Klinefelfter's Syndrome에 관한 임상적 관찰

Abstract

Klinefelter's syndrome is a form of testicular failure characterized by small testes, azoospermia and male phenotype and is the most common cause of frank hypogonadism. Frequency of the Klinefelter's syndrome in large scale surveys has estimated as 0.2% of all live-born males. Thus it may be approximated that we have nearly 40,000 cases of Klinefelter's syndrome in Korea. Clinical observations were made on a total of 64 cases of Klinefelter's syndrome in OUf Department of Seoul National University Hospital for the past 15 years. The results obtained were as follows: 1. Age distribution of the patients ranged from 12 to 34 with mean of 26 (Table 1). 2. Their chief complaints were small testicles in 29 cases (45%), infertile marriages in 16 cases (25%), small penis in 12 cases (19%), gynecomastia in 6 cases (9%), and other in 1 case (2%) (Table2). 3. The testicular sizes of the patients ranged from Irnl to 6ml with mean of 4ml (Table 3). 4. The penile sizes of the patients ranged from 2.5cm to 7.3cm with mean of 5.7cm in length, and from 2. Scm to 8. Oem with mean of 5. gem in circumference (Table 4). 5. The distribution of pubic hair were normal type in 10 cases (1696), horizontal type in 25 cases (39%), hypotrichosis pubis in 26 cases (4196), and atrichosis pubis in 3 cases (5%) (Table 5). 6. Gynecomastia occurred in 29 cases (45%). 7. Azoospermia was found in all cases. 8. The frequency of ejaculation per week ranged from 0.5 to 5 with mean of 1. 9/week in married cases and 2.1/week in single cases (Table 6). 9. The chromosomal study showed 47, XXY in 63 cases (9896) and 46, XX/47, XXY in 1 case (2%) (Table 8). Sex chromatin was positive in all patients (Table 7). 10. The average levels of plasma testosterone, FSH and LH were 2.43ng/ml (ranges: O. 27~8. Sl ng /rnl), 37. 92IU/L (ranges: 1.12~141. 03 IU/L), and 22.08 IU/L (ranges: O. 99~51. 88 IU/L), respectively(Tables 9 and 10). 11. Histological structures of the testes were hyalinization with fibrosis of the seminiferous tubules and pseudoadenomatous clumping of the Leydig cell. 12. These patients were treated with testosterone.