NEONATAL

HYPERBILIRUBINEM
IA
By: UMALI, JOZA A.
BILIRUBIN
PRODUCTION
 1g Hgb = 35mg Bilirubin

 Newborn
 2-3fold greater rate
compared to adults
 6-10mg/kg/24hrs vs
3mg/kg/24hrs
UNCONJUGATED BILIRUBIN
 Indirect
 Toxic to the CNS

 Insoluble to water; lipid soluble

 Binds to albumin (1g : 8.5mg Bilirubin)

 Transferred across the placenta  conjugated by

maternal hepatic enzyme
CONJUGATED BILIRUBIN
 Direct
 Water soluble

 Mostly excreted through the bile  small intestine 

stool
 When hydrolysed  unconjugated by glucuronidase

 With bacteria: converted to: (limits bilirubin

reabsorption)
 Urobilinogen
 Stercobilinogen
ETIOLOGY OF INDIRECT
UNCONJUGATED BILIRUBIN
 Physiologic Jaundice
 Criggler Najjar Syndrome
 Gilbert Disease
 Breastmilk Jaundice
 Jaundice on 1st day of life
PHYSIOLOGIC JAUNDICE
 Common cause of increased bilirubin in NB
 Diagnosis of exclusion

 Results from ↑ Bilirubin production

↑ RBC mass
 Shortened RBC life span
 Hepatic immaturity of ligandin & gluconyltransferase

 Term: not >12mg/dL on D3

 Preterm: 15mg/dL on D5

 Breastfed infant: 15-17mg/dL vs 12mg/dL

CRIGLER-NAJJAR SYNDROME
 Serious, rare, permanent deficiency of
gluconyltransferase
 Severe indirect hyperbilirubinemia

 Autosomal Dominant
 Responds to enzyme induction by phenobarbital  ↑enzyme
activity and ↓bilirubin level
 Autosomal Recessive
 Does not respond to phenobarbital
 Persistent indirect hyperbilirubinemia  kernicterus
GILBERT DISEASE
 Caused by a mutation of the promoter region of
gluconyltransferase
 Results in mild indirect hyperbilirubinemia

 If with icterogenic factor  more severe jaundice

BREAST MILK JAUNDICE
 Associated with unconjugated hyperbilirubinemia
 No hemolysis at 1st & 2nd wk of life

 Bilirubin >20mg/dL

 TX: interruption of breastfeeding for 1-2days

 Breastmilk
 May contain an inhibitor of bilirubin conjugation
 May ↑ enterohepatic recirculation
JAUNDICE ON 1ST DAY OF LIFE
 Pathologic
 Early onset results from:
 Hemolysis
 Internal Hemorrhage
 Infection

 Bilirubin
 0.5mg/dL/hr
 Peak: >13mg/dL (term)
 Direct: >1.5mg/dL
ETIOLOGY OF DIRECT CONJUGATED
HYPERBILIRUBINEMIA
 Cholestasis (i.e. Biliary Atresia)
 Hepatocellular injury

 Direct Bilirubin >2mg/dL or >20% of Total Bilirubin

 Do not respond to phototherapy/exchange transfusion
KERNICTERUS
 Indirect Bilirubin crossing BBB
 Disrupts neuronal metabolism and function esp. in basal
ganglia
 Caused by increase indirect bilirubin exceeding the
binding capacity of albumin
 May be noted if Bilirubin
 >25mg/dL
 <20mg/dL
 i.e. sepsis, meningitis, hemolysis, asphyxia, hypoxia, hypothermia,
hypoglycemia, sulfa-drugs, prematurity
 TX: Exchange transfusion
KERNICTERUS
MANIFESTATION
 Early (Within 4DOL)  Late
 Lethargy  Bulging fontanelle
 Hypotonia  Opisthotonic posturing
 Irritability  Pulmonary hemorrhage
 Poor Moro response  Fever
 Poor feeding  Hypertonicity
 High pitched cry  Paralysis of upward gaze
 emesis  seizure
KERNICTERUS
COMPLICATIONS
 Nerve deafness
 Choreoathetoid cerebral palsy

 Mental retardation

 Enamel displasia

 Discoloration of teeth
THERAPHY FOR INDIRECT
HYPERBILIRUBINEMIA
 Phototherapy
 Exchange Transfusion
PHOTOTHERAPY
 Effective  Complications
 Safe  ↑insensible water loss
 Diarrhea
 Started if IBL between 16
 Dehydration
& 18mg/dL
 Macular-papular red skin
 Max: 425-275nm
rash
wavelength  Lethargy
 Indirect Bilirubin 
 Masking of cyanosis
isomers; lumirubin  Nasal obstruction
 Retinal damage
 Skin-bronzing
EXCHANGE TRANSFUSION
 For infants with dangerously ↑IBL and at risk of
kernicterus
 Rule of Thumb:
 IBL 20mg/dL (exchange #) with hemolysis for infants with
hemolysis weighing >2000g
 Breastfed: no need unless IBL>25mg/dL
 exchangeable level of indirect bilirubin for other infants
may be estimated by:
 10% of the birth weight in grams: the level in an infant
weighing 1500 g would be 15 mg/dL
 Infants <1000g if IBL >10mg/dL
EXCHANGE TRANSFUSION
 Small infusions of whole blood crossmatched with that
of the mother and infant are alternated with withdrawals
of an equivalent quantity of the infant's blood
 Aliquots of 5-20ml/cycle
 Depends on infant’s size
 Duration: 45-90mins
 Amt of Bld Exchange = Wt(kg) x 85ml/kg x 2
EXCHANGE TRANSFUSION
COMPLICATION
 Blood  Unusual
 Transfusion reaction  Thrombocytopenia
 Metabolic instability  Graft vs Host disease
 infection

 Catheter
 Vessel
perforation
 Hemorrhage

 Procedure
 Hypotension
 Necrotizing enterocolitis
THANK YOU!