11. GI Pathoma pt 2 (exocrine, gallbladder, liver)

Question 1

Annular Pancreas

what does this present as?

Answer 1

when the ventral pancreatic bud strangles the 2nd part of the duodenum.

-nonbilious vomiting

Question 2

Acute Pancreatitis

which enzyme gets activated

two most common causes?

how do you diagnose this?

clinical presentatoin

Answer 2

Autodigestion of pancreas by pancreatic enzymes

• -trypsin*
• -gallstone and ethanol*
• -*incr. serum amylase + lipase
• epigastric pain that radiates to the back

Question 3

Chronic Pancreatitis

major cause?

major clinical finding?

Answer 3

caused by multiple bouts of acute pancreatits –> fibrosis

-alcohol abuse

pancreatic insufficiency / calcification of pancreas

Question 4

Pancreatic Adenocarcinoma

clinical presentation if in head of pancreas?

whats a serum tumor marker

tx?

Answer 4

Adenocarcinoma arising from pancreatic ducts

-Obstructive jaundice w/ weigh loss & palpable gall bladder

-CA 19-9

-Whipple procedure:removal of head of pancreas, duodenum, and gall bladder

Question 5

what causes the formation of gall stones?

Answer 5

Increase bilirubin/ increase cholesterol

(or decreased Bile Salts) (or gall bladder stasis)

Question 6

Cholesterol gall stone

Answer 6

Radiolucent

associated w/ obesity, Crohns dz, old age, Native american.

Question 7

Pigment/ billirubin stone

risk factors?

Answer 7

Black stones: Radiopaque

RF: extravascular hemolysis

Question 8

Where does gall bladder pain radiate to?

Answer 8

The right scapula

Question 9

Porcelain gallbladder

tx?

Answer 9

Calcified gallbladder due to chronic cholecystitis.

take out dat GB

Question 10

Walk through bilirubin metabolism starting tat Hb

Answer 10

RBCs gets destroyed by macrophages in the splee.

Hb gets broken down into Heme and globin.

Globin gets broken down to amino acids

Heme gets broken down to Iron and Protoporphyrin

Proto gets transformed into uncojugated billi. this gets transferd to liver to become conjugated to be stored in the GB.

Once GB releases conjugated into intestines- conjugated gets turned into Urobillinogen (makes stool brown)

Question 11

Which enzyme in the liver conjugates billirubin

Answer 11

UGT: udp-glucuronosyltransferase

Question 12

Why do babies get jaundice?

what bad thing can this lead to?

Tx? how does it work

Answer 12

Their livers arent fully developed so they dont have enough UGT to keep up with unconjugated billirubin

-Kernicterus (billirubin deposition in brain-basal ganglia)

-Phototherapy: makes the uncojugated billirubin water soluble

Question 13

GIlbert’s Syndrome

What does it lead to?

Answer 13

they have mildly low UGT (UDP-glucuronosyltransferase) activity

-increase in Uncojugated BIllirubin

Question 14

Crigler-Najjar syndrome

type 1 vs type 2

Answer 14

Absence of UGT(udp-glucuronosyltransferase)

• very high levels of uncojugated billi. (can lead to kernicterus)
• usually fatal

1 is fatal/ 2 is less severe and responds to phenobarbital (increases liver enzyme synthesis)

Question 15

Dubin-Johnson syndrome

how does it present?

Rotor Syndrome

Answer 15

Conjugated Hyperbillirubinemia due to defective excretion by the liver.

-Black liver

__________

Same as Duby but no liver discoloration

Question 16

Cirrhosis

Answer 16

Bridging fibrosis (via stellate cells) and regenerative nodules

-leads to portal hypertension

Question 17

Portal hypertension

Answer 17

Incr. in portal venous pressure

Question 18

Fatty liver (alc. liver dz)

-hepatic steatosis-

Answer 18

Macrovesicular Fatty change (accumulation of fat in hepatocytes)

reversible with alc cessation

Question 19

Alcoholic hepatitis

whats a hallmark finding on histo

how do liver enzyme labs look

Answer 19

Sustained Long term consumption of alc

direct chemical injury to hepatocytes by Acetylaldehyde

-Mallory bodies (eosinophilic inclusion of keratin filaments)

AST>ALT

Question 20

Alcoholic chirrosis

Answer 20

Final and Irreversible form

shrunken liver. Sclerosis around central vein

ZONE 3

Question 21

Non Alcoholic Fatty Liver Dz

Answer 21

fatty infiltration of hepatocytes but NOT due to alcohol.

ALT > AST

Question 22

Hemochromatosis

whats the mutation?

whats the classic triad presentation?/other presentations?

which stain do you use to determine?

Answer 22

Excess IRON leading to deposition in tissues and organ damage. (generation of free radicals)

• HFE gene mutation
• cirrhosis, diabetes mellitus, skin pigmentation

Bronze diabetes

dilated cardiomyopathy and hypogonadism

-Prussian blue

Question 23

Lipofuscin

Answer 23

wear and tear brown pigment in hepatocytes

Question 24

Wilsons Dz

whats the genetics/mutation

hallmark clinical finding

Answer 24

Autosomal Recessive defect in ATP7B gene

-inadequate copper excretion into bile/ cant incorporate copper into ceruloplasmin

-Kayser- Fleischer rings in cornea

Question 25

Primary Biliary Cirrhosis (PBC)

which antibody is present in the serum

Answer 25

Autoimmune destruction of intrahepatic bile ducts

Bitches over 40

Anti-​mitochondrial antibody

Question 26

Primary Sclerosing cholangitis

whats the classic appearance?

what gi problem is this associated with?

what does this have an increased risk for?

Answer 26

inflammation and fibrosis of both intra and extrahepatic bile ducts

• onion skin appearance of bile duct & Beads on a string of ducts
• Ulcerative Colitis

Positive (+) P-ANCA

-cholangiocarcinoma

Question 27

Reye’s Syndrome

what part of the cells are damaged

Answer 27

Liver failure and encephalopathy in children when given aspirin for viral illness

-mitochondrial damage of the hepatocytes

Aspirin metabolites decr. B-oxidation of mito enzymes

Question 28

Hepatic Adenoma

what can cause this?

Answer 28

benign liver tumor of hepatocytes

-Oral contraceptive use

Risk of rupture

Question 29

Budd-chiari

what can lead to this

Answer 29

Thrombosis of hepatic vein w/ centrilobular congestion and necrosis

nutmeg liver

hepatocellular carcinoma