Pseudohermaphroditism in Female and Male Symptoms, Causes, Treatment
Pseudohermaphroditism is a term used to describe a condition in which person born with primary sex characteristics of one sex and develop secondary sex characteristics different from what expected. In simple words, person develop a different sex characteristics from one which is expected on the basis of gonadal tissue. This term is different from true hermaphroditism because in true hermaphroditism, person have both male and female sex characteristics i.e. testes and ovaries respectively. It is divided into two main terms on the basis of sexual characteristics. If ovaries are present then it is termed as female pseudohermaphrodite and if testes are present then the term male pseudohermaphrodite is used.
Male pseudohermaphrodite involves male gonads in combination with 46 XY karyotype. The male genitalia appear different from usual one because of a hormone named dihydrotestosterone. It is synthesized via testosterone which in turn synthesize with the help of an enzyme i.e. 5 a reductase. When mutation occurs, this enzyme is blocked and there will be no more synthesis of testosterone. As a result structure deformity occurs leading to male pseudohermaphrodite. Men with pseudohermaphroditism develop feminine external sex organs and behaviour which is a clear symptom of male pseudohermaphrodite.
When pseudohermaphroditism occurs in females, it appears with ambiguous external genitalia along with 46 XX karyotype and ovaries. It occurs due to exposure of androgens to developing fetus during 1st trimester of pregnancy. And this exposure have a reason of congenital suprarenal hyperplasia, tumor in suprarenal gland or ongoing androgen therapy in pregnancy. The external appearance of female genitalia involves large clitotis, and scrotum is missing. Sometimes an obliterated vagina is formed in female pseudohermaphroditism. The internal organs of female genitalia i.e. ovaries, vaginal internal structure and fallopian tubes are normal and there are chances of pregnancy.
Pseudohermaphroditism is a congenital problem in which person have secondary sex characteristics. People with this condition may have sexual structural deformity and develops an abnormal external genitalia. Patient with pseudohermaphroditism may either have deformed testes or deformed vagina. In rare cases, person may develop a new external genitalia which is the combination of both penis and vagina. In female pseudohermaphrodite vagina may develop with enlarged clitoris and an empty scrotum. Obliterated vagina may form. While in case of male pseudohermaphrodite, penis is not much enlarged and testes are smaller in size than usual. Male patient may suffer feminine behavior and external sex structure.
As it is divided into two main types on the basis of gender. So both the types have different leading causes behind them. In male pseudohermaphrodite, mutation occurs and 5 a reductase is missing. So there is no more testosterone synthesis. Due to this dihydrotestosterone do not synthesize and leads to sexual structure deformity. Well in case of female pseudohermaphrodite, it occurs due to androgen exposure to developing fetus during 1st trimester of pregnancy. Other causes may include congenital suprarenal hyperplasia, androgen therapy during pregnancy or tumor in suprarenal gland.
The symptoms of pseudohermaphroditism appear at the age of puberty. And when external sexual organs fully grown or mature than a surgery may be performed for the correction of structure deformity of external genitalia. Surgeries in number are performed on intersex people. In female pseudohermaphrodite, only the external structure is deformed but the internal sex organs are proper so mostly women do not prefer surgery because of normal feminine functioning. The intra abdominal testes should be removed via surgery because there is increased risk of tumor formation in abdomen. Hormonal therapy is also effective if treated on time.
Pseudohermaphroditism in Males
Male pseudohermaphrodite involves male gonads in combination with 46 XY karyotype. The male genitalia appear different from usual one because of a hormone named dihydrotestosterone. It is synthesized via testosterone which in turn synthesize with the help of an enzyme i.e. 5 a reductase. When mutation occurs, this enzyme is blocked and there will be no more synthesis of testosterone. As a result structure deformity occurs leading to male pseudohermaphrodite. Men with pseudohermaphroditism develop feminine external sex organs and behaviour which is a clear symptom of male pseudohermaphrodite.
Pseudohermaphroditism in Females
When pseudohermaphroditism occurs in females, it appears with ambiguous external genitalia along with 46 XX karyotype and ovaries. It occurs due to exposure of androgens to developing fetus during 1st trimester of pregnancy. And this exposure have a reason of congenital suprarenal hyperplasia, tumor in suprarenal gland or ongoing androgen therapy in pregnancy. The external appearance of female genitalia involves large clitotis, and scrotum is missing. Sometimes an obliterated vagina is formed in female pseudohermaphroditism. The internal organs of female genitalia i.e. ovaries, vaginal internal structure and fallopian tubes are normal and there are chances of pregnancy.
Pseudohermaphroditism Symptoms
Pseudohermaphroditism is a congenital problem in which person have secondary sex characteristics. People with this condition may have sexual structural deformity and develops an abnormal external genitalia. Patient with pseudohermaphroditism may either have deformed testes or deformed vagina. In rare cases, person may develop a new external genitalia which is the combination of both penis and vagina. In female pseudohermaphrodite vagina may develop with enlarged clitoris and an empty scrotum. Obliterated vagina may form. While in case of male pseudohermaphrodite, penis is not much enlarged and testes are smaller in size than usual. Male patient may suffer feminine behavior and external sex structure.
Pseudohermaphroditism Causes
As it is divided into two main types on the basis of gender. So both the types have different leading causes behind them. In male pseudohermaphrodite, mutation occurs and 5 a reductase is missing. So there is no more testosterone synthesis. Due to this dihydrotestosterone do not synthesize and leads to sexual structure deformity. Well in case of female pseudohermaphrodite, it occurs due to androgen exposure to developing fetus during 1st trimester of pregnancy. Other causes may include congenital suprarenal hyperplasia, androgen therapy during pregnancy or tumor in suprarenal gland.
Pseudohermaphroditism Treatment
The symptoms of pseudohermaphroditism appear at the age of puberty. And when external sexual organs fully grown or mature than a surgery may be performed for the correction of structure deformity of external genitalia. Surgeries in number are performed on intersex people. In female pseudohermaphrodite, only the external structure is deformed but the internal sex organs are proper so mostly women do not prefer surgery because of normal feminine functioning. The intra abdominal testes should be removed via surgery because there is increased risk of tumor formation in abdomen. Hormonal therapy is also effective if treated on time.
Pseudohermaphroditism in Female and Male Symptoms, Causes, Treatment
Reviewed by Simon Albert
on
August 18, 2019
Rating:
Reviewed by Simon Albert
on
August 18, 2019
Rating:
