L14- GIT Pathology VII (liver) Flashcards

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1
Q

list the autoimmune cholangiopathies

A

Primary biliary cholangitis (PBC)

Primary sclerosing cholangitis (PSC, think UC)

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2
Q

PBC, formerly named (1):

  • affects (2) population, age/gender
  • 90% of cases are caused by (3)
  • (4) are key evidence of PBS in serum
A
(primary biliary cholangitis)
1- primary biliary cirrhosis
2- middle aged women
3- Antimitochondrial Igs
4- elevated ALP, GGT
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3
Q

PBC:

  • (1) gross appearance
  • (2) is the main histological feature, described as (3)
  • in (2), (4) is seen centrally and (5) on the periphery
  • (6) is the end progression
A

1- rough, nodular, greenish tinge

2- Florid duct lesion
3- non-suppurative, granulomatous destruction of small / medium sized intrahepatic bile ducts

4- fusing macrophages (forming granuloma and destroying bile duct)
5- surrounding inflammatory cells

6- ductopenia and cirrhosis

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4
Q

describe the cause of Secondary Biliary Cholangitis

A

partial or total bile duct obstruction, due to:

  • tumor
  • strictures
  • gallstones
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5
Q

PSC:

  • involves the destruction of (1) with (2) lesions surrounding it
  • ERCP/MRCP, in order to visualize biliary tree, will show (3)
  • (4) is often positive / found in serum
  • (5) is the main associated condition, (6) is the main risk / complication
A

(primary sclerosing cholangitis)
1- intra-hepatic and extra-hepatic bile ducts (large)
2- ‘onion-skin’ lesions: periductal inflammation and fibrosis

3- dilatation and beading of biliary tree

4- p-ANCA (MPO)
5- UC
6- cholangiocarcinoma

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6
Q

PSC:

  • (1) MRCP appearance
  • (2) ERCP appearance
  • (3) main histological feature
A

(primary sclerosing cholangitis)
1- (magnetic resonance cholangiopancreaticography) loss of brightness / contrast in portions of biliary tree, dilation in other areas

2- (endoscopic retrograde cholangiopancreaticography) focal dilatation in some bile ducts

3- degenerating bile duct trapped in dense scar

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7
Q

indicate if the following refers to PBC or PSC:

  • (1) mostly affects females
  • (2) mostly affects younger population
  • (3) predictable and progressive
  • (4) strictures and beading seen on radiography
  • (5) inflammatory destruction of large extra-/intra-hepatic ducts, obliteration of medium/small ducts
  • (6) florid duct lesions + loss of small hepatic ducts
A

primary (biliary / sclerosing) cholangitis
1- PBC, 90% (PSC is 70% male)
2- PSC, ~30y/o (PBC is ~50y/o)

3- PBC (PSC is progressive and unpredictable)

4- PSC- affects large ducts (PBC appears normal- affect small ducts)

5- PSC
6- PBC

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8
Q

Hemochromatosis:

  • (1) definition
  • primary cause has (2) as the main genetic defect (+ inheritance pattern)
  • (2) mainly affects (males/females), presents at (4) age, and causes (5)
A
1- excessive Fe accumulation
2- HFE gene mutation, AR inheritance
3- males
4- rarely seen before 40y/o
5- inc Fe absorption
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9
Q

Hemochromatosis:

  • (1) definition
  • (2) method of secondary form
  • (3) architectural changes in liver in primary/secondary form
  • (4) is the most common cause of death / progression
  • (5) Tx
A
1- excessive Fe accumulation
2- acquired
3- liver fibrosis --> cirrhosis (micronodular)
4- HCC
5- phlebotomy
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10
Q

List causes of secondary hemochromatosis

A

Parenteral Fe overload: repeated blood transfusions, Fe dextran injections

Ineffective erythropoiesis: β-thalassemia. other chronic hemolytic anemias

Increased oral intake: Bantu disease (example of drinking beer made in iron pot –> increasing iron intake)

Chronic liver disease: alcohol, HepC

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11
Q

Hemochromatosis effects in Pancreas:

  • (1) appearance
  • (2) histological changes
  • Fe is in (3) form / cells
  • (4) associated disease
A

1- intensely pigmented
2- diffuse interstitial fibrosis
3- hemosiderin in both acinar and islet cells
4- DM

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12
Q

describe Hemochromatosis effects in Heart

A
  • hemosiderin in myocardial fibers = cardiomyopathy

- delicate interstial fibrosis

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13
Q

describe Hemochromatosis effects in Skin:

  • (1) color change
  • Fe is in (2) form / cells
  • (3) is the main physiological effect
A

1- gray

2- free Fe in dermal melanophages

3- inc melanin production

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14
Q

Wilson’s disease:

  • (1) brain effects
  • (2) eye effects
  • (3) blood effects
A

1- basal ganglia Cu deposits => neurodegeneration –> chorea, tremors, abnormal gait

2- Cu deposits in Descemet membrane of corneal limbus = Kayse Fleischer ring (dark gold rings around Iris)

3- intravascular hemolysis (Cu damages RBCs)

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15
Q

Wilson’s disease clinical presentation

A

-asymptomatic w/ abnormal liver enzymes (initially)

  • acute hepatitis
  • acute hepatic failure (childhood –> young adulthood)
  • cirrhosis
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16
Q

Wilson’s disease clinical presentation

  • (1) initial Sxs
  • (2) progression Sxs
  • (3) Tx
A

1- asymptomatic w/ abnormal liver enzymes (initially)

2:

  • acute hepatitis
  • acute hepatic failure (childhood –> young adulthood)
  • cirrhosis

3- chelation therapy

17
Q

Wilson’s disease investigations results

A
  • low serum Ceruloplasmin
  • inc urinary Cu excretion
  • inc hepatic Cu (>250 µg/g dry weight)
18
Q

Wilson’s disease morphological changes (liver)

A

(ranges mild to severe)

  • fatty change, acute/fulminant/chronic hepatitis
  • Cu accumulation
  • progresses to cirrhosis
19
Q

α1-antitrypsin deficiency:

  • (1) inheritance pattern
  • (2) α1-antitrypsin normal and mutant genotype
  • (3) α1-antitrypsin normal function and (4), the effect of mutant form
A

1- AR
2- PiMM normal, PiZZ mutant
3- Pi = protease inhibitor
4- (PiZZ) retained in hepatocytes

20
Q

α1-antitrypsin deficency:

  • α1-antitrypsin is made in (1)
  • (2) and (3) are the affected organs, include particular changes
  • (4) is noted in the cells of (2)
A

1- liver
2- liver, cirrhosis
3- lungs, emphysema
4- cytoplasmic globules in hepatocytes

21
Q

α1-antitrypsin deficiency histology (liver):

  • (1) stain is used
  • (2) is seen on EM
A

1- PAS w/ diastase digestion (PASD)

2- ER dilated due to accumulation of misfolded protein

22
Q

α1-antitrypsin deficiency:

  • (1) clinical presentation
  • (2) is the main complication or progression
  • (3) test is not reliable, so (4) is used
  • (5) Tx
A

1- neonatal hepatitis with or without cholestasis; chronic hepatitis; cirrhosis

2- HCC

3- serum levels
4- Pi typing / genetic testing

5- liver transplantation (only option)