First ERC

[Guest guest]

Hi,

I sent more or less the same message on pscmoms but I thought I would

ask also you guys for an opinion. Sorry for the long message.

I have a son who got suddenly very sick with a liver disease over

four years ago at the age of six. It was first called PSC, then AIH

for quite a while, and now PSC again two-three years. He has had

multiple MRIs and last time in November 2006 his disease was found to

be active and to have spread also to the right lobe (it was first in

the left lobe only). His liver enzymes have been more or less ok

since he has been on azathioprine for almost two years but now he has

elevated ALT and ALP (not as bad as they have been but rising). He

was just prescibed metronidatsole for four weeks and lab tests to see

if the numbers come down with that. If not, it is time for

prednisolone again (which I HATE). In the MRIs he has had dilated

bile ducts and an uncommonly large gallbladder. He has not been found

to have strictures in the bile ducts but I am under the impression

that even though the MRI does not show strictures, there can be some.

I took my son to a liver specialist in August (a private one) and he

said an ERC is absolute necessary to confirm the diagnosis, to do

bile duct brushings and possibly to do balloon dilatation of the

strictures (which have not been found but can exist) - I hope I got

the terms correct. So far my son has been treated by public sector

pediaticians some of whom have specialised in gastroenterology but

none of them in hepatology. I much prefer to get advice from a person

really familiar with liver diseases. I do not trust the good opinion

of the pediatricians because we live in a small country with not

many young psc patients. In my view there is no way the pediatricians

can be very experienced in treating psc. However, I do not doubt their

understanding and knowledge when it comes to IBD which is much more

common.

Me and my husband are trying to decide how strongly we go for the

more invasive examinations/treatments such as ERC and a renewed

colonscopy which was recommended due to the great risk of psc

patients developing IBD later on - some have it already before the

psc diagnosis. We would like to have the disease type diagnosed so

that the treatment can be targeted (no extra medications which do not

help in the kind of diesease our son has). So far many medications

have just been tried out and it feels the doctors are experimenting

on our son. It does not feel nice to give him a lot of medications

without knowing if they do any good - especially since there are

often side-effects such as the major weight gain from prednisolone.

What is your experience on the timing of the first ERC (endoscopic

retrograde cholangiography), when did you or your chidren have it,

meaning at what stage of the liver disease (and at what age did the

children have it)? Did you/they have any post-ERC cholangitis,

pancreatitis or other complications? I would value any comments on

the matter. By the way, it just turned out our son has elevated IgG4

which I take to mean his liverdisease is the kind that reacts to

cortocosteroids ( recently sent a posting regarding the

PSC vs SC-AIP which was very interesting for us). Did you/your

children have the IgG4 test taken and what have you been told about

what it means?

With greetings from Finland,

Taru-Mari, mother of Eemeli 10 yrs, psc 2003

OT: Arne, I just visited Trondheim, Norway. There was an

international educators conference there with people from all over

the world. I did not have time to check if Myrabo was close to that

region.

[Guest guest]

Hi Taru-Mari;

You mention multiple MRIs, but not MRCP. MRCP is quite good for

visualizing the bile ducts and might give you some answers about

whether bile duct strictures are present or not without an invasive

ERCP:

Pediatr Radiol. 2002 Jun;32(6):413-7.

Magnetic resonance cholangiopancreatography in primary sclerosing

cholangitis in children.Ferrara C, Valeri G, Salvolini L, Giovagnoni

A.

Department of Radiology, MR Centre F. Angelini, University of Ancona,

Ospedale Torrette, Via Conca, 60020 Torrette, Ancona, Italy.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic

cholestatic syndrome of unknown aetiology. Diagnosis is based on

clinical and laboratory data in conjunction with imaging of the

biliary tree using endoscopic retrograde cholangiopancreatography

(ERCP). OBJECTIVE:: To evaluate the clinical usefulness of MR

cholangiopancreatography (MRCP) in the diagnosis of PSC in children.

MATERIALS AND METHODS: Twenty-one children with clinical and

laboratory suspicion of PSC were enrolled. MRCP was performed using a

superconductive system with a phased-array coil. Rapid acquisition

with relaxation enhancement (RARE) T2-weighted and half-Fourier

single-shot turbo-spin-echo (HASTE) sequences were used. The

distribution and extent of biliary tree changes were evaluated and

classified according to Majoie's classification. A comparison between

MRCP and ERCP was performed blind in all cases to evaluate the

usefulness of MRI. RESULTS: In 13 cases (62%), MRCP showed

abnormalities of the biliary tree which were considered positive for

PSC, while in 8 cases there were no signs of PSC. Both MRCP and ERCP

correctly identified changes in 13 cases and excluded abnormalities

in 5. MRCP had a sensitivity of 81%, specificity of 100%, negative

predictive value of 62%, positive predictive value of 100% and an

accuracy of 85%. CONCLUSIONS: MRCP can be proposed as the preliminary

non-invasive imaging modality for the diagnosis of PSC in children.

PMID: 12029341 [PubMed - indexed for MEDLINE]

For additional references on MRCP please see:

http://www.psc-literature.org/MRCP.htm

Best regards,

Dave

(father of (22); PSC 07/03; UC 08/03)

[Guest guest]

Hi ,

Thank you for the response and links. I checked the name of the imaging

study from my son's papers, and it is actually called MRC, not MRI as I

mistakenly wrote. In the first one (January 2004) they did not use the

dye, in the two following ones (November 2005 and 2006) they used it.

In the last one the changes had progressed from the left to the right

lobe and the PSC was described as 'active'.

So maybe it is still the case that Eemeli does not have strictures. But

still, the liver specialist's view of the need for ERC was totally

different from that of the pediatrician's. I am still happy we saw the

liver specialist and that he recommended taking the IgG4 because it

gives at least some grounds for 'feeding' my son the Prednisolone if it

comes to that again.

Thank you for the comments!

Taru-Mari, mother of Eemeli (10), PSC July 2003