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clinical alimentary system > 3.6. Liver + Biliary Tree Disease - Primary Biliary Cirrhosis > Flashcards

3.6. Liver + Biliary Tree Disease - Primary Biliary Cirrhosis Flashcards

1
Q

What is the known Aetiology of Primary Biliary Cirrhosis?

A

Trick Question - It is Unknown

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2
Q

What is the Suspected Aetiology of Primary Biliary Cirrhosis?

A

An autoimmune disorder

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3
Q

Why is this thought to be an Autoimmune disorder?

A

Due to immunological mechanisms playing a part

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4
Q

Is Primary Biliary Cirrhosis more common in Males or Females?

A

Females

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5
Q

In what age group is Primary Biliary Cirrhosis most apparent?

A

40-50 years old

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6
Q

What is the term used for an early, preceding lesion?

A

Chronic, Non-Suppurative, Destructive Cholangitis

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7
Q

What does Non-Suppurative mean?

A

Without pus formation on inflammation

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8
Q

What does the preceding lesion highlight?

A

That it is only Late-Stage which ends in Cirrhosis

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9
Q

What is the Pathology of Primary Biliary Cirrhosis?

A

There is a progressive destruction of the Small Bile Ducts, eventually leading to Cirrhosis

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10
Q

What is found in most patients with Primary Biliary Cirrhosis?

A

Serum Anti-Mitochondrial Antibodies (AMA positive disease)

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11
Q

What causes the initiation of the Pathology of Primary Biliary Cirrhosis?

A

It is most likely that an Environmental Factor acts on a Genetically Predisposed Host - via Molecular Mimicry - initiating autoimmunity

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12
Q

What does the Molecular Mimicry (initiating the Autoimmunity) cause?

A

Granulomatous Formation

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13
Q

What does Granulomatous Formation result in?

A

Reduction in the effectiveness of the Intrahepatic Ducts, eventually leading to Death and Cirrhosis

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14
Q

What are the Clinical Signs of Primary Biliary Cirrhosis?

A
  1. Serum Anti-Mitochondrial Antibodies
  2. A Raised Serum Alkaline Phosphatase (ALP)
  3. Hepatomegaly
  4. Xanthelasma (and other Cholesterol Deposits)
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15
Q

Why is there raised Serum Alkaline Phosphatase (ALP)?

A

This is present in Hepatic Canalicular and Sinusoidal Membranes, and is released into the Blood in Intra/Extrahepatic causes of Cholestatic Disease due to an increase in its synthesis

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16
Q

Why does Hepatomegaly occur?

A

Due to the damage and inflammation of the Liver

17
Q

What is Xanthelasma?

A

A Yellowish Fat Deposition around the Skin of the Eye

18
Q

Why does Xanthelasma occur (and other Cholesterol Deposits)?

A

Due to the Liver Inability to deal with Cholesterol any more

19
Q

What are the Symptoms of Primary Biliary Cirrhosis?

A
  1. Asymptomatic (usually)
  2. Pruritus
  3. Jaundice
  4. Fatigue (and other generalized symptoms)
20
Q

How is Primary Biliary Cirrhosis normally discovered, if Asymptomatic?

A
  1. On a routine examination for something else

2. Screening

21
Q

What is Pruritus?

A

Itching

22
Q

Why does Pruritus occur in Primary Biliary Cirrhosis?

A

Due to the Elevated Levels of Bilirubin in the Blood

23
Q

What type of Jaundice will occur in Primary Biliary Cirrhosis?

A

Intrahepatic / Post-Hepatic Jaundice

24
Q

What investigations are required for Primary Biliary Cirrhosis?

A
  1. Liver Function Test (and Biochemistry)
  2. Serum Anti-Mitochondrial Antibody (AMA) test
  3. Abdominal Ultrasound
  4. Magnetic Resonance Cholangio-Pancreatography (MRCP) with Biopsy
25
Q

What is the purpose of the Liver Function Test (and Biochemistry)

A
  1. To assess for Alkaline Phosphatase Levels

2. To look at the Blood Lipid and IgM content

26
Q

What is the purpose of the Serum Anti-Mitochondrial Antibody test?

A

To look for the associated antibodies with this condition

27
Q

What is the purpose of the Abdominal Ultrasound / MRCP with Biopsy?

A

To assess if the Common Bile Duct is effected

28
Q

What is the treatment for Primary Biliary Cirrhosis?

A
  1. Vitamin Supplements
  2. Ursodeoxycholic Acid
  3. Antihistamine / Cholestryamine
29
Q

What is the purpose of giving Vitamin Supplements?

A

As these can, sometimes, not be readily absorbed due to the decrease in Bile Secretion

30
Q

What Vitamins will need to be Supplemented?

A
  1. Vitamin A
  2. Vitamin D
  3. Vitamin E
  4. Vitamin K
31
Q

What is the purpose of giving Ursodeoxycholic Acid?

A

This improves Bilirubin and Aminotransferase Levels

32
Q

What is the purpose of giving Antihistamines / Cholestryamine?

A

Symptomatic Relief of Pruritus (itching)

clinical alimentary system (64 decks)

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