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GIR Test 2 > B5.025 - Prework Patterns of Hepatic Injury > Flashcards

B5.025 - Prework Patterns of Hepatic Injury Flashcards

1
Q

What is bile accumulation associated with

A

Feathery degeneration

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2
Q

Intracellular accumulation of fat

A

Steatosis

obesity, alcohol, diabetes

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3
Q

Intracellular iron accumulation

A

Hemochromatosis

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4
Q

Copper intracellular accumulation

A

Wilson disease

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5
Q

Protein in RER

A

alpha 1 antitrypsin deficiency

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6
Q

Lipofuscin intracellular accumulation

A

Old age, poor health

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7
Q
A

Bile, feathery degeneration

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8
Q
A

Steatosis

fat vacuoles

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9
Q
A

Ballooning degeneration, Mallory hyaline

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10
Q
A

Iron

hemachromatossi

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11
Q
A

Perl’s iron stain

hemachromatosis

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12
Q

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)

A

Hereditary hemochromatosis

AR

treat with weekly phlebotomy and deferoxamine

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13
Q

Describe genetics of hereditary hemochromatosis

A

AR (HFE gene)

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

Chromosome 6 mutation responsible

HFE regulates hepcidin

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14
Q

Pathophysiology of hereditary hemochromatosis

A

innappropriately high intestinal iron absorption

results in excess body iron stores

Normal body stores: 4 g

tissue injury - 25 g

cirrhosis - 30-40 g

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15
Q
A

Alpha 1 antitrypsin deficiency

PAS diastase resistant globules

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16
Q

What is alpha 1 antitrypsin deficiency

A

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

17
Q
A

Copper rhodanine stain

wilsons disease

18
Q

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

A

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

19
Q
A

Wilson

20
Q
A

Lipofuscin

21
Q

Liver cell death histo

A

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

22
Q
A

Apoptosis, eosinophilia body in viral hepatitis (dead red)

23
Q
A

Interface hepatitis

lymphocytes in right

24
Q
A

Confluent necrosis

GIR Test 2 (17 decks)

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