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AL-maaref University College
Department of Medical Laboratory Techniques
Dec 2021
Subject Name : Advanced Clinical Chemistry [Practical]
Topic : Jaundice
4th-Stage
EXECULTED BY
M.Sc. Med. Biochemistry
MOHANAD ALI ABDULHADI
• Jaundice refers to the yellow discolouration
of the sclera and skin that is due
to hyperbilirubinaemia, occurring at bilirubin
levels roughly greater than 50 µmol/L
Pre-Hepatic Hepatocellular Post-Hepatic
* excessive red cell
breakdown
* unconjugated
hyperbilirubinemia.
•Hemolytic anemia
•Gilbert’s syndrome
•Criggler-Najjar
syndrome
* dysfunction of the
hepatic cells
* unconjugated and
conjugated bilirubin in
the blood
•Alcoholic liver disease
•Viral hepatitis
•Hereditary
hemochromatosis
•Autoimmune hepatitis
•Biliary cirrhosis
•Hepatocellular
carcinoma
* Refers to obstruction
of biliary drainage
* conjugated
hyperbilirubinaemia
•Gallstones
•cholangiocarcinoma
•pancreatic cancer or
abdominal masses (e.g.
lymphomas)
Types of Jaundice
Inherited disorders of bilirubin metabolism causing
Unconjugated Hyperbilirubinemia
• Crigler-Najjar syndrome
type 1:
• Crigler-Najjar syndrome
type 2:
• Gilbert syndrome:
Virtually no UGT1A1 activity
UGT1A1 activity below 10%
UGT1A1 activity ~30%
• UDP-glucuronosyltransferases (UGTs)
• UGT’s are a family of enzymes concentrated in the liver.
• One UGT isoform, UGT1A1, conjugates bilirubin and is
essential for its excretion.
• Inherited UGT1A1 deficiency causes jaundice.
Inherited disorders of bilirubin metabolism causing
Unconjugated Hyperbilirubinemia
• Crigler-Najjar syndrome
type 1:
• Crigler-Najjar syndrome
type 2:
• Gilbert syndrome:
Serum bilirubin 18-40 mg/dl:
Kernicterus, unless treated
vigorously
Serum bilirubin 8-18 mg/dl:
Kernicterus is rare
Serum bilirubin normal to
5 mg mg/dl
(increases during fasting,
intercurrent illness, etc.
No cerebral toxicity.
Phenobarbital treatment:
little or no effect.
Phenobarbital reduces serum
bilirubin is by >25%
Inherited disorders of bilirubin metabolism causing
Conjugated + Unconjugated Hyperbilirubinemia
• Dubin Johnson syndrome
• Rotor syndrome * Hepatic storage disorder
* No pigmentation
•Abnormality of biliary excretion
causes the retention of a pigment
in the liver [organic anions, but
not bile salts ]
•Accumulation of pigments
•serum bilirubin is only mildly
elevated (3-5 mg/dl)
Jaundice.pptx

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Jaundice.pptx

  • 1. AL-maaref University College Department of Medical Laboratory Techniques Dec 2021 Subject Name : Advanced Clinical Chemistry [Practical] Topic : Jaundice 4th-Stage EXECULTED BY M.Sc. Med. Biochemistry MOHANAD ALI ABDULHADI
  • 2. • Jaundice refers to the yellow discolouration of the sclera and skin that is due to hyperbilirubinaemia, occurring at bilirubin levels roughly greater than 50 µmol/L
  • 3. Pre-Hepatic Hepatocellular Post-Hepatic * excessive red cell breakdown * unconjugated hyperbilirubinemia. •Hemolytic anemia •Gilbert’s syndrome •Criggler-Najjar syndrome * dysfunction of the hepatic cells * unconjugated and conjugated bilirubin in the blood •Alcoholic liver disease •Viral hepatitis •Hereditary hemochromatosis •Autoimmune hepatitis •Biliary cirrhosis •Hepatocellular carcinoma * Refers to obstruction of biliary drainage * conjugated hyperbilirubinaemia •Gallstones •cholangiocarcinoma •pancreatic cancer or abdominal masses (e.g. lymphomas) Types of Jaundice
  • 4. Inherited disorders of bilirubin metabolism causing Unconjugated Hyperbilirubinemia • Crigler-Najjar syndrome type 1: • Crigler-Najjar syndrome type 2: • Gilbert syndrome: Virtually no UGT1A1 activity UGT1A1 activity below 10% UGT1A1 activity ~30% • UDP-glucuronosyltransferases (UGTs) • UGT’s are a family of enzymes concentrated in the liver. • One UGT isoform, UGT1A1, conjugates bilirubin and is essential for its excretion. • Inherited UGT1A1 deficiency causes jaundice.
  • 5. Inherited disorders of bilirubin metabolism causing Unconjugated Hyperbilirubinemia • Crigler-Najjar syndrome type 1: • Crigler-Najjar syndrome type 2: • Gilbert syndrome: Serum bilirubin 18-40 mg/dl: Kernicterus, unless treated vigorously Serum bilirubin 8-18 mg/dl: Kernicterus is rare Serum bilirubin normal to 5 mg mg/dl (increases during fasting, intercurrent illness, etc. No cerebral toxicity. Phenobarbital treatment: little or no effect. Phenobarbital reduces serum bilirubin is by >25%
  • 6. Inherited disorders of bilirubin metabolism causing Conjugated + Unconjugated Hyperbilirubinemia • Dubin Johnson syndrome • Rotor syndrome * Hepatic storage disorder * No pigmentation •Abnormality of biliary excretion causes the retention of a pigment in the liver [organic anions, but not bile salts ] •Accumulation of pigments •serum bilirubin is only mildly elevated (3-5 mg/dl)