Klinefelter`s syndrome was described in 1942 by Klinefelter, Reifenstein, and Albright and is characterized classically by small testes, azoospermia, gynecomastia, and elevated gonadotropins. The features become apparent during puberty. The cause of this syndrome is a developmental defect of the gonad resulting from a sex chromosome abnormality. Clinical observation was made on 8 patients with Klinefelter`s syndrome in the Department of Urology,
Seoul National University as follows;
1) The are was distributed from 19 years to 29 years.
2) The testes were small in size. Gynecomastia was noticed in 4 cases. Body hairs, beard and axillary hairs were rudimentary. The pubic hairs were absent, scanty or female type. The prostates were rudimentary in all cases.
3) chromosome study revealed 47, XXY in 6 cases and 46, XX in 2 cases.
4) The semen samples were devoid of spermatozoa.
5) The urinary gonadotropins for 24 hours elevated in 6 cases. The urinary 17-KS levels were between 6mg/day and 15.6mg/day.
6) Histologically there were atrophy and hyalinization with fibrosis of the seminiferous tubules. Increased number of Leydig cells in clumps were noticed adjacent to seminiferous tubules.
7) Treatment was hormonal therapy with testosterone.
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