Liver Function and Disorders
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Questions and Answers

Which function of the liver is primarily responsible for the conversion of ammonia into urea?

  • Detoxification
  • Deamination of amino acids (correct)
  • Bilirubin metabolism
  • Protein synthesis
  • What process describes the formation of glucose from non-carbohydrate sources in the liver?

  • Lipogenesis
  • Gluconeogenesis (correct)
  • Glycogenolysis
  • Glycogenesis
  • What role does the hepatic portal vein play in liver function?

  • Supplies nutrients from the gastrointestinal tract to the liver (correct)
  • Transports bile from the liver to the intestine
  • Carries oxygen-rich blood to the liver
  • Drains waste products from the liver
  • Which component is NOT part of bile produced by the liver?

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

    The liver plays a critical role in lipid metabolism by converting carbohydrates into which of the following?

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

    What is the main genetic inheritance pattern of Gilbert Syndrome?

    <p>Autosomal recessive (C)</p> Signup and view all the answers

    What is the primary laboratory finding in Crigler-Najjar Syndrome Type I?

    <p>Elevated total bilirubin (D)</p> Signup and view all the answers

    Which symptom is NOT commonly associated with Dubin-Johnson Syndrome?

    <p>Severe unconjugated hyperbilirubinemia (D)</p> Signup and view all the answers

    What is the remaining enzymatic activity of glucuronyl transferase in Gilbert Syndrome?

    <p>30% (A)</p> Signup and view all the answers

    How does Crigler-Najjar Syndrome Type II differ from Type I?

    <p>It has normal liver enzymes (A)</p> Signup and view all the answers

    Which of the following best describes the function of Alanine aminotransferase (ALT)?

    <p>Indicator of hepatocyte integrity and acute damage (B)</p> Signup and view all the answers

    What is the primary clinical significance of elevated indirect (unconjugated) bilirubin?

    <p>Hemolysis and impaired liver function (C)</p> Signup and view all the answers

    Which statement about Alkaline Phosphatase (ALP) is incorrect?

    <p>ALP is specific to liver function only. (A)</p> Signup and view all the answers

    What condition is characterized by an excess of bilirubin greater than 50 µmol/L?

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

    Which of the following causes an increase in prothrombin time (PT)?

    <p>Impaired hepatic synthesis function (C)</p> Signup and view all the answers

    What substance is primarily produced by the liver and has a half-life of around 20 days?

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

    Which of the following conditions is NOT typically associated with an increase in Alkaline Phosphatase (ALP)?

    <p>Viral hepatitis (D)</p> Signup and view all the answers

    What causes an increase in indirect bilirubin levels?

    <p>Impaired liver function or hemolysis (D)</p> Signup and view all the answers

    Which condition is particularly associated with increased levels of Gamma-Glutamyl Transpeptidase (GGT)?

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

    What is a common cause of acute liver disease?

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

    Which type of liver disease progresses to cirrhosis as a late stage?

    <p>Nonalcoholic fatty liver disease (C)</p> Signup and view all the answers

    What condition results from advanced cirrhosis of the liver?

    <p>Liver failure (D)</p> Signup and view all the answers

    Which method is considered the golden standard for diagnosing liver conditions?

    <p>Liver biopsy (D)</p> Signup and view all the answers

    What could lead to increased blood ammonia levels in acute liver disease?

    <p>Acute hepatic failure (D)</p> Signup and view all the answers

    Which laboratory test is not recommended when platelet count is low?

    <p>Liver biopsy (D)</p> Signup and view all the answers

    What is a primary consequence of chronic liver damage?

    <p>Progression to cirrhosis (D)</p> Signup and view all the answers

    What is the most common symptom experienced by individuals diagnosed with hereditary hemochromatosis?

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

    Which laboratory finding is commonly associated with hereditary hemochromatosis?

    <p>Transferrin saturation of 70% or greater (B)</p> Signup and view all the answers

    What is a distinguishing feature of Wilson disease compared to hereditary hemochromatosis?

    <p>Presence of Kayser-Fleischer rings (C)</p> Signup and view all the answers

    What condition is NOT typically associated with hereditary hemochromatosis?

    <p>Active hepatitis (C)</p> Signup and view all the answers

    In Wilson disease, what is the consequence of the body’s inability to excrete excess copper?

    <p>Damage to the liver and other organs (C)</p> Signup and view all the answers

    What is the primary mode of transmission for Hepatitis A?

    <p>Fecal-oral route (C)</p> Signup and view all the answers

    Which symptom is commonly associated with acute hepatitis?

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

    What is a potential complication of chronic Hepatitis C?

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

    Which laboratory test is specific for diagnosing Hepatitis B?

    <p>Hepatitis B surface antigen (HBsAg) (A)</p> Signup and view all the answers

    Hepatitis C is primarily transmitted through which of the following?

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

    What percentage of adults typically recover from acute Hepatitis B within 6 months?

    <p>70% to 80% (C)</p> Signup and view all the answers

    Which type of hepatitis virus does NOT have a vaccine available?

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

    What is the greatest risk period for transmission of Hepatitis A?

    <p>2 weeks before symptoms appear (C)</p> Signup and view all the answers

    Flashcards

    Liver Function: Carbohydrate Metabolism

    The liver plays a crucial role in regulating blood glucose levels, using hormones like insulin and glucagon to control glycogen storage and breakdown (glycogenolysis and gluconeogenesis).

    Liver Function: Lipid Metabolism

    The liver manufactures lipoproteins, processes fatty acids and triglycerides for energy, changes carbohydrates and proteins into fats, and produces bile from cholesterol.

    Liver Function: Protein Synthesis

    The liver is essential for producing plasma proteins, including clotting factors, and breaking down amino acids, converting them into urea, a waste product.

    Bilirubin Metabolism: Role of Liver

    The liver processes bilirubin, a byproduct of red blood cell breakdown. It converts unconjugated bilirubin into a form that can be excreted in bile.

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    Liver Blood Supply

    The liver receives blood from two main sources: the hepatic artery and the hepatic portal vein.

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    Alanine aminotransferase (ALT)

    An enzyme found in liver cells. Elevated levels indicate liver damage.

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    Bilirubin

    A yellowish pigment produced from the breakdown of hemoglobin. Elevated levels can cause jaundice.

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    Alkaline Phosphatase (ALP)

    An enzyme, mostly from the liver and bones. Elevated levels can point to issues with bile flow (cholestasis).

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    Jaundice

    Yellowing of the skin and eyes due to excessive bilirubin.

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    Albumin

    A protein made in the liver, important for maintaining blood pressure and transporting various substances.

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    Prothrombin Time (PT)

    A measure of how quickly blood clots. Elevated PT indicates liver's trouble producing clotting factors.

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    Hemolysis

    Breakdown of red blood cells, releasing bilirubin.

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    Elevated Bilirubin (causes)

    Elevated levels can mean hemolysis, liver disease, or blocked bile ducts.

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    Gilbert Syndrome

    A genetic liver disease with reduced bilirubin conjugation due to decreased glucuronyl transferase activity.

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    Crigler-Najjar Syndrome Type I

    A rare, severe genetic disorder causing no bilirubin conjugation, leading to potentially life-threatening high levels of unconjugated bilirubin.

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    Crigler-Najjar Syndrome Type II

    A less severe genetic disorder with decreased, not absent, bilirubin conjugation, characterized by chronic bilirubinemia.

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    Dubin-Johnson Syndrome

    An autosomal recessive genetic disorder where bilirubin isn't effectively transported out of liver cells, causing conjugated bilirubinemia.

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    Hereditary Hyperbilirubinemia

    A group of genetic conditions that lead to an abnormal accumulation of bilirubin in the blood.

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    GGT

    Gamma-Glutamyl Transpeptidase, a sensitive marker for liver problems, found in hepatocytes' endoplasmic reticulum, involved in detoxification.

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    Acute Liver Disease

    Sudden liver damage caused by poisoning, infection or poor blood flow.

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    Paracetamol Overdose

    A common cause of acute liver disease, resulting from taking too much paracetamol.

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    Chronic Liver Disease

    Long-term liver damage leading to potential liver complications, including cirrhosis.

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    Cirrhosis

    The late stage of chronic liver disease, where liver tissue is scarred, leading to liver failure.

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    Liver Failure

    Liver's inability to perform its normal functions, which can be a consequence of cirrhosis.

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    Liver Biopsy

    A medical procedure/test, which is the best way to diagnose cirrhosis.

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    Chronic Hepatitis

    Long term liver inflammation, a type of liver disease.

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    Hepatitis

    Inflammation of the liver caused by viruses, bacteria, toxins, drugs, or alcohol.

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    Types of Hepatitis Viruses

    There are five main types of hepatitis viruses: A, B, C, D, and E.

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    Acute Hepatitis

    Hepatitis lasting for 6 months or less. It usually involves symptoms like fatigue, jaundice, fever, nausea, dark urine, and pale feces.

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    Hepatitis A Transmission

    Hepatitis A spreads through the fecal-oral route, often through contaminated food or water.

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    Hepatitis B Transmission

    Hepatitis B spreads through contact with infected blood, semen, or vaginal fluids.

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    Hepatitis C Transmission

    Hepatitis C spreads primarily through contaminated blood, such as from blood transfusions (before 1990s) or shared needles.

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    Hepatitis B Vaccine

    A vaccine exists for Hepatitis B, providing protection against infection.

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    Hereditary Hemochromatosis

    A genetic disorder where the body absorbs too much iron, leading to iron overload in organs. This can cause various symptoms if left untreated.

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    What symptoms might a person with hemochromatosis have early on?

    Early symptoms of hemochromatosis include fatigue, sexual dysfunction (impotence), and joint pain (arthralgia).

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    What happens if hemochromatosis is left untreated?

    Untreated hemochromatosis can lead to serious organ damage, diabetes mellitus, and skin discoloration. This can also lead to cirrhosis and liver cancer.

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    Wilson Disease

    A genetic disorder where the body doesn't properly process copper, leading to copper buildup in organs. This causes damage to the liver, brain, and eyes.

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    What are some signs of Wilson Disease?

    Signs of Wilson disease include low serum copper, elevated liver enzymes like AST, ALT, GGT, bilirubin, and decreased ceruloplasmin. Kayser-Fleischer rings in the eyes are also a key indicator.

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    Study Notes

    Liver Disorders

    • Liver has four lobes and hepatic lobules
    • Two primary sources of blood supply are hepatic artery and hepatic portal vein
    • Bile is collected by bile canaliculi and transported in the common hepatic duct

    Functions of Liver

    • Metabolism of carbohydrates, lipids, and proteins
    • Breakdown of red blood cells (reticuloendothelial system)
    • Detoxification of endogenous and exogenous compounds
    • Bilirubin metabolism
    • Protein synthesis (e.g., albumin, acute phase proteins, coagulation factors)
    • Storage of substances

    Liver and Carbohydrate Metabolism

    • Carbohydrate metabolism is linked to blood glucose levels
    • Glucose levels are largely regulated by the liver, influenced by:
      • Insulin
      • Glycogen
      • Glucagon
      • Glycogenolysis
      • Gluconeogenesis

    Liver and Lipid Metabolism

    • Lipids are water-insoluble and transported as lipoproteins
    • Liver produces lipoproteins, metabolizes fatty acids (acetyl-CoA) and triglycerides (energy)
    • Converts carbohydrates and proteins into fats
    • Converts cholesterol to bile
    • Bile is continuously produced with water, bile salts, bilirubin, fatty acids, and cholesterol

    Liver and Protein Metabolism

    • Most important functions of liver:
      • Production of plasma proteins
      • Production of clotting factors
      • Deamination of amino acids
      • Formation of waste products (ammonia)
      • Production of urea

    Liver and Bilirubin Metabolism

    • Bilirubin is derived from hemoglobin
    • Unconjugated bilirubin is conjugated to glucuronic acid
    • High concentrations of unconjugated bilirubin can cause cell damage, jaundice, and toxicity to the brain
    • Total bilirubin is the sum of unconjugated and conjugated bilirubin

    Liver Function Tests (LFTs)

    • Most LFT panels include:
      • Alanine aminotransferase (ALT) (integrity of hepatocytes)
      • Bilirubin
      • Alkaline phosphatase (ALP) (abundant in bile canaliculi)
      • Albumin

    Aminotransferases

    • Alanine aminotransferase (ALT) test is sensitive and nonspecific; a marker of acute hepatocyte damage
    • Aspartate aminotransferase (AST) test is produced by hepatocytes, skeletal muscles, kidney, brain, and heart; less specific information for liver assessment

    Bilirubin

    • Bilirubin is derived from heme
    • Unconjugated bilirubin is excreted in bile
    • Conjugated bilirubin is degraded by bacteria in the intestine to stercobilinogen
    • Small amounts are excreted in urine as urobilinogen
    • Elevated total bilirubin suggests liver disease, hemolysis, or biliary obstruction

    Jaundice

    • Jaundice is caused by excess bilirubin
    • Three main causes are hemolysis (pre-hepatic), failure of hepatic conjugation mechanism (hepatic), and biliary system obstruction (post-hepatic)
    • Laboratory considerations include hemolysis (increased indirect bilirubin), hepatocellular damage (elevated bilirubin, ALT and ALP), and biliary obstruction (elevated bilirubin and ALP)

    Alkaline Phosphatase (ALP)

    • ALP is derived from liver, bone, intestine, and placenta
    • Increased ALP indicates increased synthesis by cells lining bile canaliculi, cholestasis, infiltrative liver diseases, or cirrhosis

    Plasma Proteins

    • Albumin is produced by the liver with a biological half-life of around 20 days
    • Hypoalbuminemia is observed in advanced chronic liver disease and severe acute liver damage

    Prothrombin Time (PT)

    • Prothrombin time measures the time it takes for blood to clot, reflecting hepatic synthesis function
    • Prothrombin is made by the liver and plays a role in the blood clotting cascade
    • Has a very short half-life (60 to 72 hours)
    • Normal PT indicates normal blood clotting protein. Increased PT indicates decreased synthesis

    Gamma-Glutamyl Transpeptidase (GGT)

    • GGT is a very sensitive marker of liver pathology, associated with detoxification and alcohol ingestion, drugs like phenytoin, and conditions like cholestasis.
    • Commonly increased GGT accompanies raised ALT

    Acute/Chronic Liver Disease

    • Acute liver disease has common causes like poisoning (paracetamol overdose, plant/fungal toxins), liver infection (hepatitis A, B, C), inadequate liver perfusion (blood loss), and others
    • It can resolve, or progress to acute hepatic failure (medical emergency, not compensated, renal failure), or lead to chronic hepatic damage.
    • Chronic liver disease includes alcoholic liver disease, chronic active hepatitis, primary biliary cirrhosis, and nonalcoholic fatty liver disease; it may progress to cirrhosis (late stage fibrosis of the liver)

    Cirrhosis

    • Cirrhosis is the final stage of chronic liver damage characterized by scarring of the liver, decreased function, and irreversible changes.
    • Commonly associated with chronic conditions, particularly alcohol abuse (though reversible in the preceding stages)
    • It can lead to liver failure.
    • Diagnosis can be challenging and often features late diagnosis (common mistaken for fatigue, fibromyalgia, etc.)

    Diagnosis of Cirrhosis and Liver Failure

    • Early cirrhosis (stable period) with years may have no biochemical indicators.
    • Late stages often showcase jaundice, encephalopathy, ascites, and bleeding.
    • Laboratory considerations include liver biopsy, antibody tests (hepatitis), copper/iron blood tests (Wilson/hemochromatosis diseases), albumin, PT, imaging.

    Gilbert Syndrome

    • Genetic, autosomal recessive liver disease usually causing hereditary hyperbilirubinemia
    • Reduced activity of glucuronyl transferase, resulting in less conjugation of bilirubin, leading to high levels of unconjugated bilirubin.

    Crigler-Najjar Syndrome

    • Rare genetic disorder, either autosomal recessive (Type I) or autosomal dominant (Type II), characterized by lack of glucuronyl transferase, resulting in severe unconjugated bilirubinemia, potentially life-threatening.

    Dubin-Johnson Syndrome

    • Autosomal recessive genetic defect in the transportation of bilirubin from hepatocytes to bile
    • Signs include jaundice (early adolescence), upper abdominal pain, anorexia, nausea, and vomiting.
    • Dark pigmentation in the liver and increased serum bilirubin.

    Hepatitis

    • Inflammation of the liver due to viruses, bacteria, toxins, chemicals, or alcohol.
    • Hepatitis can be acute (less than 6 months) or chronic (longer than 6 months)
    • Types A, B, C, D, and E are viral. Early symptoms include fatigue, jaundice, fever, nausea, tea-coloured urine, and clay-coloured feces.
    • Diagnosis panel may include testing for anti-HAV IgM, HBAg, anti-HBc IgM, anti-HCV.

    Hepatitis A

    • Single-stranded RNA virus transmitted fecally
    • Greatest risk 2 weeks before symptoms. 70-80% jaundice.
    • Prevented by vaccination.

    Hepatitis B

    • DNA virus transmitted through contact with blood, semen, and vaginal fluid.
    • Incubation (60-90 days) with potential for anorexia, vomiting, abdominal pain, dark urine, jaundice in 30-50% of cases. 15-25% develop chronic disease, requiring testing for hepatitis B core antigen and other antibodies.

    Hepatitis C

    • Enveloped single-stranded RNA virus transmitted by blood transfusion or contaminated needles.
    • Incubation (2-26 weeks); 60-70% asymptomatic.
    • Symptoms include anorexia, malaise, fatigue, abdominal pain, and jaundice.
    • Higher risk of chronic cases compared to types A & B, and potentially cancers.

    Laboratory Diagnosis of Viral Hepatitis

    • Non-specific tests (bilirubin, liver enzymes) and specific tests (antibodies to individual viral antigens, antigens of hepatitis B, etc.) aid in identifying type, acute/chronic, and past infection.

    Hereditary Hemochromatosis

    • Autosomal recessive genetic disease characterized by iron overload/accumulation, potentially asymptomatic in the early stages.
    • Common cause is increased iron absorption, and 75% of impacted individuals are asymptomatic.
    • Early symptoms may include fatigue, impotence, and arthralgia, and later can result in organ damage, diabetes, skin hyperpigmentation, arthropathy, etc.

    Diagnosis of Hereditary Hemochromatosis

    • Clinical evaluation might mistake symptoms for other conditions (fatigue, fibromyalgia, depression).
    • Blood tests are essential for diagnosis (serum iron, ferritin, transferrin saturation, liver function tests, total protein, albumin, glucose levels.)
    • Genetic testing can confirm the diagnosis.

    Wilson Disease

    • An autosomal recessive disorder of copper metabolism impacting the liver, brain, eyes, heart, etc.
    • Copper absorption in the intestine but is not processed nor excreted properly resulting in copper buildup.
    • Signs include Kayser-Fleischer rings and liver, brain, kidney issues, and hepatic disturbances.
    • Laboratory features can show low serum copper but high urine copper, and low ceruloplasmin. Elevated AST, ALT, GGT, and bilirubin are also indicators.

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