Anybody has e.g MVP?

yep - son with KP/UO symptoms has

Dear Anne what kind of problem does he have?

transposition of the great arteries i.e. born with pulmanory and aorta around the wrong way - arterial switch was performed at 7 days old.

Also has a bicuspid valve on the aorta

what does MVP stand for???

PS: did you know that KPAF and UO often appear with other congenital abnormalities and that quite a few syndromes involving the heart have KPAF and UO as part of them - I am not sure if KPRF also appears in the mix as well.

Hey - just looked it up myself - mitrial valve prolapse!

Just read this on some site:

The mitral valve prolapse (MVP) syndrome has a strong hereditary tendency, although the exact cause is unknown. Affected family members are often tall, thin, with long arms and fingers, and straight backs. It is seen most commonly in women from 20 to 40 years old, but also occurs in men

just out of interest does this description of tall thin etc fit you? - seems strange that a heart defect would be connected with having long fingers doesn't it?

I'm not so tall (5 ft 9 in) but I'm quite thin (124 lb). I've got KPRF. Regards

Can somebody please tell me the difference between KPAF, KPRF, and UO? I'm not exactly sure what I have anymore. Thanks

KPRF atrophicans - ulerythema etc

here you can find great explanation by anne

I was born with Pulmonary Atresia with VSD (ventricular septal defect).
You can read about it here: http://www.rch.org.au/cardiology/def...fm?doc_id=5084

Yes it is hereditary.
My sister have heart murmur, also my grandmother has it and my grandgrandmother had it. They also have/had some redness on their cheeks...


I strongly believe that there is some connection "bad heart - redness" but i cannot give you any proof or anything that interpret that i could be a connection :P

Yes I do to - if you look at CFC syndrome, Rubinstein–Taybi Syndrome and Noonans syndrome you will see the whole heart thing and ulerythema ophryogenes combined.

The only article I have ever read that showed someone with KPRF having a heart condition was the one posted on this site recently by ANUNU which said one of the patients with KPRF had transpostion of the great arteries.

However, as not much is written about KPRF fullstop there may well be a higher percentage of congenital heart condition occuring in people with this condition.

Statistically approximately 1% of the population is born with some heart defect (it may just be a small whole in the heart). As we have less than 100 on this site with KPRF (I think!) and at least two of you have mentioned a definate congenital heart problem it would be fair to say that is above the usual percentage!

Not very scientific - but Pehr your family history certainly would make someone think there was a connection.

I had open heart in 2000 to patch my hole and valve repair and I have KP. I believe all of this goes together. PP

Oh, very intresting anne. I have never heard of those syndromes before.
Intresting.

Heh, not very scientific but it might be correct that we KP/KPRF-people are have a higher percentage of heart defects

We could make a poll about it?

I wonder how it would be if I had another operation and this time get an almost normal heart. And then do some cardio exercise
Who knows, I might get rid of my redness ;D

Not sure if fixing the heart problem would help the KPRF as the KPRF is genetic and it appears than the gene that causes KPRF must be near or linked with a gene that causes heart defects. This is why you get these syndromes shown above which have KP variants appearing along with heart defects.

I agree it would be good to do some kind of survey seeing if people with KPRF, KPAF and UO do actually have a higher case of heart defects than those without.

I have never read though that normal KP is assoicated without any any other syndrome - it seems that the syndromes tend to attract the rarer variants of KP.

My KP is constant in my body. It will flare to severe during stress or immune disturbance. I am still going through a flare up since October. The keritan in my fingers swell the tips of my finger 2x's the normal size and the skin bursts and the keritan comes out in large strips. It also comes out in my eye lashes; scalp and nose. It will drain from my nose to the back of my throat and now I have asthma and cough at night.

You can squeeze my finger tips and see the Keritan squirt out of my finger nails and skin. As soon as it hits the air it turns to a white flacky stuff.

I also get it on my face and it will make blisters and leave scars.

It is painful and I itch constantly and other than some cream that the dermatoligist gave to liquify the plugs the doctors can not do anything.

PP

For the heart problems look up Marfran's Syndrome. This fits what I have. PP