Table 4.
Histological Features of Specific Causes of Neonatal Cholestasis
Disorder | Histology |
---|---|
Biliary obstruction | Portal tract bile duct plugs, ductular reaction, portal tract fibrosis |
A1AT deficiency | Periodic acid Schiff-positive, diastase-resistant hepatocyte globules |
Alagille syndrome | Bile duct paucity |
Neonatal sclerosing cholangitis | Necro-inflammatory bile duct lesions |
Inborn Errors of Metabolism | Hepatic steatosis and pseudo-acinar formation of hepatocytes |
PFIC types 1, 2 | Electron microscopy - abnormal canalicular bile |
Storage diseases | Electron microscopy - lysosomal storage material |
Transient Neonatal Cholestasis | Multinucleated giant cells, extramedullary hematopoiesis, and hepatocellular cholestasis without portal tract involvement |
Viral infections | CMV inclusions and HSV, CMV on immunohistochemistry |