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Glossary of pathology, bitches

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What is hemosiderosis?
Deposit of iron (hemosiderin) - this causes hemochromatosis
The traid of sx. are?
micronodular cirrhosis, pancreatic fibrosis, and skin pigmentation ("bronze" diabetes)
Possible complications include?
CHF, increased risk of hepatocellular carcinoma
What is primary form?
inherited, Autosomal recessive
What is secondary form due to?
chronic transfusion therapy
What are ferritin, iron, TIBC and transferrin saturation levels?
Ferritin increased, iron increased, TIBC decreased => transferrin saturation is increased
How much iron may the body contain?
As much as 50g, enough to set off airport metal detectors
How is this condition treated?
Phlebotomy, deferoxamine
bacteria convert conjugated bilirubin into what? what happens to some of this?
urobilinogen --> some which is reabsorbed, some which is excreted. (urobilinogen that's reabsorbed is converted to urobilin= yellow pigment in urine)
which is soluble in water/urine: conjugated or unconjugated bilirubin?
conjugted of course!
check out the 3 jaundice types: what are they? For each type: bilirubin type, urine bilirubin?, urine urobilinogen?
heptocellular (conjugated/uncongugated; urine bilirubin inc; urnine urobilinogen normal) obstructive (conjugated; urine bilirubin increased; urine urobilinogen decreased); hemolytic (unconjugated; urine bilirubin absent; urine urobilinogen increased)
What is the biochemical disorder in GILBERTS SYNDROME?
mild ↓ in UDP-glucuronyl transferase (this is key enzyme in conjugation of bilirubin)
what are the symptoms?
asymptomatic
what type of bilirubin is seen upon the lab findings in Gilbert's? Do you have do you have major hemolysis?
unconjugated bilirubin is elevated w/o overt hemolysis
What is the syndrome called when you have an ABSENCE of UDP-glc-ur-transferase?
Crigler-Najjar (CN) syndrome type 1 (type 2 is LESS severe)
when does it present in life? What is the prognosis?
presents early in life: pts die in a few years.
name 3 findings of Crigler Najjar syndrome?
jaundice, kernicterus, ↑ unconjugated bilirubin
type 2 is more sever or less severe? What do you treat it with?
less severe than type 1; treat type 2 with phenobarbital.
what do you treat type 1 with (name 2)?
plasmapheresis + phototherapy (breaks down unconj. Bilirubin)
What is Dubin Johnson syndrome?
↑↑ hyperbilirubin (conjugated) due to defective liver EXCRETION
what does the liver look on gross exam?
black liver grossly
what is the name of the less severe syndrome akin to Dubin Johnson?
Rotor's syndrome: also no black liver.
what is the pathophysiology of primary sclerosing cholangitis
segmental inflammation and fibrosis of bile ducts: unaffected parts are dilated.
what test do you do to see stricture/dilating/beading pattern in primary sclerosing cholangitis?
ERCP
what disease is primary sclerosing cholagitis associated with?
Ulcerative Colitis
What can prim. Scl. Cholangitis lead to?
2° biliary cirrhosis/sclerosis
what is the cause of Primary Biliary Sclerosis (PBS)? (compare the name/disease of PBS vs. PSC: primary sclerosing cholangitis)
autoimmune disorder with antimitochondrial antibodies
name 3 signs associated with PBS:
1) severe obstructive jaundice 2) itching 3) hypercholesterolemia (xanthoma)
What is the cause of SBS: secondary biliary sclerosis?
EXTRA-hepatic biliary obstruction → ↑ pressure in INTRA-hepatic ducts → ↑ injury/sclerosis
what is 2° biliary sclerosis associated with (name 3 things)?
ascending cholangitis (bacterial infection), bile stasis, and bile lakes
in liver cancers, is this common?
yes, #1 most common!
there is an ↑ incidence of hepatoma due to what diseases/exposures (name 7)
1) hep B, 2) hep C, 3) Wilson's disease, 4) hemochromatosis, 5) a-antitrypsin def 6) EtOH cirrhosis 6) carcinogens (e.g. aflatoxin B1)
How do hepatomas spread? How does this differ with renal cell ca. spread?
They BOTH commonly spread hematogenously!
What is major lab finding with hepatocelluar ca?
↑ AFP (alpha fetoprotein)
What is Reye's syndrome?
Hepato-encephalopathy: fatal: occurs in childhood
name 3 finding in Reye's:
1) fatty liver w/ microvesicular fatty change 2) hypoglycemia, 3) coma
is this associated with any outside factors/triggering factors?
yes: 1) viral infection (esp. VZV or influenza B) 2) salicylates!!!
if baby/child has fever what do you use in place of aspirin?
use acetaminophen (but with caution): i.e. acetaminophen OD hepato-toxicity
name 3 types of stones and tell me how they show up on CT scan:
1) cholesterol stone (radiolucent but 10-20% opaque due to calcification) 2) mixed stone = cholesterol + pigment (radiolucent) 3) pigment stone (radio-opaque)
Which is the most common type of gall stone?
mixed stone
name at least 5 out 9 risk factors for Cholesterol stones: is there a mnemonic?
1) obesity 2) Crohn's D 3) Cystic fibrosis 4) elderly 5) clofibrate 6) estrogens 7) multiparity 8) rapid weight loss 9) native American origin mnemonic = 4 F's fat, female….
name 4 risk factors for pigment stones:
1) pts with chronic RBC hemolysis 2) alcoholic cirrhosis 3) elderly 4) biliary infection
What is a good way to diagnosis stones?
ultrasound
What is CHARCOT'S TRIAD
1) epigastric/RUQ pain 2)fever 3) jaundice. present in 70% of those with bacterial cholangitis

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