Neonatal Cholestasis

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Definition

-  Prolonged conjugated hyperbilirubinaemia in neonate

-  >17 if total bilirubin <85.5

-  >20% of bilirubin if total bilirubin >85.5

 

Features

-  1:2,500 births

-  Any child with jaundice at 2 weeks needs Ix

-  At 3 weeks if breast fed + Hx & Ex NAD

 

Causes

-  70-80% due to

-  Biliary atresia

-  Neonatal hepatitis

-  Alpha-1 antitrypsin deficiency 5-15%

-  TPN

-  Sepsis

-  Galactosaemia

-  Choledocal cysts

 

Complications

-  As related to cause

-  Kernicterus

 

Ix

-  Bilirubin: total & conjugated

-  LFTs

-  New Born Screening results

-  +/- TFT, FBE: hypothyroidism

-  +/- septic screen: Urine MCS, BC

-  +/- CF screen: CF

-  +/- Alpha-1 antitrypsin screen: deficiency

-  +/- Urinary reducing substances: galactosaemia

 

Mx

-  Exclude sepsis, hypothyroidism, panhypopituitarism & inborn errors of metabolism

-  US Abdo

-  Choledocal cysts, biliary atresia

-  +/- DISIDA scan

-  +/- liver biopsy