APPROACH TO A CHILD

WITH JAUNDICE

S.BALASANKAR
2009 MBBS
S.BALASANKAR
2009 MBBS
Jaundice is the visible manifestation of increased
level of bilirubin in the body.
It is not a disease rather a symptom of diseases.
In adults sclera appears jaundiced when serum
bilirubin exceeds 2 mg/dl.
However it is difficult to see sclera in newborn
due to difficulty in opening eye. But in new born
it is very easy to see jaundice in skin.

Important problem in the 1
st
week of life.
Almost all neonates (60% Term and 80% Preterm)
will have bilirubin > 5 mg/dl in the 1
st
week of
life and become visibly jaundiced, vast majority
being benign
Some of the term babies (8 to 9%) have levels
exceeding 15 mg/dl in 1
st
7 days of life.
High bilirubin level is toxic to the developing
CNS.(KERNICTERUS; Bilirubin25mg/dl)

End product of hemoglobin metabolism that is
excreted in bile.
In neonates
-75% : from catabolism of circulating RBCs
-25% :*from ineffective erythropoiesis
(bone marrow)
*from turnover of heme proteins &
free heme( liver).
Hyperbilirubinemia
-Direct( Conjugated)
-Indirect( Un-conjugated)

Conjugated Hyperbilirubinemia is present,
* >20% of total bilirubin is conjugated
* >2mg/dl is conjugated

If neither criteria is met, hyperbilirubinemia is
classified as Un-conjugated.
a) Prolonged direct Jaundice
Neonatal hepatitis (common)
Extra-hepatic biliary atresia
Breast milk jaundice
Metabolic disorders
Intra-hepatic biliary atresia
Amino acid toxicity
Inspissated bile syndrome (uncommon)
b) Prolonged Indirect Jaundice

> Criggler Najjar Syndrome
> Breast milk jaundice
> Hypothyroidism
> Pyloric stenosis
> Ongoing hemolysis, malaria

PHYSIOLOGICAL JAUNDICE


PATHOLOGICAL JAUNDICE
In new born babies bilirubin
metabolism is immature which results in the
occurrence of hyperbilirubinemia in the first few
days of life. Also there is increased bilirubin load
on the hepatic cell due to physiological
polycythemia.

Immaturity could be at various steps of bilirubin
metabolism like:

Defective uptake from plasma into liver cell(
deficiency of LIGANDIN)
Defective conjugation( UDP-glucoronosyl
transferase: <1% of adult activity during the
first 10 days of life)
Decreased excretion
Increased entero-hepatic circulation

Increased Bilirubin production:


Larger circulating red blood cell volume

Shortened RBC lifespan (70-90 days vs 120 days
in adult)

Substantial production from other sources

First appears between hours of age
Maximum intensity seen on 4-5th day in term
and 7th day in preterm neonates
Does not exceed 15 mg/dl
Clinically undetectable after 14 days.
No treatment is required but baby should be
observed closely for signs of worsening
jaundice.

Presence of any of the following signs denotes that
the jaundice is pathological.

Clinical jaundice detected before 24 hours of
age
Rise in serum total bilirubin by more than 5
mg/dl/ day (>5mg/dl on first day , 10 mg/dl on
second day and 12- 13 mg/dl thereafter in term
babies)

Serum bilirubin more than 15 mg/dl
Clinical jaundice persisting beyond 14 days of
life
Clay/white colored stool and/or dark urine
staining the nappy yellow
Direct bilirubin >2 mg/dl at any time.

Treatment is required in the form of
phototherapy or exchange blood transfusion. One
should investigate to find the cause of pathological
jaundice.
It can be


Overproduction Hyperbilirubinemia


Under-secretion Hyperbilirubinemia
Overproduction Hyperbilirubinemia:
Blood group incompatibilities
Maternal-fetal or feto-fetal transfusions
Non Immune Hemolytic anemias
Structurally Abnormal Red cells
Extra-vascular Hemolysis
Rh negative mother & Rh positive infant

ABO incompatibilities

Strongly considered if there is jaundice in the
first 24 hours of life

1. G6PD Deficiency:
Deficiency-decreased NADPH- decreased
reduced Glutathione decreased protection of
RBCs from oxidants-hemolysis.

2. Excess of Vitamin K given IM
Spherocytosis

Pyknocytes ( irregular borders)
Under-secretion Hyperbilirubinemia:
Enzymatic Deficiency( Glucoronyl transferase)
Hormonal suppression (Breast milk jaundice)
Inhibition of conjugation
Hepatic cell injury due to Infections
Substrate deficiency (hypoglycemia)
Mechanical obstruction (biliary atresia)
Pregnandiol present in maternal breast milk
suppresses bilirubin conjugation.
Breast feeding may be stopped and restarted in a
period of 48hours.
Thyroxine increases the activity of Glucoronyl
transferase which promotes conjugation of
bilirubin.
Sulfonamides and Vitamin K results in
competitive conjugation inhibition of bilirubin.

GALACTOSEMIA:
Absent or deficient Galactose 1-phosphoate
uridyl transferase which is needed in
glucoronidaton of indirect bilirubin.
Maternal and Perinatal History:
Delivery at period of gestation, Postnatal age in
hours.
Maternal illness during pregnancy which also
includes diabetes; drug use.
Previous history of malaria.
Traumatic delivery, delayed cord clamping,
oxytocin use.
Birth asphyxia, delayed feeding, delay in
meconium passage.
Family history of jaundice, liver disease
Previous sibling with jaundice for blood group
incompatibility.
Kernicterus: Lethargy, poor feeding, and
hypotonia. Some advanced signs are seizures,
retrocollis, paralysis of upward gaze and shrill
cry.
Breast feeding.

Baby lethargic, poor feeding, temperature
instability, with apnea: Sepsis

Small for gestation: polycythemia

Cataract, rash: TORCH infections

Extra vascular bleed: Cephalhematoma

Pallor: hemolysis, blood loss

Petechiae: sepsis, TORCH infections.

Hepatosplenomegaly: Rh-isoimmunization,
sepsis, TORCH infections.

Dysmorphic features, congenital heart disease
(pulmonary stenosis), Intra-hepatic biliary atresia
:Alagille syndrome

HOW DO YOU LOOK FOR
ICTERUS?
1. Dermal staining :progresses from head to toe
Examined in good day light skin of forehead,
chest, abdomen, thigh, legs, palms, and soles.
Blanched with digital pressure and the
underlying color of the skin and subcutaneous
tissue should be noted.

2. Trans-cutaneous bilirubinometer .


Clinical Jaundice
> 12 mg/dl and infant < 24 hr
old
Fraction of Bilirubin
Indirect Direct
< 12 mg/dl and infant > 24 hr
old
Follow bilirubin level
Measure Bilirubin
Direct bilirubin
Evaluate for treatable causes
1.Infections-blood, urine culture, VDRL.
2.Metabolic disorders-urine reducing substances, serum amino
acids,T4,TSH.
If no abnormality ;screen for Identifiable causes
*-1 antitrypsin deficiency
*cystic fibrosis
*congenital viral infections
If no abnormality; evaluate for anatomical abnormalities
*Stool color
*USG
*Hepato-biliary scintigraphy
*Liver biopsy

Indirect
High(>65%)
Polycythemia
Hematocrit
Low/Normal
Reticulocyte count
Blood Type
Coombs test
Platelets
Evaluate smear
Normal Reticulocyte count
Look for evidence of,
*Infections-blood, urine culture
*Enclosed hemorrhage
*Congestive cardiac failure
*Hypoxia
*Hypothyroidism
*Drugs/toxins

None found

Familial Disorders
*Criggler Najjar
*Gilbert
Elevated Reticulocyte Count
Consider:
1.Iso-immunization
2.Erythrocyte membrane defect
3.Erythrocyte enzyme defect
Explain about benign nature of the disease

Encourage to breastfeed frequently & exclusively

Ask Mother to bring baby back if baby looks
deep yellow or palms & soles have yellow
staining.
Mainly 2 modalities of Treatment:


PHOTOTHERAPY

EXCHANGE TRANSFUSION
Mainstay of treatment
Under blue-green light(460-490nm), insoluble
bilirubin is converted into soluble isomers that
can be excreted in urine & feces.
To be effective, bilirubin must be present in skin;
hence nor role for prophylactic phototherapy
The American Academy of Pediatrics (AAP) has
laid down criteria for managing babies with
elevated serum bilirubin based on gestational
age and other risk factors( hemolysis ,
asphyxia, low albumin level, hypothermia)
CONFIGURATIONAL ISOMERIZATION

STRUCTURAL ISOMERIZATION

PHOTO OXIDATION
Z-isomer converted to E-isomer
Reaction is instantaneous but reversible.
After exposure of 8-12 hrs, this constitutes about
25% of the TSB which is non-toxic.
Excreted slowly from the body; hence not a
major mechanism for decrease in TSB.
Irreversible reaction
Bilirubin is converted into Lumirubin.
This product forms 2-6% of TSB which is rapidly
excreted from body thus is mainly responsible
for phototherapy induced decline in TSB.
Minor reaction
Optimum ambient room temperature( 25-
28celcius) to prevent hypothermia.
Remove all clothes of baby except diaper
Cover babys eyes with an eye patch(to prevent
retinal degeneration) ensuring that it does not
block the babys nostrils.
Place the baby under the lights in a cot if weight
is more than 2kg or in an incubator if baby is
small(<2kg)
Keep the distance between the baby & the light
30-45cm.
Ensure optimum breastfeeding as intermittent
feeding sessions.
Monitor temperature of the baby every 2-4hrs
Measure TSB every 12-24hrs.
Discontinue, once 2 TSB values 12hr apart fall
below current age-specific cut-offs.
Monitor for rebound bilirubin rise within 24hrs.
Loose stools
Erythematous macular rash
Purpuric rash associated with transient
porphyrinemia
Over-heating
Dehydration
Bronze baby syndrome


Intense grey-brown discoloration of
the skin, serum, and urine, especially
in premature infants; when
phototherapy was used to reduce
hyperbilirubinemia. Pre-existing
hepatic disease is suspected as a
cause of the jaundice and may have
prevented the biliary excretion of the
photo oxidation products of
bilirubin; their retention resulted in
the bronze discoloration.
Double Volume Exchange Transfusion (DVET) :
160-180ml/kg; is to be performed if TSB levels
reach age-specific cut-offs or if the infant shows
signs of bilirubin encephalopathy, irrespective of
TSB levels.
If baby shows signs of cardiac decompensation at
birth, partial exchange transfusion with 50ml/kg
of packed red cells should be done to quickly
restore oxygen carrying capacity of blood.
Umbilical venous route.
Babies with serum bilirubin>20 mg/dl & those
who required ET should be kept under follow-up
in high-risk clinic for neuro-developmental
outcome.
Hearing assessment should be done at 3months
of age.
Ante-natal screening to detect Rh iso-
immunization & prompt administration of Anti D
after first obstetric event.
Ensure adequate breast feeding.
Educate parent about danger signs to ensure
immediate checkup.
Follow-up high risk babies( large cephalo-
hematoma, family history of jaundice) for 2-3
days of discharge.
HISTORY:
Age at onset of symptoms. E.g.: Wilsons disease
commonly manifests in pre-adolescents &
adolescents.
Past/present use of any drugs
H/o of blood transfusion/ dialysis
Exposure to viral hepatitis
Any h/o of chronic illness; hemoglobinopathies.
Family h/o of inheritable disorders; Wilsons.
Auto-immune hemolytic anemias
Drug induced hemolytic anemias
Erythrocyte membrane defects
Erythrocyte enzyme defects
Hemoglobinopathies
Congestive cardiac failure
Sepsis
Gilbert syndrome.

1. Obstructive
Gall stones
Primary sclerosing cholangitis
Choledochal cyst
Tumors
2. Infections
Hepatitis A, B, C, D, E
EBV,CMV
Varicella zoster
HIV
Leptospirosis
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