The Korean Journal of Medicine

  • 제79권6호
  • /
  • Pages.701-704
  • /
  • 2010
  • /
  • 1738-9364(pISSN)
  • /
  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지

클라인펠터증후군과 동반된 제2형 당뇨병 1예

A case of diabetes mellitus associated with Klinefelter's syndrome

  • 윤지영 (한양대학교 의과대학 내과학교실) ;
  • 홍상모 (한양대학교 의과대학 내과학교실) ;
  • 박용수 (한양대학교 의과대학 내과학교실) ;
  • 김동선 (한양대학교 의과대학 내과학교실) ;
  • 최웅환 (한양대학교 의과대학 내과학교실) ;
  • 김태화 (한양대학교 의과대학 내과학교실) ;
  • 안유헌 (한양대학교 의과대학 내과학교실)
  • Yoon, Ji-Young (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Hong, Sang-Mo (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Park, Yong-Soo (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Kim, Dong-Sun (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Choi, Woong-Hwan (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Kim, Tae-Wha (Department of Internal Medicine, Hanyang University College of Medicine) ;
  • Ahn, You-Hern (Department of Internal Medicine, Hanyang University College of Medicine)
  • 투고 : 2009.10.08
  • 심사 : 2009.12.22
  • 발행 : 2010.12.01
⟨ 이전 논문 다음 논문 ⟩

초록

제2형 당뇨병 환자가 경구 혈당강하제로 불규칙적인 치료를 해오던 중 뒤늦게 성선기능저하증이 발견되어 클라인 펠터증후군을 진단받고 테스토스테론 보충요법과 인슐린치료를 시행한 증례로서 당뇨병 및 인슐린저항성과 성호르몬결핍 간의 밀접한 연관성에 대한 인식을 제고하는 바이다.

An increased frequency of diabetes or impaired glucose tolerance in Klinefelter's syndrome has been previously reported. Insulin resistance is considered to be the cause of this phenomenon in Klinefelter's syndrome, which is associated with low serum SHBG and sex hormone deficiency. Sex hormone deficiencies also result in dyslipidemia and metabolic syndrome. The interrelationship between diabetes, metabolic syndrome, and androgen deficiency is complex. Here we report a case of an 18-year-old man first diagnosed with diabetes mellitus 3 years ago. Upon physical examination the patient showed characteristic phenotypes compatible with primary hypogonadism. Subsequently, tests aimed at determining the cause of hypogonadism, including a chromosomal analysis, suggested Klinefelter's syndrome. The patient's HOMA-IR score was compatible with insulin resistance. Therefore, when diabetes mellitus develops at a young age with characteristic phenotypes, a careful history and physical examination may be needed to determine whether the patient might have primary hypogonadism caused by Klinefelter's syndrome.

키워드

참고문헌

  1. Lee YJ, Choe YJ, Chin KC, Lee EB, Kim HA, Song YW. A case of Klinefelter's syndrome associated with systemic lupus erythematosus. Korean J Med 59:331-334, 2000
  2. Bojesen A, Gravholt CH. Klinefelter syndrome in clinical practice. Nat Clin Pract Urol 4:192-204, 2007 https://doi.org/10.1038/ncpuro0775
  3. Spark RF. Testosterone, diabetes mellitus, and the metabolic syndrome. Curr Urol Rep 8:467-471, 2007 https://doi.org/10.1007/s11934-007-0050-4
  4. Kalyani RR, Dobs AS. Androgen deficiency, diabetes, and the metabolic syndrome in men. Curr Opin Endocrinol Diabetes Obes 14:226-234, 2007 https://doi.org/10.1097/MED.0b013e32814db856
  5. 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 29:1062-1073, 1969 https://doi.org/10.1210/jcem-29-8-1062
  6. Becker KL, Hoffman DL, Underdahl LO, Mason HL. Klinefelter's syndrome: clinical and laboratory findings in 50 patients. Arch Intern Med 118:314-321, 1966 https://doi.org/10.1001/archinte.118.4.314
  7. Forbes AP, Engel E. The high incidence of diabetes mellitus in 41 patients with gonadal dysgenesis, and their close relatives. Metabolism 12:428-439, 1963
  8. Jackson IM, Buchanan KD, McKiddie MT, Prentice CR. Carbohydrate metabolism in Klinefelter's syndrome. J Endocrinol 35:169-172, 1966 https://doi.org/10.1677/joe.0.0350169
  9. Takeuchi Y, Murata Y, Sintani J, Masukawa H, Nakamura R, Oi K, Kato Y, Niinomi M. Klinefelter's syndrome accompanied by mixed connective tissue disease and diabetes mellitus. Intern Med 38:875-881, 1999 https://doi.org/10.2169/internalmedicine.38.875
  10. Aoki N. Klinefelter's syndrome, autoimmunity, and associated endocrinopathies. Intern Med 38:838-839, 1999 https://doi.org/10.2169/internalmedicine.38.838
  11. Kocar IH, Yesilova Z, Ozata M, Turan M, Sengul A, Ozdemir I. The effect of testosterone replacement treatment on immunological features of patients with Klinefelter's syndrome. Clin Exp Immunol 121:448-452, 2000 https://doi.org/10.1046/j.1365-2249.2000.01329.x
  12. Lee KW, Cha CK, Uhm C, Kwon OY, Lee SK, Chung YS, Kim HM, Kim YJ, Hong EK, Chae BN. Impaired glucose tolerance associated with Klinefelter syndrome. J Korean Soc Endocrinol 13:495-500, 1998
  13. Abate N, Haffner SM, Garg A, Peshock RM, Grundy SM. Sex steroid hormones, upper body obesity, and insulin resistance. J Clin Endocrinol Metab 87:4522-4527, 2002 https://doi.org/10.1210/jc.2002-020567
  14. Farrell JB, Deshmukh A, Baghaie AA. Low testosterone and the association with type 2 diabetes. Diabetes Educ 34:799-806, 2008 https://doi.org/10.1177/0145721708323100
  15. Cho IR, Lee MS. Klinefelter's syndrome: 87 cases. Korean J Urol 32:642-648, 1991
  16. Kim CW, Lee MY, Nam SM, Koh JH, Shin JY, Shin YG, Chung CH. A case of Klinefelter's syndrome associated with idiopathic thrombocytopenic purpura. Korean J Med 72:241-243, 2007
  17. Ota K, Suehiro T, Ikeda Y, Arii K, Kumon Y, Hashimoto K. Diabetes mellitus associated with Klinefelter's syndrome: a case report and review in Japan. Intern Med 41:842-847, 2002 https://doi.org/10.2169/internalmedicine.41.842
  18. Lanfranco F, Kamischke A, Zitzmann M, Nieschlag E. Klinefelter's syndrome. Lancet 364:273-283, 2004 https://doi.org/10.1016/S0140-6736(04)16678-6