Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

Ota, Kikuko; Suehiro, Tadashi; Ikeda, Yukio; Arii, Kaoru; Kumon, Yoshitaka; Hashimoto, Kozo

doi:10.2169/internalmedicine.41.842

Cited by 24 publications

(20 citation statements)

References 18 publications

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“…Klinefelter's syndrome, the most frequent form of primary hypogonadism, is associated with insulin resistance and diabetes (34). In our study, approximately onethird of the hypogonadal men had either primary or secondary hypogonadism.…”

Section: Sex Hormones and Body Compositionsupporting

confidence: 42%

Clinical and Biochemical Assessment of Hypogonadism in Men With Type 2 Diabetes

Kapoor

Aldred²,

Clark³

et al. 2007

Diabetes Care

458

368

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OBJECTIVE -The aim of our study was to assess the prevalence of clinical hypogonadism, based on both symptoms and biochemical available measures of testosterone deficiency, in men with type 2 diabetes.RESEARCH DESIGN AND METHODS -In a cross-sectional study of 355 type 2 diabetic men aged Ͼ30 years, total and bioavailable testosterone, sex hormone-binding globulin, BMI, and waist circumference were measured and free testosterone was calculated. Overt hypogonadism was defined as the presence of clinical symptoms of hypogonadism and low testosterone level (total testosterone Ͻ8 nmol/l and/or bioavailable testosterone Ͻ2.5 nmol/l). Borderline hypogonadism was defined as the presence of symptoms and total testosterone of 8 -12 nmol/l or bioavailable testosterone of 2.5-4 nmol/l. RESULTS -A low blood testosterone level was common in diabetic men, and a significant proportion of these men had symptoms of hypogonadism. Overt hypogonadism was seen in 17% of men with total testosterone Ͻ8 nmol/l and 14% with bioavailable testosterone Ͻ2.5 nmol/l. Borderline hypogonadism was found in 25% of men with total testosterone 8 -12 nmol/l and bioavailable testosterone between 2.5 and 4 nmol/l; 42% of the men had free testosterone Ͻ0.255 nmol/l. BMI and waist circumference were both significantly negatively correlated with testosterone levels in men, with the association being stronger for waist circumference.CONCLUSIONS -Testosterone levels are frequently low in men with type 2 diabetes, and the majority of these men have symptoms of hypogonadism. Obesity is associated with low testosterone levels in diabetic men. Diabetes Care 30:911-917, 2007

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Section: Sex Hormones and Body Compositionsupporting

confidence: 42%

Clinical and Biochemical Assessment of Hypogonadism in Men With Type 2 Diabetes

Kapoor

Aldred²,

Clark³

et al. 2007

Diabetes Care

458

368

View full text Add to dashboard Cite

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“…However, low testosterone levels have been associated with diabetes mellitus and prediabetic status [15, 16], and plasma testosterone concentration was inversely related to insulin resistance in patients with Klinefelter syndrome and idiopathic gonadotropin deficiency [17]. Moreover, testosterone replacement improved glycemic control in a case of Klinefelter syndrome with diabetes mellitus [18]. It also has been reported that early treatment of Klinefelter syndrome with testosterone can significantly improve the patient’s quality of life and prevent serious consequences [19].…”

Section: Discussionmentioning

confidence: 99%

49,XXXXY Syndrome with Diabetes Mellitus

Kim

Kim²,

Shin³

et al. 2006

Horm Res Paediatr

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49,XXXXY syndrome is a rare sex chromosome aneuploidy and characterized by mental retardation, skeletal defects, craniofacial anomalies and hypogonadism. The increased frequency of diabetes mellitus in patients with Klinefelter syndrome and other types of X-chromosome polysomy has been reported, but no cases of diabetes mellitus in adult with 49,XXXXY syndrome have been reported so far. We report an 18-year-old patient with 49,XXXXY syndrome accompanying diabetes mellitus.

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“…Since Mirouze and coworkers coined the term "Prediabetes in KS" (19) in 1966, most studies reported an increased incidence of diabetes mellitus (DM) in KS (5,7,19,20,21,22,23,24,25,26). In large registrybased studies, Bojesen (5) and Swerdlow (7), taking into account cause-specific mortality ratios, showed a relative risk (RR) of DM of 1.64 and 7.07, respectively.…”

Section: Diabetesmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Klinefelter syndrome, cardiovascular system, and thromboembolic disease: review of literature and clinical perspectives

Salzano

Arcopinto

Marra

et al. 2016

European Journal of Endocrinology

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Klinefelter syndrome (KS) is the most frequently occurring sex chromosomal aberration in males, with an incidence of about 1 in 500 -700 newborns. Data acquired from large registry-based studies revealed an increase in mortality rates among KS patients when compared with mortality rates among the general population. Among all causes of death, metabolic, cardiovascular, and hemostatic complication seem to play a pivotal role. KS is associated, as are other chromosomal pathologies and genetic diseases, with cardiac congenital anomalies that contribute to the increase in mortality. The aim of the current study was to systematically review the relationships between KS and the cardiovascular system and hemostatic balance. In summary, patients with KS display an increased cardiovascular risk profile, characterized by increased prevalence of metabolic abnormalities including Diabetes mellitus (DM), dyslipidemia, and alterations in biomarkers of cardiovascular disease. KS does not, however, appear to be associated with arterial hypertension. Moreover, KS patients are characterized by subclinical abnormalities in left ventricular (LV) systolic and diastolic function and endothelial function, which, when associated with chronotropic incompetence may led to reduced cardiopulmonary performance. KS patients appear to be at a higher risk for cardiovascular disease, attributing to an increased risk of thromboembolic events with a high prevalence of recurrent venous ulcers, venous insufficiency, recurrent venous and arterial thromboembolism with higher risk of deep venous thrombosis or pulmonary embolism. It appears that cardiovascular involvement in KS is mainly due to chromosomal abnormalities rather than solely on low serum testosterone levels. On the basis of evidence acquisition and authors' own experience, a flowchart addressing the management of cardiovascular function and prognosis of KS patients has been developed for clinical use.

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Diabetes Mellitus Associated with Klinefelter's Syndrome: A Case Report and Review in Japan.

Cited by 24 publications

References 18 publications

Clinical and Biochemical Assessment of Hypogonadism in Men With Type 2 Diabetes

Clinical and Biochemical Assessment of Hypogonadism in Men With Type 2 Diabetes

49,XXXXY Syndrome with Diabetes Mellitus

MANAGEMENT OF ENDOCRINE DISEASE: Klinefelter syndrome, cardiovascular system, and thromboembolic disease: review of literature and clinical perspectives

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