin Secretion, Adult, Blood Glucose, Female, Glucose, Glucose Tolerance Test, Humans, Insulin, Turner Syndrome, Case-Control Studies, Primary Ovarian Insufficiency, Area Under Curve
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