Question

define Male Pseudohermaphroditism

Answer 1

Persons with this intersexual condition have a 46, XY chromosome complement. Consequently, their cells
are chromatin- negative, ie, their nuclei do not show the Barr body*. Male pseudohermaphroditism is produced by insufficient production of testosterone by the fetal testes. It has been pro­posed that mutations that affect the enzymes required for the synthesis of tes­tosterone cause a deficiency or ab­sence of this hormone. The resulting pseudohermaphroditism affects the de­velopment of all those structures that are dependent upon testosterone for their differentiation. The external geni­talia develop into a female type, the testes do not descend and mesonephric ducts do not differentiate into male genital ducts. However, because Sertoli cells of testis produce MIS, the paramesonephric ducts degenerate and the uterus and the feminization is not reversed at the time of puberty be­cause the blood testosterone level fails to rise and, hence, the person contin­ues to resemble a female even in the adulthood.
Another variety of male pseudo­hermaphroditism is seen in persons suffering from androgen insensitivity syndrome (formerly called testicular feminization syndrome). Persons with this syndrome have the external ap­pearance normal of females, although the chromosomal constitution is 46, XY and testes are present. The androgen insensitivity syndrome results from the absence of androgen receptors. The
The Barr body or nucleolar satellite represents the resting X chromosome and is seen in the cells of the females uterine tubes are absent.
testosterone produced by the fetal tes­tes fail to induce the development of male genitalia because the target tis­sues (in the genital tubercle, urethral folds and labioscrotal swellings) do not respond and development proceeds as though androgens were absent.
The MIS produced by the fetal testes induces the regression of paramesonephric ducts and, hence, the uterus and uterine tubes are absent.
The vagina ends blindly in a pouch. The testes are usually present in the abdomen or inguinal canals but may descend into the labia majora. These abnormally located testes are liable to develop malignant tumors and, there­fore, should be removed as soon as they are discovered. Androgen insensitivity syndrome is an X-linked recessive disorder caused by mutations in the gene encoding the androgenreceptors.