Jaundice: Causes and Metabolism
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Questions and Answers

What is the earliest sign of jaundice?

  • Dark urine
  • Increased abdominal pain
  • Yellow discoloration of the skin
  • Yellow discoloration of the sclera (correct)
  • Which condition is characterized by high levels of unconjugated bilirubin (UCB) overwhelming the liver's conjugating ability?

  • Extravascular hemolysis (correct)
  • Dubin-Johnson syndrome
  • Crigler-Najjar syndrome
  • Gilbert syndrome
  • In which type of jaundice does phototherapy serve as an effective treatment?

  • Physiologic jaundice of the newborn (correct)
  • Gilbert syndrome
  • Crigler-Najjar syndrome
  • Dubin-Johnson syndrome
  • What is the cause of kernicterus in physiologic jaundice of the newborn?

    <p>Low UGT activity (C)</p> Signup and view all the answers

    Which syndrome presents with a deficiency of the bilirubin canalicular transport protein?

    <p>Dubin-Johnson syndrome (B)</p> Signup and view all the answers

    What is a common clinical feature observed in Gilbert syndrome?

    <p>Jaundice during stress or infection (D)</p> Signup and view all the answers

    What distinguishes Crigler-Najjar syndrome from other forms of jaundice?

    <p>It results from an absence of uridine glucuronyl transferase. (D)</p> Signup and view all the answers

    Which of the following accurately describes a consequence of increased unconjugated bilirubin (UCB) in the blood?

    <p>Yellow discoloration of skin and sclera (D)</p> Signup and view all the answers

    Flashcards

    Jaundice

    Yellow discoloration of the skin, most often due to increased bilirubin levels in the blood. It usually signifies a problem with the liver's ability to process bilirubin.

    Bilirubin Metabolism: Step 1

    The breakdown of heme from red blood cells into unconjugated bilirubin (UCB), which then has to be processed by the liver.

    Bilirubin Metabolism: Step 2

    Unconjugated bilirubin (UCB) is transported by albumin to the liver, where it's processed further.

    Bilirubin Metabolism: Step 3

    Enzymes within hepatocytes convert unconjugated bilirubin (UCB) into a water-soluble form called conjugated bilirubin (CB).

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    Bilirubin Metabolism: Step 4

    Conjugated bilirubin (CB) is excreted through the bile ducts into the small intestine.

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    Bilirubin Metabolism: Step 5

    Intestinal bacteria convert conjugated bilirubin (CB) into urobilinogen. Some urobilinogen is reabsorbed into the blood, giving urine its yellow color, while the rest is excreted in feces, making them brown.

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    Unconjugated Hyperbilirubinemia

    A condition where the liver has trouble conjugating and excreting bilirubin, resulting in high unconjugated bilirubin levels.

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    Conjugated Hyperbilirubinemia

    A condition characterized by high levels of conjugated bilirubin in the blood, often due to problems with bile flow or liver cell function.

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    Study Notes

    Jaundice

    • Yellow discoloration of skin and sclera, caused by elevated serum bilirubin (usually > 2.5 mg/dL) This is the earliest sign
    • Result of disturbances in bilirubin metabolism.

    Normal Bilirubin Metabolism

    • Breakdown of red blood cells (RBCs) by macrophages in the reticuloendothelial system.
    • Conversion of protoporphyrin from heme to unconjugated bilirubin (UCB).
    • Transport of UCB to the liver via albumin.
    • Conjugation of bilirubin by uridine glucuronyl transferase (UGT) in hepatocytes to form conjugated bilirubin (CB).
    • Transfer of CB to bile canaliculi and formation of bile, stored in the gallbladder.
    • Release of bile into small intestine for digestion and absorption.
    • Conversion of CB to urobilinogen by intestinal flora, then to stercobilin (for brown feces) and urobilin (for yellow urine).

    Clinical features and Laboratory findings in Jaundice

    • Extravascular hemolysis or Ineffective erythropoiesis: High UCB levels overwhelm the liver's ability to conjugate it resulting in dark urine due to increased urine urobilinogen . Risk of pigmented bilirubin gallstones.
    • Physiological jaundice of the newborn: Transiently low UGT activity in newborns, resulting in increased UCB. Increased risk of kernicterus (brain damage) if untreated.
    • Gilbert syndrome: Mildly low UGT activity; autosomal recessive, increased UCB.
    • Crigler-Najjar syndrome: Absence of UGT; autosomal recessive, increased UCB, kernicterus usually fatal.
    • Dubin-Johnson syndrome: Deficiency of bilirubin canalicular transport protein, autosomal recessive. Increased CB, decrease urine urobilinogen. Usually not clinically significant unless the liver is dark/brown.
    • Rotor syndrome: Similar to Dubin-Johnson, but without liver discoloration. Increased bilirubin in blood.
    • Biliary tract obstruction: Associated with gallstones, pancreatic carcinoma, cholangiocarcinoma, parasites, liver fluke. Increased CB, decreased urine urobilinogen, and increased alkaline phosphatase.
    • Viral hepatitis: Inflammation disrupts hepatocytes; Increased CB, and UCB.

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    Description

    This quiz explores the causes and physiological processes associated with jaundice, focusing on bilirubin metabolism and its clinical implications. Understand the normal breakdown of red blood cells, the conversion of bilirubin, and the signs and symptoms of jaundice through clinical findings and laboratory results.

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