Question regarding intra- and extra- hepatic bile duct damage

[Guest guest]

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

[Guest guest]

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

[Guest guest]

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

[Guest guest]

Hi ;

I'd tend to agree with Arne that PSC with only small-duct

(intrahepatic) involvement seems to have a better prognosis than PSC

with large-duct involvement. According to Adolf Stiehl, when the

large-ducts get strictured (he calls these large-duct

structures 'dominant stenoses') there seeems to be a higher risk of

bacterial infections of the bile. This can lead to deterioration of

liver function.

Eur J Gastroenterol Hepatol. 2006 Jan;18(1):69-74.

The role of dominant stenoses in bacterial infections of bile ducts

in primary sclerosing cholangitis.

Pohl J, Ring A, Stremmel W, Stiehl A.

Department of Internal Medicine IV, Ruprechts Karls University,

Heidelberg, Germany.

OBJECTIVE: Primary sclerosing cholangitis (PSC) is characterized by

progressive fibrotic inflammation and strictures of the biliary

system. We studied the role of dominant stenoses in bacterial

biliary infections and the effect of routine antibiotic

administration with cholangiography. DESIGN: A prospective clinical

trial without blinding or randomization. SETTING: The endoscopy unit

in a university hospital. PARTICIPANTS: Fifty patients with PSC

entered and finished the study. INTERVENTIONS: A total of 103

endoscopic retrograde cholangiographies (ERC) was performed in 37

PSC patients with dominant stenosis and 13 controls with PSC but no

dominant stenosis. After selective cannulation of the bile duct,

bile samples were obtained during each procedure. All patients

received systemic antibiotic treatment with ciprofloxacin for one

week after ERC. RESULTS: Enteric bacteria were detected in the bile

specimens of 15 out of 37 PSC patients (40.5%) with dominant

stenosis but never in the absence of dominant stenosis (P=0.004).

Positive cultures for enteric bacteria were associated with elevated

serum C-reactive protein, high leukocyte counts in bile (P<0.05) and

the deterioration of liver function assessed by increasing bilirubin

levels during the follow-up period lasting a median of 7 months

(P=0.06). Despite the high rate of susceptibility in vitro,

ciprofloxacin treatment eradicated enteric bacteria in only two out

of 12 cases. CONCLUSION: Bacterial infection of the bile ducts with

dominant stenosis is a frequent finding and may play a role in the

progression of PSC. Short-course antibiotic treatment is not very

effective in eradicating acteria from the bile ducts.

PMID: 16357622

Best regards,

Dave

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

[Guest guest]

Hi ;

I'd tend to agree with Arne that PSC with only small-duct

(intrahepatic) involvement seems to have a better prognosis than PSC

with large-duct involvement. According to Adolf Stiehl, when the

large-ducts get strictured (he calls these large-duct

structures 'dominant stenoses') there seeems to be a higher risk of

bacterial infections of the bile. This can lead to deterioration of

liver function.

Eur J Gastroenterol Hepatol. 2006 Jan;18(1):69-74.

The role of dominant stenoses in bacterial infections of bile ducts

in primary sclerosing cholangitis.

Pohl J, Ring A, Stremmel W, Stiehl A.

Department of Internal Medicine IV, Ruprechts Karls University,

Heidelberg, Germany.

OBJECTIVE: Primary sclerosing cholangitis (PSC) is characterized by

progressive fibrotic inflammation and strictures of the biliary

system. We studied the role of dominant stenoses in bacterial

biliary infections and the effect of routine antibiotic

administration with cholangiography. DESIGN: A prospective clinical

trial without blinding or randomization. SETTING: The endoscopy unit

in a university hospital. PARTICIPANTS: Fifty patients with PSC

entered and finished the study. INTERVENTIONS: A total of 103

endoscopic retrograde cholangiographies (ERC) was performed in 37

PSC patients with dominant stenosis and 13 controls with PSC but no

dominant stenosis. After selective cannulation of the bile duct,

bile samples were obtained during each procedure. All patients

received systemic antibiotic treatment with ciprofloxacin for one

week after ERC. RESULTS: Enteric bacteria were detected in the bile

specimens of 15 out of 37 PSC patients (40.5%) with dominant

stenosis but never in the absence of dominant stenosis (P=0.004).

Positive cultures for enteric bacteria were associated with elevated

serum C-reactive protein, high leukocyte counts in bile (P<0.05) and

the deterioration of liver function assessed by increasing bilirubin

levels during the follow-up period lasting a median of 7 months

(P=0.06). Despite the high rate of susceptibility in vitro,

ciprofloxacin treatment eradicated enteric bacteria in only two out

of 12 cases. CONCLUSION: Bacterial infection of the bile ducts with

dominant stenosis is a frequent finding and may play a role in the

progression of PSC. Short-course antibiotic treatment is not very

effective in eradicating acteria from the bile ducts.

PMID: 16357622

Best regards,

Dave

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

[Guest guest]

Hi ;

I'd tend to agree with Arne that PSC with only small-duct

(intrahepatic) involvement seems to have a better prognosis than PSC

with large-duct involvement. According to Adolf Stiehl, when the

large-ducts get strictured (he calls these large-duct

structures 'dominant stenoses') there seeems to be a higher risk of

bacterial infections of the bile. This can lead to deterioration of

liver function.

Eur J Gastroenterol Hepatol. 2006 Jan;18(1):69-74.

The role of dominant stenoses in bacterial infections of bile ducts

in primary sclerosing cholangitis.

Pohl J, Ring A, Stremmel W, Stiehl A.

Department of Internal Medicine IV, Ruprechts Karls University,

Heidelberg, Germany.

OBJECTIVE: Primary sclerosing cholangitis (PSC) is characterized by

progressive fibrotic inflammation and strictures of the biliary

system. We studied the role of dominant stenoses in bacterial

biliary infections and the effect of routine antibiotic

administration with cholangiography. DESIGN: A prospective clinical

trial without blinding or randomization. SETTING: The endoscopy unit

in a university hospital. PARTICIPANTS: Fifty patients with PSC

entered and finished the study. INTERVENTIONS: A total of 103

endoscopic retrograde cholangiographies (ERC) was performed in 37

PSC patients with dominant stenosis and 13 controls with PSC but no

dominant stenosis. After selective cannulation of the bile duct,

bile samples were obtained during each procedure. All patients

received systemic antibiotic treatment with ciprofloxacin for one

week after ERC. RESULTS: Enteric bacteria were detected in the bile

specimens of 15 out of 37 PSC patients (40.5%) with dominant

stenosis but never in the absence of dominant stenosis (P=0.004).

Positive cultures for enteric bacteria were associated with elevated

serum C-reactive protein, high leukocyte counts in bile (P<0.05) and

the deterioration of liver function assessed by increasing bilirubin

levels during the follow-up period lasting a median of 7 months

(P=0.06). Despite the high rate of susceptibility in vitro,

ciprofloxacin treatment eradicated enteric bacteria in only two out

of 12 cases. CONCLUSION: Bacterial infection of the bile ducts with

dominant stenosis is a frequent finding and may play a role in the

progression of PSC. Short-course antibiotic treatment is not very

effective in eradicating acteria from the bile ducts.

PMID: 16357622

Best regards,

Dave

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

[Guest guest]

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?

[Guest guest]

These all sound like great questions to me, but I get the impression the state of the art is such that cause and effect relationships on the finer grain points that you suggest is in many cases still unknown. For example why some have debilitating fatigue, itching, etc.etc. years before needing a transplant and others see it late in the progression or not at all. I bet you could come up with enough great research questions to keep Mayo busy in this department for the next 50 years. I too am at times annoyed at the things we don't know (this topic and many others!!)

Bestjd, 45 goin to 46UC 1973, Jpouch 2000, Chronic Pouchitis 2001, PSC 2004, Stage 3Southern, IL_