Re: PSC - The arteriosclerosis of the bile duct (LONG reply to Nina

[Guest guest]

Well, Ross, clearly you have a MUCH better grasp of this than I do,

so plz ignore my now-ridiculous-looking explanation! I love the golf

analogy, though, , I really do think it's perfect.

Ross, I have one follow-up question for YOU, now that I've read

through your post and *THINK* i understand it - based on other posts

my understanding of the PXR receptor is that its availability is also

dictated by genetics - couldn't the CFTR mutation affect the PXR

receptor? Or maybe the constellation of genes that seems to be

responsible for IBD controls PXR?

The other things that I think may further complicate the issues are

that I'm still not convinced that PSC is just one thing. Given its

incredibly varying course and symptoms, I would not at all be

surprised if there are two or three systemic problems whose

main " symptom " is what we consider to be PSC - i.e. the narrowing of

the bile ducts. So maybe there's one " disease " that is somehow

related to elevated IgG4, another that's related to elevated ANCA,

another that seems to affect children, etc. etc.

Also, i think i understand that they're now thinking that genetic

susceptibility for IBD is through a combination of genes - maybe a

CFTR mutation is just one of the genes that controls PSC? My husband

has never been tested for the CFTR gene, but he did test heterozygous

for 's disease - given the increased copper retention of many

PSCers maybe yet another variation of PSC is some CF/'s

hybrid?

Anyway, thanks for indulging me in the " heavy science " discussion -

even though i don't understand it all it makes me feel like i'm doing

something when i at least try to learn it... tx,

nina