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ObjectivesObjectives
• By end of this period, you will be able to
explain the clinical and pathological
approach to a patient suffering from
jaundice.
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DefinitionDefinition
• Jaundice is defined as yellowish
discolouration of skin, conjunctivae,
sclerae and mucous membrane due to
increased level of bilirubin in blood.
• Syn:
– Icterus,
– Hyperbilirubinaemia (Hyperbil)
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IntroductionIntroduction
• French word, ‘jaune’ means yellow.
• Serum Bilirubin levels < 2 mg/dl (30 μmol/
liter) in adults does not cause jaundice.
• Serum Bilirubin level < 5 mg/dl (75 μmol/L)
in newborn does not cause jaundice.
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Differential DiagnosisDifferential Diagnosis
• Carotenemia:
– Palms and soles are yellow,
– Skin may also be yellow, but
– Sclera is never yellow,
– Not found in newborn.
• Chloroquine (?).
• Turmeric application.
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Some clues to diagnosis
• Fever, pain abdomen, vomiting, acute
onset, tender hepatomegaly with jaundice:
– Acute Viral Hepatitis.
• Chronic jaundice, low grade fever or
afebrile, features of liver failure with or
without features of portal hypertension:
– Chronic Liver Disease (CLD)
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Clues:Clues:
• Mild jaundice (lemon yellow) with severe
pallor with splenomegaly, sometimes
hepatomegaly also, typical facial features:
– Congenital Haemolytic Anaemia.
• Preceding h/o diarrhoea, pallor, jaundice,
oliguria or even anuria:
– HUS.
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CluesClues
• Biphasic course with fever, mild jaundice,
haemorrhages, meningitis and h/o rats in
kitchen or access to food stored in house:
– Leptospirosis.
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How to look for jaundice?
• To look for jaundice, press the infant’s skin over
the forehead with your fingers to blanch, remove
your fingers and look for yellow discolouration
under natural light.
• If there is yellow discoloration, the infant has
jaundice.
• To assess severity, repeat the process over
chest, abdomen, thighs, legs and palms and
soles.
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According to age of
appearance of jaundice
• Early Jaundice (< 10 days):
– First 24 hours,
– After 24 hours.
• Prolonged Jaundice (> 10 days):
– Prolonged unconjugated hyperbilirubinaemia
– Prolonged conjugated hyperbilirubinaemia
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Approach to the newborn with jaundiceApproach to the newborn with jaundice
Jaundice in
in a term
Neonate
< 24 hours old > 24 hours old
• Rule out hemolysis.
• No discharge.
• Appropriate Therapy
• Close monitoring.
Diagnosis
Treatment
AAP guideline Disposition
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And if the infant has
jaundice
And if the infant has
jaundice
SIGNS CLASSIFY AS
• Palms and soles yellow or
• Age < 24 hours or
• Age 14 days or more.
SEVERESEVERE
JAUNDICEJAUNDICE
•Palms and soles not yellow JAUNDICE
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Need of investigation
• Check if,
– Onset of jaundice is within 24 hrs of life, or
– Baby is more than 14 days of age, or
– Jaundice is below abdomen on clinical
assessment.
• If any of the above is ‘yes’ then do the
investigations.
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Unconjugated HyperbilirubinaemiaUnconjugated Hyperbilirubinaemia
Haemolysis
Physiological Pathological
• Well appearing,
• DCT negative,
• Peaks at day 3,
• Disappears by day 5,
• Peak < 12 mg/dl.
• ABO/Rh incompatibility,
• RBC defects,
• Enzyme defects
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Clinical Features and Recommended Tests for Genetic
Disorders with UnconjugatedUnconjugated Hyperbilirubinaemia.
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Crigler Najjar Type ICrigler Najjar Type I
• Features:
– Severe lifelong jaundice with high risk of
kernicterus,
– No haemolysis or significant hepatocellular
dysfunction.
• Tests:
– UGT1A1 enzyme assay in liver.
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Indicators of HaemolysisIndicators of Haemolysis
• Rapidly rising bilirubin,
• Falling hemoglobin.
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History and Physical Examination
• Note adequacy of feeding, passing stools, and
voiding (risk factors for increased enterohepatic
circulation of bilirubin).
• Examine for the following:
– Well being (no sepsis),
– Growth parameters,
– Bruising and cephalhematoma (increased bilirubin
production),
– Pallor, oedema, and hepatosplenomegaly (indicators
of haemolysis and CHF).
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Growth ParametersGrowth Parameters
• SGA infants are likely to be plethoric
resulting in higher bilirubin and requiring
earlier phototherapy.
• This may be symptomatic of intrauterine
infection and hence likely conjugated
jaundice.
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Types of Prolonged JaundiceTypes of Prolonged Jaundice
• Unconjugated Hyperbilirubinaemia.
• Conjugated Hyperbilirubinaemia.
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Causes of ProlongedCauses of Prolonged
Unconjugated HyperbilirubinaemiaUnconjugated Hyperbilirubinaemia
• Breast Milk Jaundice.
• Hypothyroidism.
• Intestinal Stasis.
• Hemolytic causes.
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Breast Milk JaundiceBreast Milk Jaundice
• Most common cause of prolonged
unconjugated Hyperbilirubinaemia.
• Total Serum Bilirubin usually < 200 mcg/L
• Conjugated bilirubin < 20% (Normally > 25%)
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Breast Milk JaundiceBreast Milk Jaundice (continued)
Beta glucuronidase present in Breast Milk
Bilirubin diglucuronide
UC Bilirubin Glucuronic Acid
Absorbed from GIT
Hyperbilirubinaemia
(Unconjugated)
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Breast Milk JaundiceBreast Milk Jaundice (continued)
• Baby well,
• No treatment required,
• Settles by 6 weeks
(occasionally up to 4 months.
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Lucy Driscoll SyndromeLucy Driscoll Syndrome
• Inhibitors of bilirubin glucuronide is
present in the mother’s milk.
• Sometimes severe enough to require
exchange transfusion.
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HypothyroidismHypothyroidism
• A heavy weight, sluggish baby with
feeding difficulty and prolonged jaundice
s/o congenital hypothyroidism.
• Investigation:
– Thyroid Function Test.
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Intestinal StasisIntestinal Stasis
• 2nd
most common cause of prolonged
unconjugated hyperbilirubinaemia.
• Due to increased enterohepatic circulation
of bilirubin.
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MechanismMechanism
• Normally 75% of conjugated bilirubin is
excreted in feces and 25% of is broken
down to unconjugated form in duodenum
and is absorbed into circulation.
• When there is stasis, the break-down to
unconjugated form increases significantly
to cause hyperbilirubinaemia.
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Causes of Intestinal StasisCauses of Intestinal Stasis
• Hirschsprung disease.
• Intestinal atresia.
• Pyloric Stenosis.
• Meconium ileus of Cystic Fibrosis.
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InvestigationsInvestigations
• Serum Bilirubin:
– Total
– Direct (conjugated)
– Indirect (Unconjugated)
• Hepatobiliary ultra-sound study or
• Technetium-99 HIDA cholescintigraphy.
• Percutaneous liver-biopsy.
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Stool ColourStool Colour
• Stool partially pale or intermittently pale +
IUGR = Neonatal Hepatitis (intrahepatic
cholestasis).
• Stool completely and persistently pale and
lasting longer that 2 weeks + hard liver or
unconjugated hyperbilirubinaemia =
Extrahepatic cholestasis (congenital biliary
atresia)
