Icterus

Neonatarum/
Neonatal Jaundice
Anatomy & Physiology
 Definition
• Yellow discoloration of the skin and the
mucosa due to accumulation of excess of
bilirubin in the tissue and plasma in neonates.
(more than 7mg/dl).
• 30-50 % of term newborn
• And more of preterm newborns 80%.
 RISK FACTORS
• J - Jaundice within first 24 hrs of life
• A - A sibling who was jaundiced as neonate
• U - Unrecognized hemolysis
• N -Non-optimal sucking/nursing
• D - Deficiency of G6PD
• I - infection
• C -Cephalhematoma /bruising
• E - East Asian/North Indian
Pathophysiology
 Mechanism/ Causes
• Relatively low activity of the enzyme glucuronosyl
transferase which normally converts unconjugated bilirubin
to conjugated bilirubin that can be excreted into the
gastrointestinal tract.
• Before birth, this enzyme is actively down-regulated, since
bilirubin needs to remain unconjugated in order to cross
the placenta to avoid being accumulated in the fetus.
• After birth, it takes some time for this enzyme to gain
function.
• Shorter life span of fetal red blood cells, being
approximately 80 to 90 days in a full term infant, compared
to 100 to 120 days in adults.
• Relatively low conversion of bilirubin to urobilinogen by the
intestinal flora, resulting in relatively high absorption of
bilirubin back into the circulation.
 Physiological Causes

1. Increased red cell volume & increased red cell

destruction.
2.Decreased conjugation of bilirubin d/t decreased
UDPG-T activity.
3.Increased enterohepatic circulation d/t decreased
gut motility.
4.Decreased hepatic excretion of bilirubin.
5.Decreased liver cell uptake of bilirubin d/t
decreased ligandin.
 Physiological jaundice
• Most infants develop visible jaundice due to
elevation of unconjugated bilirubin concentration
during their first week. This common condition is
called physiological jaundice. This pattern of
hyperbilirubinemia has been classified into two
functionally distinct periods.
Phase one
• Term infants - jaundice lasts for about 10 days
with a rapid rise of serum bilirubin up to 12
mg/dL.
• Preterm infants - jaundice lasts for about two
weeks, with a rapid rise of serum bilirubin up to
15 mg/dL
Phase two
• bilirubin levels decline to about 2 mg/dL for
two weeks, eventually mimicking adult
values.
• Preterm infants - phase two can last more
than one month.
• Exclusively breastfed infants – phase two can
last more than one month.
 Physiological jaundice

Characteristics
• Appears after 24 hours
• Maximum intensity by 4th-5th day in term & 7th
• day in preterm
• Serum level less than 15 mg / dl
• Clinically not detectable after 14 days
• Disappears without any treatment
• Note: Baby should, however, be watched for
worsening jaundice
 Why does physiological
jaundice develop?

• Increased bilirubin load

• Defective uptake from plasma
• Defective conjugation
• Decreased excretion
• Increased entero-hepatic circulation
 Pathological Causes
1. Excessive Red cell hemolysis.
2. Defective conjugation of bilirubin.
3. Breast milk jaundice.
4. Metabolic and endocrine disorders.
5. Increased enterohepatic circulation.
6. Substances and disorders that affect binding.
7. Miscellaneous.
 Pathological jaundice

• Appears within 24 hours of age

• Increase of bilirubin > 5 mg / dl / day
• Serum bilirubin > 15 mg / dl
• Jaundice persisting after 14 days
• Stool clay / white colored and urine
• staining clothes yellow
• Direct bilirubin> 2 mg / dl
 Causes
Non Conjugated: Hemolytic
• Intrinsic causes of hemolysis
Membrane conditions
• Spherocytosis
• Hereditary elliptocytosis
Systemic conditions
• Sepsis
• Arteriovenous malformation
Enzyme conditions
• Glucose-6-phosphate dehydrogenase deficiency (also called G6PD
deficiency)
• Pyruvate kinase deficiency
Globin synthesis defect
• sickle cell disease
• Alpha-thalassemia, e.g. HbH disease
 Extrinsic causes of hemolysis

• Hemolytic disease of the newborn (ABO)

• Rh disease
• Other blood type mismatches
 Non-hemolytic causes

• Breast milk jaundice

• Cephalohematoma ( hemorrhage of blood
between the skull)
• Polycythemia( increase in no of RBCs)
• Urinary tract infection
• Sepsis
• Hypothyroidism
• Gilbert's syndrome
• Crigler-Najjar syndrome
• High GI obstruction
 Conjugated : Hepatic causes
Infections
• Sepsis
• Hepatitis A
• Hepatitis B
• TORCH infections vertically transmitted infections
Metabolic
• Galactosemia
• Cystic fibrosis
• Dubin-Johnson Syndrome
• Rotor syndrome
Drugs
Total parenteral nutrition
Idiopathic
 Post-hepatic

• Biliary atresia or bile duct obstruction

• Alagille syndrome ( genetic disorder that
affects the liver, heart, kidney, and other
systems of the body)
• Choledochal cyst
 Common causes in India

• Physiological
• Blood group incompatibility
• G6PD deficiency
• Bruising and cephalhaematoma
• Intrauterine and postnatal infections
• Breast milk jaundice
 Breast Milk jaundice

• breast milk jaundice is a biochemical

occurrence
• Breast milk jaundice occurs later in the
newborn period, with the bilirubin level
usually peaking in the sixth to 14th days of
life. This late-onset jaundice may develop in
up to one third of healthy breastfed infants.
 Breast Milk jaundice

• At birth, the gut is sterile, and normal gut flora takes

time to establish. The bacteria in the adult gut convert
conjugated bilirubin to stercobilinogen which is then
oxidized to stercobilin and excreted in the stool.
• In the absence of sufficient bacteria, the bilirubin is
deconjugated by brush border β-glucuronidase and
reabsorbed. This process of re-absorption is called
enterohepatic circulation. It has been suggested that
bilirubin uptake in the gut (enterohepatic circulation) is
increased in breast fed babies, possibly as the result of
increased levels of epidermal growth factor (EGF) in
breast milk.Breast milk also contains glucoronidase
which will increase deconjugation and enterohepatic
recirculation of bilirubin.
 Approach to jaundiced baby

• Ascertain birth weight, gestation and

postnatal age
• Assess clinical condition (well or ill)
• Decide whether jaundice is physiological or
pathological
• Look for evidence of kernicterus* in deeply
jaundiced NB
• *Lethargy and poor feeding, convulsions
 Workup

• Maternal & perinatal history

• Physical examination
• Laboratory tests (must in all)*
– Total & direct bilirubin*
– Blood group and Rh for mother and baby*
– Hematocrit, retic count and peripheral smear*
– Sepsis screen
– Liver and thyroid function
– TORCH titers, liver scan when conjugated
• hyperbilirubinemia
 Complications

• Kernicterus
• Most Important, Often Fatal.
 Management

• Rationale: to reduce level of serum bilirubin

and prevent bilirubin toxicity
• Prevention of hyperbilirubinemia: early feeds,
adequate hydration
• Reduction of bilirubin levels: phototherapy,
exchange transfusion, drugs
 Technique
• 6-8 daylight tubes are mounted on a stand and all electrical
outlets are well grounded.
• Baby is placed naked 45 cm away from the tube lights in a crib or
incubator.
• Eyes are covered with eye-patches to prevent damage to the
retina by the bright lights; gonads should also be covered.
• Phototherapy is switched on. Baby is turned every two hours or
after each feed.
• Temperature is monitored every two to four hours.
• Weight is taken at least once a day.
• More frequent breastfeeding.
• Urine frequency is monitored daily.
• Serum bilirubin is monitored at least every 12 hours.
• Phototherapy is discontinued if two serum bilirubin values are <
10 mg/dl.
 Side effects of phototherapy

•Increased insensible water loss: Frequent Breast

feeding.
•Loose green stools: weigh often and compensate with
breast milk.
•Skin rashes: Harmless, no need to discontinue
phototherapy.
•Bronze baby syndrome: occurs if baby has conjugated
hyperbilirubinemia. If so, discontinue phototherapy.
•Hypo or hyperthermia: monitor temperature
frequently
 Exchange transfusion

Indications:
• Rise of bilirubin >1mg/dl/hour
• To improve anemia & CCF
• Sr. Bilirubin > 20mg/dl in first 24 hrs
• Cord hemoglobin is < 12mg/dl & bilirubin is >
5mg/dl

It is still the most effective and reliable method to

reduce serum bilirubin
 Technique
• The procedure involves the incremental removal
of the patient's blood and simultaneous
replacement with fresh donor blood, saline or
plasma.
• The patient’s blood is slowly drawn out
• And an equal amount of fresh, prewarmed blood,
plasma or physiologic saline is transfused.
• The cycle is repeated until a predetermined
volume of blood has been replaced.
 Risk and Complications

• Cardiac and respiratory disturbances

• Shock due to bleeding or inadequate
replacement of blood
• Infection
• Clot formation
• Rare but severe complications include: air
embolism, portal hypertension and
necrotizing enterocolitis
 Conjugated hyperbilirubinemia

Suspect
• High colored urine
• White or clay colored stool
Caution
• Always refer to hospital for investigations so
that biliary atresia or metabolic disorders can
be diagnosed and managed early
 Conjugated hyperbilirubinemia

Causes
• Idiopathic neonatal hepatitis
• Infections -Hepatitis B, TORCH, sepsis
• Biliary atresia, choledochal cyst
• Metabolic -Galactosemia, tyrosinemia,
• hypothyroidism
• Total parenteral nutrition
 Prevention
• Breastfeeding
– Should be encouraged for most women
– 8-12 times/day for 1st several days
– Assistance and education
– Avoid supplements in non-dehydrated infants
• Ongoing assessments for risk of developing
severe hyperbilirubinemia
– Monitor at least every 8-12 hours
– Don’t rely on clinical exam
– Blood testing
• Prenatal : ABO & Rh type, antibody
• Infant cord blood
 Nursing support
• Infant will receive appropriate therapy if
needed to reduce serum bilirubin levels.
• Infant will experience no complications from
therapy.
• Family will receive emotional support.
• Family will be prepared for home
phototherapy (if prescribed).
THANK YOU