A case of diabetes mellitus associated with Klinefelter's syndrome

클라인펠터증후군과 동반된 제2형 당뇨병 1예

  • 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)
  • 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)
  • Received : 2009.10.08
  • Accepted : 2009.12.22
  • Published : 2010.12.01

Abstract

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.

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.

Keywords

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