Hepatobiliary System

Question 1

What are the diseases where Mallory Hyaline bodies are found?

Answer 1

New Indian WATCH

1. NASH
2. Indian Childhood Cirrhosis
3. Wilson’s disease
4. ALD (m/c)
5. Tumor- HCC
6. Cirrhosis- Primary Biliary Cirrhosis
7. Hyperplasia- Focal Nodular Hyperplasia

Question 2

What are the morphological features of ALD.

Answer 2

ALD includes:
1. Alcoholic steatosis
Gross: Enlarged, soft, greasy and yellow liver
Microscopy:
Fat vacuoles within the hepatocytes, which coalesce to form larger droplets.
M/c is macrovesicular steatosis
Absence of inflammation of fibrosis

2. Alcoholic Hepatitis
   Gross- Enlarged, yellow and slightly firm due to fibrosis
   a) Ballooning degeneration of hepatocytes
   b) Mallory Hyaline - Intermediate filaments CK8 and CK18
   c) Steatosis
   d) Inflammation (mainly neutrophilic, plus lymphocytic+macrophages)
   e) Fibrosis
   Starts as perivenular(perisinusoidal) —> periportal —> bridging fibrosis —> cirrhosis
3. Alcoholic Cirrhosis
   - Regerating nodules -macro or micro
   - Fibrosis (bands or scars surrounding the nodules)
   - Loss of normal architecture (entire liver)

Question 3

Name 2 liver diseases where Mallory Hyaline is not seen.

Answer 3

1. Hemochromatosis

2. Primary Sclerosing Cholangitis

Question 4

Briefly mention the morphological features of Acute and Chronic Hepatitis.

Answer 4

ACUTE HEPATITIS
- Gross: Enlarged or normal. Cut surface is muddy-red, mushy with yellow-green discoloration d/t jaundice

• Microscopy:
  
  1. Hepatocyte injury (Ballooning degeneration, Dropout necrosis, Acidophil/Councilman bodies)
  2. Inflammation (mainly lympho, mixed with plasma cells and eosino)
  3. Kupffer Cells- Hypertrophy & hyperplasia & contain Lipofuschin (brown pigment)
  4. Lobular disarray- loss of normal architecture

CHRONIC HEPATITIS
Gross: Normal initially; later shrunken and firm due to fibrosis.

Microscopy:

1. Portal inflammation (mainly lymphoplasmacytic)
2. Interface hepatitis
3. Lobular Inflammation and necrosis
4. Fibrosis - Hallmark of chronic liver damage- starts as periportal & progresses to bridging fibrosis

Question 5

Define cirrhosis.

Answer 5

Cirrhosis is an end stage of any chronic liver disease. It is a diffuse process (entire liver is involved) characterized by fibrosis and conversion of normal architecture to structurally abnormal regenerating nodules of liver cells.

Question 6

Hemochromatosis — definition, classification, Pathogenesis, clinical features.

Answer 6

Def: Excessive accumulation of iron in the body.

Classification:

1) Primary/Hereditary: Mutation in HFE/HAMP/HJV/TFR-2. More common in M>F
2) Secondary: Bantu siderosis, blood transfusion, ineffective erythropoiesis (thalassemia)

Pathogenesis:

1) HAMP mutation - Decreased hepcidin- increased iron absorption
2) Iron causes hepatocyte injury by
- Catalysing free radical reactions causing Lipid peroxidation
- Activating stellate cells to cause fibrosis
- Iron and ROS with DNA causing lethal DNA damage

Clinical features:

• Liver: Micronodular cirrhosis
• Pancreas: Diabetes mellitus
• Skin: Bronze like pigmentation (Increased melanin produced)
• Joint: Pseudogout
• Heart: Hemosiderosis
• Pituitary: Decreased production of Gonadotropins leading to atrophy of testes

Question 7

What are the complications of gallstones?

Answer 7

In gall bladder:
1. Cholecystitis: It can cause both acute and chronic cholecystitis. Rarely it may cause gangrenous or emphysematous cholecystitis

2. Empyema of gallbladder: distention of gallbladder with pus.
3. Hydrops : distended with clear watery fluid.
4. Mucocele: distended by cloudy, mucoid material
5. Perforation of gallbladder
6. Carcinoma: Gallstones increase the risk of carcinoma of gallbladder

Biliary tree:

1. Biliary obstruction (obstructive cholestasis).
2. Acute cholangitis (inflammation of the biliary tree)
3. Acute pancreatitis

In the Intestine

1. Biliary fistulas: Fistula may develop between biliary system and bowel or gallbladder and skin
2. Gallstone ileus/ Bouveret’s syndrome: Rarely a gallstone erodes into an adjacent loop of small intestine— intestinal obstruction

Question 8

Differences between Brown stones and Black stones.

Answer 8

1. Black= Ca bilirubinate+ Ca phosphate/ carbonate+ Mucin
   Brown= Ca bilirubinate+ Ca palmitate+ Mucin
2. Black in chronic hemolytic states, cirrhosis
   Brown in biliary stasis and infection
3. Black due to increase in unconjugated bilirubin.
   Ca precipitates with bilirubin forming Ca bilirubinate.
   Ca bilirubinate precipitates around a nucleus of mucin

Brown-Stasis of bile—
Infection— microbes produce:
a)Beta glucuronidase- Conjugated to unconjugated bilirubin conversion.
Unconjugated+Ca= Ca bilirubinate
b)Phospholipase—Phospholipid to fatty acid
Ca + fatty acid = Ca palmitate
Ca palmitate and bilirubinate precipitate around nucleus of dead bacteria, parasites
c) Hydrolase - Deconjugates Bile acids= Free bile salts

Question 9

Types of stones according to location.

Answer 9

1. Intrahepatic: Mostly brown stones
2. Gallstones: Mostly cholesterol stones
3. Choledocholithiasis (Bile duct stones): Mostly mixed stones