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Jaundice Pathology
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- 2. Jaundice •Hyperbilirubinemia- > 0.3-1.2mg/dl •Jaundice- > 2.0-2.5 mg/dl •Icterus- Yellow discoloration of Sclera •Cholestasis- All Bile elements
- 3. Source Of Bilirubin •Major-(85%) Derived from catabolism of Hb by RE system (breakdown of senescent red cells. )- 0.2-0.3 mg/dl •Minor- (15%) Degradation of Heme produced from other sources (e.g. the P-450 cytochromes) and from premature destruction of hemoglobin in developing red cell precursors in the bone marrow.
- 5. Kernicterus In hemolytic diseaseof new born(Erythroblastosis fetalis) High concentrationof free unconjugated Bilirubin Cross Blood Brain Barrier, reaches Brain Cause severe irreversible neurological damage
- 6. Kernicterus - Deposition ofexcess bilirubin to brain mainly Basal Ganglia -C/f – 3 phases • Decreased alertness, Hypotonia, poor feeding, •Hypertonia, Opisthotonus •Hypotonia.
- 7. Types Of Bilirubin •Unconjugatedbilirubin- - Insoluble in water pass. - Cannot be excreted in the urine even when Its levels are high in the blood. - Present in the circulation forming tight complexes with serum albumin. •Conjugated bilirubin- - Water-soluble, nontoxic - Loosely bound to albumin in the plasma - Excess conjugated bilirubin in plasma can be excreted in urine
- 8. Jaundice It is definedas Yellowish pigmentation (discoloration) of Skin, Mucus membrane & Sclera due to increased levels of bilirubin (Hyperbilirubinemia) in the blood. - Normal bilirubin level- 0.3-1.2 mg/dl - Jaundice- 2.0-2.5 mg/dl - Latent Jaundice- 1.2-2.0 mg/dl- wthout clinical menifestation
- 9. Classification of Jaundice •Basedon the underlying cause - - Predominantly unconjugated hyperbilirubinemia - Predominantly conjugated hyperbilirubinemia • Based on pathological mechanism - - Hemolytic jaundice. - Hepatocellular jaundice. - Obstructive jaundice.
- 10. Other Classification •1- - Pre-hepatic(Haemolytic) - Hepatic (Toxic) - Post-hepatic (Obstructive) • 2- - Medical - Surgical
- 11. Mechanism of Jaundice 1-Predominantly Unconjugated Hyperbilirubinemia 2- Predominantly Conjugated Hyperbilirubinemiaz Eqilibirium between bilirubin production & clearance is disturbed.
- 12. Predominantly Unconjugated 1- ExcessiveExtrahepatic Production of Bilirubin- - Hemolytic Anemias - Ineffective erythropoiesis (Thalassemia, Pernicious anemia ) - Resorption of blood from internal hemorrhage (GIT bleeding, Hematomas)
- 13. Predominantly Unconjugated 2- ReducedHepatic Uptake- - Drug interference with membrane carrier systems - Some cases of Gilbert syndrome 3- Impaired Bilirubin Conjugation- - Physiological Jaundice of Newborn - Diffuse hepatocellular disease (Viral or drug induced hepatitis, cirrhosis) ….
- 14. .... - Crigler–Najjar syndrometypes I and II - Gilbert syndrom Breast Milk Jaundice- Neonatal jaundice, may be exacerbated by breastfeeding, due to the presence of bilirubin- deconjugating enzymes in breast milk.
- 15. Gilbert Syndrome - Geneticallyinherited disorder with no clinical consequences. (Most commonest) - It is inherited as Autosomal recessive - It leads to mutations in the UGT1 gene. - Affecting 2-5% of population - Mild, chronic unconjugated hyperbilirubinemia which is not due to haemolysis. - Usually jaundice devoloped is asymptomatic, or mild & intermittent.
- 16. Crigler-Najjar Syndrome Two formsof this condition - Crigler–Najjar Syndrome Type 1 - Crigler–Najjar Syndrome Type 2 Crigler–Najjar syndrome: Basic abnormality is impaired conjugation of bilirubin
- 17. Crigler–Najjar Syndrome Type1- - Rare, autosomal recessive disorder due to complete absence of hepatic UGT1A1. - Characterized by chronic, severe, unconjugated hyperbilirubinemia produce severe jaundice, icterus and death secondary to kernicterus within 18 months of birth. - Liver is morphologically normal by light and electron microscopy - Extreme elevation of Unconjugated bilirubin (usually more than 20 mg/dl)
- 18. Crigler–Najjar Syndrome Type2- - Autosomal dominant disorder. - Partial deficiency of UGT1A1 enzyme. - Less severe, nonfatal disorder - Unconjugated hyperbilirubinaemia is mild to moderate (usually less than 20 mg/dl)
- 19. Predominantly Conjugated 1- DecreasedHepatocellular Excretion- - Drug-induced canalicular membrane dysfunction (Oral contraceptives, Cyclosporine) - Hepatocellular damage or Toxicity (Viral or drug induced hepatitis, systemic infection, Total parenteral nutrition )
- 20. Predominantly Conjugated 2- ImpairedBile Flow (Intra/Extra Hepatic)- - Inflammatory destruction of intrahepatic bile ducts (Primary biliary cirrhosis, Primary sclerosing cholangitis, liver transplantation - Gallstones(Most common surgical cause of obstructive jaundice is- Common bile duct stones) - External Compression (carcinoma of pancreas)
- 22. Clinical Features Common signsand symptoms seen in individuals with jaundice include- - Yellow discoloration of the skin, mucous membranes, and the whites of the eyes - Light-colored stools - Dark-colored urine - Itching of the skin
- 23. The underlying diseaseprocess may result in additional signs and symptoms. These may include- • Nausea and vomiting, • Abdominal pain, • Fever, • Weakness, • Loss of appetite, • Headache, • Confusion, • Swelling of the legs and abdomen
- 24. Lab Diagnosis Laboratory Tests- •Liverfunction test (Serum Bilirubin) •FBC/Peripheral blood film •Urine for urobilinogen (haemolytic) or bilirubin (cholestatic) •Reticulocyte count •Serology for viral hepatitis •PT/APTT The following tests may be performed-
- 25. Lab Diagnosis Imaging (Noninvasive)- • Ultrasound hepato-biliary system • Computerised Tomography (CT) Scan • Magnetic resonance cholangiopancreatography (MRCP)/Magnetic resonance imaging (MRI)
- 26. Lab Diagnosis Invasive procedure-diagnostic and therapeutic)- • Endoscopic retrograde cholangiopancreatography (ERCP) • Percutaneous transhepatic cholangiography (PTC) • Endoscopic ultrasonography (EUS)
- 27. Van Den BerghTest Test done to determine type of Jaundice- There are three main reactions • Direct Reaction a) Immediate or Prompt b) Delayed • Biphasic Reaction • Indirect Reaction
- 28. Van Den BerghTest Conclusions- • Vanden Berg test is direct & prompt-Obstructive • Vanden Berg test is indirect- Haemolytic • Vanden Berg test is delayed direct/ biphasic- Toxic jaundice where parenchymatous liver damage is seen
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