بسم ال الرحمن الرحيمبسم ال الرحمن الرحيم

Jaundice in the newbornJaundice in the newbornDR.TOSIF AHMADDR.TOSIF AHMAD

TMO-PAEDSTMO-PAEDS

HISTORYHISTORY

A neonate of 5 days age presented to A neonate of 5 days age presented to nursery with fever, jaundice, reluctant to nursery with fever, jaundice, reluctant to take feed and fits.take feed and fits.

Baby was delivered in home at full term Baby was delivered in home at full term pregnancy and uneventful labour.pregnancy and uneventful labour.

One of the elder brother of the baby died One of the elder brother of the baby died of jaundice at the age of 7 days.of jaundice at the age of 7 days.

EXAMINATIONEXAMINATION

The baby is deeply jaundiced, pale, and The baby is deeply jaundiced, pale, and has hypertonia and decorticate posture, has hypertonia and decorticate posture, although there are no fits at the moment.although there are no fits at the moment.

INVESTIGATIONSINVESTIGATIONS

HB 12gHB 12gSBR 27mgSBR 27mgBlood group of baby B +veBlood group of baby B +veBlood group of mother B –veBlood group of mother B –veThe mother has not received any injection The mother has not received any injection

after deliveryafter delivery

DIAGNOSISDIAGNOSIS

Rh incompatibility leading to neonatal Rh incompatibility leading to neonatal jaundice and kernicterusjaundice and kernicterus

Jaundice in the newbornJaundice in the newborn

ClinicalClinical jaundice jaundice appear at SBr 5 mg/dlappear at SBr 5 mg/dl

25% to 50% of term 25% to 50% of term newborns have clinical newborns have clinical jaundice.jaundice.

Jaundice may be Jaundice may be caused by serious caused by serious illness & lead to illness & lead to keriniectrus.keriniectrus.

75% of bilirubin comes 75% of bilirubin comes from haemoglobin and from haemoglobin and 25% from other sources25% from other sources

Neonatal jaundiceNeonatal jaundice

Neonatal jaundice

physiological pathological

unconjugatedconjugated

Intrahepatic

Hepatic injures

Extrahepaitc

infectious

metabolic

Neonatal hepatitis

Paucity of hepatic ducts

Physiological jaundicePhysiological jaundice

Start after the first 24hours.Start after the first 24hours.Peak in the fourth or fifth day {not >12 Peak in the fourth or fifth day {not >12

mg/dl} in term babies and not more than mg/dl} in term babies and not more than 15 mg/dl in premature15 mg/dl in premature

The baby is well.The baby is well.Clear in 2 weeks in term and 3 weeks in Clear in 2 weeks in term and 3 weeks in

premature.premature.Bilirubin is unconjucated.Bilirubin is unconjucated.The rise is not more than 0.5 mg /hThe rise is not more than 0.5 mg /h

Causes of physiological jaundiceCauses of physiological jaundice

High haemoglobinHigh haemoglobin Decrease RBC life Decrease RBC life

span due to HbFspan due to HbF Increase Increase

enterohepatic enterohepatic circulation.circulation.

Defective conjugation.Defective conjugation. Decrease hepatic Decrease hepatic

excretionexcretion

Pathological jaundicePathological jaundice

Jaundice is pathological if:Jaundice is pathological if: PrePresent on 1sent on 1stst day of life. day of life. SBr level increases more then SBr level increases more then

0.5mg/dl/hr.0.5mg/dl/hr. Peak SBr is greater than13mg/dl in Peak SBr is greater than13mg/dl in

term infant or 15mg/dl in preterm term infant or 15mg/dl in preterm infant.infant.

Pathological jaundicePathological jaundice

Direct bilirubin fraction is greater than 1.5-Direct bilirubin fraction is greater than 1.5-2mg/dl2mg/dl

Hepatosplenomegaly and anemia are Hepatosplenomegaly and anemia are present.present.

Causes of unconjucated Causes of unconjucated hyperbiliruniemiahyperbiliruniemia

Glucuronyl transferase defect.(Crigler Glucuronyl transferase defect.(Crigler Najjar syndrome,Gilbert syndrome)Najjar syndrome,Gilbert syndrome)

Defective conjugation.Defective conjugation.Jaundice of prematurity.Jaundice of prematurity.Breast milk jaundice.Breast milk jaundice.Hypothyroidism.Hypothyroidism.Other conditions Pyloric stenosis,infant of Other conditions Pyloric stenosis,infant of

diabetic mother, down's syndromediabetic mother, down's syndrome

G6PD deficiencyG6PD deficiency

Investigation of unconj-Investigation of unconj-hyberbilirubinneamiahyberbilirubinneamia

Split biliurubin.Split biliurubin.Blood groups and Rh of mother and baby.Blood groups and Rh of mother and baby.coomb’s test of mother and baby.coomb’s test of mother and baby.CBC and reticulocyte.CBC and reticulocyte.G-6-P-D estimationG-6-P-D estimationBlood film and osmotic fragility test.Blood film and osmotic fragility test.

TFT and urine for reducing substance.TFT and urine for reducing substance.

Causes of conjugated Causes of conjugated hyberbilirubineamiahyberbilirubineamia

Hepatitis: Hepatitis: CMV.toxoplasmosis.rubella.herpes.Hep A and CMV.toxoplasmosis.rubella.herpes.Hep A and b,syphilis,E coli.b,syphilis,E coli.

Metabolic: Metabolic: Galctosemia,Tyroseanemia,Fructoseamia.Galctosemia,Tyroseanemia,Fructoseamia.

Cystic fibrosis.Cystic fibrosis. Alpha one anti trypsin deficiency.Alpha one anti trypsin deficiency. Gauchers and neimman pickGauchers and neimman pick Biliary Artesia (intrahepatic and extrahepatic)Biliary Artesia (intrahepatic and extrahepatic) Choldoccal cyst.Choldoccal cyst. T.P.NT.P.N

Investigation of conjugated Investigation of conjugated hyperbiliruniemiahyperbiliruniemia

L.F.TL.F.T PT.PTT.PT.PTT. Urine for glucose and Urine for glucose and

reducing substance.reducing substance. Serum and urine amino Serum and urine amino

acid determinations.acid determinations. TORCH serology.TORCH serology. Ultrasound.Ultrasound. Liver scanLiver scan Duodenal aspiration.Duodenal aspiration. Liver biopsy.Liver biopsy.

ManagementManagement

Prevention:Prevention: Rh incompatibility----- Anti D Rh incompatibility----- Anti D Syphlis---PencillineSyphlis---Pencilline

Specific therapy:Specific therapy: PhototherapyPhototherapy Exchange transfusionExchange transfusion Septicaemia---- Antibiotic.Septicaemia---- Antibiotic. Surgery------------ Ex hepatic biliary Artesia.Surgery------------ Ex hepatic biliary Artesia. Lactose free formula for galactosemia.Lactose free formula for galactosemia.

PhototherapyPhototherapy

Wave length 450-460 Wave length 450-460 ---- Reduce bilirubin Reduce bilirubin To harmless To harmless compound excreted in compound excreted in the urine.the urine.

Side effects:Side effects:

Retinal damage, Nasal Retinal damage, Nasal obstruction, Mild obstruction, Mild diarrhea,Dehydration,diarrhea,Dehydration,Bronze baby Bronze baby syndromesyndrome

Exchange TransfusionExchange Transfusion

Indicated when Indicated when bilirubin reach toxic bilirubin reach toxic level.level.

Mortality1%Mortality1% Remove bilirubin Remove bilirubin

,antibodies ,correct ,antibodies ,correct anaemia.anaemia.

Double blood volume Double blood volume is used 85 ml /kgis used 85 ml /kg

Exchange TransfusionExchange Transfusion

Side effectsSide effects::HypervolemiaHypervolemiaHypothermiaHypothermiaSkin rashesSkin rashesCardiac failureCardiac failureHypocalcemiaHypocalcemiaAir embolismAir embolism

PhenobarbitonePhenobarbitone

This act as enzyme inducer which This act as enzyme inducer which increase amount of glucoreny transferase increase amount of glucoreny transferase and protein z.and protein z.

Used in Crigler Najjar syndrome ,Gilbert Used in Crigler Najjar syndrome ,Gilbert syndrome.syndrome.

KernicterusKernicterus

Yellow staining of Yellow staining of nuclear centres of the nuclear centres of the brainbrain

Due to high level of Due to high level of indirect bilirubin.indirect bilirubin.

Bilirubin cause neural Bilirubin cause neural loss.loss.

Bilrubin inhibit cell Bilrubin inhibit cell respiration, protein respiration, protein synthesis,glucouse synthesis,glucouse metabolism.metabolism.

KERNICTERUSKERNICTERUS

Poor feeding and lethargy-Poor feeding and lethargy- fits,rigidityfits,rigidityspasticity spasticity deafnes,athetosisdeafnes,athetosis

PathophysiologyPathophysiology

UCB is lipophilic and crosses the Blood-UCB is lipophilic and crosses the Blood-Brain BarrierBrain Barrier

UCB has an affinity for the basal ganglia, UCB has an affinity for the basal ganglia, hippocampus, cranial nerve nucleihippocampus, cranial nerve nuclei

UCB interrupts metabolism in glial cells UCB interrupts metabolism in glial cells and causes apoptosis of neuronsand causes apoptosis of neurons

Clinical ManifestationsClinical ManifestationsBilirubin EncephalopathyBilirubin Encephalopathy

Acute Bilirubin EncephalopathyAcute Bilirubin Encephalopathy11stst phase: hypotonia, poor suck-present in the phase: hypotonia, poor suck-present in the

first few daysfirst few days22ndnd phase: Hypertonia (retrocollis and phase: Hypertonia (retrocollis and

opisthotonos), feveropisthotonos), fever33rdrd phase: Gradual disappearance of the phase: Gradual disappearance of the

hypertonia-Up to years after the first weekhypertonia-Up to years after the first week

Clinical Manifestations:Clinical Manifestations:Bilirubin EncephalopathyBilirubin Encephalopathy

Chronic Encephalopathy: Chronic Encephalopathy: Extrapyramidal abnormalities: Facial grimacing, Extrapyramidal abnormalities: Facial grimacing,

drooling, dysarthria, and athetosis--may develop by drooling, dysarthria, and athetosis--may develop by 18mo or delayed to 8or9 years.18mo or delayed to 8or9 years.

Hearing loss is usually due to injury of the cochlear Hearing loss is usually due to injury of the cochlear nuclei in the brainstem. It may be the only nuclei in the brainstem. It may be the only manifestationmanifestation

Gaze abnormalities: Limitation of upward gaze, Gaze abnormalities: Limitation of upward gaze, palsiespalsies

Cerebral cortex is relatively spared, so intelligence is Cerebral cortex is relatively spared, so intelligence is often close to normaloften close to normal

PreventionPrevention

Treatment of Hyperbilirubinemia by:Treatment of Hyperbilirubinemia by:PhototherapyPhototherapyExchange transfusionExchange transfusion

Thank youThank you