<unk>  is a type of female hypogonadism in which no functional ovaries are present to induce puberty in an otherwise normal girl whose karyotype is found to be 46,XX. With nonfunctional streak ovaries, she is low in estrogen levels (hypoestrogenic) and has high levels of FSH and LH. Estrogen and progesterone therapy is usually then commenced. This syndrome is inherited as an autosomal disease. It affects both males and females, but the phenotype differs. In both sexes, sensorineural deafness occurs, but in females ovarian dysgenesis also occurs. The term “pure<unk>  dysgenesis” (PGD) has been used to distinguish a group of patients from<unk>  dysgenesis related to Turner syndrome. In the latter a distinct chromosomal aberration is present, while in PGD the chromosomal constellation is either 46,XX or 46,XY. Thus<unk>  is also referred to as PGD, 46 XX, and<unk>  as PGD, 46,XY or Swyer syndrome. Patients with PGD have a normal chromosomal constellation but may have localized genetic alterations.
XX gonadal dysgenesis
XY gonadal dysgenesis
Testicular dysgenesis syndrome
XX male syndrome
Complete androgen insensitivity syndrome
XXXY syndrome
Mild androgen insensitivity syndrome
Thyroid dysgenesis