Contents
What are Dysgenetic gonads?
Gonadal dysgenesis is classified as any congenital developmental disorder of the reproductive system in the male or female. It is the atypical development of the gonads in an embryo, with reproductive tissue replaced with functionless, fibrous tissue, termed streak gonads.
Why is Turner syndrome called gonadal dysgenesis?
Turner’s Syndrome. Turner’s syndrome (gonadal dysgenesis) is due to the absence of a second X chromosome. Manifestations of the syndrome include primary amenorrhea, genital immaturity, and short stature; intelligence is usually normal.
What causes streak gonads?
Underdeveloped gonads are called “dysgenetic” or “streak” gonads. These gonads may not work or make sex hormones before birth. One or both gonads may also have testis tissue and / or ovary tissue. These gonads may make some hormones.
What is Swyer syndrome?
Swyer syndrome is a rare disorder characterized by the failure of the sex glands (i.e., testicles or ovaries) to develop. Swyer syndrome is classified as a disorder of sex development (DSD), which encompasses any disorder in which chromosomal, gonadal or anatomic sex development is abnormal.
How is gonadal dysgenesis diagnosed?
Complete (pure) XY gonadal dysgenesis (XY CGD) Endocrine evaluation usually shows hypergonadotropic hypogonadism with elevated basal LH and FSH, as the gonads are not functional. Imaging studies, including pelvic ultrasound or MRI, demonstrate the presence of a uterus and may show bilateral streak gonads.
What are the two gonads?
The gonads, the primary reproductive organs, are the testes in the male and the ovaries in the female.
What is a gonadal?
The gonads, the primary reproductive organs, are the testes in the male and the ovaries in the female. These organs are responsible for producing the sperm and ova, but they also secrete hormones and are considered to be endocrine glands.
What is gonadal hypoplasia?
What is Gonadal Hypoplasia (GH): In simple terms the lack of or underdeveloped gonads in either a male or female. Gonadal hypoplasia is characterised by aberrantly small and underdeveloped gonads, in both sexes.
Are gonads endocrine glands?
What is Ovotesticular DSD?
Ovotesticular disorder of sex development (ovotesticular DSD) is a very rare disorder in which an infant is born with the internal reproductive organs (gonads) of both sexes (female ovaries and male testes). The gonads can be any combination of ovary, testes or combined ovary and testes (ovotestes).
Are males XY or YY?
Typically, biologically male individuals have one X and one Y chromosome (XY) while those who are biologically female have two X chromosomes. However, there are exceptions to this rule. The sex chromosomes determine the sex of offspring.
Is there a YY gender?
Males with XYY syndrome have 47 chromosomes because of the extra Y chromosome. This condition is also sometimes called Jacob’s syndrome, XYY karyotype, or YY syndrome. According to the National Institutes of Health, XYY syndrome occurs in 1 out of every 1,000 boys.
What does it mean to have mixed gonadal dysgenesis?
Mixed gonadal dysgenesis refers to an individual who usually has a differentiated gonad on one side and a streak gonad or streak testis on the other side Either (a) testis plus contralateral streak gonad, (b) testis and contralateral gonadal agenesis, (c) hypoplastic gonads with tubules in one gonad or (d) streak gonad with contralateral tumor
Is it possible to remove a dysgenetic gonad?
In addition, the dysgenetic gonad is at risk for development of a tumor and should be removed, particularly if the gonad cannot be brought down into the scrotum (Forest, 2001). However, sex assignment is likely to be guided by the degree of virilization, with the more virilized cases being assigned as males.
How does hormonal failure affect gonadal dysgenesis?
In gonadal dysgenesis, the accompanying hormonal failure prevents the development of secondary sex characteristics in either sex, resulting in the appearance of a sexually infantile female and infertility
Are there fallopian tubes on the side of the streak gonad?
Fallopian tubes are always found on the side of the streak gonad and often on the side with the dysgenetic gonad. Wolffian structures may be developed on the side with the dysgenetic gonad. A genitourethrogram is likely to demonstrate internal müllerian structures that can be confirmed at laparoscopy.