80: PRENATAL LH-DEFICIENCY AS POSSIBLE CAUSE OF MALE PSEUDOHERM-APHRODITISM, HYPOSPADIAS, HYPOGENITALISM AND CRYPTORCHIDISM

摘要

Some boys with gonadotropin (Gn)deficiency have hypogenitalism (very small penis). In some patients with cryptorchidism LHRH stimulated LH is low while FSH is high or normal (our experience and Canlorbe et al. 1974). The following two observations suggest that fetal LH is not only important for genital growth and testicular descent, but also for genital differentiation. Patient 1 is a girl aged 10 with ambiguous genitalia, XY karyotype, inguinal testes and no pregnantriol and testosterone response to HCG. LHRH stimulated LH was low and remained low 1 week and 6 months after bilateral castration while FSH increased to very high levels as seen in agonadal subjects, Patient 2 is a male adolescent with mild hypospadias,bilateral cryptorchidism, XY karyotype and Gn deficiency with anosmia (Kallmann syndrome).- In the male rabbit, fetal Gn deficiency produced by decapitation prior to or during genital differentiation results in female genitalia or hypospadias (Jost 1951). So far it has been assumed that in the human fetus, testosterone secreted by the Leydig cells and responsible for the differentiation of the male genitalia is not controlled by the fetal Gn as in the rabbit but rather by HCG, and that the fetal Gn take over their control of testicular function only after the period of genital differentiation. The experience presented here suggests that this concept may be wrong and that prenatal hypothalamo-pitultary disorders with LH deficiency may cause male pseudohermaphroditism, hypospadias, hypogenitalism and cryptorchidism, depending on the severity and timing of the deficiency.