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Kallmann Syndrome – frequently asked questions. Part 3.

January 28, 2012

What are hormones?

In a general sense hormones are the body’s messenger service. There are over 40 known hormones that act within the body. They are each released by a specific endocrine gland (such as the pituitary gland, adrenal glands, pancreas or testes / ovaries)  in response to a specific stimulus, possibly another hormone. A hormone is released to generate a specific result. In normal circumstances the hormonal system works on a negative feedback mechanism.

 A stimulus causes the endocrine gland to release a hormone -> The hormone causes the stimulus to be stopped or reduced -> The endocrine gland stops producing the hormone

The classic example is the role of insulin in the role of maintaining the blood sugar levels. Insulin is only released in response to an increase in sugar levels, as soon as blood sugar levels falls; the production of insulin is stopped. The breakdown in this regulation causes the most common hormonal diseases – diabetes mellitus.

 In normal puberty the pathway is:

 Hypothalamus gland produces GnRH (gonadotrophin releasing hormone)

Which causes the

Pituitary gland to produce LH & FSH (luteinsing hormone / follicle stimulating hormone)

Which cause the

Testes to produce testosterone & sperm

Or

Ovaries to produce progesterone and oestrogen & allow ovulation to occur 

The sex hormones also have other effects around the body, not just linked to puberty and fertility.

In KS or HH either the pituitary gland does not receive the GnRH, or it is unable to respond to the GnRH. Without the first signal the pituitary will not produce its hormones (LH and FSH) that in turn will prevent the testes or ovaries producing their own hormones at the required time to cause puberty. For some people with KS / HH there is no physical problem with the testes or ovaries, they just have not had correct signal from the pituitary gland in order to function correctly.

The treatments people get with KS / HH will be replacing one of the hormones missing in the chain. Normally this is either testosterone or oestrogen / progesterone. However it is also possible to be given FSH / LH or GnRH in certain circumstances, especially if fertility is desired.

Can a person with KS or HH become fertile?

Yes, possibly, but only with specialist treatment and if other circumstances are favourable. There have been many cases of people with KS / HH  having children, sometimes through a form of IVF or other assisted fertilisation programmes. As with any type of fertility treatment there are many other factors to consider and it can take many months of treatment and there is no guarantee as with any form of fertility treatment. It has been noted that fertility can be achieved with Kallmann’s women more quickly than with other patients.

Is there any effect on expected lifespan?

There is no reliable evidence that KS or HH has any effect on the life span on an individual. It is worth point bearing in mind though that there are some rare symptoms that can occur with KS and HH that may have an effect on life span. These other symptoms may be connected to KS or HH or may have arisen regardless.

Are there any risks if KS or HH is left untreated?

Yes there can be. The major problem with a person with untreated KS or HH is the increased risk of osteoporosis or ‘brittle bone disease’. The greatly reduced levels of sex hormones seen with KS and HH have a detrimental effect on the strength of the bones. This can be easily treated with the appropriate drugs that will reduce the risk of osteoporosis to that seen in the rest of the population. It is advised that a person with KS or HH should have a bone scan (DEXA) at least every 5 years to assess their bone age and to assess the risk of osteoporosis. There is increasing evidence that Vitamin D levels play a vital role general health, not just bone strength. It is not unknown for people with KS / HH to have their Vitamin D levels monitored and prescribed tablets if the level is too low.

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One Comment
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