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Jaundice – neonatal, Jaundice – neonatal, prolonged and beyondprolonged and beyond
Dr. KW So
Department of Paediatrics
The Chinese University of Hong Kong
JaundiceJaundice
Clinical diagnosis – yellow discoloration of skin Clinical diagnosis – yellow discoloration of skin due to hyperbilirubinemiadue to hyperbilirubinemia
Commonest reason for admission in the Commonest reason for admission in the neonatal unitneonatal unit
During neonatal period, most are benign but During neonatal period, most are benign but may cause irreversible brain damagemay cause irreversible brain damage
Beyond neonatal period, all have underlying Beyond neonatal period, all have underlying causescauses
ClassificationClassification Pre-hepaticPre-hepatic
(Increase bilirubin load)(Increase bilirubin load) Increase breakdown of hemoglobinIncrease breakdown of hemoglobin
Hepatic Hepatic (Reduce conjugation or excretion)(Reduce conjugation or excretion) Reduce uridine diphosphate glucuronosyltransferase (UDPGT) Reduce uridine diphosphate glucuronosyltransferase (UDPGT)
activityactivity Hepatocellular dysfunctionHepatocellular dysfunction
Post-hepatic Post-hepatic (Reduce excretion outside the liver or increase reabsorption)(Reduce excretion outside the liver or increase reabsorption) Biliary tract obstructionBiliary tract obstruction Increase enterohepatic circulationIncrease enterohepatic circulation
Diagnosis of JaundiceDiagnosis of Jaundice
Clinical examinationClinical examination Sclera Vs SkinSclera Vs Skin
Transcutaneous bilirubinometerTranscutaneous bilirubinometer
Serum bilirubinSerum bilirubin
Early Neonatal JaundiceEarly Neonatal Jaundice
Within 48 hour after deliveryWithin 48 hour after delivery
Common or important causes:Common or important causes: HemolysisHemolysis
Blood group incompatibilityBlood group incompatibility G6PD deficiencyG6PD deficiency
Poor feeding / dehydrationPoor feeding / dehydration InfectionInfection
Blood group incompatibilityBlood group incompatibility
ABO group incompatibilityABO group incompatibility Commonest cause of early neonatal jaundice in HKCommonest cause of early neonatal jaundice in HK Blood group A or B baby of group O motherBlood group A or B baby of group O mother
Rhesus IncompatibilityRhesus Incompatibility Rhesus +ve baby of sensitized Rhesus –ve motherRhesus +ve baby of sensitized Rhesus –ve mother
Minor blood group incompatibilityMinor blood group incompatibility
ABO IncompatibilityABO Incompatibility
Early onset jaundice – within 24 hour after birthEarly onset jaundice – within 24 hour after birth
Baby blood group A or B, Mother blood group OBaby blood group A or B, Mother blood group O
Direct Coomb’s test +veDirect Coomb’s test +ve
Blood smear show increase spherocytesBlood smear show increase spherocytes
Usually can be controlled with phototherapyUsually can be controlled with phototherapy
Glucose-6-phosphate dehydrogenase deficiencyGlucose-6-phosphate dehydrogenase deficiency
Incidence: male 4.4%, female 0.35% in HKIncidence: male 4.4%, female 0.35% in HK Essential for the converting oxidized Essential for the converting oxidized
haemoglobin back to haemoglobinhaemoglobin back to haemoglobin
Oxidized Oxidized HbHb
HbHb
GSHGSH
GSSHGSSH
NADPNADP
NADPHNADPH
G6PG6P
6GP6GP
G6PD
G6PD deficiencyG6PD deficiency
Early or prolonged jaundiceEarly or prolonged jaundice
Acute severe jaundice with precipitating factorsAcute severe jaundice with precipitating factors
Massive intravascular haemolysis => free haemoglobin Massive intravascular haemolysis => free haemoglobin in urine (urine haemstix +ve but no red cell seen under in urine (urine haemstix +ve but no red cell seen under microscopy)microscopy)
Prone to bilirubin encephalopathyProne to bilirubin encephalopathy
May complicate with acute renal failureMay complicate with acute renal failure
Approach to Early Neonatal JaundiceApproach to Early Neonatal Jaundice
HistoryHistory
Age of onsetAge of onset General well beingGeneral well being Maternal blood groupMaternal blood group Screening result & family history of G6PD deficiencyScreening result & family history of G6PD deficiency Type & amount of feedingType & amount of feeding Urine output & colorUrine output & color Body weight changeBody weight change
Approach to Early Neonatal JaundiceApproach to Early Neonatal Jaundice
Physical ExaminationPhysical Examination General conditionGeneral condition Hydration stateHydration state Clinical jaundiceClinical jaundice
Dermal zoneDermal zone
Feature of kernicterusFeature of kernicterus
Dermal ZoneDermal Zone Bilirubin level (umol/L)Bilirubin level (umol/L)
11 70 - 13070 - 130
22 90 - 20090 - 200
33 130 - 280130 - 280
44 190 - 300190 - 300
55 >250>250
Approach to Early Neonatal JaundiceApproach to Early Neonatal Jaundice
InvestigationsInvestigations
Serum bilirubinSerum bilirubin Blood group of mother & babyBlood group of mother & baby G6PD screening resultG6PD screening result Urine for haemstix, RBCUrine for haemstix, RBC
Neonatal Jaundice D3 to 1 weekNeonatal Jaundice D3 to 1 week
Common causesCommon causes
Physiological jaundicePhysiological jaundice
Breast feed jaundiceBreast feed jaundice
G6PD deficiencyG6PD deficiency
Increase red cell loadIncrease red cell load CephalhaematomaCephalhaematoma PolycythaemiaPolycythaemia
Blood group incompatibilityBlood group incompatibility
Physiological JaundicePhysiological Jaundice
Transition from fetal to adult bilirubin metabolismTransition from fetal to adult bilirubin metabolism
Start from D2 to D4Start from D2 to D4
Reach maximum at D4 to D6Reach maximum at D4 to D6
Back to normal from D5 to D7 (up to 2 week in preterm Back to normal from D5 to D7 (up to 2 week in preterm infants)infants)
Clinically well except jaundiceClinically well except jaundice
Physiological JaundicePhysiological Jaundice Criteria that rule out physiological jaundiceCriteria that rule out physiological jaundice
Jaundice within the first 24 hoursJaundice within the first 24 hours
Jaundice persist >1 week in term or >2 week in preterm Jaundice persist >1 week in term or >2 week in preterm infantsinfants
Velocity of rise of bilirubin > 100 umol/l/dayVelocity of rise of bilirubin > 100 umol/l/day
Bilirubin level > 250 umol/lBilirubin level > 250 umol/l
Conjugated bilirubin > 34 umol/lConjugated bilirubin > 34 umol/l
Management : Management : Reassurance and monitor SBReassurance and monitor SB
Breast feed jaundiceBreast feed jaundice Inadequate intake in the few few daysInadequate intake in the few few days
Wt lost > 8% of birth weightWt lost > 8% of birth weight
Increase serum bilirubinIncrease serum bilirubin
Increase serum sodiumIncrease serum sodium
FeverFever
Mx: Rehydration +/- phototherapyMx: Rehydration +/- phototherapy
Approach to jaundice D3 to 1 weekApproach to jaundice D3 to 1 week
HistoryHistory
Age of onsetAge of onset General well beingGeneral well being Type & amount of feeding Type & amount of feeding Body weight changeBody weight change Urine output & colorUrine output & color Maternal blood groupMaternal blood group Screening result of G6PD & HypothyroidismScreening result of G6PD & Hypothyroidism
Approach to jaundice D3 to 1 weekApproach to jaundice D3 to 1 week
Physical ExaminationPhysical Examination
General conditionGeneral condition Body temperatureBody temperature Hydration stateHydration state Clinical jaundiceClinical jaundice
Dermal zoneDermal zone
Feature of kernicterusFeature of kernicterus
Approach to jaundice D3 to 1 weekApproach to jaundice D3 to 1 week
InvestigationsInvestigations
Serum bilirubinSerum bilirubin Blood group of mother & babyBlood group of mother & baby G6PD & TSH screening resultG6PD & TSH screening result Urine for haemoglobin, RBC & WBCUrine for haemoglobin, RBC & WBC
Prolonged Neonatal JaundiceProlonged Neonatal Jaundice
Beyond 1 week in term infantsBeyond 1 week in term infants Beyond 2 week in preterm infantsBeyond 2 week in preterm infants
Common & important causesCommon & important causes Breast milk jaundiceBreast milk jaundice Obstructive jaundiceObstructive jaundice Neonatal hepatitisNeonatal hepatitis HaemolysisHaemolysis Metabolic - HypothyroidismMetabolic - Hypothyroidism
Breast milk jaundiceBreast milk jaundice
Breast milkBreast milk Inhibits conjugationInhibits conjugation Enhance hydrolysation of conjugated bilirubinEnhance hydrolysation of conjugated bilirubin Enhance enterohepatic circulationEnhance enterohepatic circulation
Clinical presentationClinical presentation Prolonged jaundice beyond 1 weekProlonged jaundice beyond 1 week Resolve in 3 to 12 weeks Resolve in 3 to 12 weeks
Breast Milk JaundiceBreast Milk Jaundice
Clinically asymptomatic except jaundiceClinically asymptomatic except jaundice No hepatosplenomegalyNo hepatosplenomegaly Normal stool & urineNormal stool & urine Normal liver function test (included conjugated Normal liver function test (included conjugated
bilirubin)bilirubin) Other pathology e.g. haemolysis, hypothyroidism Other pathology e.g. haemolysis, hypothyroidism
ruled outruled out
Management: ReassuranceManagement: Reassurance
Obstructive jaundiceObstructive jaundice
Common causesCommon causes Biliary atresiaBiliary atresia Choledochal cystCholedochal cyst
Clinical featuresClinical features Prolonged jaundiceProlonged jaundice Pale stool & tea color urinePale stool & tea color urine Elevated direct bilirubin, Elevated direct bilirubin, -GTP-GTP Normal or mildly elevated liver enzymesNormal or mildly elevated liver enzymes
Obstructive JaundiceObstructive Jaundice
US Biliary tractUS Biliary tract Diagnose choledochal cyst & other anatomical obstructionDiagnose choledochal cyst & other anatomical obstruction
E-Hida ScanE-Hida Scan Radioisotope excrete via the CBD to doudenumRadioisotope excrete via the CBD to doudenum
Liver BiopsyLiver Biopsy Histological diagnosis of biliary atresia & hepatitisHistological diagnosis of biliary atresia & hepatitis
Intra-operative CholangiogramIntra-operative Cholangiogram Cannulation & inject contrast into CBD under direct visionCannulation & inject contrast into CBD under direct vision
Neonatal hepatitisNeonatal hepatitis
Congenital infectionCongenital infection TORCHTORCH
Acquired infectionAcquired infection Hepatitis virusesHepatitis viruses Cytomegalovirus (CMV)Cytomegalovirus (CMV) Epstein-Barr virus (EBV)Epstein-Barr virus (EBV)
Neonatal hepatitis without organism identifiedNeonatal hepatitis without organism identified AutoimmuneAutoimmune IdiopathicIdiopathic
Neonatal HepatitisNeonatal Hepatitis Elevated serum bilirubinElevated serum bilirubin
both direct & indirectboth direct & indirect
Elevated liver enzymesElevated liver enzymes
US – mild hepatomegalyUS – mild hepatomegaly
E-Hida: normal (may have false positive due to E-Hida: normal (may have false positive due to cholestasis)cholestasis)
Viral titre – Hepatisis viruses, TORCH, CMV, EBVViral titre – Hepatisis viruses, TORCH, CMV, EBV
Urine – CMV isolationUrine – CMV isolation
Haemolysis cause prolonged jaundiceHaemolysis cause prolonged jaundice
G6PD deficiencyG6PD deficiency
RBC Membrane defectRBC Membrane defect Hereditary spherocytosisHereditary spherocytosis
HaemoglobinopathyHaemoglobinopathy -thalassaemia-thalassaemia Usually present with anaemia rather than jaundiceUsually present with anaemia rather than jaundice
HypothyroidismHypothyroidism
Common in HK (1 in 4,000)Common in HK (1 in 4,000)
Clinical features like coarse face, macroglossia, Clinical features like coarse face, macroglossia, constipation & abdominal distention all develop constipation & abdominal distention all develop after 2 ~ 3 months of ageafter 2 ~ 3 months of age
Prolong jaundice may be the only early signProlong jaundice may be the only early sign
Check Hypothyroid screening result can prevent Check Hypothyroid screening result can prevent irreversible brain damageirreversible brain damage
Approach to prolonged neonatal jaundiceApproach to prolonged neonatal jaundice
HistoryHistory
Antenatal history suggested congenital Antenatal history suggested congenital infectioninfection
Onset & progress of jaundiceOnset & progress of jaundice Type of feedingType of feeding Urine & Stool colourUrine & Stool colour Newborn cord blood screening resultNewborn cord blood screening result Family history of jaundiceFamily history of jaundice
Approach to prolonged neonatal jaundiceApproach to prolonged neonatal jaundice
Physical ExaminationPhysical Examination
General well beingGeneral well being Rash or petechiaeRash or petechiae HepatosplenomegalyHepatosplenomegaly Features suggest hypothyroidismFeatures suggest hypothyroidism Examine stool & urineExamine stool & urine
Approach to prolonged neonatal jaundiceApproach to prolonged neonatal jaundice
InvestigationInvestigation
Serum bilirubin – direct & indirect, liver enzymes, Serum bilirubin – direct & indirect, liver enzymes, --GTPGTP
Urine for bile & urobilinogenUrine for bile & urobilinogen
Conjugated hyperbilirubinaemiaConjugated hyperbilirubinaemia Viral titre & urine for CMVViral titre & urine for CMV US biliary tract +/- E-Hida scanUS biliary tract +/- E-Hida scan
Unconjugated hyperbilirubinaemiaUnconjugated hyperbilirubinaemia CBC, Blood smear if unconjugated hyperbilirubinaemiaCBC, Blood smear if unconjugated hyperbilirubinaemia
Jaundice beyond neonatal periodJaundice beyond neonatal period Heterogenous etiologiesHeterogenous etiologies
Common or important causesCommon or important causes
Pre-hepaticPre-hepatic Hemolysis: Hereditary spherocytosis, Thalassaemia, G6PD Hemolysis: Hereditary spherocytosis, Thalassaemia, G6PD
deficiencydeficiency
HepaticHepatic Infective hepatitisInfective hepatitis Drug induced hepatitisDrug induced hepatitis Hepatocellular failure: cirrhosis, malignancyHepatocellular failure: cirrhosis, malignancy Metabolic: Wilson’s disease, Metabolic: Wilson’s disease, -1-antitrypsin deficiency-1-antitrypsin deficiency
ObstructiveObstructive Biliary stonesBiliary stones Cystic fibrosisCystic fibrosis
Approach Approach
HistoryHistory
Onset & progress of jaundiceOnset & progress of jaundice Associated symptomsAssociated symptoms
Fever, abdominal painFever, abdominal pain Urine colourUrine colour Stool colourStool colour
Drug historyDrug history Neonatal screening of G6PD statusNeonatal screening of G6PD status Family history of jaundiceFamily history of jaundice
Physical ExaminationPhysical Examination
Jaundice – ScleraJaundice – Sclera
PallorPallor
Stigmata of chronic liver diseaseStigmata of chronic liver disease
Abdominal ExaminationAbdominal Examination Hepatomegaly – tender liverHepatomegaly – tender liver SplenomegalySplenomegaly AsciteAscite
InvestigationInvestigation Direct & Total bilirubinDirect & Total bilirubin
Liver enzymesLiver enzymes
Viral titre for hepatitisViral titre for hepatitis HAV, HBV, EBVHAV, HBV, EBV
Urine for bile, urobilinogenUrine for bile, urobilinogen
CBC, blood smearCBC, blood smear
Hb pattern, reticulocyte count if indicatedHb pattern, reticulocyte count if indicated
US Abdomen if indicatedUS Abdomen if indicated
PhototherapyPhototherapy
converting bilirubin to converting bilirubin to lumirubin that bypass lumirubin that bypass liver conjugating liver conjugating systemsystem
Effectiveness depends Effectiveness depends on on light irradiancelight irradiance exposed body areaexposed body area wavelength of light, best wavelength of light, best
450nm i.e. blue light450nm i.e. blue light
Side effects of PhototherapySide effects of Phototherapy
body tempbody temp & & fluid lostfluid lost due to radiant heat due to radiant heat
loose stoolloose stool : photodegradation products : photodegradation products
retinal damageretinal damage : eye shield : eye shield
photo rashphoto rash : UV light induced mast cell : UV light induced mast cell damagedamage
bronze baby syndromebronze baby syndrome : phototherapy in : phototherapy in obstructive jaundice, ? accumulation of obstructive jaundice, ? accumulation of lumirubin under skinlumirubin under skin
Exchange TransfusionExchange Transfusion
MechanismMechanism Remove bilirubinRemove bilirubin Remove antibodiesRemove antibodies
Type of blood usedType of blood used citrate phosphate dextrose (CPD) banked bloodcitrate phosphate dextrose (CPD) banked blood freshly collected < 5 daysfreshly collected < 5 days compatible with baby & mother’s blood groupcompatible with baby & mother’s blood group Rh-ve in case of Rh incompatibilityRh-ve in case of Rh incompatibility
Exchange TransfusionExchange Transfusion
Volume of exchange transfusionVolume of exchange transfusion 2 times blood volume2 times blood volume remove 87% of total bilirubin theoreticallyremove 87% of total bilirubin theoretically 45% of original level may still remain & rebound to 45% of original level may still remain & rebound to
60% due to redistribution60% due to redistribution
MethodMethod Umbilical vein catheterizationUmbilical vein catheterization peripheral artery & veinperipheral artery & vein
Push & PullPush & Pull Vs VsIsovolumetric techniqueIsovolumetric technique
Time
Blood V
olum
e
Complication of Exchange TransfusionComplication of Exchange Transfusion
umbilical catheterizationumbilical catheterization: embolism, portal vein : embolism, portal vein thrombosisthrombosis
peripheral arteryperipheral artery : ischaemia of extremity : ischaemia of extremity
haemodynamic distrubancehaemodynamic distrubance : hypotension, : hypotension, arrhythmiaarrhythmia
coagulationcoagulation : thrombocytopenia : thrombocytopenia
metabolicmetabolic : hyperkalemia, hypocalcemia, : hyperkalemia, hypocalcemia, hypoglycemiahypoglycemia
infectioninfection : HIV, CMV, Hepatitis viruses : HIV, CMV, Hepatitis viruses