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Hyperbilirubinemia
Bilirubin physiology
Physiological Jaundice
PHASE 1
Rapid rise in serum bilirubin levels to 12 to 15
mg / dl
plasma
Defective bilirubin conjugation
Defective bilirubin excretion
Pathological jaundice
or 5 mg/dl/24 hours
Total bilirubin >15 mg/dl
Direct bilirubin > 2 mg/dl
Causes of
Unconjugated
Hyperbilirubinemia
INCREASED PRODUCTION
1) Fetomaternal blood group incompatibility :
Rh,ABO
2) Hereditary spherocytosis
3) Non spherocytic hemolytic anemia:G6PD
deficiency,PK deficiency,Vit.k induced
hemolysis
4) Sepsis
5) Increased enterohepatic circulation ,eg large
bowel obstruction
DECREASED CLEARANCE
1) Inborn errors of metabolism : Crigler najjar
syndrome type 1 & 2
KERNICTERUS
KERNICTERUS
Yellowish staining & necrosis of neurons in
basal ganglia,hippocampus,subthalamic
nuclei & cerebellum
CLINICALLY :
I. Phase 1:poor
suck,lethargy,hypotonia,depressed
sensorium
II. Phase 2:fever,hypertonia,opisthotonus
III. Phase 3:high pitched cry,convulsions,death
TREATMENT
2.)EXCHANGE
1.)PHOTOTHERAPY
TRANSFUSION
Phototherapy – Mechanism of Action
OXIDATIO
N
STRUCTUR
AL
ISOMERISA
TION
GEOMETRIC
PHOTOISOMERIS
ATION
Phototherapy
Indications
When bilirubin levels may be hazardous to
infant,but not yet reached exchange
transfusin levels.
Prophylactically in extremely low birth weight
or severely bruised infants.
Phototherapy
Complications
1. Increased insensible water loss
2. Diarrhoea
3. Retinal damage
4. Bronze baby syndrome
5. Hypocalcemia
Exchange Transfusion
Indications
1. No response to phototherapy
2. To correct anemia & improve CCF in
hydropic infants
3. To stop hemolysis & production by removing
antibodies & sensitised rbc s
4. Septicemia
Exchange transfusion
Complications
o Bacterial sepsis
o Thrombocytopenia
o Portal vein thrombosis
o Umblical or portal vein perforation
o Arrythmia,cardiac arrest
o Hypocalcemia,hypoglycemia,hypomagnesemia
o Metabolic acidosis,alkalosis
o HIV,HBV,HCV,GVHD
Conjugated Hyperbilirubinemia
Directbilirubin > 2mg/dl or >15% of total
bilirubin
Causes –
Biliary atresia
Neonatal hepatitis
Sepsis
Biliary atresia
Clay coloured stools from day 4-5 of life
Gradual decline in liver function
Anemia & fat soluble vitamin deficiencies set
in
Types
1) Extra hepatic – good prognosis,CBD or CHD
involved,surgery successful within 60 days
2) Intra hepatic – poor prognosis,orthotopic
liver transplant needed
Neonatal Hepatitis
Presents at 4-6 weeks of age
Symptoms are usually intermittent,unlike
biliary atresia
Presence of cholestatic inflammatory process