Jaundice Flashcards
Jaundice in neonates can be split into 3 categories
1) Early (<24hrs old)
2) Intermediate (<2wks)
3) Prolonged (>2wks old)
What are the major causes of early/intermediate jaundice?
Physiological Jaundice Breast Milk Jaundice Sepsis Haemolysis Abnormal Conjugation (genetic problems)
All forms of Early/intermediate jaundice are unconjugated. What causes physiological jaundice?
Short RBC life span
Relative Polycythaemia
Immature Liver
What causes haemolysis in neonates?
ABO incompatible
Rhesus Disease
Spherocytosis
G6PD deficiency
What genetic causes can cause abnormal conjugation of bilirubin?
Gilbert’s Disease
Crigler-Najjar Syndrome
How do we test to determine the source of early/intermediate jaundice?
Split bilirubin, LFTs, Albumin, Coagulation tests, Glc & ammonia
Urine & blood cultures + TORCH screen (For sepsis)
Blood group, DCT, blood film & G6PD Assay (for haemolysis)
Genetic Testing
How do we manage early/intermediate jaundice?
Treat the cause
If really bad can do Blue Light phototherapy (only works for unconjugated jaundice)
The blue light converts bilirubin to a water soluble isomer
Kernicterus is a dangerous complication of unconjugated jaundice. What happens in it?
Unconjugated bilirubin is fat soluble
Crosses BBB
Deposited in brain (mostly basal ganglia)
Neurotoxic –> encephalopathy
How does kernicterus present?
Early –> Encephalopathy –> Poor feeding, lethargy & seizures
Late –> Cerebral palsy, LDs & SN deafness
What are the major causes of prolonged jaundice? (>2wks old, 3 if preterm)
Conjugated = Biliary Atresia, Choledochal cyst, Alagille Syndrome or neonatal hepatitis
Unconjugated = Hypothyroid or breast milk jaundice
Assume Biliary Atresia until proven otherwise
What happens in Biliary Atresia?
Fibroinflammatory disease –> destruction of extrahepatic ducts
Other signs include pale stool, dark urine and liver failure
What happens in alagille syndrome?
Intrahepatic cholestasis (leading to conjugated jaundice), dysmorphism & congenital HDs
What are some causes of neonatal hepatitis?
Viral Alpha-1-antitrypsin deficiency Haemochromatosis Parenteral Nutrition High ammonia etc etc
How do you test someone who presents with prolonged jaundice?
1st) Split bilirubin
2nd) Check stool colour
Other Tests:
- US for cysts & Atresia
- Liver biopsy for Atresia and some hepatitises
- Genotyping for Alagille Syndrome
- Specific tests for hepatitis causes e.g. serology, TFTs, A-1-A level, Ammonia & Fe studies
How do we treat Biliary Atresia after confirming it with US & biopsy?
Kasai Portoenterostomy
