Conjugated Hyperbilirubinemia

Jaundice in the period after birth due to conjugated hyperbilirubinemia is called neonatal cholestasis.

Biliary atresia and other congenital liver diseases are distinguished from physiological prolonged neonatal jaundice as they are associated with conjugated (direct), rather than unconjugated, hyperbilirubinemia.

Causes of neonatal cholestasis as examples of many

Extrahepatic disorders:

- biliary atresia

Intrahepatic disorders

- “ Idiopathic “ neonatal hepatitis

- Intrahepatic cholestasis (persistent): example Alagille syndrome

Anatomic

Metabolic and endocrine disorders as

- tyrosinemia,

- Niemann-Pick disease,

- galactosemia,

- alpha 1 antitrypsin deficiency

Infectious diseases

Chromosomal anomalies

It important to differentiate between biliary atresia and other causes of cholestasis, because biliary atresia needs timely surgical treatment  

Last modified
28 August 2019
LOINC
1968-7 Bilirubin.direct [Mass/volume] in Serum or Plasma
1975-2 Bilirubin.total [Mass/volume] in Serum or Plasma
HPO
HP: 0006579 Prolonged neonatal jaundice
Feature

Neonatal jaundice is a yellowish discolouration of the white part of the eyes and skin in a newborn baby due to high bilirubin levels.  The...

Investigations of serum bilirubin are indicated in the first days of life when yellowness rapid appears.

And when the child remains to have a yellow...

Rare Condition

Alagille Syndrome

Alagille syndrome is a multisystem disorder possibly affecting :

- liver: chronic cholestasis due to paucity of intrahepatic bile ducts, 

- heart: peripheral pulmonary artery...

Disease

Neonatal Cholestasis 

Prognosis of cholestasis due to specific disorders (as...