B5.025 - Prework Patterns of Hepatic Injury

Question 1

What is bile accumulation associated with

Answer 1

Feathery degeneration

Question 2

Intracellular accumulation of fat

Answer 2

Steatosis

obesity, alcohol, diabetes

Question 3

Intracellular iron accumulation

Answer 3

Hemochromatosis

Question 4

Copper intracellular accumulation

Answer 4

Wilson disease

Question 5

Protein in RER

Answer 5

alpha 1 antitrypsin deficiency

Question 6

Lipofuscin intracellular accumulation

Answer 6

Old age, poor health

Question 7

Answer 7

Bile, feathery degeneration

Question 8

Answer 8

Steatosis

fat vacuoles

Question 9

Answer 9

Ballooning degeneration, Mallory hyaline

Question 10

Answer 10

Iron

hemachromatossi

Question 11

Answer 11

Perl’s iron stain

hemachromatosis

Question 12

Clinical case:

55 yo man presents with weakness, fatigability, weight loss, anorexia

he notes sets thirstier and urinating more

PE - sclera icterus, jaundice, hyperpigmentation of skinon trunk and extremities

serum ferritin 1500 (30-300)

Answer 12

Hereditary hemochromatosis

AR

treat with weekly phlebotomy and deferoxamine

Question 13

Describe genetics of hereditary hemochromatosis

Answer 13

AR (HFE gene)

disrupted iron regulation results in toxic iron accumulation and tissue iron deposition

Chromosome 6 mutation responsible

HFE regulates hepcidin

Question 14

Pathophysiology of hereditary hemochromatosis

Answer 14

innappropriately high intestinal iron absorption

results in excess body iron stores

Normal body stores: 4 g

tissue injury - 25 g

cirrhosis - 30-40 g

Question 15

Answer 15

Alpha 1 antitrypsin deficiency

PAS diastase resistant globules

Question 16

What is alpha 1 antitrypsin deficiency

Answer 16

Piz allele —> mutant protein is abnormally folded and cannot move from ER to golgi for secretion

All with PiZZ accumulate alpha 1 antitrypsin in hepatocytes

liver - hepatitis, cirrhosis, HCC

lung - emphysema

PiMM - normal

PiZZ - mutant

Question 17

Answer 17

Copper rhodanine stain

wilsons disease

Question 18

8 yo boy presents with strange, jerky movements and drastic changes in behavior and personality

resting tremor in both arms and hands

brownish ring around cornea

ceruloplasmin - decreased

urin copper - elevated

Answer 18

wilson disease

AR

mutation of copper transporting protein (ATPase) leading to defecting conjugation of copper to ceruloplasmin

10-30

increased copper absorption in gut

accululation in basal ganglia, live,r kidney, cornea

treat with penicillin I’ve for copper chelation

Question 19

Answer 19

Wilson

Question 20

Answer 20

Lipofuscin

Question 21

Liver cell death histo

Answer 21

Apoptosis - eosinophilia bodies in viral haptitis

focal (spotty) or interface periportal necrosis in many forms of hepatitis

zonal toxic necrosis - centrilobular, midzonal, periportal due to toxins/ischemia

Question 22

Answer 22

Apoptosis, eosinophilia body in viral hepatitis (dead red)

Question 23

Answer 23

Interface hepatitis

lymphocytes in right

Question 24

Answer 24

Confluent necrosis