Infantile Cholestasis

NC (Neonatal Cholestasis)

• Assess clinical status → Sick OR Not Sick

Sick

• Branch on sepsis screen → Sepsis Screen +ve OR Sepsis Screen –ve

Sick → Sepsis Screen +ve

• Treat sepsis (Galactosemia needs to be excluded by urine reducing sugar by Benedict's test as sepsis can be associated with Galactosemia)

Sick → Sepsis Screen –ve

• Test for CMV, HSV, Urinary Succinylacetone, Ferritin, GALT if urine reducing sugar is positive

Not Sick

• Branch on stool color → Pale stools OR Pigmented stools

Not Sick → Pale stools

• Fasting USG (see for contractility post-feed)
• → Normal · Small gall bladder · Absent gall bladder · Choledochal cyst

Pale stools → Fasting USG results

• Normal: → Liver biopsy → Specific treatment
• Small gall bladder: → Biliary atresia to be excluded
• Absent gall bladder: → Biliary atresia to be excluded
• Choledochal cyst: → Surgery

Not Sick → Pigmented stools

• GCT*, Fasting USG (If the USG show a choledochal cyst, the child needs to be referred for surgery), Metabolic tests, Genetic tests, Liver Biopsy
• → Specific treatment

Notes

• ----------> Optional
• * GGT is normal in PFIC I and II and some inborn errors of bile acid metabolism