Take a look at ZE Syndrome, Zollinger−Ellison. In
Endocrinology, our discussion of Zollinger−Ellison
will be in greater detail. It’s a gastrinoma. And
this gastrinoma is located in the pancreas perhaps.
It could be at organs as well and part of MEN−1.
It’s a hypersecretion of gastrin from tumors in the
pancreas or perhaps in the wall of the duodenum. It
is a gastrinoma. How is it possible that you have a
gastrinoma outside of the stomach when gastrin
physiologically is coming out of the G cells of the
antrum of the stomach? It’s a cancer, it’s a
malignancy; it can do whatever it darn well pleases.
What are you looking for with ZE Syndrome?
Ulcerations. Ulcerations not only in the stomach.
Then you have, think of this of being like a lava
when a volcanic eruption has taken place. When a
volcano blows out lava, everything that it encounters
in front of it is going to be obliterated. So here’s
my acid that’s being produced in great quantity from
your parietal cell. I don’t care where a gastrin is
being released or where it’s coming from, it’s always
going to work on your parietal cell. That’s where you
form acid, period. So all these acids that’s being
formed in great quantity will come out from the stomach
into the duodenum and kill everything in it’s path.
So you might have issues of gastric ulcers, duodenal
ulcers. Have you studied separately peptic ulcer disease
of the jejunum? No. Peptic ulcer disease either in
the gastric or in the duodenum. But if you, by chance,
look for or your symptom description of an ulcer down
in the jejunum, high on your differential should be
gastrinoma or Zollinger−Ellison. Is that clear?
Now there’s also diarrhea that is involved,
the secretory type, because you’re producing all
these acid. Malabsorption is a huge problem
and refractory to treatment. Diarrhea due to secretion
of all that gastric acid. Because there’s malabsorption,
you can't properly take up your lipids because the,
what are these fingerlike projections in the duodenum?
They are called microvilli. And by Chemistry and Physiology,
you have dealt with quite a bit and we have as well
in hyperlipidemia with vascular pathology with the
proper handling of your lipid. If the Brush border
had been blunted or had been completely destroyed,
then maybe lipid then starts accumulating in your stool,
steatorrhea. Association with MEN−1. Once again with
MEN−1, PAN-pancreas most likely where you would find this.
Parathyroid; PIT–pituirary with a prolactinoma; Part
of MEN−1. 2/3rd of your gastrinomas are malignant tumors.
Majority are. ZE, your serum gastrin levels will be
ridiculously high as will be the acid. That is not normal.
Whenever your acid levels are high, physiologically
it tells my G cells to shutdown. So my gastrin levels
should be low or with that increased levels of acid,
somatostatin will tell my G cells to no longer release
gastrin, I have enough acid. So you do have physiologic
feedback normally. However, in ZE, please understand
that that high levels of acid will not shut down the
gastrin because the gastrin, this is a malignant tumor.
An important integration between physiology and pathology
is the next statement. Secretin. Acids coming out,
physiologically, from the stomach and into the duodenum.
Acid into the duodenum. That pH in the acid
or in the stomach is maybe at a pH of 2. Coming in
to the duodenum, that’s dramatic. Yeah,
and it is absolutely. It’s lava coming in to duodenum.
So therefore the duodenum is now incredibly
sensitive to this acid. It wants to do everything
in its power to neutralize this acid as quickly
as possible. What is the neutralizer for this acid
in the duodenum? Bicarb. Where is the bicarb coming from?
Pancreas. So give me the hormone that’s responsible
for stimulating the pancreas to secret the bicarb
That’s your secretin, right? That’s your normal physio.
Let’s take a look at what happens here in ZE
and how you will utilize a physiologic Secretin test
to help you diagnose ZE. The Secretin Stimulation Test
needed for gastrin levels less than 1000. Positive
test shows lack of gastrin suppression by administration
of secretin. In other words, the secretin stimulation
they're needed to gastrin levels less than 1000.
So want this so that you can decrease the gastrin.
If the secretin comes out and the gastrin levels
are still high, this is going to then help you diagnose
ZE. Elevated basal output level greater than
10 milliequivalents per hour. That is how much acid
that is being produced and that's quite a bit.
So you have three major physiologic issues that have
now gone awry that you will be using to your advantage
with the gastrin, secretin and actual basal output.
Further, you want to localize the tumor by using
octreotide scan. You’ll do what’s known as a
ultrasound so that you can properly localize your
tumor. A CT scan, angiography and surgical exploration,
well, all of them help you properly identify your tumor
so that you can then excise it. Remember, you can
manage it by using certain drugs. However, in order
for you to cure your patient you have to resect this tumor.
What you’re seeing here is the fact that you are
destroying everything in the path of your intestine.
So as acids coming in, as you see the cartoon here,
you’ll notice the duodenum with scattered, scattered,
scattered ulcers, completely refractory to any
type of treatment. H2 blocker or your PPIs.
All should indicate that your patient maybe is suffering
from ZE. I've gave you some diagnostic tools
including the fact that hypergastrinemia is present,
the secretin test is not effective or shows that the
gastrin levels are still high and number three, the
basal output. Management. High dose of PPI.
Surgical resection, if no metastasis or not associated
with MEN−1. Parietal cell vagotomy for gastric hypersecretion.
Chemotherapy for metastatic disease. It’s important
that you pay attention to the normal feedback mechanism
because the angles in which they could possibly ask
you, experimentally, for ZE is every possibilty
because this could be a wonderful pathophysio question
in which if you know that there’s too much acid
coming into parietal cell then you should be thinking
about all the different receptors that you have on there
including gastrin receptors for secretion, your H2
receptor for secretion, your PP on the luminal side.
And the fact that you have an M3 receptor, referring
to my vagal tone, right. Or in other words, your vagus