Question regarding intra- and extra- hepatic bile duct damage

July 19, 2006

Okay, you all know I

really struggle with this issue and the incessant confusion that I have but

what happens if you just have the intrahepatic damage? Is that advanced? Or does

that just mean that is where the disease started with that patient? Or will it

go back out and catch the extrahepatic ducts? (which would not be all bad in my

mind.) Does having intrahepatic damage cause more symptoms in the way of

fatigue or what? Can any one speak to this? Labs? Just curious what this

does to the disease course in general.

Thank you in advance.

Would all 4 portal tracts being involved mean intra- or extra- or does that

alone not indicate either? Can you tell that I still don’t quite get it?

www.caringbridge.org/pa/nwmartens

July 20, 2006

" Just " intrahepatic damage may be indicative of small duct PSC, which mainly involves the intrahepatic ducts (within the liver). Small duct PSC is thought to have a better long-term outlook (takes much longer to get to transplant). See http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=12029635 & dopt=Abstract, for example. I can't recall if you said which diagnosis he has.

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On Behalf Of

Okay, you all know I really struggle with this issue and the incessant confusion that I have but what happens if you just have the intrahepatic damage? Is that advanced? Or does that just mean that is where the disease started with that patient? Or will it go back out and catch the extrahepatic ducts? (which would not be all bad in my mind.) Does having intrahepatic damage cause more symptoms in the way of fatigue or what? Can any one speak to this? Labs? Just curious what this does to the disease course in general.

Thank you in advance. Would all 4 portal tracts being involved mean intra- or extra- or does that alone not indicate either? Can you tell that I still don’t quite get it?

July 20, 2006

" Just " intrahepatic damage may be indicative of small duct PSC, which mainly involves the intrahepatic ducts (within the liver). Small duct PSC is thought to have a better long-term outlook (takes much longer to get to transplant). See http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=12029635 & dopt=Abstract, for example. I can't recall if you said which diagnosis he has.

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On Behalf Of

Okay, you all know I really struggle with this issue and the incessant confusion that I have but what happens if you just have the intrahepatic damage? Is that advanced? Or does that just mean that is where the disease started with that patient? Or will it go back out and catch the extrahepatic ducts? (which would not be all bad in my mind.) Does having intrahepatic damage cause more symptoms in the way of fatigue or what? Can any one speak to this? Labs? Just curious what this does to the disease course in general.

Thank you in advance. Would all 4 portal tracts being involved mean intra- or extra- or does that alone not indicate either? Can you tell that I still don’t quite get it?

July 20, 2006

" Just " intrahepatic damage may be indicative of small duct PSC, which mainly involves the intrahepatic ducts (within the liver). Small duct PSC is thought to have a better long-term outlook (takes much longer to get to transplant). See http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=12029635 & dopt=Abstract, for example. I can't recall if you said which diagnosis he has.

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On Behalf Of

Okay, you all know I really struggle with this issue and the incessant confusion that I have but what happens if you just have the intrahepatic damage? Is that advanced? Or does that just mean that is where the disease started with that patient? Or will it go back out and catch the extrahepatic ducts? (which would not be all bad in my mind.) Does having intrahepatic damage cause more symptoms in the way of fatigue or what? Can any one speak to this? Labs? Just curious what this does to the disease course in general.

Thank you in advance. Would all 4 portal tracts being involved mean intra- or extra- or does that alone not indicate either? Can you tell that I still don’t quite get it?

July 20, 2006

Well, I cannot

remember either to be honest and my doctor has confused the matter by doing

some flip-flopping. She seems to answer my question with what I seem to her

that day as wanting to hear. <grrrr> So, we are getting records for an

eyes-on look.

This conversation

came up after Clint (relaytech1960) got his ERCP results and started asking me

and asked me to ask the group because he was too tired…hmmm wonder why.

(I hate this disease.) So my questions are really for two of us and I really

have his newbie thoughts in mind as well as my own. He only had intrahepatic

damage. So that is a GOOD thing right!!! That would be small duct PSC? The

supposed slow-moving train. It will not go back out and cause damage to the

extrahepatic ducts will it? Is fatigue worse with intrahepatic or does it just

depend on how far you are into the disease? Clint is historically a very

active fellow (biker, runner) and is really suffering from the fatigue end of

things. And it seems to have slammed him all of a sudden…like in the

last 3-6 months which is VERT frustrating to someone who WANTS to be out there

doing things. Does anyone have any words of wisdom for him. I KNOW he won’t

ask, but he should if there is anything to help.

God Bless you

all, this is a wonderful group,

Mom of Zoe (13) My very normal (teenager normal) soccer player;

Noah (8 1/2) Indeterminate colitis, PSC, Osteopenia (1-4

lumbar vertebrae), Enthesopathy;

Aidan (4 1/2) Moderately-severe SNHL bilaterally

Recycle

Yourself

Become an

Organ Donor

July 20, 2006

I think it just depends. Slow-moving does not mean there are not symptoms,

just that it is generally much longer to transplant. Some have fatigue with

no other symptoms. Some never experience fatigue even up to transplant.

Some only have itching, some never do.

One thing that's certain about PSC is everyone appears to be different!

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On

Behalf Of

....He only had intrahepatic damage. So that is a GOOD thing right!!! That

would be small duct PSC? The supposed slow-moving train. It will not go

back out and cause damage to the extrahepatic ducts will it? Is fatigue

worse with intrahepatic or does it just depend on how far you are into the

disease?..

July 20, 2006

I think it just depends. Slow-moving does not mean there are not symptoms,

just that it is generally much longer to transplant. Some have fatigue with

no other symptoms. Some never experience fatigue even up to transplant.

Some only have itching, some never do.

One thing that's certain about PSC is everyone appears to be different!

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On

Behalf Of

....He only had intrahepatic damage. So that is a GOOD thing right!!! That

would be small duct PSC? The supposed slow-moving train. It will not go

back out and cause damage to the extrahepatic ducts will it? Is fatigue

worse with intrahepatic or does it just depend on how far you are into the

disease?..

July 20, 2006

I think it just depends. Slow-moving does not mean there are not symptoms,

just that it is generally much longer to transplant. Some have fatigue with

no other symptoms. Some never experience fatigue even up to transplant.

Some only have itching, some never do.

One thing that's certain about PSC is everyone appears to be different!

Arne

55 - UC 1977, PSC 2000

Alive and (mostly) well in Minnesota

________________________________

From: [mailto: ] On

Behalf Of

....He only had intrahepatic damage. So that is a GOOD thing right!!! That

would be small duct PSC? The supposed slow-moving train. It will not go

back out and cause damage to the extrahepatic ducts will it? Is fatigue

worse with intrahepatic or does it just depend on how far you are into the

disease?..

July 20, 2006

Dear ;

PSC can start with damage to the intrahepatic ducts, and then later

can affect the larger and extrahepatic ducts. Adolf Stiehl notes

that the majority of patients with PSC will eventually develop

dominant strictures (stenoses), and when this occurs, he advocates

opening then with endoscopic procedures:

J Hepatol. 2002 Feb;36(2):151-6.

Comment in: J Hepatol. 2002 Feb;36(2):278-9.

Development of dominant bile duct stenoses in patients with primary

sclerosing cholangitis treated with ursodeoxycholic acid: outcome

after endoscopic treatment.

Stiehl A, Rudolph G, Kloters-Plachky P, Sauer P, S.

Department of Medicine, University of Heidelberg, Medizinische

Universitatsklinik, Bergheimerstrasse 58, D-69115 Heidelberg,

Germany. adolf_stiehl@...

BACKGROUND/AIMS: Primary sclerosing cholangitis is characterized by

progressive fibrotic inflammation and obliteration of intra- and/or

extrahepatic bile ducts. METHODS: In a prospective study of 106

patients treated for up to 13 years with ursodeoxycholic acid, the

development of major bile duct stenoses and the efficacy of

endoscopic measures have been evaluated. RESULTS: Of 106 patients

ten had major duct stenoses at entry, and during a median follow-up

period of 5.0 years another 43 developed a dominant stenosis. Fifty-

two patients with dominant stenoses were treated endoscopically by

repeated balloon dilatations and five patients were temporarily

stented. Complications of endoscopic procedures were pancreatitis

(5.2%), bacterial cholangitis (3.3%) and bile duct perforation

(0.5%). Five years after the first dilatation of a dominant stenosis

the Kaplan-Meier survival rates free of liver transplantation were

100% in stage 2, 72% in stage 3 and 50% in stage 4 disease. The

actuarial survival free of liver transplantation of the whole group

at 3, 5 and 7 years were 0.987, 0.935 and 0.891 and the

corresponding survival rates predicted with the Mayo multicenter

survival model were 0.860, 0.775 and 0.737 (P<0.001). CONCLUSIONS:

In advanced disease, occlusion of major bile ducts with time occurs

in the majority of patients. Endoscopic opening of dominant stenoses

is effective and appears to be a valuable addition to the medical

treatment of such patients.

Publication Types:

Clinical Trial

PMID: 11830325

Intrahepatic bile-duct blockages can produce all of the classic

symtoms ... elevated liver function tests, pruritus, fatigue etc.

PBC only affects the small-ducts, and PBCers have all of these

symptoms!

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)

>

> He only had intrahepatic damage. So that is a GOOD thing right!!!

> That would be small duct PSC? The supposed slow-moving train. It

will not go back out and cause damage to the extrahepatic ducts will

it? Is fatigue worse with intrahepatic or does it just depend on

how far you are into the disease?

July 20, 2006