Labs

Total BDirectType of hyperbilirubinemia
<5 mg/dl
1.5 mg/dl0.6 mg/dlNormal
25.2 mg/dl20.5 mg/dlConjugated
20.260.8 mg/dlunconjugated

CAUSES OF HYPERBILIRUBINEMIA

Unconjugated Hyperbilirubinemia:

  • ABO incompatibility
  • Rh isoimmunization
  • G6PD deficiency
  • Cephalhematoma or extensive bruising
  • Hemoglobinopathies or other RBC membrane defects
  • Gilbert’s syndrome & Crigler-Najjar syndrome
  • Hypothyroidism
  • Breastfeeding jaundice
  • Breast milk jaundice
  • Ileus
  • Intestinal obstruction
  • UTI

Conjugated Hyperbilirubinemia:

  • Biliary atresia
  • Choledochal cyst
  • Alagille syndrome
  • Tyrosinemia
  • Galactosemia
  • Sepsis
  • TORCH infection
  • Idiopathic neonatal hepatitis
  • Parenteral nutrition related cholestasis

Jaundice starting at <24 hours of age:

  • Haemolytic disorders:
    • Rhesus incompatibility
    • ABO incompatibility
    • G6PD deficiency
    • Spherocytosis, pyruvate kinase deficiency
  • Congenital infection

Jaundice at 24 hours to 2 weeks of age:

  • Physiological jaundice
  • Breast milk jaundice
  • Infection, e.g., urinary tract infection
  • Haemolysis, e.g., G6PD deficiency, ABO incompatibility
  • Bruising
  • Polycythaemia
  • Crigler–Najjar syndrome

Causes of prolonged (persistent) neonatal jaundice

Unconjugated:

  • Breastmilk jaundice
  • Infection (particularly urinary tract)
  • Haemolytic anaemia, e.g., G6PD deficiency
  • Hypothyroidism
  • High gastrointestinal obstruction
  • Crigler–Najjar syndrome

Conjugated (>25 µmol/L): Bile duct obstruction:

  • Biliary atresia
  • Choledochal cyst

Neonatal hepatitis syndrome:

  • Congenital infection
  • Inborn errors of metabolism
  • α₁-Antitrypsin deficiency
  • Galactosaemia
  • Tyrosinaemia (type 1)
  • Errors of bile acid synthesis
  • Progressive familial intrahepatic cholestasis
  • Cystic fibrosis
  • Intestinal failure-associated liver disease (associated with long-term parenteral nutrition)

Intrahepatic biliary hypoplasia:

  • Alagille syndrome

Neonatal Jaundice

When does it appear?

A

  • Before 24h: Usually either Sepsis or Blood diseases (Rhesus disease, ABO incompatibility or HS [Hereditary spherocytosis])
  • 24h to 2 weeks: Most commonly Physiological or Breastfeeding Jaundice
  • Prolonged Jaundice: Breastfeeding jaundice, biliary atresia, sepsis, thyroid problems (hypo), CF

S+S

  • Jaundiced! Skin and sclera are orange!
  • Signs of underlying disease are evident:
    • Pallor and Hemolysis
    • Lemon color + conjugated
    • Biliary Atresia = Pale stools
    • Rhesus – Splenomegaly

Signs of Kernicterus (Bilirubin >350μmol/L)

  • Lethargic
  • Poor feeding
  • ↑ Muscle tone
  • Fits
  • Coma
  • Death!

P

  • Jaundice in general = ↑bilirubin (breakdown of haemoglobin) in blood.
  • Physiological Jaundice: The fetal Hb has a short life span, and the neonatal liver often has difficulty in metabolizing it all. Fetal Jaundice happens in around about 60% of babies, so is very common.
  • Breastfeeding Jaundice: Breastfed babies are more likely to be jaundiced for longer. Multifactorial.
  • Kernicterus = ↑↑bilirubin levels cause bilirubin to cross BBB and deposit in basal ganglia and brainstem. This can give long-lasting neurological damage, but with good treatment, this is avoided.

I

  • Good history and examination.
  • Bloods (Bilirubin, FBC, LFT, Blood groups, Blood film, blood culture – TORCH SCREEN, Coombs test)
  • Urine
  • If persistent jaundice, consider USS of the biliary tree.

T

  • Each center has its own treatment chart, which looks like this →
  • Supportive therapy (hydration status, etc.), if breastfeeding, keep doing so!
  • Phototherapy – 450nm wavelength light converts unconjugated bilirubin into a harmless substance. The baby must be completely naked but wear eye protection. Can be done via fibre-optic blanket.
  • Transfusion – Either done through UVC or peripheral vein and arterial line. 2x baby’s blood volume is transfused.

Scenario 1

mother of ali 1 month complaining yellowish discoloration of sclera, since was 2 weeks of age. persistant jaundice

information

  • jaundice is lemon yellow extending to the mid-abdomen
  • all is B+ blood group, mother is B+ (No ABO/RHO incompatibility)
  • normal vaginal delivery, uneventful pregnancy and birth, no admission to NICU
  • All is is exclusively breast fed, taking frequent feds, he gained baout 500 mg. during last 2 weeks (normal growth of baby) - (no suboptimal intake)
  • stool become clay colored, urine is dar (Obstructive JAundice)
  • abdominal distension is noticed (Biliary atresia????)

Chief of complant

what brings you today?

  • theres yellowish discoloration

HOPI

When did you notice?

  • 1 month ago For how long?

Tone of color?

  • Yellow Progress?
  • first just sclera, extending to abdomen. Associated?
  • stool/urine color changes
  • constipation
  • hematuria
  • polyuria
  • fever
  • weight loss
  • irritations
  • Blood group of mothter & baby
  • Hypothyroidism - prolonged
  • increased tummy size - hepatomegaly, spleenomegaly.
  • constipation (hypothyroidism)
  • vomitting
  • diarrhea

Review systems

CNS

  • Irritiability
  • high pitched cry
  • seizures

Respiratory

  • tachypnea
  • Cough

CVS

  • Cyanosis
  • breathing
  • fatigue

Hematology

  • Bruises
  • hematuria

Developments

  • follow up finger to mid line
  • awareness to sound

Neonatal History

less in two years, related to neonatal diseases if 7+ “is there any complications during delivery?”

Parinatal

  • D-Diseases during pregnancy? (diabetes)
  • A- Admissions: during pregnancy, age of mother
  • T- Drugs: supplementation, any drug not prescribed by doc, medications

Neonatal

  • Gestational Age (Preterm)
  • Mode of delivery (complicated/noncomplicatied - prolonged/nonprolonged)
  • Cried immediatly?
  • Apgar score
  • Cut of umbilical cord (delayed; polycyt?)

Postnatal

  • admission to neonatal? How long? what drugs? - if yes take full history of it.
  • Screening test (hypothyroidism) - transucutantous???

T

travel, transfusion … truama, photo therapy

Family history

  • consanguinity
  • Siblings with similar diseases (if yes, take full details)
  • Blood diseases in family (G6PD)
  • Thyroid, Liver, syndromes (Gilbert- recurrent attacks of jaundice) diseases

Social History

  • depends on case - not for jaundice.

Scenario 2

10 days old complaining yellow discoloration of skin and sclera since was 3 days old

Diagnosis: Physiological jaundice / (suboptimal? open diagnosis)

Chief of complaint

What is your child complaining of?

  • 7 days

HOPI

Progression, Tone, Extension, Frequency urine, blood group mother and child ABO/RHO, color urine and stool, abdomen distension, tone, lethargy, vomiting, fever, Nutrition(breast fed?); weight changes, does it come and go,

Neonatal

Perinatal

  • Blood transfusions
  • Vaccinations
  • Diabetes
  • Admission to hospital (flu, rash)
  • Drugs (without prescription)

Natal

  • GA
  • Mode delivery
  • Complication

Postnatal

  • admission to NICU (take details if yes)
  • screening

Family history

siblings similiar conditions…