HHT Complex article

I found a complicated article at http://www.emedicine.com/derm/topic782.htm on the net. Some bits in the article are interesting.

• HHT is a disorder that is inherited in an autosomal dominant fashion, although 20% of patients are unaware of a positive family history, partly because the lesions may be minimal and because 10% of patients have no episodes of bleeding. The homozygous condition probably is fatal. Histopathologic studies reveal large, irregular, thinly walled blood vessels, but the pathogenesis has not been fully established. One current theory states that systemic nevus vascular damage may not be equally expressed in all individuals with HHT. Individuals with blood group type O are affected more often, whereas males and females are affected equally. Coagulation abnormalities and increased fibrinolytic activity in the lesions may contribute to the tendency for bleeding.

I still have to work out what autosomal dominant is - but I think that it means roughly it's more than likely to carry through in the genes

It is really interesting - I recently had my blood group confirmed - just for interest - and its O+

• United States

  HHT is rare. Prevalence is 1-2 cases per 100,000 population.

Ok, so I'm not 1 in a million - but 1:100,000 is "special" !!


I read somewhere a while ago that this condition is more prevalent in Scandinavian (viking) heritage. Does that mean I have some viking in my family history?!

• International

  The onsets, courses of illness, and sex distributions are the same worldwide. The condition is much more common on the Danish island of Fyn, in the Dutch Antilles, and in parts of France.

I wonder what is "special" about these ethnic groups?