Pseudohermaphroditism Health Dictionary

An individual in whom both ovarian (see OVARIES) and testicular (see TESTICLE) tissue is present. Hermaphrodites may have a testis on one side and an ovary on the other; or an ovotestis on one side and an ovary or testis on the other; or there may be an ovotestis on both sides. Both gonads are usually intra-abdominal. The true hermaphrodite usually has a UTERUS and at least one Fallopian tube (see FALLOPIAN TUBES) on the side of the ovary, and on the side of the testis there is usually a VAS DEFERENS. Most true hermaphrodites are raised as males, but external virilisation is not usually complete. Even when signi?cant phallic development is present, HYPOSPADIAS and CRYPTORCHIDISM are common. At puberty, GYNAECOMASTIA develops and MENSTRUATION is common, as ovarian function is usually more nearly normal than testicular function. The condition is rare. A more common condition is pseudohermaphroditism: these are individuals who possess the gonads of only one sex but whose external genitalia may be ambiguous. The cause is a hormonal imbalance and can usually be corrected by hormone treatment.... hermaphrodite

A congenital disorder in which gonads of both sexes are present, and the external genitalia are not clearly male or female.

True hermaphroditism is extremely rare and its cause unknown.

A more common condition is pseudohermaphroditism, in which the gonads of only 1 sex are present, but the external genitalia are not clearly either male or female.... hermaphroditism

A rare inherited condition in which a genetic male with internal testes has the external appearance of a female. The syndrome is a form of intersex and is the most common form of male pseudohermaphroditism.

The cause is a defective response of the body tissues to testosterone.

The causative genes are carried on the X chromosome, and so females can be carriers. Affected individuals appear to be girls throughout childhood, and most develop female secondary sexual characteristics at puberty; but amenorrhoea occurs, and a diagnosis is usually made during investigations to find its cause. Chromosome analysis shows the presence of male chromosomes and blood tests show male levels of testosterone. Treatment of testicular feminization syndrome involves surgical removal of the testes, to prevent cancerous change in later life, and therapy with oestrogen drugs. An affected person is not fertile but can live a normal life as a woman.... testicular feminization syndrome

a rare disorder consisting of the triad of *nephroblastoma (Wilms’ tumour), congenital nephropathy, and intersex disorders, resulting from mutations in the Wilm’s tumour suppressor gene (WT1). Incomplete forms exist; congenital nephropathy, with diffuse mesangial sclerosis, is the constant feature with either Wilms’ tumour or intersex disorders, usually in the form of male *pseudohermaphroditism. [P. Denys (20th century), French physician; A. Drash (20th century), British physician]... denys–drash syndrome