STUDIES ON PITUITARY-GONADAL ENDOCRINE FUNCTION IN PATIENTS WITH MALE HERMAPHRODITISM

Abstract

Pituitary-gonadal endocrine function was studied by measuring serum LH, FSH and testosterone (T) levels under various conditions using radioimmunoassay methods in 18 patients with various types of male hermaphroditism. Among them, 11 patients were classified as group A1 (;male hermaphroditism with no breast development and rudimentary or no mullerian structures), 3 patients as group A2 (;male hermaphroditism with well formed mullerian structures) and 2 patients as group B2 (;male hermaphroditism with breast development and ambiguous external genitalia) according to the classification modified from that proposed by Jones. In the other 2 patients, it was not determined whether they belonged to group A1 or group B2 because of their prepubertal age.
In addition, 4 patients with true hermaphroditism and 3 patients with gonadal dysgenesis were studied for comparison. The pubertal and postpubertal patients with male hermaphroditism excluding those of group B2 showed strikingly elevated levels of both serum LH and FSH. The levels of serum LH and FSH were further elevated by LH-RH administration. Serum T levels in them were widely ranged from subnormal to normal adult male levels, and hCG stimulation test revealed a poor Leydig cell reserve capacity in some cases.
In the pubertal and postpubertal patients with male hermaphroditism of group B2, serum FSH values were in the upper limit of normal adult male range, but the levels of serum LH were abnormally elevated despite their normal or high levels of serum T. Serum gonadotropin levels in two patients with male hermaphroditism of group A2 were studied along with usual course of clinical treatment. It was observed that further increased levels of serum gonadotropins after castration were suppressed to almost undetectable levels by estrogen replacement therapy. The serum gonadotropin levels in one of the patients with male hermaphroditism of group B2 were not suppressed by single administration of testosterone depot 100mg.
In contrast to these abnormally elevated levels of serum gonadotropins in adult patients with male hermaphroditism, the prepubertal cases showed normal age-matched male levels of serum gonadotropins.
Four patients were studied longitudinally for 3 or 5 years and abrupt rises of serum gonadotropin levels to the abnormally high range were observed with the onset of puberty. These pubertal rises of serum gonadotropin levels from the normal range to an abnormally high range were also observed in the studies on patients with true hermaphroditism.
Meanwhile, the patients with gonadal dysgenesis of prepubertal age showed somewhat elevated levels of serum gonadotropins. It is concluded that development of puberty causes abnormal changes in pituitary gonadal endocrine function in patients with male hermaphroditism. The aspects of these abnormalities are thought to be compatible in some cases with those of primary gonadal failure and in some cases with those of androgen insensitivity in target organs.
Possible mechanism was discussed for these abnormal alterations in pituitary gonadal endocrine axis during puberty in patients with male hermaphroditism.