Hormonal Investigation on Klinefelter's Syndrome

Shim, Hong Bang; Lee, Hee Yong

Abstract

Klinefelter's syndrome was described in 1942 as a syndrome of hypogonadal men with small testes, gynecomastia and elevated urinary gonadotropin by Klinefelter et al. for the first time. As the pathogenesis has become better understood the original description has been expanded to include additional features. The presence of supernumerary X chromosome is considered to be the fundamental underlying etiological factor. The clinical triad of the Klinefelter`s syndrome are sterility, male phenotype, and small firm testes. Clinical observation and plasma testosterone, FSH(follicle stimulating hormone), LH(luteinizing hormones measurement were made on the 11 Klinefelter`s syndromes in the Department of Urology, Seoul National University Hospital as follows: 1. The age distribution was from l9 years old to 33 years old and the average span was 28 years old. 2. A11 of the patients had small and firm testes, and showed decreased pubic hair and sparse body hair. Gynecomastia occurred in 5 patients(48%). 3. The average height was 169.3cm and average span was 170. 1 cm and average lower leg length 96.3 cm 4. The chromosomal study showed 47 XXY in the all patients and sex chromatin was positive in the all patients. 5. All of the patients was azoospermia on the semen analysis. 6. Histological structures of the testes were hyalinization with fibrosis of the seminiferous tubule and psuedoadenomatous clumping of the Leydig cell. 7. The average level of plasma testosterone, FSH, LH were 184 ng/d1(range 80-349 ng/d1) 35.25 mI U/ml(range 27-43.3mI U/ml), 14.5mI U/ml(range 7.6-26.52mI U/ ml). 8. Treatment of the patients was hormonal therapy with testosterone.