So today I have the pleasure of looking at a family tree that Ellen's
Mum and I have put together, I have made it anonymous but that doesn't mean we
can't discuss it. So as you can see from the above tree only o is Ledderhose
only with one possibly case seen in C. This goes to show that even in a family
here with about 50 people shown we can only see 1 case where there is an
individual with Ledderhose only and this was initiated by a massive trauma
event. In total there are 10 cases of Dupuytren's and many of the people are
under 50 years old so who knows how this would look if seen in 20 or 30 years
times. There were however 4 people with both Ledderhose and Dupuytren's and
interestingly 3 of these cases appear in the 9 and A lineage.
9 and A:
So I am going to start by looking at 9 and A as this part of the tree
has a very high incidence of the disease. Here we have data on 9 individuals
including 9 and A and 6 of them have these diseases in one form or another. So
this part of the tree has 66% prevalence and you have to wonder what
the odds are for (a) seeing as both A and P are people that are not blood
relatives to 1 and 2 and they are the only others in this lineage that do not
have the disease. I think this is really interesting as it suggests that there
is a strong genetic link that has been passed down by 9 to M, N and O and this
has in turn been passed down to b and c. Of course I am not aware of all
factors but as far as I am aware there is no diabetes in this tree but they may
smoke or drink excessive amounts of alcohol which could contribute towards
this.
F and G and the rest of the tree:
Ok so this part of the tree makes me less concerned about the genetic
element. Ok so take out 9 and A and that part of the tree and suddenly you are
down to 7 confirmed cases of Dupuytren's or Ledderhose and one of these comes
from outside of the 1+2 blood line and that is in about 40 cases so down to
less than a 25% prevalence compared to the 66% is quite low. I think that
the interesting thing about the F and G line is that it skips a couple of
generations and then only appears in o after trauma and o also has a
grandparent from a different lineage that has Dupuytren's so it could have come
from their rather than any genetics link passed down from 1 and 2.
Summary:
What this tree seems to show is exactly what is mentioned in all of the
papers and that is that this disease is dominant but has different rates of
appearing and maybe requires a trigger so that if you
are susceptible to getting the disease you may still need a trigger
such as trauma or excessive alcohol to actually cause the disease to show
itself.
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Dearest Gary,
ReplyDeleteThanks for your visit and comment on my post about Dupuytren's disease, on the 29th.
Yes, I did read about the Lederhosen disease and was surprised by the mass misspelling of this German name. It is with only ONE 'd'.
Lederhosen meaning leather pants.
Probably it was a German docter who named it after him.
It is good to know that there are other cases out there.
Wishing you all the best,
Mariette
It is named after someone so although as you say there is the word Lederhosen meaning leather pants it is Ledderhose disease after Georg Ledderhose as far as I am aware this does have 2 d and that is why the disease has 2 d.
ReplyDeletehttp://en.wikipedia.org/wiki/Georg_Ledderhose
Hope you are well and you don't get to the stage where you need treatment.
Gary