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00:00:01
i've stuff i think is that ah yeah five i think it's a pleasure for
00:00:04
me to be here today and actually to talk about one of our
00:00:08
favourite diseases at our whole but i can convince you that this is really
00:00:13
a disease well we have to watch the patients very very closely
00:00:17
so up to now really the cults that are not for the likenesses of primaries carousing
00:00:23
calling judges is imaging studies and yeah i brought you to well yeah see picture
00:00:30
with mountain well it's the noses that also creates an article stations
00:00:34
and this is really the classical picture you see in advance p. s. c.
00:00:40
so why is it actually worthwhile really talking about yes
00:00:44
yeah i mean it's a really really rare disease
00:00:47
and we think that that probably less than two
00:00:50
but ten thousand patients oh those things affected
00:00:55
it's a disease that is increasing in prevalence or incidence
00:01:00
this is about that somewhat older data shown here
00:01:06
increased and i am female patients on population based
00:01:10
study but we also have access to
00:01:14
um john and health care insurance data is different
00:01:20
i also see that actually all three option you later diseases p. b.
00:01:25
c. p. s. c. and altering in hepatitis however rice in
00:01:30
german um health insurance databases so we see that this
00:01:34
is really an increasing problem within the complete population
00:01:42
so what really is primaries carousing columns ideas well if
00:01:47
maybe i should say we don't why no
00:01:50
it is a chronic slow roasting inflammatory disease affecting all the file that's
00:01:56
sometimes the very large file that's the medium size follow that
00:02:00
and then the special entity which i will come too late
00:02:03
to on also the very smallest file that stuff males
00:02:07
other than all other option you diseases affected somewhat more often n. t. s. c.
00:02:14
so this makes yes he really different from the vast majority of other autoimmune diseases
00:02:20
and the median age diagnosis is in the thirty fourth decade of life
00:02:25
but we do see sometimes very young children maybe then more presenting as all to
00:02:31
immune cullen giant is and sometimes even really older patients also for that
00:02:37
the me entranced non free survival for population based um study is
00:02:43
about eighteen years but may vary dramatically between different individuals
00:02:49
and so far we do not have any approved medical treatment conservative treatment
00:02:57
approximately seventy percent of all p. s. patients far i. b.
00:03:02
t. inflammatory bowel disease in addition of and an
00:03:07
mousse patients presenting with five fifteen may have only few sentence from i. b. d.
00:03:12
often if it's the case that they haven't also to fly to this
00:03:17
um and often they have rectal sparing which also
00:03:20
explains the rather mild symptoms and the station
00:03:24
please feel free to interrupt me if you have any questions um well so um
00:03:32
um
00:03:34
so how do you actually diagnose this disease well first you need to have the suspension and and then do you really
00:03:40
would have the suspicion is that young patients presenting with for
00:03:44
right yes these are young patients presenting with jaundice
00:03:48
or just with elevated term let that enzymes
00:03:52
often close tactic liver enzymes and um
00:03:56
some months ago we had a symposium for all patients in hamburg and
00:04:01
puzzle was the off into the audience four hundred patients sitting there
00:04:06
in whom was your disease diagnosis but not donation and i
00:04:10
would say about one third of the patients showed out
00:04:13
so early disease maybe just diagnosed by chance that just got sampling
00:04:19
in addition to studies as i said in the beginning out the major diagnostic feature for p. s. c.
00:04:25
histology may help in all patients in whom the situation is
00:04:30
unclear and you do not have this very typical pattern
00:04:34
what you see on histology well what you see very exactly yes the files are
00:04:39
and you see this concentric i'm in like slow motion pattern and he's not actually an effect
00:04:46
sorry oh it varies cost information up but if you which certainly will tell us later
00:04:52
but if there's one from actually also to see overlaps syndromes but this is really the very
00:04:57
typical picture for p. s. c. but i want to caution you that awesome p. b.
00:05:03
c. patients who also may show test pattern in
00:05:08
that the histology so she'd be easy crime
00:05:11
rate bill yuri college i just has to be excluded prior to making the diagnosis
00:05:17
although i'm a very big fan often alter antibodies actually in
00:05:22
ah p. s. see if they don't really help
00:05:26
some patients may hot tea i'm close but they're not diagnostic some patients may this lame
00:05:32
billy every at least epithelial cell antibodies but even these are not really diagnostics so
00:05:39
um well to antibodies and to quality doesn't really help in this case
00:05:45
small that primaries liberal think all enjoyed yes
00:05:49
we don't know if this is a special entity well if this
00:05:54
is just a very early for what he is c.
00:05:58
right now it may be the case that this is a special entity because they also differ
00:06:04
regarding the age of eight patterns so we think that this is actually separate disease
00:06:10
it's even more for yeah then recollection is c. and only the small
00:06:15
bile ducts affected so you won't see any changes on imaging studies
00:06:20
no changes and and ah m. r. i. no changes them yes
00:06:22
see so histology is the scenic found one of these patients
00:06:27
and this disease has probably very favourite i'll calm compared to regular large talk p. s. c.
00:06:37
now
00:06:39
how um how to
00:06:42
um diagnose i just want to briefly summarise and we
00:06:47
do nowadays initially in m. r. i. e.
00:06:51
has the sensitivity of about eighty five percent of the specificity about
00:06:55
and ninety five percent accuracy is only performed if there's any doubt
00:07:01
about the diagnosis so if you still haven't unclear picture
00:07:05
despite having performed all the other tests all if you have dominance that knows this
00:07:10
and want to fill a take this no service if you suspect malignancy
00:07:15
and if you have a patient with an acute column titus and you need to
00:07:20
have of microbiology micro by your biological work out of the bile through it
00:07:26
and of course as i said if you have consistent suspicion of p. u. c.
00:07:33
talking about the prognosis about p. c. s. that in the beginning
00:07:36
that um it has a rather corporate moses given the fact
00:07:41
that um we have young patients and there's a median
00:07:44
transplant three survival of only about eighteen years
00:07:48
this is quite different if you look at the population based school board or the patients that
00:07:53
actually has seen it centres su wasn't oh us so we probably have just the
00:07:59
was cases really that we see and we see transplant free survival rates of more less thirteen years
00:08:05
or so so it's a great disease and it's important that these pay patients also get
00:08:10
as socially um social advise them concerning um that jolts
00:08:15
that family planning and so on and so forth
00:08:20
complications of p. s. c. and i would like to spend some
00:08:25
while on this topic because i think it's of major importance
00:08:29
first of all up obviously it's the disease than that we can only react in a very um
00:08:37
single weight on damage it develops starring so roses in
00:08:43
t. i. c. patients see roses often leads to
00:08:48
very significant portal hypertension we re only see that patients
00:08:54
decreased liver function tests or only mildly decrease love affection test
00:08:59
so let the synthesis is still good but then i have very bad is of local terraces
00:09:06
and also i'm pleading events so this is something you really have to watch for this patients
00:09:11
and also rather good hint and this disease is that the screen size
00:09:17
increases and we know now from our patients from a colleague has looked at
00:09:22
the data that the delta increase in spleen size actually will tell you
00:09:29
if this is the patient actress to develop complications on on so it's a good idea to perform
00:09:35
an ultrasound and actually measure the spleen size and also keep that track because those patients
00:09:42
increases in size more than two centimetres the yeah they have a proper
00:09:47
noses so watch that is of course if sort of good
00:09:55
well we in hamburg don't be even monstrosity portal hypertension
00:10:00
and the so um i'll i'll idea is that
00:10:04
these diseases t. v. c. and actually p.
00:10:07
s. c. also affect the live uh and very heterogeneous way and yes you have of
00:10:13
large sampling error off and if in your sample in your i'm thinking a sample of
00:10:18
you have a um as to fibrosis this shit just may not really be representative
00:10:24
we performed across the t. on almost i would
00:10:29
yes ninety percent of a patient to perform liver biopsy and we also
00:10:34
we often see large discrepancies from the uh microscopic oh picture and
00:10:40
then drop all the just tells us so i would we think
00:10:43
this is all sample size um uh all sample the problem
00:10:51
does that answer your question
00:10:57
we've column titus college i just if it will increase the speed of
00:11:03
the calls of p. s. c. so it's important that you really
00:11:07
check for college i despair early on so we
00:11:10
actually perform the aussie open just for bile
00:11:15
a sampling and michael bay o. m. we'll we'll work up because we see strange but
00:11:21
unless it one battery would and often we also see fungus growing yeah so it's important that you
00:11:27
have a really good sampling that you can target the bucks that actually grown the buyer
00:11:33
and because college i just bacterial or fungal contractors will
00:11:37
just speed on the process of p. s. c.
00:11:42
don't instructions and this is a very difficult topic if you're see pictures and i think we
00:11:48
all agree this is the dominant structure here right and this priest an object alliteration
00:11:54
and um we know that really dominant structures can only be defined by
00:12:00
yeah see my colleague one to multi did a rather sobering
00:12:05
study the other which i think it was last year
00:12:09
and um she asked um experts in p.
00:12:13
s. c. high volume centre experts
00:12:17
to basically he gave them case vignettes and show them some an r.
00:12:22
s. and also the the um cut them arises not i
00:12:27
use that for uh also i'm moving but also um slide and
00:12:32
basically um these experts around the wells well i asked
00:12:38
is that um the dominance the noses and do you think there's malignancy and these were
00:12:44
the two questions are all these experts around the world and use the sobering presided
00:12:53
seven judge adjust the sixteen vignettes
00:12:58
um have at least a an agreement of more than fifty percent
00:13:04
so this is really and our eyes no good to tightness dominance noses
00:13:10
and i think we just should be very much and we have that and you see yeah the different
00:13:15
places and you see that there's just um intrepid accept
00:13:18
hectic and basically it was completely different for
00:13:23
posting scoring this
00:13:26
okay so we need yeah see if we really be heaped that they s. of
00:13:30
dominance notices me needlessly and then we also need to perform brash september actually
00:13:37
and money no matter this is something that actually as a physician really focused need probably the most
00:13:45
cullen your casino mouth will occur in p. s. t. patient are up
00:13:50
to ten percent of p. s. c. patients within their lives than
00:13:55
and actually one third of these climb your casino mass will not come up with an thirsty of diagnosis
00:14:02
you can use of of course is is always mentioned we've got them through the
00:14:08
phone or don't don't move you started already been nice of him oh
00:14:16
uh_huh rules that we've been through
00:14:20
yeah uh_huh
00:14:22
yes sir that's not true there's room for sure in real time
00:14:31
um in fact i have some more curves for you to show i i don't
00:14:35
i don't know what i mean it really doesn't matter um because i think
00:14:39
we need to watch these patients just within the thursday very
00:14:43
very closely anyways if we can believe this or not
00:14:48
it's a disease awful right
00:14:54
through the price of course and of course the notre israel it's it's agreement in oh
00:15:02
when is your vision through
00:15:06
okay you're right if if
00:15:10
get rerun information you know
00:15:14
actually you could also look at it in another way and actually believe that
00:15:20
maybe they have happy you see for one or two wires and cullen your casino my actually makes
00:15:25
that they get symptomatic and ten yeah i agree
00:15:29
um and colorectal cancer second problem and
00:15:35
patients who have i. p. t. in addition to p. s. c. have significantly elevated trust
00:15:44
so if he is one of the coolest um
00:15:49
so that's as the cumulative incidence of patients
00:15:53
here three patients with happy to i'm delirious malignancy and you see that your patients
00:15:59
have a low risk that actually all the patients and that the ration of disease increases the risk
00:16:06
that does not mean unfortunately that young patients may not develop such
00:16:13
a cost no more and interesting he if we i personally have never seen and happen
00:16:20
to sell a casino money p. s. c. patient of then made beef soul cases
00:16:27
vast majority of chances have party kansas auckland you haas uh_huh in p. c. patients
00:16:35
yep
00:16:37
and this is one patient i brought to from hamburg and he it's of true case but
00:16:42
we've seen i think two years ago and it's a thirty year old male patients
00:16:47
p. c. for a couple of years that really wasn't very affected by the disease
00:16:52
and yeah this ultrasound study joel this too small
00:16:56
polyps flat not larger quality was five millimetres
00:17:00
and we said mm doesn't look so good and let's see them three months later
00:17:07
and this is what happened three months and at that point we had and they said gall bladder cancer off and
00:17:14
turn if he's still alive today and this well um
00:17:19
but this is just a reminder any pollard scene
00:17:23
other then polyps and you and me any polyps seemed and
00:17:28
p. s. c. patients should caution you and really should
00:17:32
maybe half the patients cotton concept myself i have to admit we
00:17:37
have colours suspected maestro have patients close affect the mice
00:17:41
and we didn't find any polyps in that respect take then i think that's the price you pay for but we don't
00:17:48
want to see this as i can um for second place
00:17:52
i brought you home of um is this picture
00:17:56
and well we thought uh_huh doesn't look so nice here um and um there's
00:18:03
a brash scientology of and actually turned out we had in a casino
00:18:10
and the next case
00:18:13
okay that he probably visible so this last spring last year and well
00:18:22
oh radiologist told us mood maybe some segment lump 'em colours faces
00:18:28
segment five six that and then yeah uh_huh maybe not
00:18:32
anyways where i'm certain and actually we perform c. s. ca ninety nine
00:18:36
of them let's sampling on our patients on a regular basis
00:18:40
and we saw an increase in ca ninety nine so we actually
00:18:44
had the next m. r. i. only six months later
00:18:48
and um i think now it's obvious that there's something wrong
00:18:53
you got 'em atypical the reflection and actually was already
00:18:57
nobody met as the size and you received an additional chemotherapy
00:19:02
on that um so just to remind you um
00:19:07
these pictures will not all be everything
00:19:14
and um
00:19:17
the last thing on line casino mark again you see
00:19:22
if if you just knows us again on yesterday
00:19:27
and this is the colour and it's got a good chance just
00:19:30
these battles that low a typical and also it's humorous aspect
00:19:34
and we were actually able to take a biopsy there
00:19:37
and i'm determined ah longer cinnamon disputes you should
00:19:47
complications and that's not a curve that looks different again um
00:19:52
but i have to know maurice compared to the normal population is
00:19:56
about increased risk of four hundred fold than the incidents well
00:20:03
depending on mapping last may each screen zero point five and one cent
00:20:10
oh no actually it's a it's a very very uh entity in the normal party
00:20:15
and usually only occurs if you have um pause line called
00:20:18
out or something we do see quite frequently see patients
00:20:25
and
00:20:27
and now
00:20:29
and
00:20:31
if the patient asks display china in his bile ducts
00:20:37
in respect right wall and even if you hackman your cost on the likelihood
00:20:44
that you find display jess elsewhere and that that is extremely high so patients with
00:20:50
heart couldn't you call c. no more need to be monitored explain closely um
00:20:57
is the actually and because we know that the risk for
00:21:02
not only recurrence but also occurrence it's different sites like
00:21:08
i'm actually not sure about um at one point there was this study where
00:21:13
these patients were actually able to receive a liver transplant but this
00:21:18
i'll send a close one so we really have to offer for these patients right now so
00:21:30
oh yes
00:21:33
germany
00:21:37
i thought gee you're
00:21:41
i know but i'm real or or or or or you know there yes
00:21:52
we can do we can you know and we we we are it no we we we are not allowed to
00:22:00
do it but you know we we did it we had is that the the cost which went rather well
00:22:06
patients went rather well and that's that's that it that this that these canoes now and
00:22:11
we don't get any organs that and she so in germany it's just not possible
00:22:17
okay coming to holler rectal cancer
00:22:21
i just remind you once more reckless staring off quite is it off and
00:22:29
the cost well the most mature uh activity of cool light is
00:22:33
in the right cool down and also ah watch it is
00:22:38
and then have some gasp control matches performs the weirdest the p. on all patients
00:22:44
and and say okay i've excluded colon cancer to seventy percent
00:22:48
no you haven't because in p. s. c. patients
00:22:51
colour like i'll call calamity found so often and
00:22:55
more often than the normal population actually because
00:22:59
and the right call on so it's really important that you really the phone call and ask the p.
00:23:04
of the right column and that the patients really thinking well prepared and also to sampling
00:23:10
and the guidelines because and you will find this page and these patients
00:23:18
in the beginning i said seventy percent of p. i. c. patients have i.
00:23:22
p. now look at it the other way around you have id patients
00:23:29
two percent will have clinical p. s. c. but a two cent
00:23:35
may have stopped the makeup yes see elevated liver enzymes
00:23:40
sharing the same risk as patients with active or
00:23:44
clinically offered p. s. c. so any elevated
00:23:49
colour statically that ends i'm sure the note during the hike patients to actually um
00:23:55
um money twelve for the presence of t. s. c. edition
00:24:03
what is the best for colorectal cancer and these patients affected
00:24:07
by p. c. and i. v. d. actually greatly elevated
00:24:11
the light rail line other patients suffering from i. b. t. only
00:24:16
and this dog and shows the patients i'm suffering from both p. s. t. n. i. d.
00:24:23
i mean the human to risk is almost twenty cents so it's significant
00:24:29
and in hamburg we to perform column column mostly p. with um he's
00:24:34
the logical sampling yearly on our patients affected by both diseases
00:24:42
the vast majority of p. c. patients to would have assertive collide to is only
00:24:46
a small proportion to have one school lightest but these patients that's cool ones
00:24:52
of significantly worse so five will then the other
00:24:56
patients so interestingly not only concerning yeah
00:25:02
uh and cool but also concerning liver disease even little
00:25:07
disease tends to be worse in these patients
00:25:10
so patients who have p. c. m. clones client is watched him closely
00:25:20
surveillance well
00:25:23
we do as as that formed yearly colour lost the p. in these patients
00:25:27
and patients will have no clinical symptoms of identity
00:25:32
but have p. s. c. should receive columnist the p. we do it at the time point
00:25:38
of tight noses and mostly we do it five years later once more just to
00:25:43
exclude but the late onset identity will uh we
00:25:48
fall ultrasound on operations every six months
00:25:52
and then our i actually want to yeah and um we monitor for other
00:25:58
diseases as well we have a high risk in these patients that they
00:26:02
develop osteoporosis early on so we to send them to our own specialists
00:26:07
and um well they perform their follow up at the um um
00:26:13
um miss schedule but and this is something to think about and
00:26:17
also many patients develop vitamin d. deficiency at least and uh
00:26:23
try so very very early on sophie we do monitor for that action
00:26:30
um so what about this i mean this huge variance apparatus cost
00:26:36
enormous amounts of money is it really worth doing it
00:26:41
and we think it is it's worse for our patients but it's also worth it
00:26:46
if you look at the outcome data and this is actually the cases
00:26:50
the and proportion of patients so fighting happen to be very malignancy
00:26:55
and these are the patients that actually have been in the surveys program
00:27:00
and these other patients that have not been in the surveillance program
00:27:04
it's an easy yes but obviously it it's a good idea to
00:27:08
civilians and you just pick up the to miss early on
00:27:12
um well at mariah is done this is something that 'cause of sham has looked in very very
00:27:18
closely and this is a huge project and what's actually out that that null states than that
00:27:25
things that the radiologists should before we do all the all pictures with contrast them with conference
00:27:33
but if you do it without contrast issue to the t. h.
00:27:36
t. one way to entity to break it down and slices
00:27:41
to look for calling choices and also to look want malignancy
00:27:48
and this is really hot looks like time this is the reconstructed m. r. i.
00:27:53
and you see this product has sort of a good and contrast and aren't
00:27:58
real seats here and the t. two way faith and also
00:28:04
here this this also command you will see no
00:28:10
so we've seen now m. r. i.'s was doing it now we look at
00:28:14
the colour rectal cancer that and even here it's worthwhile doing survey and
00:28:20
patients are but significantly small uh c. s. u. related
00:28:25
mortality than those who do not undergo survey
00:28:28
and so again we pick up the tombs early and are able to treat them rather well
00:28:36
now i spent you with the anti slide empty
00:28:39
hands uh_huh what about treatment for our patients
00:28:44
difficult topic
00:28:46
well the only really
00:28:49
approved treatment for p. c. is the transplantation and these are
00:28:54
high but it's not i'm looking at our own
00:28:57
cool water and looking at patients with a likeness of roses and p. c. s. lest ye see patients
00:29:04
and i think i can convince you that p. s. c. n. p. v. c. patients just had better long term outcome
00:29:11
which is somewhat surprising because the c. t. s. c. relapse thing
00:29:18
and why did he find proportion of patients after the transplantation
00:29:23
but obviously this is a condition that is treatable and working
00:29:30
anyways
00:29:32
there are you know
00:29:36
uh_huh
00:29:39
maybe
00:29:42
um i'm sorry at this in german and i'm sorry i just like the wording of this so much
00:29:48
so i copied the original um text and bought the majority of you may understand it anyways
00:29:56
in hamburg we get all our patients on ninety nine percent of
00:30:00
patients those results call i guess it really that's normal
00:30:04
no proof that it helps and this is an extraction from
00:30:08
the um german guideline forty new diseases than that well
00:30:13
but also needs to decrease and colour statically that enzymes in those
00:30:18
it all thirteen to twenty three milligrams per kilogram body weight
00:30:22
can be
00:30:24
given to operations um well and we we don't know what to do we very sure know
00:30:32
all that we should not give high doses of rousseau because this is called doctor
00:30:38
and and the study whether this was examined and they get more than twenty eight milligrams per kilogram
00:30:44
we usually give those this from thirteen to fifteen
00:30:48
milligrams that we don't go option twenty should
00:30:53
what is it is the really tune out patients o'clock from lowering
00:30:58
the fans arms i think that's a really it tells us
00:31:03
who has the corporate noses that doesn't
00:31:07
and we see this is the the and from two thousand thirteen which shows that very nicely
00:31:13
they gave patients either so oh placebo
00:31:18
some patients responded with a live and size to assault or
00:31:22
placebo and these actually the patients have a good outcome
00:31:26
we are the ones who did not show em enamel
00:31:30
your ration of liver enzymes campus so all placebo
00:31:34
that paul from notices and really it was that tells you if you have good prognosis on
00:31:41
we still like you to give a troll page
00:31:46
so what do we have next we have a new substance on it in development now
00:31:53
paul no i was oh we're being prescribed felicity and anti
00:31:58
productive and if that project and anti inflammatory and um
00:32:03
and a a and b.
00:32:10
great thank thank you properties and um it's actually rather well
00:32:14
tolerated and patients so this substance does not lead
00:32:18
to for right as as we've seen them that from a number of other um file s. it
00:32:23
and really has very mild celtic side effect only and here's the data
00:32:28
from the phase two study showing that there's actually those depended effect
00:32:33
showing a significant decrease in a few levels
00:32:38
and this that is underway now we also is the logical changes will be
00:32:45
hopefully through and we don't know yet so we have very curious to see
00:32:49
what will happen with this substance and we think this is really
00:32:53
hopefully going to ram give us some new treatment option for this difficult disease
00:33:01
this is that the that was presented at the little congress last yeah most of
00:33:04
you may know it and it's a very ambitious french study and so on
00:33:10
here was the idea to compare spent insertion
00:33:15
versus balloon that haitian of dominance the noses in p. s. c.
00:33:21
and the red definitions for yeah both for the volume that station
00:33:24
and also for the standard insertion was very very similar
00:33:29
that
00:33:32
the shortens panting and i think it was designed that they had this than
00:33:36
two weeks and then this then was removed by augustus the p.
00:33:41
but you see that the side effects us some different so we are
00:33:46
are almost half of the patients who received to stand out
00:33:51
i uh complication um current charges that packer tied to us or k.
00:33:57
and only very few independent adaptation and i think this is a very very strong document not
00:34:02
inserts then some patients the p. s. c. unless there's no other way to do so
00:34:11
and then
00:34:12
one of my last slide um and is he asked yet
00:34:17
all safe in patients and can be good form it
00:34:20
the same project patients is it may be unsafe and several to patients this is
00:34:25
something that most chrysler had looked at um recently and it was funny
00:34:31
really patience with supposes that fewer complications really because they have who are um
00:34:38
and packet hiatus and after i am u. s. c.
00:34:42
so yes even supported patients it may be
00:34:45
worthwhile if you have dominance noses to try and to the tate it um to um
00:34:53
and then you read the calls
00:34:56
yeah summarising the spot was sold well i don't know i like to stop sons
00:35:01
and stop the treatment of dominant well that's analysis of calls in
00:35:06
time and very very consequently and of columns ideas these patients
00:35:10
on not find with five days off active i don't take
00:35:14
any prolonged courses of antibiotics watchful fungus in these patients
00:35:19
in use oppression and it'll if you and i actually i i i'm i
00:35:25
i i i i i i thought and i mean i i could refer to you but now you have to refer
00:35:31
to be so immune suppression of course indicate you have an
00:35:36
overlap syndrome where information off the dialogue acts actually
00:35:41
uh maps over to the to the hottest side and of course treatment of additional disease osteoporosis
00:35:48
and i definitely think we should try to include out page to any kind of investigation
00:35:53
or study because otherwise this disease just will not we will not move forward
00:36:00
uh_huh
00:36:03
i hope i could convince you that cause that to me maybe you
00:36:07
good idea if you see even small polyps in the gallbladder
00:36:12
browse scientology is a good idea if you have a good site colleges
00:36:17
that actually can tell you whether these really are atypical samples
00:36:21
um so variance is a good idea
00:36:25
yeah and now differential diagnosis only part reviewed very modern deceased
00:36:31
um i did before related to and common choices
00:36:34
and ah i. g. four really helpless and the tightness well maybe in some patients but really gotten
00:36:41
all so if in doubt take a biopsy osgood apologised to perform an i. g. for staining
00:36:47
and tell you how many i. g. for positive sell c. actually
00:36:51
sees that i call flow and to get the diagnosis
00:36:56
of course malignancy he's can also men make primaries rousing
00:37:01
column jot is so you we have that
00:37:05
and who's actually
00:37:08
uh_huh the typical oh my g. four related problem child to
00:37:12
station well it's the blue collar work uh it's usually
00:37:15
older male patient without inflammatory pile these these people have
00:37:20
an excellent responses to read it usually be completely
00:37:25
um it without any sentence after weakest there but it may also have
00:37:29
other i. g. four related diseases as option you hang for titles
00:37:34
and this is the case i saw i think last year and i just want to briefly
00:37:40
um talk about this also we believe that this diagnosis is much more
00:37:46
all fun and much more frequent in our patients than actually
00:37:50
and we believe this is an eighteen year old go i'm actually
00:37:56
not this dominance the noses here as you can see
00:37:59
and um we thought it would be meeting and we thought this would be like that's the
00:38:05
noses and we actually wanted to hop so i'm i'm search search your surgically and explore
00:38:13
she didn't want to search we said okay we perform um guided
00:38:18
needed biopsy actually got the like um with this um
00:38:24
very dense or for the right or the or close got that we're very lucky that we didn't
00:38:31
get it and what we saw with these very inflamed bile ducts or what have you
00:38:38
can you see all these very is is initially excels within so this is
00:38:43
what you see in isn't isn't it feel like all enjoy it is
00:38:46
nipples small teams that easy finding isn't if it they got that information up to two percent of the patients
00:38:52
and we do believe that this is really quite frequent um clinical picture
00:38:58
okay coming to the end in one to consider g. s. c. elevated and slightly the enzymes in
00:39:04
i. d. d. patients or elevated liver enzymes jaundiced
00:39:08
top right isn't young patients especially young adult
00:39:12
well that's a nose is actually very large told that uh it's a very diagnostics line
00:39:18
of slap you see these patients very frequently have very large and dysfunctional call that
00:39:26
and uh and it's a rare disease affecting the bile ducts ah you need imaging
00:39:32
studies to diagnose the disease and actively look five feet in addition um
00:39:39
as the latencies are major complication the calls up yes yes it rains shown
00:39:44
to improve life expectancy it's probably a good idea to to show
00:39:49
and lost the only approved treatment right now at least
00:39:53
in german means that transplantation we like those so
00:39:57
um we also like to form and stopping the notations in operations
00:40:03
and tend to treat calling charges and need more investigational studies for patients
00:40:10
and with this i will read from harm buck puzzles of
00:40:14
message on and this is basically the team and caring about the patients and the option you operation give it to pop
00:40:21
happy to take your questions thank you very much
00:40:31
i i i i
00:40:35
or
00:40:36
yeah
00:40:39
first
00:40:44
ah you know
00:40:53
yeah
00:40:55
we don't perform so we perform yes you really only if we see it on
00:41:00
the noses of we have the feeling that very high up on the latency there like they can walls also
00:41:07
we do not perform brash scientology on protocol place it's
00:41:11
a thing that's something we don't give uh_huh
00:41:16
yeah
00:41:18
uh_huh
00:41:22
yes
00:41:25
uh_huh
00:41:27
are you
00:41:31
so i actually i don't know all the different from sponsors the mike that's right
00:41:38
and no i'm in germany um so with the uh melts go up that
00:41:44
we need for these things really very bad for our p. c. patients
00:41:49
so we have nonstandard exception help lines and being um
00:41:54
dominance in houses um college items and and so on and so
00:41:58
forth and we usually list of patients when they become
00:42:03
physically you know which usually takes quite a while we do not we empty the stealth mission
00:42:14
is your sculpture
00:42:17
yeah oh i mean i was also yeah yes both
00:42:22
uh and also in my clinic or a feeling
00:42:27
he is the patients are very willing to work and very much within live
00:42:34
and if you see patient comes to me and says oh i cannot go well if you're bad
00:42:39
i think then that's really the point when to this and going to think about what we talk
00:42:45
operations early on and say that it may be a possibility but it's necessary at one point
00:42:52
and but um when they develop will group in that doesn't go away after visitation and
00:42:59
second pin yeah and call enjoyed it's really these are major things well
00:43:08
three or so
00:43:13
sorry red rolls of uh_huh oh i would say uh in about half of the patients one
00:43:20
i mean became older
00:43:24
and then sort with the question is it scheming type billion illusion so it's
00:43:30
the t. c. i mean it's probably the same disease displays patients right
00:43:38
you go
00:43:41
description row
00:43:45
you are
00:43:52
if you would cost me personally i would answer yes to um but
00:43:59
because actually we have seen quite a number of comical scene no
00:44:03
mouse also of to be problem spot and suppression that just
00:44:08
one gotten on the the sedans um
00:44:11
would probably be wise aged but this sniper cousin you there that day
00:44:21
to the system
00:44:25
and and
00:44:27
you are
00:44:30
i use a lot of work
00:44:35
it should but
00:44:39
oh look good
00:44:42
and on it goes mm it's or it's for a good read
00:44:54
we we don't we don't vomit on a regular basis either but
00:44:59
mm
00:45:02
we're
00:45:07
uh_huh
00:45:09
there is zero nine or if there's really some years

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Conference program

PSC: from diagnosis to treatment
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Overlap syndromes: cholestatic liver diseases and auto-immune hepatitis
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