The incomplete male.
The incomplete male.
复制标题
不完整的男性。
DOI：
10.1136/adc.53.9.701
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发表时间：
1978
影响因子：
5.2
通讯作者：
D. Grant
中科院分区：
文献类型：
--
作者：
M. Savage;D. Grant
During the last 20 years a great deal has been learned about the aetiology and management of intersex states. In particular, the importance of congenital adrenal hyperplasia as a cause of female pseudohermaphroditism has become widely recognised. Cytogenetic methods have clarified the cause of the genital anomalies associated with sex chromosome abnormalities and while techniques such as banding and Y-fluorescence have failed to define the aetiology of true hermaphroditism, this has not generally interfered with correct clinical management. However, the investigation and management of patients with a male chromosomal complement but incomplete masculinisation of the external genitalia (male pseudohermaphroditism) remain among the most intriguing and difficult aspects of paediatric endocrinology. Male intersexuality represents a disturbance of the normal process of fetal sexual differentiation and is characterised by genital ambiguity and deficient masculinisation at puberty. In his classical experiments with gonadectomy in fetal rabbits, Jost' showed that the mammalian embryo has an inherent tendency to develop as a female. The development of the male phenotype is more complex than that of the female and depends on the differentiation and action of the fetal testis. Human testicular differentiation appears to be controlled by the Y chromosome. At a critical period in fetal life (10-18 weeks after fertilisation) the testicular Leydig cells secrete testosterone which directly stimulates the formation of the internal genitalia (the vas deferens, epididymis, and seminal vesicles) from the Wolffian ducts. The external genitalia are masculinised by dihydrotestosterone which is derived from testosterone by the action of the enzyme 5-a.-reductase. A second testicular hormone known as muellerian inhibiting factor, a polypeptide, is secreted during the same period by the Sertoli cells and causes suppression of the female muellerian ducts.2 The aetiology ofmale intersexualitymay be divided into three basic categories 3 (Table). These are abnormal testicular differentiation, abnormal testicular function, and target organ unresponsiveness to androgen.
