Jaundice in early infancy is often physiological and usually disappears within the first fortnight. However, unlike the physiological variety, direct jaundice (conjugated hyperbilirubinemia) is always pathological and needs careful investigation and early management. Unfortunately, due to various reasons, attention is drawn to the problem only after the first few precious weeks or even later after the liver and biliary tree have suffered irreparable damage.

Q. How does one suspect conjugated hyperbilirubinemia?
A. Conjugated hyperbilirubinemia is the leading cause of chronic liver disease in Indian children. The direct nature of jaundice can be suspected by observing the 'off-white stools' and 'off- white urine' with the presence of jaundice.

Q. What are the causes of conjugated hyperbilirubinemia in the infants?
A. In the neonate, sepsis is the most frequent cause and later biliary atresia (BA) is seen in as many as 75% of cases. The list of causes is given below:

 Infective :

• Viral
  CMV, Rubella, Reovirus III, Hepatitis B
• Bacterial
  E. Coli, Listeria,
• Protozoal
  Toxoplasma

• Niemann-Pick Type C
• Galactosemia
• Alpa-1 antitrypsin deficiency
• Biliary Hypoplasia (Syndromic)
• Cholestasis with actin and microfilament accumulation
• Progressive intrahepatic cholestasis
• Iron storage disorders
• Hepatosteatosis
  
  

• Trisomy 13
• Trisomy 18
• Trisomy 21
  
  

• Biliary atresia - Neonatal Hepatitis
• Choledochal cyst
  
  

• Anemia
• Hypoxia
  
  

• TPN
• Hypothyroidism
• Maternal alcohol ingestion
• Erythromycin estolate
• Frusemide
• Hemolytic disease
  
  

Since the etiopathology of biliary atresia-neonatal hepatitis (BA-NH) syndrome has not yet been understood, one may speculate that the atretic biliary tree is a consequence of continued neonatal infection. This is borne our from the finding that though many cases of biliary atresia are diagnosed only after 6-8 weeks, the jaundice has existed earlier- usually as the continuation of the physiological jaundice. Moreover, we have seen that hepatobiliary scintiscans which were normal can become obstructive (showing no excretion tracer into the gut upto 24 hrs) with the passage time.