Re: my nephew - potential overlap syndrome

[Guest guest]

Hi Rachna;

Welcome to the group. Sorry that your nephew's health brings you

here, but glad that you found the group.

Can I recommend that you read the following article on " Autoimmune

hepatitis: Making sense of all those antibodies " by Bruce A. Luxon,

MD, PhD in POSTGRADUATE MEDICINE /VOL 114 / NO 1 / JULY 2003:

http://www.postgradmed.com/issues/2003/07_03/3luxon.htm

It sounds like the antibody profile that you describe for your nephew

is consistent with type 1 autoimmune hepatitis. It is a good sign

that most of his liver enzymes have returned to normal in response to

the medications, since Jan 1. I think it is also a good sign that the

frequency of his fevers has declined. In the article cited above it

states that .... " A substantial proportion of patients who normalize

serum transaminase levels may continue to have ongoing active

hepatitis involving inflammation and fibrosis. An important caveat is

that histologic improvement may lag behind clinical and laboratory

improvement by as much as 6 months. Therefore, a second liver biopsy

should be considered only after that time to ensure adequate

histologic resolution of the disease process. " So, it might be a few

more months before the full effects of the medication are seen?

I am sorry, I cannot explain the fevers (I am assuming that

infections have been ruled out!). I am assuming that these fevers are

not due to the medications your nephew is taking, since you said that

he had these fevers more frequently before taking the medications.

It is quite possible that his contined tiredness may be related to

Imuran (which I think is the same as azathioprine). Some of the side

effects of azathioprine are:

More common

Cough or hoarseness; fever or chills ; lower back or side pain;

painful or difficult urination; unusual tiredness or weakness

Less common

Black, tarry stools; blood in urine or stools; pinpoint red spots

on skin; unusual bleeding or bruising

Rare

Fast heartbeat; fever (sudden); muscle or joint pain; nausea,

vomiting, and diarrhea (severe); redness or blisters on skin ;

shortness of breath; sores in mouth and on lips; stomach pain;

swelling of feet or lower legs; unusual feeling of discomfort or

illness (sudden)

http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202077.html

With regards to the possibility of your nephew having PSC, probably

an ERCP would be required to confirm this.

Hope that this answers some of your questions.

Best regards,

Dave

http://home.insightbb.com/~rhodesdavid/

[Guest guest]

Rachna,

It sounds like your nephew has had a very hard time of it--as has everyone who cares about him. It does appear as if the doctors have been thorough. I didn't see that you mentioned a ERCP (endoscopic retrograding cholangio pancreatography) I think that is right. That is the test to determine PSC. Also, what about his gall bladder? PSC usually has higher GGTP and Alkaline Phosphotase and moderate AST and ALT. During the early stage of liver diseases, it is more difficult to diagnose. I'm not sure what meds are making him so tired. Prednisone usually gives an anxious sort of energy. I've been on all those meds myself at one time or other. You didn't mention itching. Many of us itch like crazy. I also presented with edema before diagnosis. Most of us with uclerative colitis or crohns had/ have diarrhrea. I am no expert by any means. Positive ANA does suggest an autoimmune disorder, and ASMA is indicative of PBC. I suppose he had a high bilirubin during his acute attack? I know it took years for many of us to be diagnosed. I wish I could help more. Maybe someone else can offer some ideas. Take care,

Cheryl Berg, Idaho 45Married 25 years, 4 children, 2 grchildrenPSC 01, UC 00, Fibromyalgia, hypothyroid,hiatal hernia, ulcer, gall baldder removed '93disability (retired English teacher)

my nephew - potential overlap syndrome

Hi all, I am a new member. I joined to get more information on PSC. I'll give a bit of the background - this is about my nephew. Am hoping some of you might point us to a direction where we may get some answers.gi dr thinks it maybe PSC or autoimmune cholangitis LIKE, the liver specialist says he doesnt really know, he was suggesting it maybe crohn's or IBD - but that was ruled out.we are really struggling and in the meantime, he is taking these heavy duty medications and since he is so tired when he takes the meds in the morning he cant go to school right now.he is 14...any ideas from any of you? i would really appreciate it. Our family is very stressed out from this.Rachna

[Guest guest]

Hi rachna

I am not really going to answer your questions but more of a talk.

I was 13 when diagnosed and am now 15(f)

It was and still is hard for me since I use to do sports(they were my life

tennis,netball,soccer,athletics and karate but then got sick) and now am way

to tired to do anything.

I am alos on prednisone ursotan and permanent antibiotics I was on nexium

but then later discovered nexium was actually giving me stomach cramps.

Does your nephew have any side effects from psc now I have tireness ,sore

joints and sever upper abdominal pain.My upper ab pain is prob the worst

since it is the most painfull and I have it 2 to 3 times a week.

I also have not got uc or any thing which normally comes with psc(like your

nephew)

Was your nephew ever on a skin drug?( i am asking cause they say that

cyclimicine,skin drug, set my psc off.)

Any way I hope your nephew is feeling beter, be strong it will really help!

From

Terr(15)

South Africa

To: < >

Sent: Friday, February 27, 2004 6:49 PM

Subject: my nephew - potential overlap syndrome

> Hi all, I am a new member. I joined to get more information on PSC.

> I'll give a bit of the background - this is about my nephew. Am

> hoping some of you might point us to a direction where we may get

> some answers.

>

> In early december, 2003, my nephew had sever constipation and fever

> and he was slightly jaundiced - looked yellow around the eyes. he

> was also feeling a little bit tired, but still very active, playing

> tennis etc.

> trip to the doctor elicited nothing, just blood tests. another trip

> to the ped, more blood tests, hep a, b, c all ruled out. ped dr sent

> us to a GI, who ordred more tests - ana, asma, ggt, other LFT's etc.

> he also asked for a stomach ultrasound. during this US, he was found

> to be severly constipated - he was given phosphate soda to clear

> this out - it took 1 whole day and during this time he got severely

> dehydrated and ran fevers of 105. he ended up at the hospital where

> his blood tests came back positive for ana and asma and high

> alt/ast/ggt - in the 500 range for these 3.

> gi dr did a liver biopsy - came back with no fibrosis, maybe acute

> cholangitis, some autoimmune, but nothing could be told from biopsy.

> they said he has AIH and started him on prednisone and then also on

> Imuran as well as Actigall. he was in the hospital for 2

> weeks..still running a fever every day.

>

> back at home on jan 1st - on prednisone and imuran along with

> nexxium and actigall. since then he is running a fever pretty much

> every day - starts in the morning when he takes his medications -

> last from 10 am till 1 pm. during this fever which can go up 10

> 103F, he is very tired. there have been 4 days in the last week and

> 4 days this week he hasnt run a fever..but he still gets very tired

> right after he takes his medications.

>

> here are my questions, except for the GGT and sedimentation rate,

> all his liver numbers have come to pretty much normal. he is tired

> only in the morning, he is playing ping pong and tennis in the

> evening. he is tired only after medication. doctors have told us -

> we went a liver specialist at mt sinai and a rheumatologist at CHOP -

> they say they dont know whats wrong - its hard to get a diagnosis.

>

> he also had a CT scan done, MRCP, and colonscopy and endoscopy - all

> of which were totally clear. gi dr did a bunch of biopsies in colon

> and intestines and they were clear.

>

> gi dr thinks it maybe PSC or autoimmune cholangitis LIKE, the liver

> specialist says he doesnt really know, he was suggesting it maybe

> crohn's or IBD - but that was ruled out.

>

> we are really struggling and in the meantime, he is taking these

> heavy duty medications and since he is so tired when he takes the

> meds in the morning he cant go to school right now.

>

> he is 14...

> any ideas from any of you? i would really appreciate it. Our family

> is very stressed out from this.

>

> Rachna

>

>

>

>

>

>

[Guest guest]

,

I went through your website and also read the article you

recommended and found that my nephew doesnt have any of the

symptoms. the only thing he has is fever/fatigue and that looks like

it maybe Imuran induced.

so other than the fatigue that comes with his fever, nothing else

matches this disease or any other that we find.

all his tests are coming up clear except his blood tests for ggt,

esr, and c-reactive protein.

we're stumped here...

Rachna

> Hi Rachna;

>

> Welcome to the group. Sorry that your nephew's health brings you

> here, but glad that you found the group.

>

> Can I recommend that you read the following article on " Autoimmune

> hepatitis: Making sense of all those antibodies " by Bruce A.

Luxon,

> MD, PhD in POSTGRADUATE MEDICINE /VOL 114 / NO 1 / JULY 2003:

>

> http://www.postgradmed.com/issues/2003/07_03/3luxon.htm

>

> >

> Dave

>

> http://home.insightbb.com/~rhodesdavid/

[Guest guest]

Cheryl,

thanks for replying. This is the weird part, Sharad (my nephew) has

none of the symptoms that he " should' have. nothing wrong with gall

bladder. he had an MRCP, not ERCP which is 85-90% as good as ERCP

and that was clear for PSC. he also had a CT scan, colonscopy and

endoscopy..

He did have hgh bilirubin, but everything but GGT, ESR, and c-

reactive protein are normal now.

he is taking 15mg of prednisone, 75 mg of Imuran, and 1200 mg of

Actigall along with Nexxium.

just dunno if any of these medications are helping him though, but

they definitely could be hurting him.

Thanks for listening.

Rachna

> Rachna,

> It sounds like your nephew has had a very hard time of it--as has

everyone who cares about him. It does appear as if the doctors have

been thorough. I didn't see that you mentioned a ERCP (endoscopic

retrograding cholangio pancreatography) I think that is right. That

is the test to determine PSC. Also, what about his gall bladder? PSC

usually has higher GGTP and Alkaline Phosphotase and moderate AST

and ALT. During the early stage of liver diseases, it is more

difficult to diagnose. I'm not sure what meds are making him so

tired. Prednisone usually gives an anxious sort of energy. I've been

on all those meds myself at one time or other. You didn't mention

itching. Many of us itch like crazy. I also presented with edema

before diagnosis. Most of us with uclerative colitis or crohns had/

have diarrhrea. I am no expert by any means. Positive ANA does

suggest an autoimmune disorder, and ASMA is indicative of PBC. I

suppose he had a high bilirubin during his acute attack? I know it

took years for many of us to be diagnosed. I wish I could help more.

Maybe someone else can offer some ideas. Take care,

> Cheryl Berg, Idaho 45

> Married 25 years, 4 children, 2 grchildren

> PSC 01, UC 00, Fibromyalgia, hypothyroid,

> hiatal hernia, ulcer, gall baldder removed '93

> disability (retired English teacher)

> my nephew - potential overlap syndrome

>

>

> Hi all, I am a new member. I joined to get more information on

PSC.

> I'll give a bit of the background - this is about my nephew. Am

> hoping some of you might point us to a direction where we may

get

> some answers.

>

>

>

> gi dr thinks it maybe PSC or autoimmune cholangitis LIKE, the

liver

> specialist says he doesnt really know, he was suggesting it

maybe

> crohn's or IBD - but that was ruled out.

>

> we are really struggling and in the meantime, he is taking these

> heavy duty medications and since he is so tired when he takes

the

> meds in the morning he cant go to school right now.

>

> he is 14...

> any ideas from any of you? i would really appreciate it. Our

family

> is very stressed out from this.

>

> Rachna

>

>

>