Heme Catabolism and Bilirubin

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Questions and Answers

What percentage of heme is obtained from hemoglobin in the body?

  • 70%
  • 60%
  • 90%
  • 80% (correct)

Which of the following is NOT a source of heme in the body?

  • Myoglobin
  • Cytochrome
  • Chlorophyll (correct)
  • Hemoglobin

What is the organic compound found in heme?

  • Carotenoid
  • Porphyrin (correct)
  • Bilirubin
  • Chlorophyll

Which bodily substances contain bilirubin?

<p>Bile, urine, and feces (D)</p> Signup and view all the answers

What is the term for the structure formed by the connection of four pyrrols rings in heme?

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

How many pyrrols-like rings are present in bilirubin?

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

What is the fate of hemoglobin in the spleen?

<p>It is broken down into its constituent amino acids, iron, and heme (B)</p> Signup and view all the answers

What is the characteristic of unconjugated bilirubin in terms of solubility?

<p>It is insoluble in water (B)</p> Signup and view all the answers

Where is unconjugated bilirubin formed?

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

What is the role of albumin in relation to unconjugated bilirubin?

<p>It binds to unconjugated bilirubin (A)</p> Signup and view all the answers

What is the product of heme ring breakdown?

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

What is the purpose of conjugating bilirubin with glucouronic acid in the liver?

<p>To make it more water-soluble and readily transported to bile (A)</p> Signup and view all the answers

What is the eventual destination of conjugated bilirubin in the body?

<p>The duodenum for fat digestion (A)</p> Signup and view all the answers

What is the role of the bile duct in the conjugation process of bilirubin?

<p>It transports conjugated bilirubin to the duodenum (A)</p> Signup and view all the answers

What is the result of the reaction between bilirubin and glucouronic acid in the liver?

<p>The formation of bilirubin diglucuronides (C)</p> Signup and view all the answers

What is the primary function of enterohepatic circulation in the context of bile?

<p>To allow for the reabsorption of bile from the intestine (C)</p> Signup and view all the answers

What is the primary contributor to the characteristic brown color of feces?

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

What is the fate of conjugated bilirubin that remains in the large intestine?

<p>It is metabolized by colonic bacteria (D)</p> Signup and view all the answers

What is the significance of elevated levels of bilirubin in blood and urine?

<p>They indicate certain diseases (B)</p> Signup and view all the answers

How do indirect and direct bilirubin react with a reagent?

<p>Indirect bilirubin reacts slowly, while direct bilirubin reacts rapidly (A)</p> Signup and view all the answers

What is the formula for total bilirubin?

<p>T = D + ID (A)</p> Signup and view all the answers

What is the difference between the reaction of indirect bilirubin with a reagent and the reaction of direct bilirubin with a reagent?

<p>Indirect bilirubin reacts slowly in methanol, while direct bilirubin reacts rapidly in solution (C)</p> Signup and view all the answers

What is the primary characteristic of jaundice?

<p>Elevation of bilirubin in blood (B)</p> Signup and view all the answers

How is jaundice classified according to the cause?

<p>Into three types: pre-hepatic, hepatic, and post-hepatic (D)</p> Signup and view all the answers

What is affected by the yellow discoloration in jaundice?

<p>Skin, sclera, and mucous membrane (B)</p> Signup and view all the answers

What is the common factor among pre-hepatic, hepatic, and post-hepatic jaundice?

<p>Elevation of bilirubin in blood (D)</p> Signup and view all the answers

What is the underlying cause of the yellow discoloration in jaundice?

<p>Elevation of bilirubin in blood due to various causes (B)</p> Signup and view all the answers

What is the underlying cause of the elevated level of direct bilirubin in the blood and urine in post-hepatic obstructive jaundice?

<p>Obstruction of the bile duct, preventing the passage of conjugated bilirubin into the intestine (D)</p> Signup and view all the answers

Which of the following laboratory tests would confirm a diagnosis of hemolytic anemia as a cause of jaundice?

<p>Increased reticulocyte count and low haptoglobin levels (B)</p> Signup and view all the answers

What is the result of an increased rate of RBCs breakdown in the body?

<p>Increased production of bilirubin in the liver, exceeding the liver's ability to convert it to the conjugated form (A)</p> Signup and view all the answers

What is the reason behind the high bilirubin level in newborns after 2-3 days?

<p>The newborn's liver is not fully mature (D)</p> Signup and view all the answers

What is the consequence of very high unconjugated bilirubin levels?

<p>It can cause brain damage (C)</p> Signup and view all the answers

Why is phototherapy used to treat high bilirubin levels?

<p>It helps to break down bilirubin and convert it to the photo isomer form (A)</p> Signup and view all the answers

What is the characteristic of the liver in Dubin-Johnson syndrome?

<p>It is dark brown in color due to the presence of a pigment similar to melanin (A)</p> Signup and view all the answers

What is the prognosis of Gilbert syndrome?

<p>Excellent, with no treatment necessary (C)</p> Signup and view all the answers

What is the characteristic of bilirubin levels in Crigler-Najjar syndrome type 1?

<p>Bilirubin levels are usually &gt; 20 mg/dl (C)</p> Signup and view all the answers

What is the characteristic of Crigler-Najjar syndrome type 2?

<p>A moderate deficiency of glucuronyl transferase (D)</p> Signup and view all the answers

What is the prognosis of Crigler-Najjar syndrome type 1?

<p>Poor, with a high risk of kernicterus (A)</p> Signup and view all the answers

What is the characteristic of bilirubin levels in patients with Gilbert syndrome?

<p>Usually below 5mg/dl (A)</p> Signup and view all the answers

What is the difference between Dubin-Johnson syndrome and Rotor syndrome?

<p>Presence of coarse dark-brown pigment in the liver (B)</p> Signup and view all the answers

What is the incidence of Gilbert syndrome in the population?

<p>3-7% of the population (B)</p> Signup and view all the answers

What is the characteristic of Crigler-Najjar syndrome type 2?

<p>Moderate deficiency of glucuronyl transferase (B)</p> Signup and view all the answers

What is the primary reason for performing a bilirubin test in newborns?

<p>To diagnose jaundice (C)</p> Signup and view all the answers

How do you convert bilirubin levels from mg/dL to μmol/L?

<p>Multiply by 17.1 (A)</p> Signup and view all the answers

What is the effect of hemolyzed sample on bilirubin levels?

<p>Increases indirect bilirubin levels (C)</p> Signup and view all the answers

What is the normal range of total bilirubin in adults in mg/dL?

<p>0.3-1.2 (B)</p> Signup and view all the answers

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Study Notes

Bilirubin and Jaundice

  • Bilirubin is a yellow pigment produced during heme catabolism, found in bile, urine, and feces.
  • Heme is composed of iron and an organic compound called porphyrin, and is found in hemoglobin, which is a principal component of RBCs.
  • 80% of heme in the body comes from hemoglobin in old RBCs, and 20% comes from other hemo-proteins such as cytochrome and myoglobin.

Heme Catabolism

  • Heme is broken down into amino acids, iron, and bilirubin in the spleen.
  • Bilirubin is initially in an unconjugated (indirect) form, which is not soluble in water.
  • Unconjugated bilirubin is bound to albumin and carried to the liver, where it is conjugated with glucouronic acid to produce a water-soluble form.

Conjugation and Enterohepatic Circulation

  • Conjugated bilirubin is excreted into bile through the bile duct, where it helps with fat digestion.
  • Most of the conjugated bilirubin is reabsorbed from the terminal ileum into the liver through the portal vein, a process known as enterohepatic circulation.
  • The remaining conjugated bilirubin is metabolized by colonic bacteria to produce urobilinogen, which can be further oxidized to urobilin and stercobilin, giving feces its brown color.

Jaundice and Bilirubin Levels

  • Elevated levels of bilirubin in the blood and urine indicate certain diseases, such as jaundice (icterus).
  • Jaundice is a yellow discoloration of the skin, sclera, and mucous membranes due to elevated bilirubin levels.
  • Total bilirubin levels can be measured, and consist of both direct (conjugated) and indirect (unconjugated) bilirubin.

Types of Jaundice

  • Pre-hepatic jaundice occurs due to increased RBC breakdown, and is characterized by an increased level of unconjugated bilirubin.
  • Hepatic jaundice occurs due to liver cell damage, and is characterized by a decreased ability to conjugate bilirubin.
  • Post-hepatic jaundice occurs due to obstruction of the bile duct, and is characterized by an increased level of conjugated bilirubin.

Causes of Jaundice

  • Hemolytic anemia (e.g. thalassemia) can cause pre-hepatic jaundice.
  • Liver cell damage (e.g. hepatitis, cirrhosis) can cause hepatic jaundice.
  • Obstruction of the bile duct (e.g. gallstones, cancer) can cause post-hepatic jaundice.

Hereditary Hyperbilirubinemia

  • Gilbert syndrome is a type of hereditary indirect hyperbilirubinemia caused by a mild deficiency of glucuronyl transferase.
  • Crigler-Najjar syndrome is a type of hereditary indirect hyperbilirubinemia caused by a severe or moderate deficiency of glucuronyl transferase.
  • Dubin-Johnson syndrome is a type of hereditary conjugated hyperbilirubinemia characterized by a chronic benign jaundice without pruritus or elevation of serum ALP.
  • Rotor syndrome is a type of hereditary conjugated hyperbilirubinemia similar to Dubin-Johnson syndrome.

Bilirubin Test

  • The bilirubin test is performed to diagnose jaundice, liver or gallbladder problems, and to monitor newborns.
  • Normal bilirubin levels in adults are:
    • Total bilirubin: 0.3-1.2 mg/dL
    • Direct bilirubin: 0.1-0.4 mg/dL
    • Indirect bilirubin: 0.2-0.7 mg/dL

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