Mayo Clinic -Research Study

[Guest guest]

Hi Everyone,

There is a research study I found on-line and am going to put my son

Ray in. He will be 18 years old on May 18th. You have to be 18. He

said that because most patients are men they don't get alot of

participants. A rich family who has a child is funding the

project. He was asking me if we ever used lawn service to spray

chemicals. We did about 5 years ago for 2 years.

My son was diagnosed in October 06,recently his liver enzymes went

wacky again. He also has an extremely high copper level. Does

anyone have this? The Doctors at Childrens Hospital of Phila. just

put him on zinc.

He is pretty itchy and probably has had this for a couple of years.

I don't think he has IBS or Crohn's. He was perfectly healthy and

active until this and all of us are fine too. He is very thin and I

am nervous that his is progressing quickly.

If there are any young people out there please write back. Thanks

[Guest guest]

Hello, and welcome to the group. Sorry to hear about your son's PSC

diagnosis. Is the research study that you mention the STOPSC study,

funded by the Foundation (and recently also by PSC Partners

Seeking a Cure)?

https://web.emmes.com/study/psc/index.html

Your son's high copper level could possibly be the result of PSC, as

it seems that elevated hepatic copper is commonly associated with PSC:

__________________

Scand J Gastroenterol. 1995 Dec;30(12):1200-3.

Hepatic retention of copper and selenium in primary sclerosing

cholangitis.

Aaseth J, sen Y, Aadland E, Fausa O, Schrumpf E

Dept. of Clinical Chemistry, Hedmark Central Hospital, Norway.

BACKGROUND: Previous studies have suggested abnormal copper

metabolism in patients with primary sclerosing cholangitis (PSC). In

the present work the trace element metabolism was studied in a group

of 32 patients with PSC. METHODS: Hepatic copper and selenium

concentrations were determined with a sensitive electrothermal atomic

absorption technique. Serum concentrations of copper and zinc were

determined by conventional atomic absorption. RESULTS: For the

patient group serum copper values (20.3 +/- 4.5 mumol/l) were higher

than those for the control group (14 +/- 3 mumol/l), and average

hepatic copper concentrations were greater by a factor of four. Serum

selenium values were slightly lower, although the average hepatic

selenium was significantly higher than in the healthy control group.

Previous studies have discussed possible toxic effects of

hepatocellular copper accumulation, which may be accompanied by

formation of activated oxygen species and depletion of glutathione.

In the present study, however, it could not be demonstrated that the

concentration of the lipoperoxidation product, malonic dialdehyde,

was higher than normal in blood. Furthermore, blood concentrations of

glutathione and glutathione peroxidase were not abnormal. CONCLUSION:

Although a protective effect of the raised selenium concentrations in

the liver might be discussed, it is apparent that the copper

accumulation in the liver cells described here did not induce

detectable changes in the indices studied. PMID: 9053974.

__________________

However, another possibility to explain the elevated copper would be

's disease:

http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm

The differences between 's disease and PSC would likely be:

1. Elevated serum alkaline phosphatase (ALP) and gamma-

glutamyltranspeptidase (GGT) in PSC ['s disease tends to cause

only elevated serum ALT and AST].

2. PSC is more likely to be associated with inflammatory bowel

disease (IBD) (our son was found to have ulcerative colitis only

after PSC diagnosis and a colonoscopy; in many PSC patients IBD can

be " silent " and have few obvious symptoms).

3. An ERCP (endoscopic retrograde cholangiopancreatography) or MRCP

(magnetic resonance cholangiopancreatography) would tend to show

characteristic narrowing and/or beading of bile-ducts in PSC. Has

your son had one of these procedures to establish his diagnosis?

4. A genetic test is available for 's disease, but not yet PSC.

Please let us know if we can answer any specific questions you may

have, or help clarify any points.

Our son was diagnosed with PSC at age 18, just before going off to

college. He's now 21 (almost 22) and mostly without symptoms. His

itching was managed quite well by a prescription of rifampin

(rifampicin). In addition he takes high-dose ursodiol, asacol,

vitamin supplements, folic acid, and fish oils. We are thankful that

he's had a good time at college, without any major health probelms,

and will be graduating next weekend.

Best regards,

Dave

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

[Guest guest]

Hello, and welcome to the group. Sorry to hear about your son's PSC

diagnosis. Is the research study that you mention the STOPSC study,

funded by the Foundation (and recently also by PSC Partners

Seeking a Cure)?

https://web.emmes.com/study/psc/index.html

Your son's high copper level could possibly be the result of PSC, as

it seems that elevated hepatic copper is commonly associated with PSC:

__________________

Scand J Gastroenterol. 1995 Dec;30(12):1200-3.

Hepatic retention of copper and selenium in primary sclerosing

cholangitis.

Aaseth J, sen Y, Aadland E, Fausa O, Schrumpf E

Dept. of Clinical Chemistry, Hedmark Central Hospital, Norway.

BACKGROUND: Previous studies have suggested abnormal copper

metabolism in patients with primary sclerosing cholangitis (PSC). In

the present work the trace element metabolism was studied in a group

of 32 patients with PSC. METHODS: Hepatic copper and selenium

concentrations were determined with a sensitive electrothermal atomic

absorption technique. Serum concentrations of copper and zinc were

determined by conventional atomic absorption. RESULTS: For the

patient group serum copper values (20.3 +/- 4.5 mumol/l) were higher

than those for the control group (14 +/- 3 mumol/l), and average

hepatic copper concentrations were greater by a factor of four. Serum

selenium values were slightly lower, although the average hepatic

selenium was significantly higher than in the healthy control group.

Previous studies have discussed possible toxic effects of

hepatocellular copper accumulation, which may be accompanied by

formation of activated oxygen species and depletion of glutathione.

In the present study, however, it could not be demonstrated that the

concentration of the lipoperoxidation product, malonic dialdehyde,

was higher than normal in blood. Furthermore, blood concentrations of

glutathione and glutathione peroxidase were not abnormal. CONCLUSION:

Although a protective effect of the raised selenium concentrations in

the liver might be discussed, it is apparent that the copper

accumulation in the liver cells described here did not induce

detectable changes in the indices studied. PMID: 9053974.

__________________

However, another possibility to explain the elevated copper would be

's disease:

http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm

The differences between 's disease and PSC would likely be:

1. Elevated serum alkaline phosphatase (ALP) and gamma-

glutamyltranspeptidase (GGT) in PSC ['s disease tends to cause

only elevated serum ALT and AST].

2. PSC is more likely to be associated with inflammatory bowel

disease (IBD) (our son was found to have ulcerative colitis only

after PSC diagnosis and a colonoscopy; in many PSC patients IBD can

be " silent " and have few obvious symptoms).

3. An ERCP (endoscopic retrograde cholangiopancreatography) or MRCP

(magnetic resonance cholangiopancreatography) would tend to show

characteristic narrowing and/or beading of bile-ducts in PSC. Has

your son had one of these procedures to establish his diagnosis?

4. A genetic test is available for 's disease, but not yet PSC.

Please let us know if we can answer any specific questions you may

have, or help clarify any points.

Our son was diagnosed with PSC at age 18, just before going off to

college. He's now 21 (almost 22) and mostly without symptoms. His

itching was managed quite well by a prescription of rifampin

(rifampicin). In addition he takes high-dose ursodiol, asacol,

vitamin supplements, folic acid, and fish oils. We are thankful that

he's had a good time at college, without any major health probelms,

and will be graduating next weekend.

Best regards,

Dave

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

[Guest guest]

Hello, and welcome to the group. Sorry to hear about your son's PSC

diagnosis. Is the research study that you mention the STOPSC study,

funded by the Foundation (and recently also by PSC Partners

Seeking a Cure)?

https://web.emmes.com/study/psc/index.html

Your son's high copper level could possibly be the result of PSC, as

it seems that elevated hepatic copper is commonly associated with PSC:

__________________

Scand J Gastroenterol. 1995 Dec;30(12):1200-3.

Hepatic retention of copper and selenium in primary sclerosing

cholangitis.

Aaseth J, sen Y, Aadland E, Fausa O, Schrumpf E

Dept. of Clinical Chemistry, Hedmark Central Hospital, Norway.

BACKGROUND: Previous studies have suggested abnormal copper

metabolism in patients with primary sclerosing cholangitis (PSC). In

the present work the trace element metabolism was studied in a group

of 32 patients with PSC. METHODS: Hepatic copper and selenium

concentrations were determined with a sensitive electrothermal atomic

absorption technique. Serum concentrations of copper and zinc were

determined by conventional atomic absorption. RESULTS: For the

patient group serum copper values (20.3 +/- 4.5 mumol/l) were higher

than those for the control group (14 +/- 3 mumol/l), and average

hepatic copper concentrations were greater by a factor of four. Serum

selenium values were slightly lower, although the average hepatic

selenium was significantly higher than in the healthy control group.

Previous studies have discussed possible toxic effects of

hepatocellular copper accumulation, which may be accompanied by

formation of activated oxygen species and depletion of glutathione.

In the present study, however, it could not be demonstrated that the

concentration of the lipoperoxidation product, malonic dialdehyde,

was higher than normal in blood. Furthermore, blood concentrations of

glutathione and glutathione peroxidase were not abnormal. CONCLUSION:

Although a protective effect of the raised selenium concentrations in

the liver might be discussed, it is apparent that the copper

accumulation in the liver cells described here did not induce

detectable changes in the indices studied. PMID: 9053974.

__________________

However, another possibility to explain the elevated copper would be

's disease:

http://www.nlm.nih.gov/medlineplus/ency/article/000785.htm

The differences between 's disease and PSC would likely be:

1. Elevated serum alkaline phosphatase (ALP) and gamma-

glutamyltranspeptidase (GGT) in PSC ['s disease tends to cause

only elevated serum ALT and AST].

2. PSC is more likely to be associated with inflammatory bowel

disease (IBD) (our son was found to have ulcerative colitis only

after PSC diagnosis and a colonoscopy; in many PSC patients IBD can

be " silent " and have few obvious symptoms).

3. An ERCP (endoscopic retrograde cholangiopancreatography) or MRCP

(magnetic resonance cholangiopancreatography) would tend to show

characteristic narrowing and/or beading of bile-ducts in PSC. Has

your son had one of these procedures to establish his diagnosis?

4. A genetic test is available for 's disease, but not yet PSC.

Please let us know if we can answer any specific questions you may

have, or help clarify any points.

Our son was diagnosed with PSC at age 18, just before going off to

college. He's now 21 (almost 22) and mostly without symptoms. His

itching was managed quite well by a prescription of rifampin

(rifampicin). In addition he takes high-dose ursodiol, asacol,

vitamin supplements, folic acid, and fish oils. We are thankful that

he's had a good time at college, without any major health probelms,

and will be graduating next weekend.

Best regards,

Dave

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