Skip to main content
Top
Published in: BMC Endocrine Disorders 1/2021

Open Access 01-12-2021 | Insulins | Research

Glucose metabolic disorder in Klinefelter syndrome: a retrospective analysis in a single Chinese hospital and literature review

Authors: Shixuan Liu, Tao Yuan, Shuoning Song, Shi Chen, Linjie Wang, Yong Fu, Yingyue Dong, Yan Tang, Weigang Zhao

Published in: BMC Endocrine Disorders | Issue 1/2021

Login to get access

Abstract

Background

We aimed to investigate the clinical characteristics and islet β-cell function in patients with Klinefelter syndrome (KS) and hyperglycemia.

Methods

This is a retrospective study. In total, 22 patients diagnosed with KS were identified from the electronic medical record system, including 9 patients with hyperglycemia (total patients with hyperglycemia, THG-KS group) and 5 hyperglycemic KS patients with oral glucose tolerance test (OGTT) results (HG-KS group). An additional 5 subjects with hyperglycemia and 5 normal glucose tolerance (NGT) subjects matched based on body mass index were included as the HG group and NGT group, respectively. Data from clinical and laboratory examinations were collected. We further performed a literature review of KS and hyperglycemia.

Results

We found that KS patients developed abnormal glucose metabolism earlier in life than those without KS, and the median age was 17 years, ranging from 10 years to 19 years. Six of 17 (35.3%) patients were diagnosed with diabetes mellitus and 3 of 17 (17.6%) patients were diagnosed with prediabetes. Among 10 patients with both fasting blood glucose and insulin results recorded, there were 8 out of 17 (47.1%) KS patients had insulin resistance. The prevalence of hypertension and dyslipidemia was higher in patients with hyperglycemia and KS than in patients with NGT KS. Compared with the HG group, insulin sensitivity levels were lower in HG-KS group, whereas homeostasis model assessment of β-cell function levels (p = 0.047) were significantly, indicating higher insulin secretion levels in the HG-KS group.

Conclusions

KS patients develop hyperglycemia earlier in life than those without KS and show lower insulin sensitivity and higher insulin secretion. These patients also have a higher prevalence of other metabolic diseases and may have different frequencies of developing KS-related symptoms.
Appendix
Available only for authorised users
Literature
9.
go back to reference Nielsen J, Johansen K, Yde H. Frequency of diabetes mellitus in patients with Klinefelter's syndrome of different chromosome constitutions and the XYY syndrome. Plasma insulin and growth hormone level after a glucose load. J Clin Endocrinol Metab. 1969;29(8):1062–73.CrossRef Nielsen J, Johansen K, Yde H. Frequency of diabetes mellitus in patients with Klinefelter's syndrome of different chromosome constitutions and the XYY syndrome. Plasma insulin and growth hormone level after a glucose load. J Clin Endocrinol Metab. 1969;29(8):1062–73.CrossRef
10.
31.
go back to reference Hara S, Anderson RS, Bland W, Crump EP. Simultaneous occurrence of diabetes mellitus and Klinefelter's syndrome in two patients. J Natl Med Assoc. 1970;62(1):42–5.PubMedPubMedCentral Hara S, Anderson RS, Bland W, Crump EP. Simultaneous occurrence of diabetes mellitus and Klinefelter's syndrome in two patients. J Natl Med Assoc. 1970;62(1):42–5.PubMedPubMedCentral
32.
go back to reference Yoshiuchi I, Itoh N, Nakano M, Tatsumi C, Yokoyama K, Matsuyama T. Case report of Klinefelter's syndrome with severe diabetes, dyslipidemia, and stroke: the effect of pioglitazone and other anti-inflammatory agents on interleukin-6 and -8, tumor necrosis factor-alpha, and C-reactive protein. Diabetes Care. 2006;29(8):1981. https://doi.org/10.2337/dc06-0651.CrossRefPubMed Yoshiuchi I, Itoh N, Nakano M, Tatsumi C, Yokoyama K, Matsuyama T. Case report of Klinefelter's syndrome with severe diabetes, dyslipidemia, and stroke: the effect of pioglitazone and other anti-inflammatory agents on interleukin-6 and -8, tumor necrosis factor-alpha, and C-reactive protein. Diabetes Care. 2006;29(8):1981. https://​doi.​org/​10.​2337/​dc06-0651.CrossRefPubMed
36.
go back to reference Seno Y, Iwasaki Y, Aizawa-Abe M, et al. Facilitating screening of Klinefelter syndrome among patients with diabetes. J Diabetes Investig. 2019. Seno Y, Iwasaki Y, Aizawa-Abe M, et al. Facilitating screening of Klinefelter syndrome among patients with diabetes. J Diabetes Investig. 2019.
37.
go back to reference Isobe K, Niwa T, Ohkubo M, Ohba M, Shikano M, Watanabe Y. Klinefelter's syndrome accompanied by diabetes mellitus and diabetes insipidus. Intern Med. 1992;31(7):917–21.CrossRef Isobe K, Niwa T, Ohkubo M, Ohba M, Shikano M, Watanabe Y. Klinefelter's syndrome accompanied by diabetes mellitus and diabetes insipidus. Intern Med. 1992;31(7):917–21.CrossRef
41.
go back to reference Mirouze J, Jaffiol C, Bernard C, Cartry E. The prediabetes of Klinefelter’s syndrome. Diabete. 1966;14(2):57–9.PubMed Mirouze J, Jaffiol C, Bernard C, Cartry E. The prediabetes of Klinefelter’s syndrome. Diabete. 1966;14(2):57–9.PubMed
45.
go back to reference Zitzmann M, Bongers R, Werler S, et al. Gene expression patterns in relation to the clinical phenotype in Klinefelter syndrome. J Clin Endocrinol Metab. 2015;100(3):E518–23.CrossRef Zitzmann M, Bongers R, Werler S, et al. Gene expression patterns in relation to the clinical phenotype in Klinefelter syndrome. J Clin Endocrinol Metab. 2015;100(3):E518–23.CrossRef
46.
go back to reference Panimolle F, Tiberti C, Granato S, Semeraro A, Gianfrilli D, Anzuini A, et al. Screening of endocrine organ-specific humoral autoimmunity in 47,XXY Klinefelter's syndrome reveals a significant increase in diabetes-specific immunoreactivity in comparison with healthy control men. Endocrine. 2016;52(1):157–64. https://doi.org/10.1007/s12020-015-0613-y.CrossRefPubMed Panimolle F, Tiberti C, Granato S, Semeraro A, Gianfrilli D, Anzuini A, et al. Screening of endocrine organ-specific humoral autoimmunity in 47,XXY Klinefelter's syndrome reveals a significant increase in diabetes-specific immunoreactivity in comparison with healthy control men. Endocrine. 2016;52(1):157–64. https://​doi.​org/​10.​1007/​s12020-015-0613-y.CrossRefPubMed
47.
go back to reference Pei D, Sheu WH, Jeng CY, Liao WK, Fuh MM. IInsulin resistance in patients with Klinefelter’s syndrome and idiopathic gonadotropin deficiency. J Formos Med Assoc. 1998;97(8):534–40.PubMed Pei D, Sheu WH, Jeng CY, Liao WK, Fuh MM. IInsulin resistance in patients with Klinefelter’s syndrome and idiopathic gonadotropin deficiency. J Formos Med Assoc. 1998;97(8):534–40.PubMed
48.
go back to reference Lee KW, Cha CK, Uhm C, Kwon OY, Lee SK, Chung YS, et al. Impaired glucose tolerance associated with klinefalter syndrome. J Korean Soc Endocrinol. 1998;13:495–500. Lee KW, Cha CK, Uhm C, Kwon OY, Lee SK, Chung YS, et al. Impaired glucose tolerance associated with klinefalter syndrome. J Korean Soc Endocrinol. 1998;13:495–500.
52.
go back to reference Grossmann M, Hoermann R, Wittert G, Yeap BB. Effects of testosterone treatment on glucose metabolism and symptoms in men with type 2 diabetes and the metabolic syndrome: a systematic review and meta-analysis of randomized controlled clinical trials. Clin Endocrinol (Oxf). 2015;83(3):344–51.CrossRef Grossmann M, Hoermann R, Wittert G, Yeap BB. Effects of testosterone treatment on glucose metabolism and symptoms in men with type 2 diabetes and the metabolic syndrome: a systematic review and meta-analysis of randomized controlled clinical trials. Clin Endocrinol (Oxf). 2015;83(3):344–51.CrossRef
Metadata
Title
Glucose metabolic disorder in Klinefelter syndrome: a retrospective analysis in a single Chinese hospital and literature review
Authors
Shixuan Liu
Tao Yuan
Shuoning Song
Shi Chen
Linjie Wang
Yong Fu
Yingyue Dong
Yan Tang
Weigang Zhao
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Endocrine Disorders / Issue 1/2021
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-021-00893-5

Other articles of this Issue 1/2021

BMC Endocrine Disorders 1/2021 Go to the issue