Neil Smith Neil Smith

Recent paper on reversal of Kallmann syndrome / CHH cases.

European Journal of Endocrinology paper on reversal cases of Kallmann syndrome / CHH.

 

This is a very interesting area of research I think.

There are basically two types of KS / CHH. One is where the GnRH releasing neurones have been blocked in their migration into the hypothalamus during early development, which normally results in KS cases as the olfactory nerves are blocked as well.

The other type is where the action of the hypothalamus is impaired. The GnRH neurones are in the right place but for some reason they do not fire in the correct manner to allow GnRH to be released correctly (called a pulse generator). This normally results in CHH where the olfactory nerves are unaffected and there is a normal sense of smell.

Whether you get a case of KS or CHH will depend on which gene or genes have been affected and where in the developmental process they have an effect.

An active area of research at the moment is looking in how the genes involved in the action of the hypothalamus operate and how they are influenced by external factors such as nutrition, stress, environment or even sexual activity. This is known as epigenetics, 

There are research groups looking to see if the reproductive cycle can be restored by switching on genes that are present but are not active by modifying external factors. It is certainly early days in terms of research but the more reversal cases they find the more chance they have of finding out crucial information which will increase the understanding of KS / CHH.

Reversal is less common in KS cases where there are less GnRH nerve cells inside the hypothalamus but reversal cases have be reported. There is a theory that GnRH neurones continue to grow / move throughout life and if the eventually reach the hypothalamus in sufficient numbers the reproductive cycle can start later in life. 

The reversal is always fragile and the hypogonadal state can return in some cases but it does provide a little bit of hope. 

It is good to know research is ongoing in this area.

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Neil Smith Neil Smith

BBC Radio interview on Kallmann syndrome.

On 23rd March 2016 I had a phone interview for a late night radio show on local BBC radio. They had heard the interview on BBC Radio 2 and wanted to talk to me about Kallmann syndrome. It was a recorded interview rather than live this time.

This is a link to the radio show. The interview starts at about 1 hour 26 mins into the programme.

Graham Torrington BBC radio show.

The link to the programme might not be active for very long. There is an audio clip of the interview on the top of this page. 

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Neil Smith Neil Smith

Interview about Kallmann syndrome on BBC Radio 2 Jeremy Vine show.

Recently I was on the Jeremy Vine show on BBC Radio 2 talking about Kallmann syndrome. It was only a brief interview but I tried to mention as many of the major points as I could. I was on the show as part of a segment on rare medical conditions with the show's resident doctor, Dr Sarah Jarvis.

This is the link to the show. The interview starts at about 1 hour 9 mins into the show.

Jeremy Vine BBC2 radio show. 21st March 2016.

This link on the BBC website might only be active for a short time. He is a link to just the interview part:

Kallmann syndrome interview

There was so much else I could have talked about and it was a shame it was such a brief interview. However I am very grateful for the opportunity to raise awareness of the condition and am quite keen to do more in the future if I can.

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Neil Smith Neil Smith

Rare Disease UK. 2016 report. "The Rare Reality"

 "The Rare Reality"

 

The Genetic Alliance UK group published its 2016 report into patient experiences dealing with rare disorders. Kallmann syndrome was a featured condition in the report and one of the four patient videos.

Rare Disease UK is a Eurodis funded organisation that aims to bring together patients with rare disorders into one organisation to give them a single voice to help raise public awareness and help to lobby government for political change to help patients and families. 

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