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Heme Catabolism /
Metabolism of Bile Pigments
By
Dr. Santhosh Kumar N
Associate Professor
FORMATION OF BILIRUBIN
(Reticuloendotheial system of liver, spleen & bone
marrow)
Microsomal heme
oxygenase
Heme
NADPH + O2 Iron, CO2 Biliverdin
Ring opens
NADPH+H
NADP+
Biliverdin
reductase
Bilirubin (unconjugated)
Transport to Liver
(further disposal of the bilirubin)
• The bilirubin formed in the RE cells is insoluble in water (lipophilic bilirubin).
• Transported in plasma bound to albumin (bind 2 moles of bilirubin)
• Bilirubin can easily dissociate from albumin.
• The binding sites for bilirubin on albumin can be occupied by aspirin, penicillin,
etc. Hence, care should be taken while administering such drugs to newborn babies
to avoid kernicterus.
Production of conjugated bilirubin
(In the hepatic cell )
Bilirubin has no function in the body and is excreted through bile
FATE OF BILIRUBIN / DEGRADATION OF BILE
PIGMENTS
Uptake
Secretion
Excretion
β- Glucoronidase
Glucuronate
(Colorless)
Unconjugated bilirubin Conjugated bilirubin
 Formed in reticuloendothelial system
 Opening of I & II pyrroles rings
 Transport the plasma proteins (albumin)
 In soluble in Water .
 Present in 2/3rd in blood.
 Not excreted through urine
 Late reaction with bilirubin.
 Increased in hemolytic jaundice.
 Formed in liver
 Conjugated with UDP-Glucuronic acid
 Transported into the intestine from liver
freely
 Soluble in water & alcohol.
 Present in 1/3rd in blood
 Excreted through the urine.
 One min bilirubin test
 Increased in obstructive jaundice
NORMAL VALUES IN SERUM:
• Total bilirubin -0.2 to1.0 mg/dl
• Unconjugated bilirubin - 0.2 to 0.6 mg/dl
• Conjugated bilirubin – 0.2 to 0.4 mg/dl
HYPER BILIRUBINEMIA
• If the plasma bilirubin level exceeds 1mg/dl- Hyper bilirubinemia.
• If bilirubin levels exceeds between 1 to 2 mg/dl - latent jaundice
more than 2mg/dl – Jaundice.
Yellow discoloration of skin & mucus membranes
due to accumulation of Bilirubin
Types of Jaundice
Hemolytic jaundice
 Increased Hemolysis of RBCs – causes malaria, incompatible blood transfusion, sickle
cell anemia).
 Over production of bilirubin beyond the ability of the liver to conjugated and excrete
the same.
 In serum: Unconjugated bilirubin increased
• In urine: Urobilinogen increased & Absents of urinary bilirubin.
• In Feces: Dark brown colour of feces due to high content of stercobilinogen.
• Malfunction of Liver due to damage of hepatic
cells. Caused by viral hepatitis, cirrhosis of liver
& alcoholic liver diseases
• Other causes Primary biliary cirrhosis leading to
an increase in conjugated bilirubin,
• Impairment of excretion of Conjugated bilirubin
into the bile
Hepato cellular jaundice
• In Serum: Both Conjugated & Unconjugated bilirubin are increased
– Increased SGOT, SGPT in circulation due damage of Hepatocytes
• In urine: Excessive excretion of bilirubin & urobilinogen.
• In Feces: Absence of stercobilinogen.
Obstructive jaundice
• Due to Obstruction in bile duct that prevents passage of bile
into the intestine, caused by gall stones, tumors etc
• In serum: conjugation bilirubin & ALP is elevated
• In urine: bilirubin is present & absent of Urobilinogen
• In feces: Clay colored feces due to absence of
stercobilinogen.
– contain excess fat indicating impairment in fat digestion &
absorption in the absence of bile.
Jaundice
due to
Genetic defect
(Hyperbilirubinemia)
Yellow discoloration of skin and mucus
membranes due to accumulation of
yellow pigment i.e Bilirubin
• Based on the cause, it may congenital & acquired
Congenital hyperbilirubemias
– Results from abnormal uptake, conjugation or excretion of bilirubin due to
inherited defects
• Crigler-Najjar syndrome
• Gilbert's disease
• Dubin Johnson syndromes
Crigler-Najjar syndrome
Type-I Type-II
Congenital non-hemolytic jaundice.
↑↑↑Unconjugated bilirubin level in serum
(>20mg/dl).
Leading to risk of kernicterus (bilirubin
encephalopathy- brain damage).
 Due to defect in the hepatic enzyme UDP-
glucuronyl transferase.
Rare inherited disorder.
Unconjugated bilirubin does not exceed
20mg/dl.
No risk of kernicterus
Patients respond to treatment with large
doses of phenobarbitone
Gilbert's disease
• It is an autosomal dominant trait,
• caused by an inability of the hepatocytes to uptake bilirubin from the blood.
• Decreased hepatic clearance of bilirubin
• result in unconjugated bilirubin accumulates.
• Defect in uptake of bilirubin by the liver & Reduced glucuronyl transferase
activity
• Bilirubin level is usually around 3 mg/dl.
Dubin Johnson syndromes
• Autosomal recessive trait.
– Caused by inability of the hepatocytes to secrete conjugated bilirubin in bile.
• Conjugated bilirubin returns to the blood (Conjugated hyperbilirubinemia).
• The bilirubin gets deposited in the liver
• Liver appears black (black liver jaundice).
Acquired hyperbilirubemias
Neonatal physical
(Newborn) jaundice
• It occurs the first week of life
• Caused by increased hemolysis coupled with immature hepatic system for
the uptake, conjugation & secretion of bilirubin
• UDP –glucuronyl transferase activity is low in the new borns.
• There is a limitation in the availability of the substrate UDP-glucuronic acid
for conjugation in liver.
• Unconjugated bilirubin is high (25mg/dl). It
is deposited in the skin (photosensitive)
Treatment:
• Exposure to visible light (Irradiation with
blue light)
unconjugated bilirubin
Photolysis
water soluble products.
(conjugated bilirubin)
Diagnosis
of
Jaundice
Estimation of Serum Bilirubin
Van Den Bergh reaction
0.3ml of serum into each of 2
test tubes. Add 0.3 ml of DW to
one part (control) & add 0.3ml
of freshly prepared diazo reagent
into second test tube (test) &
mix both tubes.
Reddish purple
color is formed.
When serum bilirubin is
allowed to react with
VD bergh’s diazo
reagent (sulphanilic acid
& sodium nitrite in conc.
HCl),a purple colored
azobilirubin is formed.
Different responses with Van Den Bergh reaction
Direct
reaction
- Immediate color
develops
- Proceeding rapidly.
Conjugated
bilirubin
(0.1 to 0.4mg/dl)
Obstructive
jaundice
Indirect
reaction
- Color develops after
addition of methyl alcohol
Unconjugated
bilirubin
(0.2 to 0.6mg/dl)
Hemolytic
jaundice
Mixed /
biphasic
reaction
- Color appears
immediately & color is
intensified on addition of
alcohol
Both UCB & CB Hepatocellular
jaundice
Estimation of Urinary Bilirubin & Urobilinogen
Condition Urine bilirubin Urine Urobilinogen
Normal Absent 0.4mg/24hr
Hemolytic jaundice Absent Increased
Hepatic jaundice Present, if micro
obstruction to bile
ductules is present
-Normal or decreased if
micro obstruction to bile
ductules is present
Obstructive
jaundice
Present Absent (due to biliary
obstruction )
Thank You

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  • 1. “All power is within you. You can do anything and everything. Believe in that”. Heme Catabolism / Metabolism of Bile Pigments By Dr. Santhosh Kumar N Associate Professor
  • 2.
  • 3. FORMATION OF BILIRUBIN (Reticuloendotheial system of liver, spleen & bone marrow)
  • 4. Microsomal heme oxygenase Heme NADPH + O2 Iron, CO2 Biliverdin Ring opens NADPH+H NADP+ Biliverdin reductase Bilirubin (unconjugated)
  • 5. Transport to Liver (further disposal of the bilirubin) • The bilirubin formed in the RE cells is insoluble in water (lipophilic bilirubin). • Transported in plasma bound to albumin (bind 2 moles of bilirubin) • Bilirubin can easily dissociate from albumin. • The binding sites for bilirubin on albumin can be occupied by aspirin, penicillin, etc. Hence, care should be taken while administering such drugs to newborn babies to avoid kernicterus.
  • 6. Production of conjugated bilirubin (In the hepatic cell ) Bilirubin has no function in the body and is excreted through bile
  • 7. FATE OF BILIRUBIN / DEGRADATION OF BILE PIGMENTS Uptake Secretion Excretion β- Glucoronidase Glucuronate (Colorless)
  • 8. Unconjugated bilirubin Conjugated bilirubin  Formed in reticuloendothelial system  Opening of I & II pyrroles rings  Transport the plasma proteins (albumin)  In soluble in Water .  Present in 2/3rd in blood.  Not excreted through urine  Late reaction with bilirubin.  Increased in hemolytic jaundice.  Formed in liver  Conjugated with UDP-Glucuronic acid  Transported into the intestine from liver freely  Soluble in water & alcohol.  Present in 1/3rd in blood  Excreted through the urine.  One min bilirubin test  Increased in obstructive jaundice
  • 9. NORMAL VALUES IN SERUM: • Total bilirubin -0.2 to1.0 mg/dl • Unconjugated bilirubin - 0.2 to 0.6 mg/dl • Conjugated bilirubin – 0.2 to 0.4 mg/dl
  • 10. HYPER BILIRUBINEMIA • If the plasma bilirubin level exceeds 1mg/dl- Hyper bilirubinemia. • If bilirubin levels exceeds between 1 to 2 mg/dl - latent jaundice more than 2mg/dl – Jaundice.
  • 11. Yellow discoloration of skin & mucus membranes due to accumulation of Bilirubin
  • 13. Hemolytic jaundice  Increased Hemolysis of RBCs – causes malaria, incompatible blood transfusion, sickle cell anemia).  Over production of bilirubin beyond the ability of the liver to conjugated and excrete the same.  In serum: Unconjugated bilirubin increased • In urine: Urobilinogen increased & Absents of urinary bilirubin. • In Feces: Dark brown colour of feces due to high content of stercobilinogen.
  • 14. • Malfunction of Liver due to damage of hepatic cells. Caused by viral hepatitis, cirrhosis of liver & alcoholic liver diseases • Other causes Primary biliary cirrhosis leading to an increase in conjugated bilirubin, • Impairment of excretion of Conjugated bilirubin into the bile Hepato cellular jaundice
  • 15. • In Serum: Both Conjugated & Unconjugated bilirubin are increased – Increased SGOT, SGPT in circulation due damage of Hepatocytes • In urine: Excessive excretion of bilirubin & urobilinogen. • In Feces: Absence of stercobilinogen.
  • 16. Obstructive jaundice • Due to Obstruction in bile duct that prevents passage of bile into the intestine, caused by gall stones, tumors etc • In serum: conjugation bilirubin & ALP is elevated • In urine: bilirubin is present & absent of Urobilinogen • In feces: Clay colored feces due to absence of stercobilinogen. – contain excess fat indicating impairment in fat digestion & absorption in the absence of bile.
  • 17. Jaundice due to Genetic defect (Hyperbilirubinemia) Yellow discoloration of skin and mucus membranes due to accumulation of yellow pigment i.e Bilirubin
  • 18. • Based on the cause, it may congenital & acquired Congenital hyperbilirubemias – Results from abnormal uptake, conjugation or excretion of bilirubin due to inherited defects • Crigler-Najjar syndrome • Gilbert's disease • Dubin Johnson syndromes
  • 19. Crigler-Najjar syndrome Type-I Type-II Congenital non-hemolytic jaundice. ↑↑↑Unconjugated bilirubin level in serum (>20mg/dl). Leading to risk of kernicterus (bilirubin encephalopathy- brain damage).  Due to defect in the hepatic enzyme UDP- glucuronyl transferase. Rare inherited disorder. Unconjugated bilirubin does not exceed 20mg/dl. No risk of kernicterus Patients respond to treatment with large doses of phenobarbitone
  • 20. Gilbert's disease • It is an autosomal dominant trait, • caused by an inability of the hepatocytes to uptake bilirubin from the blood. • Decreased hepatic clearance of bilirubin • result in unconjugated bilirubin accumulates. • Defect in uptake of bilirubin by the liver & Reduced glucuronyl transferase activity • Bilirubin level is usually around 3 mg/dl.
  • 21. Dubin Johnson syndromes • Autosomal recessive trait. – Caused by inability of the hepatocytes to secrete conjugated bilirubin in bile. • Conjugated bilirubin returns to the blood (Conjugated hyperbilirubinemia). • The bilirubin gets deposited in the liver • Liver appears black (black liver jaundice).
  • 23. • It occurs the first week of life • Caused by increased hemolysis coupled with immature hepatic system for the uptake, conjugation & secretion of bilirubin • UDP –glucuronyl transferase activity is low in the new borns. • There is a limitation in the availability of the substrate UDP-glucuronic acid for conjugation in liver.
  • 24. • Unconjugated bilirubin is high (25mg/dl). It is deposited in the skin (photosensitive) Treatment: • Exposure to visible light (Irradiation with blue light) unconjugated bilirubin Photolysis water soluble products. (conjugated bilirubin)
  • 26. Estimation of Serum Bilirubin Van Den Bergh reaction 0.3ml of serum into each of 2 test tubes. Add 0.3 ml of DW to one part (control) & add 0.3ml of freshly prepared diazo reagent into second test tube (test) & mix both tubes. Reddish purple color is formed. When serum bilirubin is allowed to react with VD bergh’s diazo reagent (sulphanilic acid & sodium nitrite in conc. HCl),a purple colored azobilirubin is formed.
  • 27. Different responses with Van Den Bergh reaction Direct reaction - Immediate color develops - Proceeding rapidly. Conjugated bilirubin (0.1 to 0.4mg/dl) Obstructive jaundice Indirect reaction - Color develops after addition of methyl alcohol Unconjugated bilirubin (0.2 to 0.6mg/dl) Hemolytic jaundice Mixed / biphasic reaction - Color appears immediately & color is intensified on addition of alcohol Both UCB & CB Hepatocellular jaundice
  • 28. Estimation of Urinary Bilirubin & Urobilinogen Condition Urine bilirubin Urine Urobilinogen Normal Absent 0.4mg/24hr Hemolytic jaundice Absent Increased Hepatic jaundice Present, if micro obstruction to bile ductules is present -Normal or decreased if micro obstruction to bile ductules is present Obstructive jaundice Present Absent (due to biliary obstruction )