Liver

Question 1

Primary Biliary Cholangitis

Answer 1

• WOMEN 10:1
• Anti-mitochondrial (M2) antibodies
• Histology: Bile duct loss + GRANULOMAS
• USS -> NO bile duct dilatation
• Autoimmune INFLAMMATORY destruction of intrahepatic bile ducts
• Chronic dmg to bile ducts leads to fibrosis -> cirrhosis

Question 2

Primary Sclerosing Cholangitis

Answer 2

• MEN
• IBD (UC)
• p-ANCA
• FIBROSIS (sclerosis) causing destruction of bile ducts
• Increased risk of cholangiocarcinoma
• Diagnosis = Bile duct imaging

Question 3

Wilson’s Disease

Answer 3

• Chromosome 13
• Kayser-Fleischer rings (copper deposits in cornea)
• Failure of hepatocyte excretion of COPPER

Can present in children >2yo as Acute Liver Failure

Question 4

Haemochromatosis

Answer 4

• Chromosome 6 - HFE gene
• IRON deposits in organs
• a.k.a. Bronzed Diabetes

Can present in children <2yo as Acute Liver Failure

Question 5

Alpha 1 antitrypsin deficiency (A1AT)

Answer 5

• Affects lungs + liver
  
  • Lungs: Lower lobe Emphysema
    
    • Classically COPD Sx in a young non-smoker
  • Liver: Cirrhosis + Hepatocellular Carcinoma

Can present in older children as Acute Liver Failure (rare)

Question 6

Classic examples of drugs taht cause damage to liver, and what zone of liver is mainly affected?

Answer 6

• Methotrexate, Paracetamol
• ZONE 3 = Involved in drug metabolism
  
  • Zone 3 also affected in Alcoholic liver disease

Question 7

Causes of Micronodular and Macronodular cirrhosis

Answer 7

MICRO (nodules <3mm) = Alcoholic hepatitis, biliary tract disease

MACRO (>3mm) = Viral hepatitis, Wilson’s, A1AT

Question 8

Which scoring system is used to determine prognosis of cirrhosis in children?

Answer 8

Modified Pugh’s score

ABCDE:

• Albumin
• Bilirubin
• Clotting (PT)
• Distension (Ascites)
• Encephalopathy

Pugh A = Good 5yr survival

Pugh C = BAD

Question 9

Fatty liver characteristics

Answer 9

Large, pale, yellow, greasy liver

FAT DROPLETS in hepatocytes (steatosis)

FULLY REVERSIBLE

Question 10

Alcoholic Hepatitis characteristics

Answer 10

FIBROTIC liver

Hepatocyte BALLOONING

Mallory Denk Bodies

Affects Zone 3 of liver

Question 11

Alcoholic Cirrhosis

Answer 11

Large yellow/fatty -> SHRINKS + non-fatty + BROWN

MICRONODULAR cirrhosis

Question 12

Auto-immune Hepatitis

Answer 12

• Active chronic hepatitis w/ PLASMA CELLS
• WOMEN
• HLA-DR3
• Type 1 = ANA, Anti smooth muscle actin Ig, anti-soluble liver Ig
• Type 2 = Anti-LKM

Question 13

Most common benign liver tumour?

Answer 13

Haemangioma

Question 14

Liver cell (Hepatic) Adenoma is associated with

Answer 14

COCP (Oestrogen) - hence more common in WOMEN

Question 15

Most common Malignant tumour of liver?

Answer 15

Metastatic Adenocarcinoma (Secondary tumour)

Usually from GI tract, breast or bronchus

Question 16

Hepatocellular carcinoma

Answer 16

• Common in Chronic Liver Disease (viral, alcoholic, NAFLD, etc)
• Ix = Alpha-fetoprotein, USS

Question 17

Cholangiocarcinoma

Answer 17

• Main causes:
  
  • PSC (Sclerosing)
  • Worms
  • Cirrhosis
• Can be intra or extrahepatic

Question 18

Gilberts

Answer 18

• Raised Bilirubin but normal LFTs
• Autosomal Recessive
• 5-6% of population (1 in 20)
• Worsened by fasting
• UDPGT activity reduced

Question 19

35yo male, chronic alcohol intake, often appeared drunk to A&E

Nausea + abdo pain + jaundice

Bilirubin 90

ALP 200, AST 1500, AST 750