L7: Diffuse Liver Diseases Flashcards

1
Q

what are examples of Diffuse Liver Diseases?

A
  1. Non-alcoholic fatty liver disease (NAFLD)
  2. Autoimmune hepatitis (AIH)
  3. Inherited Metabolic liver diseases:
    a) Wilson’s Disease
    b) Hemochromatosis
    c) α 1 antitrypsin deficiency
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2
Q

Def of NAFLD

A
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3
Q

Prevelance of NAFLD

A
  • Most common cause of liver disease in North America
  • Growing in Parallel With Obesity and Metabolic Syndrome
  • Most common liver disorder in Western industrialized Countries
  • Affects ~25% of adult population worldwide
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4
Q

Causes & Risk Factors of NAFLD

A
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5
Q

CP of NAFLD

A

Symptoms
 Asymptomatic
 Rt hypochondrial pain
 Dyspepsia
 Symptoms of the cause as DM

General Signs
 Increased body weight
 May be xanthoma & Xanthelasma
 Signs of the cause as DM

C) Local examination
 Enlarged, soft liver with rounded boarders & may be tender

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6
Q

Investigations of NAFLD

A
  • Lab
  • Rad
  • Bx
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7
Q

Lab investigations in NAFLD

A
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8
Q

Rad investigations in NAFLD

A
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9
Q

Bx in NAFLD

A
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10
Q

TTT of NAFLD

A

1) Treatment of the cause as malnutrition
2) Reduction of body weight
3) Reduce CHO & fats in diet
4) Statins
5) Insulin sensitizers as metformin & TZDs
6) Anti-oxidants as vitamin A, C &E

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11
Q

Role of Vit E in NAFLD

A
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12
Q

Role of Pioglitazone in NAFLD

A
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13
Q

Is metformin Recommended in NAFLD?

A

Nope

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14
Q

Progression of steatosis into cirrhosis

A
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15
Q

Def of Autoimmune Hepatitis

A
  • It is a chronic (>6 months) progressive immune-mediated inflammation of the liver
  • Disease in which the body’s own immune system attacks the hepatocyte and causes it to become inflamed.
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16
Q

Incidence of Autoimmune Hepatitis

A

About 70 percent of those with autoimmune hepatitis are female.

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17
Q

Types of Autoimmune Hepatitis

A
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18
Q

Pathology of Autoimmune Hepatitis

A
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19
Q

Mild Autoimmune Hepatitis

M

A
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20
Q

Severe Autoimmune Hepatitis

A
21
Q

Cirrhosis Autoimmune Hepatitis

A
22
Q

CP of Autoimmune Hepatitis

A

 Asymptomatic.

 Vague symptoms: Fever, headache, anorexia, malaise.

 Hepatic symptoms: RUQ pain, jaundice, LCF is late.

 Liver: enlarged & firm, may be splenomegaly

 Extrahepatic symptoms: GN, arthritis.

23
Q

Investigations for Autoimmune Hepatitis

A
24
Q

TTT of Autoimmune Hepatitis

A
25
Q

Def of Wilson’s Disease

A
  • An AR defect in copper metabolism characterized by copper accumulation in hepatocytes and other extra hepatic organs.
  • Deficit in gene ATP7B on chromosome 13
26
Q

Pathophysiology of Wilson’s Disease

A
  • Decreased biliary excretion of copper
  • Decreased incorporation of copper into ceruloplasmin
27
Q

CP of Wilson’s Disease

A
28
Q

Investigations of Wilson’s Disease

A
29
Q

LFTs in Wilson’s Disease

A
30
Q

Ceruplasmin in Wilson’s Disease

A
31
Q

Genetic Testing in Wilson’s Disease

A

Mutation analysis of the ATP7B gene

32
Q

Bx in Wilson’s Disease

A
33
Q

TTT of Wilson’s Disease

A
34
Q

what is another name of Hemochromatosis?

A

Bronzed DM

35
Q

Def of Hemochromatosis

A
  • Inherited condition leading to increased total body iron with excess deposition in tissues.
  • Total body stores of iron increased to 20-40 g (normal 1 g)
36
Q

Pathology of Hemochromatosis

A
37
Q

Etiology of Hemochromatosis

A
  1. Primary (hereditary) hemochromatosis
  2. Secondary hemochromatosis
     Parenteral iron overload (e.g. transfusions)
     Chronic hemolytic anemia: thalassemia.
     Excessive iron intake
     Sidroblastic anemia
38
Q

Causes of Secondary Hemochromatosis

A
  • Parenteral iron overload (e.g. transfusions)
  • Chronic hemolytic anemia: thalassemia.
  • Excessive iron intake
  • Sidroblastic anemia
39
Q

CP of Hemochromatosis

A
40
Q

Investigations for Hemochromatosis

A
  • Blood Tests
  • Genetic Testing
  • bx
41
Q

Blood tests in Hemochromatosis

A
42
Q

Genetic Testing in Hemochromatosis

A

for the specific genetic markers —> HFE gene

43
Q

Bx in Hemochromatosis

A
44
Q

TTT of Hemochromatosis

A
45
Q

Main TTT of Hemochromatosis

A

phlebotomy

46
Q

Alternative therapy in Hemochromatosis

A
47
Q

First steps in suspected NAFLD

A
48
Q

What is a fibroscan?

A
  • Fibro Scan is an imaging-based diagnostic test developed by Echosens that looks at liver fibrosis (stiffness, scarring) and steatosis (fat) using transient- elastography technology.
  • Fibro Scan can be used on patients in need of staging of their NAFLD/NASH
49
Q

FibroScan Score Ranges

A