Master Of Medicine

Unconjugated hyperbilirubinemia

1) Increased production– hemolytic anemia (Hereditary spherocytosis, G6PD deficiency)
ineffective erythropoesis (Thalassemia, Pernicious anemia)
2) Reduced hepatic intake from bilirubin-albumin complex – drug induced
3) Impaired hepatic conjugation
a) Physiological – functionally immature liver
b) Breast milk jaundice – pregnendiol and FFA in breast milk interferes conjugation (in susceptible)
c) Genetic deficiency of UDPG transferase – Crigler-Najjar, Gilberts
d) Hepatocellular dysfunction– Viral or drug induced Hepatitis, Cirrhosis
e) Hypothyroidism
f) Cephalhematoma

Conjugated Hyperbilirubinemia

1) Impaired secretion of conjugated bilirubin into bile – Dubin-Johnson, Rotor’s
2) Impaired bile flow

a) Obstructive jaundice
b) Primary biliary cirrhosis
c) Neonatal cholestasis – extrahepatic biliary atresia/neonatal idiopathic hepatitis
Choledochal cyst,sclerosing cholangitis,Caroli’s disease
d) Metabolic– Tyrosinemia, Wolman disease, Niemann Pick, Galactosemia, Fructosemia

Appearance of Jaundice – Area of infant body to s.bilirubin levels

Head&neck — 5mg/dl
Upper trunk & proximal Upper limb — 10mg/dl
Lower trunk & thigh — 12mg/dl
Legs — 15mg/dl
Palms & sole — >15mg/dl

Causes of jaundice and time of onset

a) within 24 hours –

• Rh incompatibility (most common)
• Concealed haemorrhage
• Sepsis
• Cong. Infections – CMV, Rubella, Syphillis, Toxoplasma

• Physiological
• Crigler najjar
• Early onset breast milk jaundice

• Bacterial sepsis
• UTI
• Polycythemia
• Cong. Infections

• After 24 hours
• progressive rise in s.bilirubin- <5mg/dl per day (no sudden rise)
• Peaks in 3-5 days, total not more than 15mg/dl
• Clinical jaundice resolved by 1 week in term infants and 2 week in preterm
• 2 phases – (phase 1 – lasts 5 days in term (12mg/dl) and 7 days in preterm (15mg/dl); phase 2 – gradual decline to 2mg/dl, which          lasts 2 weeks, before falling to adult values)

• After 30 hrs, exclusively breast fed infants,
• May continue way into 3rd month
• bilirubin may reach 20mg/dl
• Temprorary intererruption of breast feeding will dramatically bring down bilirubin levels, once within control values restart breast feed.

Type 1

• Inheritance –    autosomal recessive
• LFT    –      normal
• Liver histology  –    normal
• Kernicterus –  yes
• UDPG transferase – absent
• response to  phenobarbitone-   no response
• hemolysis –  absent

Type 2

Dubin Johnson Syndrome

1. AR, conjugated hyperbilirubinemia becoz of mutation in canalicular multidrug resistance protein 2(MRP2)
2. LFT normal; accumulation of black granular pigment in lysosomes of centrilobar hepatocytes
3. Increased urinary coprophorphyrin but total coprophorphyrin is normal
4. Gall bladder is not visualised on oral cholecystography

Rotor syndrome

• AR, congenital conjugated hyperbilirubinemia. decreased biliary excreation
• Liver is not pigmented, urine as well as total coprophorphyrin will be increased
• Gall bladder will be visualized

This post was written by my friend Kim George, you can contact him @drkimgeorge@gmail.com