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Neonatal Hyperbilirubinem IA: By: Umali, Joza A

This document discusses neonatal hyperbilirubinemia or jaundice in newborns. It describes that newborns have higher rates of bilirubin production compared to adults. Unconjugated or indirect bilirubin is insoluble and toxic to the central nervous system. Physiologic jaundice is common in newborns and results from increased bilirubin production and hepatic immaturity. Kernicterus can occur if high levels of indirect bilirubin exceed albumin binding capacity. Treatment options include phototherapy and exchange transfusion for dangerously high indirect bilirubin levels to prevent kernicterus and its complications.

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50 views22 pages

Neonatal Hyperbilirubinem IA: By: Umali, Joza A

This document discusses neonatal hyperbilirubinemia or jaundice in newborns. It describes that newborns have higher rates of bilirubin production compared to adults. Unconjugated or indirect bilirubin is insoluble and toxic to the central nervous system. Physiologic jaundice is common in newborns and results from increased bilirubin production and hepatic immaturity. Kernicterus can occur if high levels of indirect bilirubin exceed albumin binding capacity. Treatment options include phototherapy and exchange transfusion for dangerously high indirect bilirubin levels to prevent kernicterus and its complications.

Uploaded by

mesho two
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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!

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