UHS PBL: Two-Day-Old with Yellowish Discoloration:

Neonatal Jaundice

Learning Objectives
 Be able to take history in neonatal jaundice
 Be able to clinically examine neonatal jaundice
 Discuss how you will reach the cause of jaundice
 Be able to council the mother and answer her questions
 Be able to recognize measures that can be taken to prevent complications of
hyperbilirubinemia after birth

Case Scenario
A two-day-old baby girl was born through vaginal delivery with Apgar score of 9 and 10 at 1 and
5 minutes. Her birth weight was 3.4 kg. The nurse called the pediatrician because she noticed
that the baby was deeply jaundiced. Bilirubin skin test was reaching 400 μmol/L (23.5 mg/dL).

Discussion Questions
1. What other information would you want to obtain from the history?
 Patient’s Demographics
 Obstetric History
o Previous pregnancies / miscarriages / abortions
o Antenatal visits / screening
o Infections / illnesses / complications during pregnancy
o Medications during pregnancy
o Mother’s blood type
 Natal History
o Gestational age & birth weight
o Type of delivery (C-section / NVD; if NVD any instrument use like forceps /
vacuum)
o Complications / need for resuscitation / NICU admission
o Is he feeding well? Breastfed? Did he pass stool?
o Blood type
 Maternal and family history

2. In your clinical examination, what points will you focus on?

General Examination
 Jaundice & its extent
o Face: around 5mg/dL o Abdomen: around 15 mg/dL
o Chest: around 10 mg/dL o Toes: >23 mg/dL
 Pallor  Growth  Vital signs
parameters
CNS Examination: neonatal reflexes (especially Morro)
 Abdominal Examination: check for hepatosplenomegaly

3. What is your impression?

2-day-old full-term baby girl born by NVD with a birth weight of 3.4 kg presented with
jaundice; bilirubin skin test was 400 umol/L.
 Physiological jaundice is a diagnosis of exclusion.

4. What is your differential diagnosis?

Direct Hyperbilirubinemia (5%):
 Obstruction:
o Intrahepatic: congenital biliary atresia / Choledochal cyst
o Extrahepatic (rare): tumors / stones
 Infections: TORCH / hepatitis B
 Metabolic: alpha-1 antitrypsin deficiency / galactosemia / Dubin Johnson / Rotor’s
syndrome
 Iatrogenic: TPN / drug-mediated
 Idiopathic: neonatal non-infectious hepatitis (exclusion)
Indirect Hyperbilirubinemia (95%)
 Hemolytic (high indirect bilirubin + low Hb + high reticulocyte count):
o Coombs & Blood Group +
 Rh incompatibility (mother is -ve whereas the child is +ve; occurs in the 2 nd
pregnancy or if the mother had a miscarriage / received a blood transfusion)
 ABO incompatibility (mother is O whereas the child is A or B; affects the 1 st
pregnancy)
 Minor: Duffy / Kell (rare; mother is -ve whereas the child is +ve)
o Coombs & Blood Group –
 Enzyme deficiency: G6PD / pyruvate kinase deficiency
 Structural: hereditary spherocytosis / elliptocytosis
 Hemoglobin defect: alpha thalassemia (no hemolysis in <6 months because
protective HbF) (SCD does not appear in neonates either)
 Non-Hemolytic:
o If prolonged (> 15 days):
 Cirgler Najjar
 Pyloric stenosis
 Breast milk jaundice (poor quality; inhibiting liver enzymes)
 Hypothyroidism
o Infections: sepsis (hemolysin toxin produced by bacteria)
o IODM (insulin inhibition of enzymes + polycythemia)
o Breast feeding jaundice (decreased feeding  decreased ejection of bilirubin 
bilirubin is broken down in the intestine & recycled thus indirect
hyperbilirubinemia) (diagnosed by weight loss of 10% within the first few days)
o Cephalhematoma (due to vacuum delivery; usually late jaundice)
 Hepatitis
Congenital Biliary
Direct (5%) atresia
Idiopathic
>20% of total Intrahepatic
Choledochal Cyst
Obstruction

Extrahepatic Stones & Tumors

Rh
Incompatibility

ABO
Coomb's +
Incompatibility

Minor antigens
Jaundice incompatibility
G6PD Deficiency
Hemolytic Enzyme
Deficiency Pyruvate Kinase
Deficiency

Hereditary
Coomb's - Membrane Spherocytosis
Defect Hereditary
Elliptocytosis

Hemoglobin Alpha
Defect thalassemia
Indirect (95%)
Crigler Najjar

Pyloric Stenosis
Prolonged:
Breast Milk
Jaundice
Infections:
hemolysin in
sepsis Hypothyroidism

IODM
Non-Hemolytic POLYCYTHEMIA
normal retics
Breastfeeding
Jaundice

Cephalhematoma

Physiologic
Jaundice <12mg
5. What are the most important investigations?
Bilirubin: total + direct / indirect
Direct Hyperbilirubinemia
 Liver Function Tests:
o ALT: very high in acute hepatitis and chronic cirrhosis (b/c it is released when the
hepatocytes rupture)
o AST: not specific for liver
o ALP & GGT: elevated in obstruction
 Abdominal Ultrasound:
o Congenital biliary atresia = collapsed gallbladder
o Choledochal cyst
o Check liver & spleen
Indirect Hyperbilirubinemia
 CBC & reticulocyte count
 Coomb’s test & blood smear
 Thyroid function test

6. What is your management?

 Supportive (enhance enteral nutrition)
 TREAT THE CAUSE
 Phototherapy:
o Can be single / double / intensive
o Intensive phototherapy may lead to dehydration b/c of high insensible water loss
 give IV fluids
o Duration: usually 1-2 days unless it is due to ABO / Rh incompatibility then 7 days
o It can be stopped when the bilirubin levels drop by 2 squares (50 umol/L) to make
sure you do not need to repeat it. Note that once discharged, the family are
unlikely to bring him back so if he develops jaundice again, the treatment was not
adequate (if he returns within 72 hours of discharge then the decision to discharge
was incorrect).
o Note: the sun is useless as the waves are harmful and are also filtered by the
windows; if the patients mention history of a previous child who improved with
sun-bathing, explain to them that jaundice can fade away on its own and that it
was not due to the sun.
 Exchange transfusion: 80ml/kg volume of blood in baby. If hes 3kg, he will be 240ml, if
exchanged twice then you need 500ml (480). indications:
o Hemoglobin < 8 g/dL
o Signs of acute bilirubin encephalopathy (hypertonia / arching / retrocollis /
opisthotonos / fever / high-pitched cry)
o Total bilirubin is > 5 mg/dL above the lines on the graph

7. How will you advice about preventing the situation?

 Follow-up 2-4 days after discharge (with the pediatrician; many cases end up requiring
admission)

PHOTOTHERAPY

EXCHANGE TRANSFUSION
Notes
Criteria for Physiological Jaundice
- Bilirubin level: < 200 umol/L (less than 12 mg/dL)
- Onset: after 48 hours (1st 48 hours is pathological)

Unit Conversion
- Divide by 17 to convert umol/L  mg/dL

Why is jaundice dangerous?

- Kernicterus (Hyperbilirubinemia Encephalopathy)
 Acute (500-600 umol/L)  effect on brain (seizures + absent morro / suckling as they
are the 1st reflexes to be affected) convulsions & not feeding / arching of back.
 Chronic  effect on basal ganglia  chorea (gross movements) + athetosis (fine
movements of the hands) extrapyramidal manifestations/encephalopathy + mental
retardation + hearing problems DEAFNESS > do a hearing test.
 Note: complications like kernicterus are usually within the first 10 days
- Liver Cirrhosis
 Due to the biliary stasis

American Academy of Pediatrics

- It has a chart for treatment of hyperbilirubinemia that indicates no phototherapy / single
phototherapy / double phototherapy / exchange transfusion
Extra Notes

Side effects of phototherapy:

- Loose stools (due to excretion of bile salts & unconjugated bile)
- Skin rash and tanning of skin
- Hyperthermia / hypothermia
- Dehydration (insensible water loss)
- Damage to exposed eye / genitalia

Drawbacks of exchange transfusion?

- Due to umbilical catheterization: embolism / thrombosis / sepsis / portal hypertension
(later in life)
- Heart failure (due to volume overload)
- Hazards of blood transfusion
- Hypocalcemia / hypoglycemia / hyperkalemia

Indications of exchange transfusion?

- Hyperbilirubinemia
 Rh incompatibility:
o Cord bilirubin > 5mg/dL
o Cord hemoglobin <10 gm/dL
o Rapid rise of bilirubin (>1 mg/dL/h) despite phototherapy
o Bilirubin level exceeding 10 mg/dL (1st day); 15 mg/dL (2nd day); 20 mg/dL (any day)
o History of kernicterus in a sibling
 Other causes: if serum bilirubin exceeds 20 mg/dL (healthy full-term; lower for pre-
term / sick babies)
- Neonatal sepsis
- Necrotizing enterocolitis
- Anemic heart failure (use packed RBCs)
- Respiratory distress syndrome
- Congenital cyanotic heart disease with marked polycythemia (use plasma)
- Sickle cell anemia crisis

Timing of Clinical Jaundice – Unconjugated

- In the 1st day of life:
 Rh incompatibility
 ABO incompatibility
- In the 2nd-3rd day of life:
 Physiologic jaundice
 Criggler Najjar syndrome
- After the 1st week:
 Breast milk jaundice
 Hemolytic anemia
- Persistent during the 1st month:
 Criggler Najjar syndrome
 Prolonged physiologic jaundice (hypothyroidism / pyloric stenosis)
 Breast milk jaundice

Timing of Clinical Jaundice – Conjugated

- In the 1st day/week of life:
 TORCH infections
- Late in the 1st week of life:
 Neonatal sepsis
- Persistent during the 1st month:
 Neonatal hepatitis (metabolic / infectious)
 Congenital biliary atresia
 Inspissated bile syndrome