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11 Liver, Gallbllader, Exocrine Pancreas > Alcoholic Metabolic Autoimmune Liver disease > Flashcards

Alcoholic Metabolic Autoimmune Liver disease Flashcards

1
Q

Child pt who presents with hypoglycemia, elevated liver enzymes, and nausea/vomiting.

-suspect what?

A

suspect Reye Syndrome!

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2
Q

What intermediate of alcohol metabolism damages liver?

  • mechs (3)
  • what enzyme is needed to metabolize this?
A

Acetaldehyde

  1. activates stellate cells to form collagen (fibrosis)
  2. Hepatocyte ballooning: microfilaments that maintain intracellular skeleton are sheared. Also, Mallory bodies are formed (bodies of intermediate filaments)
  3. kupffer cells produce TNF-A (Pentoxifylline inhibitis this)

Ethanol –> Acetaldehyde –> Acetic acid

Rxn 1. Alcohol DH and CYP2E1

Rxn 2. Aldehyde DH (50% asians have this mutated)

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2
Q

Wilson’s disease

  • clinical presentation (3)
  • typical population
A
  • presentation at childhood
    1. cirrhosis
    2. neurologic (dementia, chorea, parkinsonian due to basal ganglia Cu deposition)
    3. Kayser-Fleisher rings (requires opthalmologist to see)
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3
Q

Hepatic inflammation:

What happens histologically from stage 1 to 4?

A

inflammatory cells surround portal triad and expand outward

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3
Q

Primary Biliary Cirrhosis (PBC)

-clinical presentation

A
  • Obstructive jaundice–symptoms include:
  • pruritis
  • xanthomas
  • steatorrhea, malabsorption of DEAK
  • often asymptomatic: incidental AMA+ finding from rheumatologist looking at other autoimmune disorders
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4
Q

Fulminant liver failure

A

Acute liver failure complicated by:

  • coagulopathy
  • encephalopathy
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5
Q

Wilson’s disease

  • genetic allele
  • inheritance pattern
A
  • Auto rec
  • ATP7B gene: ATP-mediated hepatocyte Cu transport protein
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6
Q

Hepatic fibrosis

  • what cells mediate this
  • what do they secrete
  • where are they located
A
  • Stellate cells, which secrete TGF-B to create fibrosis
  • Space of Disse, beneath endothelial sinusoid cells
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6
Q

Hereditary hemochromatosis

-increased risk of what complication?

A

-hepatocellular carcinoma (b/c of free radial damage to DNA from Fe Fenton reaction)

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7
Q

Liver disease pt has high INR. How to tx immediately? (2)

A
  1. Use FFP (clotting factors)
  2. Vit K (only works for pts with cholestatic disease b/c malabsorption)
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9
Q

Autoimmune hepatitis

characteristic lab findings (3)

A
  1. ALT/AST >1000 (very high )
  2. elevated IgG
  3. autoantibodies

Type 1: (ANA–anti nuclear Ab)

(ASMA–Anti smooth m Ab)

Type 2: anti-liver kidney microsomal Ab

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10
Q

Hepatic Fibrosis:

what happens histologically from stage 1-4?

A

Fibrotic bands expand from portal triads to other portal triads.

  1. portal fibrosis
  2. periportal fibrosis
  3. bridging fibrosis
  4. cirrhosis (nodules created)
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10
Q

Metabolism of ethanol

-steps, enzymes, intermediates

A

75-80%:

Ethanol –> Acetaldehyde –> Acetic acid

Rxn 1. Alcohol DH and CYP2E1

Rxn 2. Aldehyde DH

20-25%:

-Ethanol is metab by MEOS (microsomal ethanol oxidizing system–P450)

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12
Q

Hepatic necrosis:

  • what is this
  • 2 most common causes
A
  • Acute hepatocyte death (not fibrosis, which takes years)
  • can lead to acute liver failure

most common causes:

  1. meds (acetaminophen)
  2. viral hepatitis
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13
Q

NAFL disease progression stages:

A
  1. Fatty Liver (NAFL)
  2. Steatohepatitis (NASH)
  3. NASH with fibrosis
  4. Cirrhosis
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14
Q

Hereditary hemochromatosis

  • classic symptoms (3)
  • other notable symptoms (3)
A

Classic triad:

  1. cirrhosis
  2. diabetes mellitus
  3. bronze skin

Other:

  1. dilated cardiomyopathy
  2. cardiac arrhythmias
  3. gonadal dysfunction (testicular atrophy)
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14
Q

What do AST and ALT require as cofactor?

What are AST/ALT alternate abbrev?

A

-PLP

AST: SGOT

ALT: SGPT

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15
Q

A1AT Deficiency

-Dx tests (3)

A
  1. serum A1AT levels
  2. phenotyping
  3. liver biopsy (A1AT globules stain red on PAS stain)
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15
Q

Autoimmune hepatitis

-histology finding

A

-Plasma cell infiltrate in liver

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16
Q

Hereditary hemochromatosis

  • Labs: ferritin, TIBC, serum Fe, % sat
  • Liver biopsy findings
A

high ferritin

low TIBC

high serum Fe

high % sat

  • Fe accumulates in hepatocytes (brown pigment)
  • distinguish from lipofuscin with Prussian blue stain (Fe is blue)
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16
Q

In Liver biopsy, you see brown pigment in hepatocytes. What could this be?

A

Fe or lipofuscin

-distinguish using Prussian blue stain (Fe is blue)

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16
Q

Hereditary hemochromatosis

  • what lab finding value do you look for when you suspect Hereditary hemochromatosis?
  • Tx
  • What else to do if pt is >40 or has elevated ALT/AST?
A

-Fe/TIBC ratio >45%. (do genetic testing if you find this)

Tx: phlebotomy

-also do biopsy to look for possible cirrhosis

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16
Q

ALT/AST >500

Differential? (4)

A
  1. Autoimmune Hep
  2. Hep A/B
  3. Meds
  4. Ischemic disease
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18
Q

NASH

-drug tx (2)

A
  1. Pioglitazone (Actos)–oral hypoglycemic
  2. Vit E (antioxidant)

Note: Use Vit E in non-diabetic pts

19
Q

Primary Biliary Cirrhosis (PBC)

-histological findings

A

“Florid Duct lesion”

-lymphocyte and monocyte proliferation around bile ducts (at portal triad)

20
Q

What is most common cause of genetic liver disease in children, and also is the most common genetic disease leading to liver transplantation in children?

A

A1AT Deficiency

21
Q

Wilson’s disease

-Tx (3)

A

Cu-chelating agents: (increase urinary excretion)

  1. D-Penicillamine
  2. Trientine
  3. Zinc (blocks intestinal absorption)
22
Q

Alcoholic liver disease:

-what amount of alcohol intake do we start seeing problems

A

Daily intake of 4 or more drinks

(>60g/day)

22
Q

Alcoholic liver disease

-progression of liver states

A

Fatty liver

/ \

Alcoholic hepatitis –> Cirrhosis

24
Q

NAFL

-How much weight do you tell your obese patient to lose?

A

3-5% to improve steatosis, but

10% weight reduction needed to improve inflammation

-shoot for 10%, in 6 months

26
Q

Mallory Bodies

A

inclusion bodies of intermediate filaments

-found in alcoholic hepatitis, among other liver diseases

28
Q

How does ethanol intake affect metabolism cycles in the liver? (2)

A

Increased NADH:

  1. inhibits Krebs cycle, reduced gluconeogenesis (alcoholic hypoglycemia)
  2. reduced fatty acid oxidation

(fatty acids are trapped, causing steatosis)

29
Q

Primary Biliary Cirrhosis (PBC)

-Tx (3)

A

autoimmune, but no steroid use!

  1. Ursodeoxycholic acid
    - synthetic hydrophilic bile acid. bile duct damage is due to accum of hydrophobic bile acids, so try to reverse ratio.

Pruritis tx:

  1. Cholestyramine (during day)
  2. Diphenhydramine (during night)
30
Q

Primary Biliary Cirrhosis (PBC)

  • what is it
  • classic population
A
  • chronic autoimmune destruction of intrahepatic bile ducts
  • white middle aged women
31
Q

Non Alcoholic Fatty Liver disease:

risk factors (3)

A
  1. obese
  2. hypertriglyceridemia
  3. diabetes
32
Q

Pts after completing gastric bypass surgery: what about their liver are you concerned about?

A

Rapid weight changes after gastric bypass surgery:

-can cause steatosis/steatohepatitis

33
Q

Alcoholic Hepatitis:

drug tx (2)

A

Anti-inflammatory:

  1. steroids
  2. pentoxifylline (inhibits TNF-A)
    - use this if steroids contraindicated
35
Q

Hemochromatosis

-mech of tissue damage

A

Free radicals from Fenton reaction of Fe

36
Q

Why is INR better indicator of liver function than Albumin?

when does Albumin become the better indicator?

A

Albumin can be affected by loss in urine/GI.

However, INR cannot be used in Warfarin tx. Must use albumin

37
Q

Don’t give aspiring to child pt with viral disease. However, what child disease mimics viral disease and requires aspirin to tx?

A

Kawasaki’s disease

38
Q

Primary Biliary Cirrhosis (PBC)

-what is target of AMA Ab?

A

-enzyme located on the membrane of biliary epithelial cells

(PDC-E2)–pyruvate DH complex

39
Q

elevated Alkaline Phosphatase:

  • what could it be? (3)
  • how to distinguish
A

If elevated ALP is isolated, could be:

  • Cholestatic disease
  • pregnancy
  • bone disease
  • order GGT and 5’ nucleotidase
  • if positive, then it’s liver
40
Q

Alcoholic liver disease:

CDC definition of ‘excessive drinking?’

A

One or both of the following:

  1. Heavy drinking-
    women: >1/day
    men: >2/day
  2. Binge drinking
    women: >4 on single occasion
    men: >5
41
Q

Wilson’s disease

-lab findings for dx (3)

A
  1. high urinary Cu
  2. low serum ceruloplasmin (not reliable test b/c it’s elevated in inflammation)
  3. Liver biopsy (Cu deposits)
43
Q

Wilson’s disease

-mech of genetic defect (2)

A
  • genetic defect in ATP7B (Cu transport protein in liver)
    1. Allows Cu to bind to ceruloplasmin
    2. Transports Cu to Bile for excretion
  • Therefore, Cu deposits elsewhere in the body and is excrete in urine
44
Q

What liver disease is autoimmune but is not tx with steroids?

A

Primary Biliary Cirrhosis (PBC)

(Use Urso, plus cholestyramine, benadryl)

46
Q

NAFL vs NASH

A

Non alcoholic fatty liver:

  • no inflammation
  • pts die of heart disease

Non alcoholic steatohepatitis:

  • inflammation with hepatocellular injury
  • pts die of heart disease or liver failure
47
Q

Reye syndrome

  • what happens
  • mech
A
  • Fulminant liver failure and encephalopathy in children with viral illness given aspirin
  • mitochondrial damage to hepatocytes
48
Q

Wilson’s disease

-dx criteria

A

2 of 3:

  1. high urinary Cu
  2. low ceruloplasmin
  3. kayser fleisher rings
49
Q

Which CYP is in alcohol metabolism?

-what else uses this?

A

CYP2E1.

-acetaminophen also uses

50
Q

How can you tell a liver is fatty on ultrasound?

A

Fat is bright on US

52
Q

A1AT Deficiency

  • genetic alleles:
  • inheritance pattern
A
  • auto co-dom
  • M: normal allele
  • S: mutated, less severe
  • Z: mutated, severe

M/M: normal

M/Z: asymptomatic

S/S: asymptomatic

Z/Z: lung and liver disease

null/null: lung disease but no liver disease b/c no abnormal protein in liver

53
Q

Primary Biliary Cirrhosis (PBC)

  • lab test values:
    1. ALP
    2. Bilirubin
    3. Cholesterol
A
  1. elevated
  2. elevated, usu rises late
  3. Highly elevated, but so is protective HDL. primary care doctors don’t need to worry about heart disease
54
Q

Hereditary hemochromatosis

  • what gene (what does it do)
  • what alleles
  • inheritance pattern
  • what populations, prevalence
A
  • HFE gene, which regulates absorption of Fe from small intestine (down-regulates transferrin when body Fe is adequate)
  • Auto rec
  • 2 genotypes with symptoms:
    1. C282Y/C282Y (most cases)
    2. C282Y/H63D
  • N. european (0.5-1% are homozygotes!)
55
Q

Primary Biliary Cirrhosis (PBC)

-these 2 are characteristically elevated in the blood:

A
  • high AMA (anti mitochondrial Ab)
  • elevated IgM
56
Q

Wilson’s disease

-increased risk of what complication

A

-hepatocellular carcinoma

57
Q

Autoimmune hepatitis

-Tx

A

Steroids, plus Azathioprine

-also use steroids in alcoholic steatohepatitis (only other liver disease for steroids)

58
Q

How does Hereditary hemochromatosis differ from Secondary Fe overload? (histologically)

A

Hereditary hemochromatosis: Fe deposits in hepatocytes

Secondary Fe overload: Fe deposits in Kupffer cells. Usu no symptoms.

59
Q

Alcoholic Hepatitis:

-how long does it take to see improvement in liver function after alcohol abstinence/nutritional support?

A

3 months. Lab values won’t change before 2-3 months.

11 Liver, Gallbllader, Exocrine Pancreas (7 decks)

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