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
b) appears in 2nd to 3rd day
- Physiological
- Crigler najjar
- Early onset breast milk jaundice
c) from 3rd day to 7th day
- Bacterial sepsis
- UTI
- Polycythemia
- Cong. Infections
d) after first week -All other caouses other than those mentioned above
Physiological Jaundice (unconjugated bilirubin)
- 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)
Breast milk Jaundice (unconjugated)
- 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.
Crigler Najjar syndrome
Type 1
- Inheritance – autosomal recessive
- LFT – normal
- Liver histology – normal
- Kernicterus – yes
- UDPG transferase – absent
- response to phenobarbitone- no response
- hemolysis – absent
Type 2
- inheritance – autosomal dominant
- LFT – normal
- Liver histology- normal
- Kernicterus- rare
- UDPG transferase- reduced
- response to phenobarbitone- decreases bilirubin
- hemolysis – absent
Dubin Johnson Syndrome
- AR, conjugated hyperbilirubinemia becoz of mutation in canalicular multidrug resistance protein 2(MRP2)
- LFT normal; accumulation of black granular pigment in lysosomes of centrilobar hepatocytes
- Increased urinary coprophorphyrin but total coprophorphyrin is normal
- 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
