A CLINICAL SYUDY ON KLINEFELTER'S SYNDROME

Abstract

A study of 56 cases of Klinefelter's syndrome encountered at the Chiba University Hospital in the years 1960-1981 is presented. Eighty per cent of them ranged in age from 25 to 34 years. Two-thirds had chief complaints of sterility. The average ages of their father and mother at their birth were 34.7 and 30.5 years, respectively. Fifty-three cases had a karyotype of 47XXY and two cases had that of 48XXXY.
The karyotype of one case was 46XY/47XXY mosaicism. From their average stature and body weight they had a common constitution of the Japanese males. Thirty per cent of them had gynecomastia. Testicular biopsies were performed in 39 cases, 31 cases of which showed no spermatogenesis and two infant cases had immature spermatogonial cells.
Before treatment, the average blood levels of both luteinizing hormone (LH) and follicle stimulating hormone (FSH) were elevated when compared with those in normal adult males, but the average blood level of testosterone was decreased.
There was no correlation between the blood levels of LH and testosterone. The difference of blood LH, FSH and testosterone levels between the groups with and without gynecomastia was not statistically significant. Human chorionic gonadotropin (hCG 4000 IU) administered im for three days led to a 1, 4-fold increase of blood testosterone level on the average. There were a fivefold increase in blood LH level and a twofold increase in blood FSH level thirty minutes after LH-releasing hormone (LH-RH, 100μg) was administered iv. Blood LH and FSH levels were observed in four cases under androgen replacement by injection of the depot testosterone. In two of these four cases both blood LH and FSH levels were decreased to normal range and the other two remained high.