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

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  • Slide 1
  • Jaundice neonatal, prolonged and beyond Dr. KW So Department of Paediatrics The Chinese University of Hong Kong
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  • Jaundice Clinical diagnosis yellow discoloration of skin due to hyperbilirubinemia Commonest reason for admission in the neonatal unit During neonatal period, most are benign but may cause irreversible brain damage Beyond neonatal period, all have underlying causes
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  • Classification Pre-hepatic (Increase bilirubin load) Increase breakdown of hemoglobin Hepatic (Reduce conjugation or excretion) Reduce uridine diphosphate glucuronosyltransferase (UDPGT) activity Hepatocellular dysfunction Post-hepatic (Reduce excretion outside the liver or increase reabsorption) Biliary tract obstruction Increase enterohepatic circulation
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  • Diagnosis of Jaundice Clinical examination Sclera Vs Skin Transcutaneous bilirubinometer Serum bilirubin
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  • Early Neonatal Jaundice Within 48 hour after delivery Common or important causes: Hemolysis Blood group incompatibility G6PD deficiency Poor feeding / dehydration Infection
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  • Blood group incompatibility ABO group incompatibility Commonest cause of early neonatal jaundice in HK Blood group A or B baby of group O mother Rhesus Incompatibility Rhesus +ve baby of sensitized Rhesus ve mother Minor blood group incompatibility
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  • ABO Incompatibility Early onset jaundice within 24 hour after birth Baby blood group A or B, Mother blood group O Direct Coombs test +ve Blood smear show increase spherocytes Usually can be controlled with phototherapy
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  • Glucose-6-phosphate dehydrogenase deficiency Incidence: male 4.4%, female 0.35% in HK Essential for the converting oxidized haemoglobin back to haemoglobin Oxidized Hb Hb GSH GSSH NADP NADPH G6P 6GP G6PD
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  • G6PD deficiency Early or prolonged jaundice Acute severe jaundice with precipitating factors Massive intravascular haemolysis => free haemoglobin in urine (urine haemstix +ve but no red cell seen under microscopy) Prone to bilirubin encephalopathy May complicate with acute renal failure
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  • Approach to Early Neonatal Jaundice History Age of onset General well being Maternal blood group Screening result & family history of G6PD deficiency Type & amount of feeding Urine output & color Body weight change
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  • Approach to Early Neonatal Jaundice Physical Examination General condition Hydration state Clinical jaundice Dermal zone Feature of kernicterus Dermal Zone Bilirubin level (umol/L) 1 70 - 130 2 90 - 200 3 130 - 280 4 190 - 300 5>250
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  • Approach to Early Neonatal Jaundice Investigations Serum bilirubin Blood group of mother & baby G6PD screening result Urine for haemstix, RBC
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  • Neonatal Jaundice D3 to 1 week Common causes Physiological jaundice Breast feed jaundice G6PD deficiency Increase red cell load Cephalhaematoma Polycythaemia Blood group incompatibility
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  • Physiological Jaundice Transition from fetal to adult bilirubin metabolism Start from D2 to D4 Reach maximum at D4 to D6 Back to normal from D5 to D7 (up to 2 week in preterm infants) Clinically well except jaundice
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  • Physiological Jaundice Criteria that rule out physiological jaundice Jaundice within the first 24 hours Jaundice persist >1 week in term or >2 week in preterm infants Velocity of rise of bilirubin > 100 umol/l/day Bilirubin level > 250 umol/l Conjugated bilirubin > 34 umol/l Management : Reassurance and monitor SB
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  • Breast feed jaundice Inadequate intake in the few few days Wt lost > 8% of birth weight Increase serum bilirubin Increase serum sodium Fever Mx: Rehydration +/- phototherapy
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  • Approach to jaundice D3 to 1 week History Age of onset General well being Type & amount of feeding Body weight change Urine output & color Maternal blood group Screening result of G6PD & Hypothyroidism
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  • Approach to jaundice D3 to 1 week Physical Examination General condition Body temperature Hydration state Clinical jaundice Dermal zone Feature of kernicterus
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  • Approach to jaundice D3 to 1 week Investigations Serum bilirubin Blood group of mother & baby G6PD & TSH screening result Urine for haemoglobin, RBC & WBC
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  • Prolonged Neonatal Jaundice Beyond 1 week in term infants Beyond 2 week in preterm infants Common & important causes Breast milk jaundice Obstructive jaundice Neonatal hepatitis Haemolysis Metabolic - Hypothyroidism
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  • Breast milk jaundice Breast milk Inhibits conjugation Enhance hydrolysation of conjugated bilirubin Enhance enterohepatic circulation Clinical presentation Prolonged jaundice beyond 1 week Resolve in 3 to 12 weeks
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  • Breast Milk Jaundice Clinically asymptomatic except jaundice No hepatosplenomegaly Normal stool & urine Normal liver function test (included conjugated bilirubin) Other pathology e.g. haemolysis, hypothyroidism ruled out Management: Reassurance
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  • Obstructive jaundice Common causes Biliary atresia Choledochal cyst Clinical features Prolonged jaundice Pale stool & tea color urine Elevated direct bilirubin, -GTP Normal or mildly elevated liver enzymes
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  • Obstructive Jaundice US Biliary tract Diagnose choledochal cyst & other anatomical obstruction E-Hida Scan Radioisotope excrete via the CBD to doudenum Liver Biopsy Histological diagnosis of biliary atresia & hepatitis Intra-operative Cholangiogram Cannulation & inject contrast into CBD under direct vision
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  • Neonatal hepatitis Congenital infection TORCH Acquired infection Hepatitis viruses Cytomegalovirus (CMV) Epstein-Barr virus (EBV) Neonatal hepatitis without organism identified Autoimmune Idiopathic
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  • Neonatal Hepatitis Elevated serum bilirubin both direct & indirect Elevated liver enzymes US mild hepatomegaly E-Hida: normal (may have false positive due to cholestasis) Viral titre Hepatisis viruses, TORCH, CMV, EBV Urine CMV isolation
  • Slide 27
  • Haemolysis cause prolonged jaundice G6PD deficiency RBC Membrane defect Hereditary spherocytosis Haemoglobinopathy -thalassaemia Usually present with anaemia rather than jaundice
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  • Hypothyroidism Common in HK (1 in 4,000) Clinical features like coarse face, macroglossia, constipation & abdominal distention all develop after 2 ~ 3 months of age Prolong jaundice may be the only early sign Check Hypothyroid screening result can prevent irreversible brain damage
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  • Approach to prolonged neonatal jaundice History Antenatal history suggested congenital infection Onset & progress of jaundice Type of feeding Urine & Stool colour Newborn cord blood screening result Family history of jaundice
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  • Approach to prolonged neonatal jaundice Physical Examination General well being Rash or petechiae Hepatosplenomegaly Features suggest hypothyroidism Examine stool & urine
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  • Approach to prolonged neonatal jaundice Investigation Serum bilirubin direct & indirect, liver enzymes, - GTP Urine for bile & urobilinogen Conjugated hyperbilirubinaemia Viral titre & urine for CMV US biliary tract +/- E-Hida scan Unconjugated hyperbilirubinaemia CBC, Blood smear if unconjugated hyperbilirubinaemia
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  • Jaundice beyond neonatal period Heterogenous etiologies Common or important causes Pre-hepatic Hemolysis: Hereditary spherocytosis, Thalassaemia, G6PD deficiency Hepatic Infective hepatitis Drug induced hepatitis Hepatocellular failure: cirrhosis, malignancy Metabolic: Wilsons disease, -1-antitrypsin deficiency Obstructive Biliary stones Cystic fibrosis
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  • Approach History Onset & progress of jaundice Associated symptoms Fever, abdominal pain Urine colour Stool colour Drug history Neonatal screening of G6PD status Family history of jaundice
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  • Physical Examination Jaundice Sclera Pallor Stigmata of chronic liver disease Abdominal Examination Hepatomegaly tender liver Splenomegaly Ascite
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  • Investigation Direct & Total bilirubin Liver enzymes Viral titre for hepatitis HAV, HBV, EBV Urine for bile, urobilinogen CBC, blood smear Hb pattern, reticulocyte count if indicated US Abdomen if indicated
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  • Phototherapy converting bilirubin to lumirubin that bypass liver conjugating system Effectiveness depends on light irradiance exposed body area wavelength of light, best 450nm i.e. blue light
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  • Side effects of Phototherapy body temp & fluid lost due to radiant heat loose stool : photodegradation products retinal damage : eye shield photo rash : UV light induced mast cell damage bronze baby syndrome : phototherapy in obstructive jaundice, ? accumulation of lumirubin under skin
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  • Exchange Transfusion Mechanism Remove bilirubin Remove antibodies Type of blood used citrate phosphate dextrose (CPD) banked blood freshly collected < 5 days compatible with baby & mother s blood group Rh-ve in case of Rh incompatibility
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  • Exchange Transfusion Volume of exchange transfusion 2 times blood volume remove 87% of total bilirubin theoretically 45% of original level may still remain & rebound to 60% due to redistribution Method Umbilical vein catheterization peripheral artery & vein
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  • Push & Pull Vs Isovolumetric technique Time Blood Volume
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  • Complication of Exchange Transfusion umbilical catheterization: embolism, portal vein thrombosis peripheral artery : ischaemia of extremity haemodynamic distrubance : hypotension, arrhythmia coagulation : thrombocytopenia metabolic : hyperkalemia, hypocalcemia, hypoglycemia infection : HIV, CMV, Hepatitis viruses