ACP-L21 & 22 HBP Pathology

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Questions and Answers

What is the most common cause of portal hypertension?

  • Portal vein thrombosis
  • Compression by mass lesion
  • Cirrhosis (correct)
  • Hepatic vein thrombosis

Which condition is associated with non-cirrhotic portal hypertension?

  • Budd-Chiari syndrome
  • Heart failure
  • Schistosomiasis (correct)
  • Congenital stenosis

What is a significant physiological change in the case of portal hypertension secondary to cirrhosis?

  • Decreased blood flow to the portal vein
  • Decreased endothelin-1 production
  • Increased nitric oxide production
  • Increased vascular resistance (correct)

Which of the following is NOT classified as a cause of pre-hepatic portal hypertension?

<p>Cirrhosis (B)</p> Signup and view all the answers

Which response to phenobarbital is generally observed in cases of moderately to severely decreased UDP-GT activity?

<p>No response (C)</p> Signup and view all the answers

What is the primary vascular supply to the liver?

<p>Portal vein (B)</p> Signup and view all the answers

What is a clinical consequence of portal hypertension related to the lower oesophagus?

<p>Esophageal varices (C)</p> Signup and view all the answers

Which route for liver biopsy is considered safer and avoids certain risks?

<p>Transjugular biopsy (B)</p> Signup and view all the answers

What does the term 'caput medusae' refer to in the context of liver pathology?

<p>Dilation of veins in the abdomen (B)</p> Signup and view all the answers

In liver histology, what is the central structure of the centrilobular region?

<p>Central vein (D)</p> Signup and view all the answers

Which symptom is NOT commonly associated with impaired liver function?

<p>Elevated blood pressure (B)</p> Signup and view all the answers

What is the role of macrophages in bilirubin metabolism?

<p>Decompose old red blood cells (B)</p> Signup and view all the answers

Which of the following is a potential risk of a percutaneous liver biopsy?

<p>Tumor seeding (A)</p> Signup and view all the answers

What is the primary location of the liver in relation to the rib cage?

<p>Between right 5th and 10th ICS (C)</p> Signup and view all the answers

Which of the following is a consequence of impaired protein synthesis in the liver?

<p>Bruising (A)</p> Signup and view all the answers

What is the incubation period for Hepatitis A virus (HAV)?

<p>15-45 days (B)</p> Signup and view all the answers

Which of the following viruses is associated with parenteral transmission?

<p>Hepatitis B (C)</p> Signup and view all the answers

What defines a fatty liver in terms of steatosis percentage?

<p>Steatosis ≥ 5% of hepatocytes (D)</p> Signup and view all the answers

Which condition is characterized by both steatosis and ballooning degeneration of liver cells?

<p>Alcoholic steatohepatitis (B)</p> Signup and view all the answers

What is the most common cause of chronic hepatitis in Hong Kong?

<p>Non-alcoholic fatty liver disease (D)</p> Signup and view all the answers

Which of the following is NOT associated with metabolic syndrome?

<p>High-density lipoprotein (A)</p> Signup and view all the answers

What is the primary treatment recommendation for individuals with alcoholic liver disease?

<p>Abstinence from alcohol (A)</p> Signup and view all the answers

Which statement about the epidemiology of NAFLD is true?

<p>It is associated with central obesity. (A)</p> Signup and view all the answers

What is the primary purpose of a transjugular intrahepatic portosystemic shunt (TIPS)?

<p>To manage portal hypertension (A)</p> Signup and view all the answers

What physiological process is triggered by variceal bleeding leading to protein degradation?

<p>Resorption of internal bleeding (B)</p> Signup and view all the answers

Cirrhosis involves the formation of what type of tissue structure?

<p>Bridging fibrous septa with regenerative nodules (D)</p> Signup and view all the answers

Which classification of cirrhosis is commonly associated with viral hepatitis?

<p>Macro-nodular cirrhosis (A)</p> Signup and view all the answers

What is the current understanding of the reversibility of cirrhosis?

<p>It is potentially reversible (A)</p> Signup and view all the answers

What type of renal failure is characterized as pre-renal?

<p>Reduced blood flow to the kidneys (D)</p> Signup and view all the answers

What effect does cirrhosis have on hepatocyte regeneration?

<p>It leads to fibrosis in conjunction with regenerative nodules (C)</p> Signup and view all the answers

Which of these is an incorrect statement about cirrhosis?

<p>All forms of cirrhosis are reversible. (B)</p> Signup and view all the answers

What is the typical sex ratio for Primary Biliary Cholangitis (PBC)?

<p>1:9 (C)</p> Signup and view all the answers

Which of the following antibodies is associated with Primary Sclerosing Cholangitis (PSC)?

<p>Anti-neutrophil cytoplasmic antibodies (ANCA) (B)</p> Signup and view all the answers

At what age does Primary Biliary Cholangitis typically onset?

<p>40-60 years (C)</p> Signup and view all the answers

What histological finding is indicative of Primary Biliary Cholangitis?

<p>Portal tract granuloma (D)</p> Signup and view all the answers

Which condition is commonly associated with Primary Biliary Cholangitis?

<p>Sjogren syndrome (C)</p> Signup and view all the answers

What is the main diagnostic procedure for identifying Primary Sclerosing Cholangitis?

<p>Cholangiogram (ERCP/MRCP) (B)</p> Signup and view all the answers

How do the age demographics differ between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis?

<p>PBC affects middle-aged women, while PSC affects younger males (C)</p> Signup and view all the answers

What is a common biochemical finding in Primary Biliary Cholangitis?

<p>Increased alkaline phosphatase (ALP) (B)</p> Signup and view all the answers

Which of the following diseases shows a 'beads on string' appearance on imaging?

<p>Primary Sclerosing Cholangitis (D)</p> Signup and view all the answers

Which of the following is a feature that distinguishes Primary Biliary Cholangitis from Primary Sclerosing Cholangitis?

<p>Seropositivity for Anti-mitochondrial antibodies (AMA) (D)</p> Signup and view all the answers

Which symptom is associated with impaired oestrogen metabolism?

<p>Palmar erythema (D)</p> Signup and view all the answers

What complication can arise from liver failure?

<p>Hepatorenal syndrome (D)</p> Signup and view all the answers

What type of bilirubin increase is seen in pre-hepatic jaundice?

<p>Unconjugated bilirubin (C)</p> Signup and view all the answers

Which imaging or laboratory results would be most useful to diagnose intrahepatic cholestasis?

<p>Check ALP &amp; GGT (C)</p> Signup and view all the answers

What condition is characterized by tea-colored urine and pale stool?

<p>Post-hepatic jaundice (A)</p> Signup and view all the answers

What is the bilirubin level threshold for clinically detectable hepatic jaundice?

<blockquote> <p>35 µmol/L (D)</p> </blockquote> Signup and view all the answers

What is a common cause of post-hepatic jaundice?

<p>Gallstones (B)</p> Signup and view all the answers

Which syndrome is associated with a mutation in UGT1A1 gene?

<p>Gilbert's syndrome (C)</p> Signup and view all the answers

Which condition can lead to the presentation of caput medusae?

<p>Portal hypertension (C)</p> Signup and view all the answers

How does cholestasis affect bilirubin levels in intrahepatic conditions?

<p>Bilirubin levels may not be increased (D)</p> Signup and view all the answers

What type of jaundice occurs when there is inadequate uptake and conjugation of bilirubin?

<p>Hepatic jaundice (D)</p> Signup and view all the answers

What is the result of ineffective erythropoiesis in relation to bilirubin levels?

<p>Increase in unconjugated bilirubin (A)</p> Signup and view all the answers

What typically defines the clinical presentation of Crigler-Najjar type I?

<p>Jaundice appears early in life (D)</p> Signup and view all the answers

What is the underlying issue in Gilbert's syndrome?

<p>Defective glucuronosyltransferase activity (D)</p> Signup and view all the answers

Flashcards

Liver location

The liver is located between the right 5th intercostal space and below the costal margin, at the mid-clavicular line.

Liver structure

The liver has 2 lobes, divided into 8 segments, and is covered by Glisson's capsule. It has a hexagonal structure.

Liver blood supply

The liver receives blood from both the portal vein (75%) and the hepatic artery (25%).

Portosystemic anastomosis

These are connections between the portal vein and the systemic circulation. There are 6 important ones.

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Oesophageal varices location

Oesophageal varices are a consequence of portal hypertension, occurring in the lower oesophagus.

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Liver biopsy indications

Liver biopsies are used to diagnose liver masses with inconclusive imaging, stage known liver disease, and plan treatment.

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Liver biopsy route - percutaneous

A percutaneous liver biopsy is done through the skin, but it carries risks like tumour seeding and bleeding.

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Bilirubin metabolism

Bilirubin, produced by the breakdown of red blood cells, is processed by the liver. It can then be excreted into bile or urine.

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Chronic Liver Disease (Stigmata)

Signs of chronic liver disease include jaundice, pruritus, and ascites.

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Portal Hypertension consequence

Portal hypertension causes varices in the oesophagus, and can lead to caput medusae and haemorrhoids.

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Jaundice

Yellowing of the skin and eyes due to high bilirubin levels in the blood.

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Hyperbilirubinemia

High levels of bilirubin in the blood.

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Pre-hepatic jaundice

Jaundice caused by increased bilirubin production.

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Hepatic jaundice

Jaundice due to liver dysfunction.

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Post-hepatic (obstructive) jaundice

Jaundice caused by blockage in bile ducts.

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Unconjugated bilirubin

Bilirubin that hasn't been processed by the liver.

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Conjugated bilirubin

Bilirubin that has been processed by the liver.

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Gilbert syndrome

Inherited condition causing mild jaundice.

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Portal hypertension

High blood pressure in the portal vein, leading to complications like esophageal varices.

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Hepatic encephalopathy

Brain dysfunction caused by liver failure.

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Hepatorenal syndrome

Kidney malfunction caused by liver failure.

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Ascites

Fluid buildup in the abdomen caused by liver disease.

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Splenomegaly

Enlarged spleen.

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Hematemesis

Vomiting blood.

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Esophageal varices

Swollen veins in the esophagus.

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What's the main cause of kernicterus?

Kernicterus is a serious neurological condition caused by high levels of bilirubin in the bloodstream. It can be fatal. The cause is a deficiency in the UDP-glucuronosyltransferase (UDP-GT) enzyme, which is responsible for processing bilirubin.

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What are the signs of portal hypertension?

Portal hypertension refers to increased pressure within the portal vein system. It can arise from pre-hepatic (before liver), hepatic (within liver), or post-hepatic (after liver) causes.

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How does cirrhosis cause portal hypertension?

Cirrhosis, a scarring of the liver, leads to portal hypertension due to increased vascular resistance and functional vasoconstriction.

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What is pre-hepatic portal hypertension?

Pre-hepatic portal hypertension is caused by problems in the portal vein before it reaches the liver. This could be due to thrombosis (blood clot), congenital stenosis (narrowing), or compression from a mass.

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What is post-hepatic portal hypertension?

Post-hepatic portal hypertension is caused by problems after the blood leaves the liver, such as heart failure, venous thrombosis, or the Budd-Chiari syndrome.

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What is cirrhosis?

Cirrhosis is a chronic liver disease where healthy liver tissue is replaced by scar tissue, causing the liver to harden and lose its ability to function properly.

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What are the consequences of cirrhosis?

Cirrhosis can lead to portal hypertension, ascites, jaundice, and other complications which can be life-threatening.

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What are the types of cirrhosis?

Cirrhosis is classified into two main types: macro-nodular (e.g., viral hepatitis) and micro-nodular (e.g., alcoholic fatty liver disease).

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What causes a transjugular intrahepatic portosystemic shunt (TIPS)?

A TIPS procedure is done to relieve portal hypertension by creating a new channel connecting the portal vein to the hepatic vein, bypassing the damaged liver.

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When is a TIPS procedure used?

A TIPS procedure is usually done when there's significant portal hypertension, often due to cirrhosis, and to prevent complications like variceal bleeding.

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What can cause variceal bleeding?

Resorption of internal bleeding leads to hemoglobin degradation, which in turn releases proteins that can contribute to variceal bleeding.

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What are the possible complications of variceal bleeding?

Complications of variceal bleeding can include anemia due to blood loss, infection, and liver failure.

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What is pre-renal failure?

Pre-renal failure is a condition where the kidneys are not getting enough blood flow, leading to a decreased ability to filter waste.

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What is NAFLD?

Non-alcoholic fatty liver disease (NAFLD) is a common condition where fat builds up in the liver, not caused by excessive alcohol consumption.

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What are the risk factors for NAFLD?

NAFLD is linked to metabolic syndrome, which includes central obesity, hypertension, hyperglycemia, hypertriglyceridemia, and low HDL-C.

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Is NAFLD common?

NAFLD is very common, with a prevalence of 27% in Hong Kong. It is the most frequent cause of chronic hepatitis in HK

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What is the impact of estrogen on NAFLD?

Estrogen has a protective effect against NAFLD, especially in pre-menopausal women.

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What is the difference between NAFLD and ALD?

Both NAFLD and Alcoholic Liver Disease (ALD) share similar pathological features, often indistinguishable.

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What is a macrovesicular steatosis?

In macrovesicular steatosis, fat accumulates within liver cells, displacing the nucleus.

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What are the manifestations of NAFLD?

NAFLD can be asymptomatic or cause hepatomegaly and mildly deranged liver function tests.

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How is NAFLD treated?

There is no specific cure for NAFLD, but lifestyle modifications like weight loss, exercise, and a healthy diet are crucial.

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Primary Biliary Cholangitis (PBC)

A chronic autoimmune disease characterized by the destruction of small bile ducts in the liver, causing inflammation and scarring. This often leads to liver failure.

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Primary Sclerosing Cholangitis (PSC)

An autoimmune disease that causes inflammation and scarring of the bile ducts, both inside and outside the liver. It can lead to cholestasis (bile buildup) and liver failure.

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What are the key differences between PBC and PSC?

PBC mainly affects intrahepatic bile ducts (within the liver), while PSC involves both intrahepatic and extrahepatic bile ducts (outside the liver). PBC is more common in women, while PSC is more common in men. PBC is associated with anti-mitochondrial antibodies, while PSC is associated with anti-neutrophil cytoplasmic antibodies.

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What is the 'beads on a string' appearance?

This describes the characteristic appearance of the bile ducts in a cholangiogram (imaging test) of someone with PSC. The bile ducts are narrowed and appear like beads strung together.

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What is the significance of the 'beads on a string' appearance?

This appearance on a cholangiogram is highly suggestive of PSC, helping in its diagnosis. It indicates the specific pattern of bile duct scarring and narrowing characteristic of the disease.

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What are the common associated diseases with PBC?

PBC often occurs alongside Sjogren's syndrome, a condition that causes dryness of the eyes and mouth.

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What are the common associated diseases with PSC?

PSC is often linked to inflammatory bowel diseases such as ulcerative colitis and Crohn's disease.

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What are cholangiograms and how are they used?

Cholangiograms are imaging tests that visualize the bile ducts. They can be performed endoscopically (ERCP) or using magnetic resonance imaging (MRCP). They help diagnose PSC and other biliary diseases.

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What is the significance of elevated Alkaline Phosphatase (ALP) level?

An elevated ALP level in the blood is often a sign of liver disease, particularly PBC and PSC. It indicates that the bile ducts are not functioning properly, leading to an accumulation of bile.

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What are some common symptoms of PBC and PSC?

Common symptoms include fatigue, jaundice (yellowing of skin), itching, abdominal pain, and dark urine. However, many patients are asymptomatic in the early stages.

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Study Notes

ACP-L21 & 22 HBP Pathology

  • Liver Anatomy & Physiology:

    • Heaviest visceral organ, ~1500g
    • Located between the 5th right intercostal space and below the costal margin, at the mid-clavicular line
    • Composed of 2 lobes and 8 segments
    • Covered by Glisson's capsule
    • Vascular supply: 75% portal vein, 25% hepatic artery
    • Contains 6 portosystemic anastomoses (HUS1)
  • Liver Location and Drainage:

    • Location: Lower oesophagus, umbilicus, left gastric vein, (portal),anal canal, inferior/middle rectal vein, and superior rectal vein.
    • Superficial epigastric vein and para-umbilical vein are also associated with the systemic drainage.
    • Portal drainage occurs via the portal vein.
    • Consequences of portal hypertension:
      • Oesophageal varices
      • Haemorrhoids
      • Caput medusae
  • Liver Histology:

    • Hexagonal structure (theoretical)
    • Centrilobular region: Contains central vein
    • Periportal region: Characterized by portal tracts, containing portal vein, hepatic artery, and bile duct
    • Trabeculae: 1-2 cells thick
  • Bilirubin Metabolism:

    • Bilirubin is produced from the breakdown of hemoglobin.
    • Unconjugated bilirubin (lipid-soluble) is transported in the blood bound to albumin.
    • The liver conjugates bilirubin, making it water-soluble, for excretion in bile.
    • Conjugated bilirubin is excreted into the bile, then the small and large intestine, where it is converted to urobilinogen.
    • Urobilinogen is then transformed into urobilin, which imparts the characteristic color to urine.
  • Liver Biopsy:

    • Indications: Diagnosing liver masses with inconclusive imaging results (30-60%) or staging known parenchymal liver disease.
    • Routes:
      • Percutaneous (risk of tumor seeding, bleeding, and organ puncture)
      • Transjugular (safer, via IJV→SVC→IVC)
      • Open wedge (laparoscopy).

Stigmata of Chronic Liver Disease

  • General: Impaired liver function, protein synthesis, biliary excretion, and waste metabolism. Associated symptoms such as malaise, fatigue, anorexia, weight loss, RUQ pain.
  • Jaundice: Yellowing of the sclera; characterized by different types of bilirubin (conjugated, unconjugated).
  • Portal Hypertension: Increased pressure in the portal venous system
    • Pre-hepatic: Thrombosis, stenosis, or compression of the portal vein
    • Hepatic: Cirrhosis, schistosomiasis, or granulomatous diseases
    • Post-hepatic: Right heart failure, tricuspid regurgitation or inferior vena cava (IVC) or hepatic vein thrombosis
  • Ascites: Excessive fluid in the peritoneal cavity. Symptoms include sodium retention, water retention, reduced systemic/local vasodilators to increased local vasoconstrictors, dilutional hyponatremia
  • Hepatorenal syndrome: Pre-renal failure due to intense renal vasoconstriction; influenced by RAAS and SNS
  • Hepatic encephalopathy: Hyperammonemia due to decreased functional hepatocytes.

Cirrhosis

  • Definition: Diffuse process of regenerative nodules surrounded by bridging fibrous septa.
  • Types: Macro-nodular and micro-nodular.
  • Child-Pugh scoring system: A clinical and laboratory parameter to assess and monitor the severity of cirrhosis.

Infectious Liver Disease

  • Viral Hepatitis (HAV, HBV, HCV, HDV, HEV) causes of infectious hepatitis.
    • Features: transmission routes, incubation periods, risk of chronic hepatitis, and HCC.
  • Acute Hepatitis: Viral infection duration <6 months, outcome can be remission or progression
  • Chronic Hepatitis: Viral infection ≥6 months
  • Non-viral Hepatitis: Infectious hepatitis caused by bacteria or parasites

Alcoholic Liver Disease

  • Etiology and Epidemiology: Related to significant/excessive alcoholic beverages intake .
  • Classification:
  • Steatosis (Fatty Liver): Fat accumulation in the liver (5% or more)
  • Steatohepatitis (ASH): Steatosis + inflammation, and liver damage

Non-alcoholic Fatty Liver Disease (NAFLD)

  • Definition: Accumulation of fat in the liver in the absence of significant alcohol consumption.
  • Epidemiology: Common, often associated with metabolic syndrome (obesity, hypertension, hyperglycemia, hypertriglyceridemia, and low HDL-C).
  • Manifestations: Usually asymptomatic, but can progress to NASH (nonalcoholic steatohepatitis) and cirrhosis if left untreated.

Drug-induced Liver Injury (DILI)

  • Definition: Liver damage/injury caused by medications.

Metabolic Liver Diseases

  • Inherited disorders: Genetic mutations affecting the way iron and copper are managed in the body (e.g. HFE haemochromatosis, Wilson's disease).
    • HFE haemochromatosis: An iron overload disease, characterized by elevated transferrin saturation, increased serum ferritin and/or hepatic iron index
    • Wilson's Disease: Copper accumulation in the liver or other organs; characterized by low serum ceruloplasmin, and high urine copper levels.

Malignant HBP Tumours

  • Epidemiology / Risk Factors: HCC (Hepatocellular Carcinoma), associated with cirrhosis, chronic hepatitis B/C, aflatoxin B1, alcohol, NAFLD.
  • Pathology: Varying cell structure and architecture

Pancreatic Disease

  • Acute pancreatitis: Inflammation of the pancreas, often caused by gallstones or alcohol abuse.
  • Chronic pancreatitis: Ongoing inflammation/damage of the pancreas, often causing pancreatic insufficiency.
  • Pancreatic cancer: Malignant tumour of the pancreas, frequently in head of pancreas.

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