This document provides information on jaundice in children. It discusses the causes of jaundice including liver diseases like hepatitis A and B, gallstones, and metabolic conditions. It outlines the approach to evaluating jaundice including assessing risk factors, laboratory tests to differentiate medical and surgical causes, and classifications. Complications of severe jaundice like kernicterus are described. Sickle cell disease is presented as another potential cause of jaundice in children.

1. Prof. Sushmita N. Bhatnagar
MBBS, M.S., M.Ch,M.Phil(Hospital Management)
HEAD, PEDIATRIC SURGERY
B.J WADIA CHILDREN’S HOSPITAL, MUMBAI
CONSULTANT PEDIATRIC SURGEON
BOMBAY HOSPITAL
JOINT SECRETARY
ASSOCIATION OF MEDICAL CONSULTANTS
JAUNDICE IN CHILDREN

2. JAUNDICE
NEWBORNS
INFANT
CHILD

3. JAUNDICE IN ADULTS

4. JAUNDICE – IS IT A DISEASE?
Yellowish staining of the skin and sclerae
High levels of bilirubin in blood

5. WHICH DISEASES CAUSE JAUNDICE?
• Feeding related jaundice
• Congenital liver infections
• Genetic/autoimmune diseases
• Blood group incompatibilities
• Congenital hepatobiliary anomalies
• Metabolic
• Iatrogenic
• Idiopathic
NEWBORNS
• Idiopathic
• Metabolic
• Infections
• Biliary atresia
• Congenital hepatobiliary anomalies
INFANTS
• Infections
• Metabolic/Genetic conditions
• Stones in bile duct
• Pancreatitis – Acute or chronic
• Tumors
CHILD

6. DIFFERENTIATION
Medical
Jaundice
Surgical
Jaundice

7. SURGICAL JAUNDICE
• Inspissated bile syndrome/Bile sludge
• Choledochal cyst
• Biliary atresia
• Rare causes
NEWBORNS
• Biliary atresia
• Choledochal cyst
• Bile duct calculi
• Spontaneous rupture of bile duct
• Injury
INFANTS
• Choledochal cyst
• Biliary calculi
• Pancreatitis
• Injury
• Tumors
CHILD

8. WHICH DISEASES CAUSE JAUNDICE?
• Increased production of bilirubin
• Acute liver inflammation
• Infiltrative liver diseases
• Bile duct inflammation
• Blockage of bile ducts
• Drugs
• Genetic disorders
• Developmental abnormalities of bile ducts
• Jaundice of pregnancy

9. • Skin and sclerae -
yellow
• Stool - light colour,
clay coloured
• Dark urine
• Pain in abdomen
• Itching
• Trouble with sleeping
• Fatigue
• Swelling
• Ascites
• Mental confusion
• Coma
• Bleeding
WHAT PROBLEMS DO JAUNDICE CAUSE?

10. JAUNDICE IN NEWBORN BABIES

11. NEONATAL JAUNDICE
Is jaundice in newborns normal?

12. NEONATAL JAUNDICE
Neonatal jaundice is quite common
• >50% of normal newborns and
• 80% of preterm infants have some degree of
jaundice
Two types of neonatal jaundice:
• Normal / physiological
• Abnormal / non-physiological

13. WHY?
• PRODUCTION :
In term newborns, bilirubin production is 2-3 times
higher than in adults
• CLEARANCE
decreased in newborns, mainly due to deficiency of
enzyme UGT
• UGT activity in term infants at 7 days is ~1% of adult
liver and doesn’t reach adult levels until 14 weeks
• CIRCULATION
Increase enterohepatic circulation of bilirubin,
further increases bilirubin load

14. PRETERM INFANTS
• Even more RBC turnover and destruction
• Physiologically impaired conjugation and
elimination of bilirubin
• An even less mature liver
• Reduced bowel motility due to inadequate
oral intake
• Delayed elimination of meconium
• Increased enterohepatic circulation

15. PHYSIOLOGIC JAUNDICE
• Jaundice appears around 72 hrs of life
• Bilirubin peaks <14 mg/dl
• Direct bilirubin <10% of total bilirubin
• Rate of rise <5mg/dL/day
• Jaundice resolves in 1-2 weeks in term
infants, 2 weeks in preterm infants

16. NON-PHYSIOLOGIC JAUNDICE
• Early jaundice
• Starts on first day of life
• Jaundice of long duration
• >14 days in term or >21 days in preterm
infants
• Deep jaundice
• Palms and soles deep yellow
• Objectively, high bilirubin lab levels
• Jaundice with fever

17. MUST CHECK

18. NORMAL ABNORMAL

19. WORK UP: LABORATORY STUDIES
• Where possible, confirm clinical jaundice with
bilirubin levels
• Possible additional investigations, depending
on likely diagnoses and lab availability:
• Hemoglobin/hematocrit (PCV) to look for hemolysis
• Blood smear
• Reticulocyte count
• WBC to look for signs of infection (WBC <5, WBC>20, or I:T
ratio >20%)
• Blood type of baby and mother, and Coombs test
• Syphilis serology (e.g. VDRL)
• G6PD screen, thyroid function tests, liver ultrasound

20. JAUNDICE AND ITS EFFECT
Deposits in
skin and
mucous
membranes
Unconjugate
d bilirubin
deposits in
the brain
Permanent
neuronal
damage
JAUNDICE
ACUTE BILIRUBIN
ENCEPHALOPATHY
KERNICTERUS
M
a
y
c
a
u
s
e
I
t
c
h
i

21. JAUNDICE IN CHILDREN

22. • Hepatitis A is transmitted by contaminated
food or water, or contact with a person
who is currently ill with the disease. The
hepatitis A virus is shed in the stools of an
infected person during the incubation
period of 15 to 45 days before symptoms
occur and during the first week of illness.
Blood and other bodily secretions may
also be infectious.
• The virus does not remain in the body
after the infection has resolved, and there
is no carrier state (a person or animal that
spreads the disease to others but does not
become ill).
• The symptoms associated with hepatitis A
are fever, poor appetite, nausea &
vomiting, abd pain ,jaundice & yellow
urine. This is because the liver is not able
to filter bilirubin from the blood.
• Risk factors include having a family
member who recently had hepatitis A,
HEPATATIS A

23. CONTAMINATING!

25. DRINK ONLY BOILED WATER

26. HEPATATIS B
Hepatitis B is transmitted via blood and other body fluids. Infection
can occur through:
• Contact with blood in healthcare settings -- this puts
physicians, nurses, dentists, and other healthcare
personnel at risk
• Blood transfusions
• Sharing needles during drug use
• Receiving a tattoo or acupuncture with contaminated
instruments
• Birth -- an infected mother can transmit the virus to the
baby during delivery or shortly thereafter

27. UNSAFE INJECTIONS

28. OTHER HEPATITIS
• Hepatitis C
• Hepatitis E
PREVENTION NECESSARY
VACCINATIONS

29. • Stones can present with jaundice,
especially the stones in the bile ducts
Obstructive jaundice
SUSPECT
INVESTIGATE
TREAT
CALCULUS DISEASE

30. CARE FOR ALL BABIES
Risk factors
identified?
Jaundi
ce
Examine for jaundice at every
opportunity especially in the
first 72 hours
An additional inspection
within 48 hours
YES NO

31. < 24 HOURS OF AGE
Visible
jaundice
MEDICAL EMERGENCY
Measure and record serum bilirubn
within 2 hours
Neonatology/Pediatric/Medical review
within 6 hours
Commence
phototherapy
Organise
transfer to
neonatal
referral center
YES

32. 4 DAYS – 10 DAYS
PHYSIOLOGICAL JAUNDICE

33. LATE ONSET OR PROLONGED JAUNDICE
Jaundice > 10 days or
prolonged
Check stool color
Further investigations required
Seek expert advice

34. THANK - YOU

35. PROBLEMS WITH DELAY
• If significant brain damage occurs before treatment, a child can develop serious and
permanent problems, such as:
• cerebral palsy – a condition that that affect a child's movement and co-ordination
• hearing loss, which can range from mild to severe
• learning difficulties
• involuntary twitching of different parts of their body
• problems maintaining normal eye movements – people affected by kernicterus have a
tendency to gaze upwards or from side to side rather than straight ahead
• the normal development of the teeth can be disrupted resulting in teeth that are
misshapen, discoloured and vulnerable to tooth decay

36. TWO FORMS OF HYPERBILIRUBINEMIA
• Unconjugated / indirect hyperbilirubinemia:
• Pre-hepatic cause, or impairment in conjugation
VS.
• Conjugated / direct hyperbilirubinemia:
• Injury at the level of the hepatocytes, or post-
hepatic obstruction
• Consider diagnosis of conjugated
hyperbilirubinemia if direct bilirubin is >3mg/dL, or
is >10% of total bilirubin

37. DIFFERENTIAL DIAGNOSIS:
UNCONJUGATED
HYPERBILIRUBINEMIA
• Breastfeeding jaundice
• Occurs at 1-3 days of age; due to dehydration and lack of stooling (treat by increasing feeding frequency)
• Breast milk jaundice
• Occurs at 4-10 days of age; substance in breast milk inhibits glucuronyl transferase (treat by temporary
switch to formula)
• Hemolysis
• ABO/Rh incompatibility
• RBC membrane defects
• Alpha thalassemia
• G6PD deficiency
• Cephalohematoma
• Polycythemia
• Infection
• Hypothyroidism
• Gilbert’s
• impaired conjugation, associated with stress, no overt hemolysis
• Crigler-Najjar’s
• absent (type 1) or diminished (type 2) UDP-glucoronyl transferase

38. DIFFERENTIAL DIAGNOSIS:
CONJUGATED
HYPERBILIRUBINEMIA
• Biliary atresia
• ~60% of cases; an obliterative process of bile ducts; diagnosed by U/S or
biopsy
• Infection
• Hepatitis B, TORCH
• Metabolic
• Galactosemia
• Alpha-1-antitrypsin deficiency: most common genetic cause
• Dubin Johnson or Rotor’s syndrome: defective liver secretion of bilirubin
• Iatrogenic
• Drug-mediated
• TPN-related: occurs in ~2/3 of infants given TPN over 2 weeks of duration;
unknown mechanism, possibly mediated by bacterial endotoxins, oxidative
stress, glutathione depletion
• Idiopathic
• neonatal non-infectious hepatitis (diagnosis of exclusion)

39. THE CONCERN: KERNICTERUS
• Bilirubin exceeds albumin-
binding capacity, crosses BBB,
and deposits on basal ganglia
and brainstem nuclei
• Risks increase with levels >20
mg/dl
• Or lower levels in setting of sepsis,
meningitis, hemolysis,
hypothermia, hypoglycemia, or
prematurity

40. SIGNS OF KERNICTERUS
• Acute sequelae:
• Poor suck, lethargy, hypotonia, seizure
• Then hypertonia (opisthotonus, retrocollis), fever, high-pitched cry
• Chronic sequelae:
• Choreoathetoid CP, gaze paresis, sensorineural hearing loss, mental
retardation

41. CAUSE ANALYSIS OF KERNICTERUS
• Early discharge <48hrs without follow-up within 48hrs
• Failure to check bilirubin level when jaundice within 24hrs of life
• Failure to recognize risk factors
• Underestimating severity by visual assessment
• Delay in initiating treatment
• Failure to respond to parental concerns
AAP Subcommittee on Neonatal Hyperbilirubinemia. Pediatrics 2001; 108: 763-765.

42. • Maternal:
• Race or ethnic group (Asian,
Mediterranean)
• ABO, Rh incompatibility
• Previous jaundiced infant
• Advanced maternal age
• Diabetes
• Infant:
• Gestation <38 weeks
• Bruising, cephalohematoma
• Infection
• G6PD deficiency
• Polycythemia
• Male gender
• Nutritional:
• Breastfeeding
• Weight loss
• Decreased feeding
frequency
• Decreased stooling
• Decreased urine output
WORK UP: ASSESS RISK FACTORS

43. JAUNDICE – CLASSIFICATION
WWW.DRSARMA.IN 43
• Normal Serum Bilirubin (SB) is 0.3 to 1.0 mg%
• Jaundice is increased levels of SB > 1.0 mg%
• Over production of Bilirubin (Hemolytic)
• From hemolysis of RBC
• Lysis of RBC precursors – Ineffective erythropoesis
• Impaired hepatic function (Hepatitic)
• Hepatocellular dysfunction in handling bilirubin
• Uptake, Metabolism and Excretion of bilirubin
• Obstruction to bile flow (Obstructive)
• Intrahepatic cholestasis
• Extrahepatic Obstruction (Surgical Jaundice)

44. FIRST STEP
WWW.DRSARMA.IN 44
Estimate Serum Bilirubin
Is it less than 1 mg % - Normal
Is it more than 1 mg % - Elevated

45. SECOND STEP : IF SB > 1.0 MG
WWW.DRSARMA.IN 45
Is it unconjugated bilirubin ?
Haemolytic Jaundice
Is it Conjugated Bilirubin ? (> 20%)
Hepatocellular jaundice
Obstructive jaundice

46. ↑ IN UNCONJUGATED BILIRUBIN
WWW.DRSARMA.IN 46
Hemolytic Jaundice - Uncommon
1. Hemolytic Disorders + Anemia
Inherited – Sphero, SS, G6PD, PK
Acquired – MAHA, PNH
2. Ineffective Erythropoesis –B12, Fe, F
3. Drugs – Rifampicin, Probenecid
4. Inherited –Crigler Najjar, Gilberts

47. THIRD STEP : IF CSB IS INCREASED
WWW.DRSARMA.IN 47
Do - AST and ALT (SGOT and SGPT)
Elevated AST and ALT
Hepatocellular jaundice
AKP, 5N, GGT will be normal
Do - Alkaline Phosphatase and GGT
AKP, GGT ↑↑ in Obstructive Jaundice
AST and ALT will be normal

48. FOURTH STEP : HEPATOCELLULAR
WWW.DRSARMA.IN 48
Hepatocellular – Features and D.D
Conjugated SB is increased
AST and ALT are increased
AKP, 5NS, GGT are normal
Hepititis – A,B,C,D,E, CMV,EBV
Toxic Hepatitis – Drugs, Alcohol
Malignancy – Primary Ca
Cirrhosis – ALD, NAFLD

49. LABORATORY TESTS
• Bilirubin level in serum (total and direct)
• Aminotransferase
• Alkaline phosphatase
• U/A for bilirubin and urobilogen
 Complete blood count
 Prothrombin time
 Other laboratory tests
pertinent to history
 Coombs test
 Electrophoresis of
hemoglobin
 Viral hepatitis panel

50. LITERATURE
• http://en.wikipedia.org/wiki/Jaundice#Neonatal_jaundice
• http://www.medicinenet.com/jaundice/article.htm
• http://www.nlm.nih.gov/medlineplus/ency/article/003243.htm

52. ANATOMY

54. SICKLE CELL DISEASE
• .
• Sickle cell anemia is an inherited blood
disease in which the red blood cells produce
abnormal pigment (hemoglobin). The
abnormal hemoglobin causes deformity of
the red blood cells into crescent or sickle-
shapes, as seen in this photomicrograph

55. • JAUNDICE
• BODY PAIN/JOINT PAIN
• PAINFUL SWELLINGS
• TIRED/NOT WORKING
• POOR GROWTH
• RECURRENT RESP INFECTION
• STROKE
BLOOD TEST AT GAH
WHEN DO YOU SUSPECT SCD AND WHAT
SHOULD YOU DO

56. WHAT TO DO FOR PATIENT WITH SCD
• PROTECT AGAINST
DIARRHOEA
• GIVE PENICILLIN TILL
AGE 5
• HYDROXYUREA
• GENETIC ADVISE

57. DISEASES FROM CONTAMINATED WATER
HEPATIC JAUNDICE
Dysentery, Amebic dysentery, Cholera, Giardia Entamoeba
histolytica
Hepatitis A,
Leptospirosis
Typhoid fever,
Cercarial dermatitis, Cryptosporiosis,
Cyclosporiasis, Cysticercosis, Dracunculiasis, Hookworm,,
Melioidosis, Neurocysticercosis, Polio, Relapsing fever,
Toxocariasis, Trachoma, Traveler's diarrhea,

58. JAUNDICE IN NEWBORN

59. WORK UP: LABORATORY STUDIES
• Where possible, confirm clinical jaundice with
bilirubin levels
• Possible additional investigations, depending on
likely diagnoses and lab availability:
• Hemoglobin/hematocrit (PCV) to look for hemolysis
• Blood smear
• Reticulocyte count
• WBC to look for signs of infection (WBC <5, WBC>20, or I:T
ratio >20%)
• Blood type of baby and mother, and Coombs test
• Syphilis serology (e.g. VDRL)
• G6PD screen, thyroid function tests, liver ultrasound

60. CLINICAL SYMPTOMS:
• Jaundice/Icterus:
• Newborn icterus notable once total bilirubin > 5-6 mg/dL (versus older
children/adults once > 2 mg/dL)
• Progresses cranially to caudally
• CAUTION: Visual assessment is subjective, inaccurate, and dependent on
observer experience!
• Keren et al Visual assessment of jaundice in term and late-preterm infants
(2009)
• Nurses at HUP used 5 point-scale to rate cephalocaudal extent of jaundice
• Showed weak correlation between predicted and actual levels

61. PRE-TERM VS. FULL-TERM
HYPERBILIRUBINEMIA:
• Pre-term infants at higher risk due to further reduced activity of liver
conjugating enzymes
• Pre-term infants can develop encephalopathy or kernicterus at lower total
bilirubin levels

62. DIRECT HYPERBILIRUBINEMIA:
• Considered elevated when:
• Level > 2.0 mg/dL (severe > 5.0 mg/dL)
• Level > 15% of total serum bilirubin
• Risk factors:
• Low gestational age
• Early and/or prolonged exposure to TPN
• Lack of enteral feeding
• Sepsis
• Clinical hallmarks: icterus, acholic stools, dark urine

63. DIFFERENTIAL DX OF DIRECT
HYPERBILIRUBINEMIA:
• More common causes:
• TPN-associated
• Hepatitis: Idiopathic, Infectious, Toxic
• Infection: Sepsis, TORCH, UTI
• Biliary atresia
• Inspissated bile plug
• Choledochal cyst
• Alpha-1-antitrypsin deficiency
• Galactosemia

64. DIFFERENTIAL DX OF DIRECT
HYPERBILIRUBINEMIA:
• Less common causes:
• Cholelithiasis
• Cystic fibrosis
• Hypothyroidism
• Rotor’s Syndrome
• Dubin-Johnson Syndrome
• Storage diseases (Niemann-Pick, Guacher’s)
• Metabolic disorders (tyrosinemia, fructosemia)
• Trisomy 21 or 18
• Drug-induced
• Shock
• Alagille Syndrome
• Zellweger Syndrome