Jaundice neonatal prolonged and beyond
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Jaundice – neonatal, prolonged and beyond. Dr. KW So Department of Paediatrics The Chinese University of Hong Kong. Jaundice. Clinical diagnosis – yellow discoloration of skin due to hyperbilirubinemia Commonest reason for admission in the neonatal unit

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Jaundice – neonatal, prolonged and beyond

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Jaundice neonatal prolonged and beyond

Jaundice – neonatal, prolonged and beyond

Dr. KW So

Department of Paediatrics

The Chinese University of Hong Kong


Jaundice

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


Classification

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


Diagnosis of jaundice

Diagnosis of Jaundice

  • Clinical examination

    • Sclera Vs Skin

  • Transcutaneous bilirubinometer

  • Serum bilirubin


Early neonatal jaundice

Early Neonatal Jaundice

  • Within 48 hour after delivery

  • Common or important causes:

    • Hemolysis

      • Blood group incompatibility

      • G6PD deficiency

    • Poor feeding / dehydration

    • Infection


Blood group incompatibility

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


Abo incompatibility

ABO Incompatibility

  • Early onset jaundice – within 24 hour after birth

  • Baby blood group A or B, Mother blood group O

  • Direct Coomb’s test +ve

  • Blood smear show increase spherocytes

  • Usually can be controlled with phototherapy


Glucose 6 phosphate dehydrogenase deficiency

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

GSH

NADP

G6P

G6PD

6GP

Hb

GSSH

NADPH


G6pd deficiency

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


Approach to early neonatal jaundice

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


Approach to early neonatal jaundice1

Approach to Early Neonatal Jaundice

  • Physical Examination

    • General condition

    • Hydration state

    • Clinical jaundice

      • Dermal zone

    • Feature of kernicterus


Approach to early neonatal jaundice2

Approach to Early Neonatal Jaundice

  • Investigations

    • Serum bilirubin

    • Blood group of mother & baby

    • G6PD screening result

    • Urine for haemstix, RBC


Neonatal jaundice d3 to 1 week

Neonatal Jaundice D3 to 1 week

  • Common causes

    • Physiological jaundice

    • Breast feed jaundice

    • G6PD deficiency

    • Increase red cell load

      • Cephalhaematoma

      • Polycythaemia

    • Blood group incompatibility


Physiological jaundice

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


Physiological jaundice1

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


Breast feed jaundice

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


Approach to jaundice d3 to 1 week

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


Approach to jaundice d3 to 1 week1

Approach to jaundice D3 to 1 week

  • Physical Examination

    • General condition

    • Body temperature

    • Hydration state

    • Clinical jaundice

      • Dermal zone

    • Feature of kernicterus


Approach to jaundice d3 to 1 week2

Approach to jaundice D3 to 1 week

  • Investigations

    • Serum bilirubin

    • Blood group of mother & baby

    • G6PD & TSH screening result

    • Urine for haemoglobin, RBC & WBC


Prolonged neonatal jaundice

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


Breast milk jaundice

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


Breast milk jaundice1

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


Obstructive jaundice

Obstructive jaundice

  • Common causes

    • Biliary atresia

    • Choledochal cyst

  • Clinical features

    • Prolonged jaundice

    • Pale stool & tea color urine

    • Elevated direct bilirubin, g-GTP

    • Normal or mildly elevated liver enzymes


Obstructive jaundice1

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


Neonatal hepatitis

Neonatal hepatitis

  • Congenital infection

    • TORCH

  • Acquired infection

    • Hepatitis viruses

    • Cytomegalovirus (CMV)

    • Epstein-Barr virus (EBV)

  • Neonatal hepatitis without organism identified

    • Autoimmune

    • Idiopathic


Neonatal hepatitis1

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


Haemolysis cause prolonged jaundice

Haemolysis cause prolonged jaundice

  • G6PD deficiency

  • RBC Membrane defect

    • Hereditary spherocytosis

  • Haemoglobinopathy

    • a-thalassaemia

    • Usually present with anaemia rather than jaundice


Hypothyroidism

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


Approach to prolonged neonatal jaundice

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


Approach to prolonged neonatal jaundice1

Approach to prolonged neonatal jaundice

  • Physical Examination

    • General well being

    • Rash or petechiae

    • Hepatosplenomegaly

    • Features suggest hypothyroidism

    • Examine stool & urine


Approach to prolonged neonatal jaundice2

Approach to prolonged neonatal jaundice

  • Investigation

    • Serum bilirubin – direct & indirect, liver enzymes, g-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


Jaundice beyond neonatal period

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: Wilson’s disease, a-1-antitrypsin deficiency

    • Obstructive

      • Biliary stones

      • Cystic fibrosis


Approach

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


Physical examination

Physical Examination

  • Jaundice – Sclera

  • Pallor

  • Stigmata of chronic liver disease

  • Abdominal Examination

    • Hepatomegaly – tender liver

    • Splenomegaly

    • Ascite


Investigation

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


Phototherapy

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


Side effects of phototherapy

Side effects of Phototherapy

  • Ý 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


Exchange transfusion

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


Exchange transfusion1

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


Push pull vs isovolumetric technique

Push & Pull VsIsovolumetric technique

Blood Volume

Time


Complication of exchange transfusion

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


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