Failure of the ovaries to produce enough follicles, because of a problem in the ovary itself, and resulting in depletion of eggs before the age of 40 years (known as premature menopause, a cause of secondary amenorrhea), or maybe even before the age puberty is expected (causing failure of puberty to happen, including primary amenorrhea). Sometimes occurs in spite of good numbers of primordial follicles that (inexplicably, so far) won't develop. The younger the woman, the more likely that an aneuploidy will be found if a karyotype is done on blood or on a biopsy of the ovary. Estrogen replacement therapy is important to prevent general jeopardy to health, including prevention of osteoporosis.

The natural process in which the older the female fetus, girl or woman gets the fewer are the eggs (as primordial follicles) left in the ovaries; the huge majority of eggs are lost because of atresia, only a tiny fraction by ovulation. Before the eggs are depleted there is a mild or moderate elevation in serum FSH when measured during the menstrual phase (often called a day 3 FSH. When the eggs are more or less depleted there will be primary ovarian failure and, in women who have had periods, the menopause will take place, perhaps prematurely (premature menopause). Infertility, however, usually precedes total egg depletion by up to 10 years. See also mitochondrion and oopause.

The commonest cause of severe mental retardation in males, caused by a long "triple repeat sequence" in a part of a gene for intelligence located on an X-chromosome, rendering this sex chromosome susceptible to breakage, which can be revealed either in a karyotype performed under special laboratory conditions or using a special PCR DNA test; females have two X-chromosomes and so may be carriers for this devastating condition, which will affect one-in-two of their male children; the carrier state in females can have clinical effects, namely a susceptibility to reduced numbers of ovarian follicles and hence primary ovarian failure and premature menopause, so should be looked for before assisted conception attempts are made in these clinical circumstances.

An abnormal condition of the bones, which are weakened by a loss of calcium. Deficient estrogen, such as after the menopause or after primary ovarian failure (premature menopause), eventually causes osteoporosis, with a tendency to bone fractures, especially of the wrist, the thigh and the backbone.

In medicine the word primary can denote primacy in one of 3 ways: (1) event-wise (e.g. primary amenorrhea is when there has been no precedent -- no prior period -- whereas secondary amenorrhea) follows prior periods, and likewise the distinction between primary infertility and secondary infertility, a distinction based on prior successful pregnancy); (2) developmentally or time-wise (e.g. a primary follicle gives rise to a secondary follicle, then to a tertiary follicle); or (3) causally (e.g. primary ovarian failure is based within the ovary itself, whereas secondary ovarian failure is secondary to failure of gonadotropins). These sort of distinctions is one reason why medical school takes a long time.

Measurement of follicle stimulating hormone in serum. Useful at the time of menstruation for indicating a significantly decreased number of eggs in the ovaries in the few years leading up to menopause (that is, indicative of depletion of eggs or primary ovarian failure); Continuously high in women after menopause, and then excreted in high amounts in the urine (from which, in turn human menopausal gonadotropin is derived).

The state of absolute infertility or complete infertility, with no chance of getting pregnant without special help. Causes include azoospermia, anovulation (especially primary ovarian failure), and blocked fallopian tubes.

A trisomy with a karyotype of 47,XXX -- a female with an extra X-chromosome. The old description of 'super female' is misleading, because fertility, if affected, is most likely reduced; primary ovarian failure is more common than with a normal chromosome complement and premature menopause then follows.

The combination of primary ovarian failure with constitutional (that is, genetically determined) shortness; often with other clinical abnormalities, including 'webbing' of the neck; an increased 'carrying angle' at the elbow; short fourth metatarsal (feet) and metacarpal (hand) bones (the bone within the hand that leads to the ring finger), and sometimes abnormalities of the heart and the thyroid gland. Associated with a karyotype that is 45,X (a monosomy, with one sex chromosome missing) or with partial loss ('deletion') of one of a pair of X-chromosomes. The single X-chromosome present can come from the mother or the father -- and, interestingly, it behaves differently: if it comes from the mother the girl is likely to be rather socially disruptive (the way little boys typically are), whereas if the X-chromosome comes from the father her behavior tends to be closer to normal for that of a girl!