Re: Sclerosing Cholangitis Epidemiology and Etiology.

[Guest guest]

-

This is one that has puzzled me for a while..by calling it primary

sclerosing cholangitis you are saying you can't identify the cause of

the sclerosing cholangitis but everyone says PSC is an autoimmune

disease so isn't that what the cause is ? By saying secondary

sclerosing cholangitis you are implying you know the trigger/cause but

that doesn't mean you can treat the underlying cause or stop

progression- so how are the two labels different ??

Lori

lucky mom to triplets including Braden w/short bowel syndrome, P or S

?? sclerosing cholangitis, immune deficiency, FAP

>

> O.K., so if we can come up with the cause of PSC, does this paper

> mean that it would no longer be called PSC, but SSC?

>

> J Gastrointest Surg. 2007 Oct 24 [Epub ahead of print]

>

> Sclerosing Cholangitis Epidemiology and Etiology.

>

> Lazaridis KN

>

> Center for Basic Research in Digestive Diseases, Division of

> Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200

> First Street SW, Rochester, MN, 55905, USA,

> lazaridis.konstantinos@...

>

> Sclerosing cholangitis represents a spectrum of chronic biliary

> diseases that either has an unknown etiology (i.e., primary) or is

> caused by identifiable insults to the biliary tree (i.e., secondary).

> To date, the epidemiology of primary sclerosing cholangitis has been

> appraised; however, its etiology continues to be unclear. In

> contrast, the etiology of secondary sclerosing cholangitis is always

> known, but the epidemiology of this clinical entity is difficult to

> study. PMID: 17957439.

>

> Dave

> (father of (23); PSC 07/03; UC 08/03)

>

[Guest guest]

Hi Lori;

These are very good questions. The distinction between secondary and

primary sclerosing cholangitis would seem to make a difference if the

SC could be associated with a specific infectious agent, which could

then be eliminated with a suitable antibiotic. This has been reported

in sclerosing cholangitis associated with immune deficiency and

colonization of the biliary tract with Cryptosporidium:

Am J Gastroenterol. 1987 Nov;82(11):1196-202.

Sclerosing cholangitis associated with chronic cryptosporidiosis in a

child with a congenital immunodeficiency disorder.

JJ, Heyman MB, Ferrell L, Kerner J, Kerlan R Jr, Thaler MM.

Department of Pediatrics, University of California, San Francisco.

Primary sclerosing cholangitis (PSC) in children is a rare and

incurable disorder of unknown etiology. We report an immunodeficient

child with chronic cryptosporidiosis of the biliary tract leading to

clinical, pathological, and radiographic findings consistent with

PSC. This case documents the ability of Cryptosporidium to

disseminate to extraintestinal organs, and suggests that chronic

cryptosporidial infection of the biliary tract may be one etiological

mechanism producing sclerosing cholangitis in immunodeficient

children. The increased incidence of PSC in immunodeficient children

may in part be due to their inability to resolve infections of the

biliary tract, which may result in sclerosing cholangitis mimicking

PSC. We submit that an aggressive diagnostic workup should be

performed to rule out an infectious etiology of sclerosing

cholangitis in immunodeficient patients who have findings of PSC,

because specific chemotherapy against the infecting organism would

potentially arrest progressive biliary obliteration. PMID: 3674002.

Has Braden been tested for anything like this (since you report that

he has immune deficiency)?

Best regards,

Dave

(father of (23); PSC 07/03; UC 08/03)

[Guest guest]

-

Thanks for all that you do for so many here !!!

The most current thinking about Braden's immune deficiency

(hypogammoglobinemia- sp ??) is that it was caused by the severe

malabsorption due to his short gut and is not a genetic immune

deficiency. Several doctors disagree though and the only thing they

agree on is that he needs monthly IVIG treatments for the immune

deficiency- his total IgG is low as are all his subclasses except for

his IgG 3 - it's in the low normal range.

I have heard that one of the theories behind the cause of PSC is

bacterial translocation with inflammatory bowel. Braden has that to

the extreme !! with short bowel syndrome, no ileocecal valve and long

term problems with small bowel bacterial overgrowth and extremely

compromised GI tract, increased EOS and inflammation....

Lori

>

> Hi Lori;

>

> These are very good questions. The distinction between secondary and

> primary sclerosing cholangitis would seem to make a difference if the

> SC could be associated with a specific infectious agent, which could

> then be eliminated with a suitable antibiotic. This has been reported

> in sclerosing cholangitis associated with immune deficiency and

> colonization of the biliary tract with Cryptosporidium:

>

> Am J Gastroenterol. 1987 Nov;82(11):1196-202.

>

> Sclerosing cholangitis associated with chronic cryptosporidiosis in a

> child with a congenital immunodeficiency disorder.

>

> JJ, Heyman MB, Ferrell L, Kerner J, Kerlan R Jr, Thaler MM.

>

> Department of Pediatrics, University of California, San Francisco.

>

> Primary sclerosing cholangitis (PSC) in children is a rare and

> incurable disorder of unknown etiology. We report an immunodeficient

> child with chronic cryptosporidiosis of the biliary tract leading to

> clinical, pathological, and radiographic findings consistent with

> PSC. This case documents the ability of Cryptosporidium to

> disseminate to extraintestinal organs, and suggests that chronic

> cryptosporidial infection of the biliary tract may be one etiological

> mechanism producing sclerosing cholangitis in immunodeficient

> children. The increased incidence of PSC in immunodeficient children

> may in part be due to their inability to resolve infections of the

> biliary tract, which may result in sclerosing cholangitis mimicking

> PSC. We submit that an aggressive diagnostic workup should be

> performed to rule out an infectious etiology of sclerosing

> cholangitis in immunodeficient patients who have findings of PSC,

> because specific chemotherapy against the infecting organism would

> potentially arrest progressive biliary obliteration. PMID: 3674002.

>

> Has Braden been tested for anything like this (since you report that

> he has immune deficiency)?

>

> Best regards,

>

> Dave

> (father of (23); PSC 07/03; UC 08/03)

>