2. STEP TO PG-MD/MS -DR.AKIF A.B
-1gm of Hemoglobin = 35mg of Bilirubin
-Bilirubin level >5mg/dl = Clinical manifestation of jaundice in neonates
-Bilirubin level > 2mg/dl = Clinical manifestation of jaundice in adults
-As bilirubin level increases jaundice involves more distal parts of body
3. STEP TO PG-MD/MS -DR.AKIF A.B
Part of body involved Bilirubin levels
Head and neck 5 mg/dl
Upper trunk and upper limb 10 mg/dl
Lower trunk and Thigh 12mg/dl
Legs 15 mg/dl
Palms and soles 20 mg/dl
4.
5. STEP TO PG-MD/MS -DR.AKIF A.B
Increased production of bilirubin from hemoglobin
1) Hemolytic anemia = Hereditary spherocytosis, G6PD deficiency
2) Ineffective erythropoiesis = Thallassemia, Pernicious Anemia
Impaired conjugation of Bilirubin
1) Physiological Jaundice
2) Breast milk Jaundice
3) Genetic disorders : Gilbert Sx , Criggler β Najjar Sx
4) Hepatitis
5) Hypothyroidism , Cephalhematoma
6. STEP TO PG-MD/MS -DR.AKIF A.B
-Due to Immaturity of Liver ( due to decreased UDPG Transferase activity)
-Hence unconjugated hyperbilirubinemia
-Jaundice appears after 24hrs of age
-Total bilirubin rise per day is less than 5mg/dl
-Peak bilirubin rise occurs at 3-5days but never exceeds 15mg/dl
-Clinical jaundice resolves by 1 week in term infants
-Clinical jaundice resolves by 2weeks in Preterm infants
-No Treatment is required
7. STEP TO PG-MD/MS -DR.AKIF A.B
-Unconjugated Hyperbilirubinemia
-Pregnanediol in Breast Milk interferes with
conjugation of Bilirubin
-Bilirubin level may reach upto 20mg/dl
-Temporary interuption of breast feeding will treat
the condition
8. STEP TO PG-MD/MS -DR.AKIF A.B
-Autosomal Dominant
-Congenital uncojugated Hyperbilirubinemia
-PathoPhysiology:
1) Decreased uptake of bilirubin by Liver
2) Decreased activity of UDPG Transferase
-LFT = Normal
-Liver Histology = Normal
-Phenobarbitone induces UDPG Transferase and thus induces conjugation of
Bilirubin and thus decreases Jaundice
9. STEP TO PG-MD/MS -DR.AKIF A.B
GILBERT Sx
CRIGGLER-NAJJAR Sx
Dubin
Johnson Sx
Rotor Sx
10. STEP TO PG-MD/MS -DR.AKIF A.B
-Unconjugated Hyperbilirubinemia
-Due to absent or Reduced UDPG Transferase activity
-Liver Histology = Normal
-LFT = Normal
CRIGGLER-NAJJAR -I CRIGGLER-NAJJAR -II
Autosomal Recessive Autosomal Dominant
Absent UDPG Transferase Reduced UDPG Transferase
More severe Less severe
Kernicterus may be seen Kernicterus is absent
11. STEP TO PG-MD/MS -DR.AKIF A.B
-Autosomal Recessive
-Conjugated Hyperbilirubinemia due to defective biliary excretion of bilirubin.
-Liver shows blackish pigmentation
12. STEP TO PG-MD/MS -DR.AKIF A.B
-Conjugated Hyperbilirubinemia
-Due to defective biliary excretion of bilirubin
-Liver histology = Normal
13. STEP TO PG-MD/MS -DR.AKIF A.B
Jaundice appearing Causes
at birth or within 24hrs of life Rh Incompatibility (MC cause) ,
Sepsis
On 2nd and 3rd day of life Physiological Jaundice, Criggler
najjar Sx, breast milk jaundice
3rd to 7th day of life All infective causes
> 7 days All other causes
14. STEP TO PG-MD/MS -DR.AKIF A.B
-It is bilirubin Encephalopathy caused by Bilirubin toxicity to Basal Ganglion
-Caused due to unconjugated bilirubin since it is lipid soluble and can cross
Blood Brain Barrier
Phase1 Phase 2 Phase 3
Hypotonia Fever High pitched cry
Poor suck Hypertonia
progressing to
Opisthotonus
Convulsions
Lethargy Death
Altered sensorium
15. STEP TO PG-MD/MS -DR.AKIF A.B
-Long term survivors demonstrates :
1) Choreoathetoid cerebral palsy
2) Upward gaze palsy
3) Sensorineural hearing loss
16. STEP TO PG-MD/MS -DR.AKIF A.B
Condition Cause
Hypoalbuminemia Normally bilirubin binds with albumin in circulation and in
hypoalbuminemia there is less albumin so increase free
unconjugated bilirubin present
Acidosis Displaces bilirubin from Albumin
Drugs
(Sulfonamides)
Displaces bilirubin from Albumin
Increased FFA Displaces bilirubin from Albumin
Asphyxia Make neurons more susceptible to toxic effects of bilirubin
Prematurity Make neurons more susceptible to toxic effects of bilirubin
Infections Make neurons more susceptible to toxic effects of bilirubin
17. STEP TO PG-MD/MS -DR.AKIF A.B
1) Pharmacological :
a) Barbiturates induces UDPG Transferase and enhances conjugation
b) Tin and Zinc : Decreases bilirubin formation by inhibiting heme oxygenase
2) Phototherapy
3) Exchange transfusion
18. STEP TO PG-MD/MS -DR.AKIF A.B
- If marking comes above this lines = Phototherapy should be done
19. -3 Types of Photochemical reactions :
1) Structural isomerisation : MC Type
2) Photoisomerisation
3) Photo-oxidation
-Maximum absorption peak of bilirubin = 450-460 nm
-Blue lamps are used = 425-475 nm