In the answer to Question 6 the normal menstrual cycle was discussed. Since both LH and FSH help to regulate the menstrual cycle, their absence results in the lack of menstruation. This is called primary amenorrhoea.
You have amenorrhoea because you have no LH or FSH and therefore your ovaries cannot manufacture the required amounts of oestrogen and progesterone. You have only a very small amount of oestrogen in your blood and this is the oestrogen made by your adrenal glands. With these extremely low hormone levels (progesterone in particular), your uterus cannot work properly because its active layer (endometrium) fails to thicken.
There is no menstruation and this can only be corrected with hormone replacement therapy. Several different types of therapy are available on the NHS and in almost all cases, the normal menstrual cycle can be simulated as closely as possible for as long as treatment is given. If treatment is stopped though, the menstrual cycle can no longer be maintained and amenorrhoea eventually returns. Hormone replacement therapy is discussed in more detail later in the answers to Questions 19 & 24.
All men have small quantities of the female sex hormone oestrogen, as well as testosterone, in their blood. Similarly, every woman has traces of the male sex hormone testosterone, as well as native oestrogens, in her body. The presence of female oestrogens in males sometimes causes a minor condition called gynaecomastia, or a female-like enlargement of one or both breasts (see answer to Question 14). Occasionally, this may also be a side-effect of the testosterone or gonadotrophin therapy offered to males with Kallmann's syndrome. However, it is generally believed that gynaecomastia does not occur any more frequently amongst the male Kallmann's syndrome population than that which often occurs in healthy teenage boys and in those who are overweight.
Virtually everybody with Kallmann's syndrome, apart from being sexually underdeveloped and infertile unless treated, have little or no sense of smell. It is not yet possible to treat anosmia.
(published with permission in writing from:http://www.hypohh.net)
