Download ppt - Jaundice – neonatal, prolonged and beyond Dr. KW So Department of Paediatrics The Chinese University of Hong Kong

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


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


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


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


Physical ExaminationPhysical Examination

General conditionGeneral condition Body temperatureBody temperature Hydration stateHydration state Clinical jaundiceClinical jaundice

Dermal zoneDermal zone

Feature of kernicterusFeature of kernicterus


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


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



General well beingGeneral well being Rash or petechiaeRash or petechiae HepatosplenomegalyHepatosplenomegaly Features suggest hypothyroidismFeatures suggest hypothyroidism Examine stool & urineExamine stool & urine


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


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