Body composition, metabolic syndrome and type 2 diabetes in Klinefelter syndrome

Acta Paediatrica, International Journal of Paediatrics

Volumn 100, Issue 6, 2011, Pages 871-877

  Gravholt, Claus H ^a   Jensen, Anne S ^a   Høst, Christian ^a   Bojesen, Anders ^b  

^a AARHUS UNIVERSITY HOSPITAL   (Denmark)

^b LILLEBAELT HOSPITAL   (Denmark)

Author keywords

Body composition; Diabetes; Klinefelter syndrome; Metabolic syndrome; Testosterone

Indexed keywords

ADIPONECTIN; C REACTIVE PROTEIN; CHORIONIC GONADOTROPIN; HIGH DENSITY LIPOPROTEIN CHOLESTEROL; INSULIN; PLACEBO; TESTOSTERONE; TRIACYLGLYCEROL;

ANDROGEN THERAPY; BODY COMPOSITION; BONE TURNOVER; CHOLESTEROL BLOOD LEVEL; DOSE RESPONSE; ENERGY EXPENDITURE; GLUCOSE TOLERANCE TEST; HORMONE SYNTHESIS; HUMAN; HYPOGONADISM; INSULIN RESISTANCE; INSULIN SENSITIVITY; KLINEFELTER SYNDROME; LEAN BODY WEIGHT; LEYDIG CELL; METABOLIC SYNDROME X; MORTALITY; MUSCLE WEAKNESS; NON INSULIN DEPENDENT DIABETES MELLITUS; NONHUMAN; OBESITY; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PROTEIN SYNTHESIS; REVIEW; TESTOSTERONE BLOOD LEVEL; TRIACYLGLYCEROL BLOOD LEVEL;

ANDROGENS; BODY COMPOSITION; DIABETES MELLITUS, TYPE 2; HORMONE REPLACEMENT THERAPY; HUMANS; HYPOGONADISM; KLINEFELTER SYNDROME; MALE; METABOLIC SYNDROME X; RISK FACTORS; TESTOSTERONE;

EID: 79955489888     PISSN: 08035253     EISSN: 16512227     Source Type: Journal    
DOI: 10.1111/j.1651-2227.2011.02233.x     Document Type: Review

References (50)

1. Klinefelter HF, Reifenstein EC, Albright F,. Syndrome characterized by gynecomastia,aspermatogenesis without a-leydigism, and increased excretion of follicle-stimulating hormone. J Clin Endocrinol 1942; 2: 615-27.
2. Swerdlow AJ, Hermon C, Jacobs PA, Alberman E, Beral V, Daker M, et al., Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study. Ann Hum Genet 2001; 65: 177-88. (Pubitemid 32452119)
3. Bojesen A, Juul S, Birkebaek N, Gravholt CH,. Increased mortality in Klinefelter syndrome. J Clin Endocrinol Metab 2004; 89: 3830-4. (Pubitemid 39071480)
4. Swerdlow AJ, Schoemaker MJ, Higgins CD, Wright AF, Jacobs PA,. Cancer incidence and mortality in men with Klinefelter syndrome: a cohort study. J Natl Cancer Inst 2005; 97: 1204-10. (Pubitemid 41258226)
5. Bojesen A, Juul S, Birkebaek NH, Gravholt CH,. Morbidity in Klinefelter syndrome: a Danish register study based on hospital discharge diagnoses. J Clin Endocrinol Metab 2006; 91: 1254-60.
6. Smyth CM, Bremner WJ,. Klinefelter syndrome. Arch Intern Med 1998; 158: 1309-14. (Pubitemid 28287523)
7. Bojesen A, Gravholt CH,. Klinefelter syndrome in clinical practice. Nat Clin Pract Urol 2007; 4: 192-204. (Pubitemid 46567152)
8. Handelsman DJ,. Update in andrology. J Clin Endocrinol Metab 2007; 92: 4505-11.
9. Swerdlow AJ, Higgins CD, Schoemaker MJ, Wright AF, Jacobs PA,. Mortality in patients with Klinefelter syndrome in Britain: a cohort study. J Clin Endocrinol Metab 2005; 90: 6516-22. (Pubitemid 41759307)
10. Jackson IM, Buchanan KD, McKiddie MT, Prentice CR,. Carbohydrate metabolism in Klinefelter's syndrome. J Endocrinol 1966; 35: 169-72.
11. Becker KL, Hoffman DL, Underdahl LO, Mason HL,. Klinefelter's syndrome. Clinical and laboratory findings in 50 patients. Arch Intern Med 1966; 118: 314-21.
12. Zuppinger K, Engel E, Forbes AP, Mantooth L, Claffey J,. Klinefelter's syndrome, a clinical and cytogenetic study in twenty-four cases. Acta Endocrinol (Copenh) 1967; 54 Suppl 113: 5-48.
13. 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: 1062-73.
14. Pei D, Sheu WH, Jeng CY, Liao WK, Fuh MM,. Insulin resistance in patients with Klinefelter's syndrome and idiopathic gonadotropin deficiency. J Formos Med Assoc 1998; 97: 534-40. (Pubitemid 28414523)
15. Yesilova Z, Oktenli C, Sanisoglu SY, Musabak U, Cakir E, Ozata M, et al., Evaluation of insulin sensitivity in patients with Klinefelter's syndrome: a hyperinsulinemic euglycemic clamp study. Endocrine 2005; 27: 11-6.
16. Bojesen A, Kristensen K, Birkebaek NH, Fedder J, Mosekilde L, Bennett P, et al., The metabolic syndrome is frequent in Klinefelter's syndrome and is associated with abdominal obesity and hypogonadism. Diabetes Care 2006; 29: 1591-8. (Pubitemid 44127586)
17. Ishikawa T, Yamaguchi K, Kondo Y, Takenaka A, Fujisawa M,. Metabolic syndrome in men with Klinefelter's syndrome. Urology 2008; 71: 1109-13.
18. Aksglaede L, Molgaard C, Skakkebaek NE, Juul A,. Normal bone mineral content but unfavourable muscle/fat ratio in Klinefelter syndrome. Arch Dis Child 2008; 93: 30-4.
19. Host C, Bojesen A, Frystyk J, Flyvbjerg A, Christiansen JS, Gravholt CH,. Effect of sex hormone treatment on circulating adiponectin and subforms in Turner and Klinefelter syndrome. Eur J Clin Invest 2010; 40: 211-9.
20. Bojesen A, Birkebaek N, Kristensen K, Heickendorff L, Mosekilde L, Christiansen JS, et al., Bone mineral density in Klinefelter syndrome is reduced and primarily determined by muscle strength and resorptive markers, but not directly by testosterone. Osteoporos Int 2010; Jul 24. [Epub ahead of print].
21. Andersen NH, Bojesen A, Kristensen K, Birkebaek NH, Fedder J, Bennett P, et al., Left ventricular dysfunction in Klinefelter syndrome is associated to insulin resistance, abdominal adiposity and hypogonadism. Clin Endocrinol (Oxf) 2008; 69: 785-91.
22. Andersen NH, Bojesen A, Christiansen JS, Gravholt CH,. Glycemia, lipidemia and systolic left ventricular function evaluated by myocardial strain rate: a tissue Doppler echocardiographic study. Ultrasound Med Biol 2008; 34: 151-4. (Pubitemid 350258592)
23. Bjorn AM, Bojesen A, Gravholt CH, Laurberg P,. Hypothyroidism secondary to hypothalamic-pituitary dysfunction may be part of the phenotype in klinefelter syndrome: a case-control study. J Clin Endocrinol Metab 2009; 94: 2478-81.
24. Jensen AS, Bojesen A, Gravholt CH,. Mild liver dysfunction in Klinefelter syndrome is associated with abdominal obesity and elevated total cholesterol. Endocrine Society Meeting 2009; P2-768 (abstarct).
25. Tsai EC, Boyko EJ, Leonetti DL, Fujimoto WY,. Low serum testosterone level as a predictor of increased visceral fat in Japanese-American men. Int J Obes Relat Metab Disord 2000; 24: 485-91. (Pubitemid 30198102)
26. Laaksonen DE, Niskanen L, Punnonen K, Nyyssonen K, Tuomainen TP, Valkonen VP, et al., Testosterone and sex hormone-binding globulin predict the metabolic syndrome and diabetes in middle-aged men. Diabetes Care 2004; 27: 1036-41. (Pubitemid 38579762)
27. Stellato RK, Feldman HA, Hamdy O, Horton ES, McKinlay JB,. Testosterone, sex hormone-binding globulin, and the development of type 2 diabetes in middle-aged men: prospective results from the Massachusetts male aging study. Diabetes Care 2000; 23: 490-4. (Pubitemid 30185792)
28. Abate N, Haffner SM, Garg A, Peshock RM, Grundy SM,. Sex steroid hormones, upper body obesity, and insulin resistance. J Clin Endocrinol Metab 2002; 87: 4522-7. (Pubitemid 35247035)
29. Tsai EC, Matsumoto AM, Fujimoto WY, Boyko EJ,. Association of bioavailable, free, and total testosterone with insulin resistance: influence of sex hormone-binding globulin and body fat. Diabetes Care 2004; 27: 861-8. (Pubitemid 38420205)
30. Lahlou N, Fennoy I, Carel JC, Roger M,. Inhibin B and anti-mullerian hormone, but not testosterone levels, are normal in infants with nonmosaic klinefelter syndrome. J Clin Endocrinol Metab 2004; 89: 1864-8. (Pubitemid 38507946)
31. Ross JL, Samango-Sprouse C, Lahlou N, Kowal K, Elder FF, Zinn A,. Early androgen deficiency in infants and young boys with 47,XXY Klinefelter syndrome. Horm Res 2005; 64: 39-45. (Pubitemid 41207077)
32. Aksglaede L, Petersen JH, Main KM, Skakkebaek NE, Juul A,. High normal testosterone levels in infants with non-mosaic Klinefelter's syndrome. Eur J Endocrinol 2007; 157: 345-50. (Pubitemid 47603540)
33. Bebakar WM, Honour JW, Foster D, Liu YL, Jacobs HS,. Regulation of testicular function by insulin and transforming growth factor-beta. Steroids 1990; 55: 266-70. (Pubitemid 20325479)
34. Pitteloud N, Hardin M, Dwyer AA, Valassi E, Yialamas M, Elahi D, et al., Increasing insulin resistance is associated with a decrease in Leydig cell testosterone secretion in men. J Clin Endocrinol Metab 2005; 90: 2636-41. (Pubitemid 40686302)
35. Pasquali R, Casimirri F, Cantobelli S, Melchionda N, Morselli Labate AM, Fabbri R, et al., Effect of obesity and body fat distribution on sex hormones and insulin in men. Metabolism 1991; 40: 101-4.
36. Dhindsa S, Prabhakar S, Sethi M, Bandyopadhyay A, Chaudhuri A, Dandona P,. Frequent occurrence of hypogonadotropic hypogonadism in type 2 diabetes. J Clin Endocrinol Metab 2004; 89: 5462-8. (Pubitemid 39518427)
37. Zitzmann M, Depenbusch M, Gromoll J, Nieschlag E,. X-chromosome inactivation patterns and androgen receptor functionality influence phenotype and social characteristics as well as pharmacogenetics of testosterone therapy in klinefelter patients. J Clin Endocrinol Metab 2004; 89: 6208-17. (Pubitemid 39628435)
38. Laaksonen DE, Niskanen L, Punnonen K, Nyyssonen K, Tuomainen TP, Salonen R, et al., Sex hormones, inflammation and the metabolic syndrome: a population-based study. Eur J Endocrinol 2003; 149: 601-8. (Pubitemid 38054707)
39. Nishizawa H, Shimomura I, Kishida K, Maeda N, Kuriyama H, Nagaretani H, et al., Androgens decrease plasma adiponectin, an insulin-sensitizing adipocyte-derived protein. Diabetes 2002; 51: 2734-41. (Pubitemid 34970926)
40. Lanfranco F, Zitzmann M, Simoni M, Nieschlag E,. Serum adiponectin levels in hypogonadal males: influence of testosterone replacement therapy. Clin Endocrinol (Oxf) 2004; 60: 500-7. (Pubitemid 38482514)
41. Page ST, Herbst KL, Amory JK, Coviello AD, Anawalt BD, Matsumoto AM, et al., Testosterone administration suppresses adiponectin levels in men. J Androl 2005; 26: 85-92. (Pubitemid 39665280)
42. Eyben FE, Graugaard C, Vaeth M,. All-cause mortality and mortality of myocardial infarction for 989 legally castrated men. Eur J Epidemiol 2005; 20: 863-9. (Pubitemid 41618751)
43. Marin P, Holmang S, Jonsson L, Sjostrom L, Kvist H, Holm G, et al., The effects of testosterone treatment on body composition and metabolism in middle-aged obese men. Int J Obes Relat Metab Disord 1992; 16: 991-7. (Pubitemid 23010023)
44. Bhasin S, Woodhouse L, Casaburi R, Singh AB, Bhasin D, Berman N, et al., Testosterone dose-response relationships in healthy young men. Am J Physiol Endocrinol Metab 2001; 281: E1172-81.
45. Singh AB, Hsia S, Alaupovic P, Sinha-Hikim I, Woodhouse L, Buchanan TA, et al., The effects of varying doses of T on insulin sensitivity, plasma lipids, apolipoproteins, and C-reactive protein in healthy young men. J Clin Endocrinol Metab 2002; 87: 136-43. (Pubitemid 34084682)
46. Mauras N, Hayes V, Welch S, Rini A, Helgeson K, Dokler M, et al., Testosterone deficiency in young men: marked alterations in whole body protein kinetics, strength, and adiposity. J Clin Endocrinol Metab 1998; 83: 1886-92. (Pubitemid 28496311)
47. Page ST, Amory JK, Bowman FD, Anawalt BD, Matsumoto AM, Bremner WJ, et al., Exogenous testosterone (T) alone or with finasteride increases physical performance, grip strength, and lean body mass in older men with low serum T. J Clin Endocrinol Metab 2005; 90: 1502-10. (Pubitemid 40464011)
48. Wang C, Cunningham G, Dobs A, Iranmanesh A, Matsumoto AM, Snyder PJ, et al., Long-term testosterone gel (AndroGel) treatment maintains beneficial effects on sexual function and mood, lean and fat mass, and bone mineral density in hypogonadal men. J Clin Endocrinol Metab 2004; 89: 2085-98. (Pubitemid 38619836)
49. Steidle C, Schwartz S, Jacoby K, Sebree T, Smith T, Bachand R,. AA2500 testosterone gel normalizes androgen levels in aging males with improvements in body composition and sexual function. J Clin Endocrinol Metab 2003; 88: 2673-81. (Pubitemid 36724441)
50. Wittert GA, Chapman IM, Haren MT, Mackintosh S, Coates P, Morley JE,. Oral testosterone supplementation increases muscle and decreases fat mass in healthy elderly males with low-normal gonadal status. J Gerontol A Biol Sci Med Sci 2003; 58: 618-25. (Pubitemid 36900168)