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Gastrointestinal 1 > Pathology of Jaundice, Liver Failure and Neoplasm > Flashcards

Pathology of Jaundice, Liver Failure and Neoplasm Flashcards

1
Q

What is the earliest sign of jaundice?

A

scleral icterus (yellow discoloration of the sclera) due to increased serum bilirubin (greater than 2.5 mg/dL).

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2
Q

*** How is bilirubin metabolized?

A
  1. At the end of RBCs lifespan (120 days), they are removed from the blood by the macrophages of the reticuloendothelial system (mostly spleen).
  2. Hemoglobin within the RBCs is broken down into HEME (Fe + protoporphyrin)and GLOBIN (amino acids to be recycled).
  3. Protoporphyrin (from heme) is converted to unconjugated (indirect) bilirubin (UCB).
  4. UCB will be carried on albumin to the liver.
  5. Uridine glucuronyl transferase (UGT) in hepatocytes conjugates bilirubin (CG) to make it water soluble.
  6. CB is transferred to bile canaliculi to form bile, which is stored in the gallbladder.
  7. Bile is released into the small bowel to aid in digestion of fats.
  8. Intestinal flora convert CB to urobilinogen, which is oxidized to STERCOBILIN (makes stool brown) and UROBILIN (partially reabsorbed into blood and filtered by kidney, making urine yellow).
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3
Q

What is extravascular hemolysis or ineffective erythropoiesis?

A
  • excessive destruction of RBCs by reticuloendothelial system or death or RBCs within the bone marrow, respectively.
  • In either case, you will get excessive production of UCB (indirect bilirubin), which will overwhelm the conjugating ability of the liver, leading to high levels of UCB in the blood.
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4
Q

*** What are the clinical features of extravascular hemolysis or ineffective erythropoiesis?

A
  • dark urine due to INCREASED urine UROBILINOGEN (UCB is not water soluble and thus is absent from urine, but the liver is conjugating as much bilirubin as it can, hence the excessive urine urobilinogen from CB).
  • increased risk for pigmented bilirubin gallstones.
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5
Q

What is physiologic jaundice of the newborn?

A
  • newborn liver has a transiently low conjugating ability due to low uridine glucuronyl transferase (UGT) activity. - This leads to increased UCB in the blood.
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6
Q

What are the clinical features of physiologic jaundice of the newborn?

A
  • UCB is fat soluble (NOT water soluble) and has no where to go, so it deposits in the basal ganglia (KERNICTERUS) leading to neurological deficits and death.
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7
Q

How do you treat physiologic jaundice of the newborn?

A
  • phototherapy (merely makes UCB water soluble; IT DOES NOT CONJUGATE IT).
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8
Q

What is Gilbert syndrome?

A
  • autosomal recessive mildly low UGT enzyme activity.

- This leads to increased UCB in the blood.

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9
Q

What is the clinical feature of Gilbert syndrome?

A
  • jaundice during STRESS (e.g. sever infection)

* Otherwise, not clinically significant.

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10
Q

What is Crigler-Najjar syndrome?

A
  • Absence of UGT enzyme!

- This leads to EXCESSIVELY HIGH UCB.

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11
Q

What is the clinical feature of Crigler-Najjar syndrome?

A
  • Kernicterus

* usually FATAL

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12
Q

What is Dubin-Johnson syndrome?

A
  • autosomal recessive condition causing a deficiency of bilirubin canalicular transport protein.
  • Causes an increase in CONJUGATED bilirubin in the blood.
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13
Q

What are the clinical features of Dubin-Johnson syndrome?

A
  • LIVER is DARK, but not clinically significant.

- derivative syndrome called ROTOR SYNDROME= similar to dubin-johnson but lacks liver discoloration.

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14
Q

What happens with biliary tract obstruction (obstructive jaundice)?

A
  • associated with gallstones, pancreatic carcinoma, cholangioicarcinoma, parasites, and liver fluke (Clonorchis sinensis).
  • Anything in the bile will then leak into the blood:
  • CONJUGATTED bilirubin will be high in the blood because once bilirubin has made it into the biliary tract, it is conjugated, remember.
  • DECREASED urine urobilinogen (because it is not making it to the bowel to be converted via bacteria).
  • INCREASED alkaline phosphatase
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15
Q

What are the clinical features of biliary tract obstruction (obstructive jaundice)?

A
  • DARK URINE (due to bilirubinuria; conjugated bilirubin is water soluble that has leaked into the blood; NOT UROBILINOGEN).
  • pale stool
  • pruritus due to increased plasma bile acids.
  • hypercholesterolemia with xanthomas (from leaking into the blood from the bile).
  • steatorrhea with malabsorption of fat-soluble vitamins.
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16
Q

What will viral hepatitis do?

A
  • causes inflammation and disruption of the hepatocytes and small bile ductules.
  • this leads to an INCREASE of both CB and UCB.
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17
Q

What are the clinical features of viral hepatitis?

A
  • DARK URINE due to increased urine bilirubin
  • urine UROBILINOGEN is normal or decreased (because less is making it to the bowel due to damaged bile ductules).
  • NOTE: if Kawasaki disease, the treatment IS ASPIRIN!
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18
Q

What is hepatic adenoma?

A
  • BENIGN tumor of hepatocytes associated with ORAL CONTRACEPTIVE use and will regress with cessation of drug.
  • no central scar like in “focal nodular hyperplasia.”
  • no connection to biliary system.
  • ESTROGEN and PROGESTERONE RECEPTORS are present in most cases.
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19
Q

What is a risk of haptic adenomas if pt is pregnant?

A
  • rupture and intraperitoneal bleeding, because the tumors are subcapsular (right underneath the capsule of the liver) and grow with exposure to estrogen.
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20
Q

*** What is hepatocellular carcinoma?

A
  • MALIGNANT tumor of hepatocytes.
  • more common in males bc males are more likely to get cirrhosis.
  • Will see SERUM ELEVATION of ALPHA-FETOPROTEIN (AFP).
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21
Q

** What are the risk factors for heptaocellular carcinoma? (HIGH YIELD)

A
  • chronic hepatitis (HBV and HCV)
  • cirrhosis (alcohol, nonalcoholic fatty liver disease, hemochromatosis, Wilson disease, and A1AT deficiency).
  • AFLATOXINS derived from Aspergillus (induce p53 mutations).
22
Q

What does hepatocellular carcinoma love to invade?

A
  • the hepatic vein and this causes BUDD-CHIARI syndrome.
23
Q

What is Budd-Chiari syndrome?

A
  • liver INFARCTION secondary to hepatic vein OBSTRUCTION causing painful hepatomegaly and ascites.
24
Q

What is the prognosis for hepatocellular carcinoma?

A

poor bc tumors (often heterogenous; aka could be trabecular, tubular…) are often detected late because symptoms are masked by cirrhosis.

25
Q

*** What is a serum marker for hepatocellular carcinoma?

A

alpha-fetoprotein

26
Q

Is metastasis more common than primary tumors?

A

YES

27
Q

What are the most common sources of metastasis to the liver?

A
  • colon, pancreas, lung, and breast carcinomas
28
Q

What will happen in the liver if the carcinoma occurs due to metastasis from somewhere else?

A
  • multiple nodules

- clinically may be detected as hepatomegaly with a nodular free edge of the liver.

29
Q

What is liver failure?

A
  • most severe clinical consequence of liver disease
  • may result from sudden and massive liver destruction such as fulminant hepatitis.
  • often presents end stage of progressive chronic damage to the liver.
30
Q

What percent of hepatic function must be lost before hepatic failure occurs?

A

80-90%

*liver transplant is often the only treatment.

31
Q

Do we see high or low levels of albumin and ammonia, respectively in the blood with liver failure?

A
  • hypoalbuminemia= peripheral edema

- hyperammonemia= cerebral dysfunction (hepatic encephalopathy).

32
Q

What is fetor hepaticus in liver failure?

A

musty body odor due to the formation of mercaptans by the action of GI bacteria on sulfur containing amino acids with shunting of splanchnic blood from the portal into systemic circulation.

33
Q

What happens due to impaired estrogen metabolism during liver failure?

A
  • vasodilation causing PALMAR ERYTHEMA and SPIDER ANGIOMAS.

- hyperestrogenemia in males gives rise to hypogonadism and gynecomastia.

34
Q

What blood problems can happen with liver failure?

A
  • coagulopathy due to impaired production of hepatic clotting factors.
  • massive GI hemorrhage
  • intestinal absorption of blood increases the metabolic load on the liver.
  • systemic inflammatory response syndrome (SIRS), which result from a massive release of inflammatory cytokines from the necrotic liver.
  • a rapid downhill course may follow with death from weeks to months.
35
Q

Can acute liver failure occur in pts without previously recognized liver disease?

A

YES. Some pts undergo hepatic regeneration and spontaneously recover.
*Most require liver transplant.

36
Q

What can cause acute liver failure?

A
  • wilson disease
  • viral hepatitis (most common worldwide).
  • autoimmune hepatitis
  • drug overdose (acetaminophen).
37
Q

What is the most common cause of death in pts with acute liver failure?

A

multi-system organ failure often caused by sepsis

38
Q

What bacteria are often a cause for infections with acute liver failure?

A
  • staph and strep, often associated with extensive invasive monitoring in these pts
39
Q

What else can occur with acute liver failure?

A
  • hypoxemia from acute lung injury and ARDS

- rising CO2 levels= cerebral vasodilation and increased intracranial pressure.

40
Q

What does hyperammonemia due in acute liver failure?

A
  • converts glutamate to glutamine, which accumulates in the astrocytes causing osmotic stress by overwhelming the ability of astrocytes to expel organic osmolytes. This leads to cerebral edema.
41
Q

What does hepatorenal syndrome cause as a result of liver failure?

A
  • drop in urine output with a rising blood urea nitrogen (BUN) and creatinine.
  • liver transplant is often treatment of choice.
42
Q

What is the classic triad of hepatopulmonary syndrome?

A
  1. hypoxemia= from V/Q mismatch
  2. chronic liver disease
  3. intrapulmonary vascular dilations
43
Q

What is focal nodular hyperplasia?

A
  • subcapsular grayish white mass, which may be pedunculated.
  • usually ASYMPTOMATIC.
  • a white depressed area of fibrosis is often present in the center of the mass with broad strands radiating from it often producing a stellate configuration.
  • fibrous septa divide the lesion into lobules resembling cirrhosis (but not and just localized).
  • presence of the RAS ONCOGENE PRODUCT P21
44
Q

Does hepatocellular carcinoma sometimes have a capsule?

A

YES (this is the exception to the rule that most benign tumors have a capsule and most malignant tumors do not).

45
Q

Review question: How do most carcinomas vs sarcomas metastasize?

A
  • carcinomas= via lymphatics
  • sarcomas= via hematogenous (blood)
  • hepatocellular carcinoma is an exception as it loves the portal vein.
46
Q

Can you see mallory bodies (seen in alcoholic cirrhosis) in hepatocellular carcinoma also?

A

YES as well as alpha-1 antitrypsin, AFP, copper, or bile pigment.

47
Q

** What is Fibrolamellar carcinoma (type of hepatocellular carcinoma)?

A
  • seen mostly in YOUNG PATIENTS
  • NOT ASSOCIATED WITH CIRRHOSIS
  • FAVORABLE PROGNOSIS
  • microscopically has fibrosis arranged in a lamellar fashion around neoplastic hepatocytes.
  • have more mitochondria
  • KNOW PICTURE
48
Q

What is hepatoblastoma? (blastoma= immature)

A
  • seen mostly in infants with other congenital anomalies.
  • no relationship with cirrhosis.
  • usually solitary tumors consisting of immature hepatocytes.
  • may be associated with Familial Adenomatous Polyposis (FAP) syndrome.
49
Q

** What is hemangioma?

A
  • most common BENIGN tumor of the liver

- spongy red purple lesion (most are cavernous type)

50
Q

What is angiosarcoma?

A
  • malignant tumor of the inner lining of the blood vessels.

- identified by FACTOR VIII related antigen and other endothelial markers.

51
Q

What are risk factors for angiosarcoma?

A
  • CIRRHOSIS and hemochromatosis
  • VINYL CHLORIDE exposure
  • ARSENIC exposure
  • THORIUM DIOXIDE exposure (thorotrast contrast medium).
  • Rembmer “VAT”

Gastrointestinal 1 (21 decks)

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