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neonatal cholestasis

Tuesday 3 May 2005

Types

- neonatal extra-hepatic cholestasis
- neonatal extra-hepatic cholestasis

  • ductopenias
  • neonatal lobular cholestasis

Etiology

- neonatal biliary duct diseases

  • neonatal extra-hepatic biliary atresia
  • neonatal biliary duct paucity (ductopenia)
    • neonatal Alagille disease
  • Aagenaes syndrome (norwegian)
  • north american indian cirrhosis (north american indian cholestasis)
  • Greenland eskimo cholestasis

- neonatal intrahepatic cholestasis

  • congenital bile acide synthesis defects (CBASs)
    • CBAS1 (MIM.607765) : 3beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency (HSD3B7 - MIM.607764)
    • CBAS2 (MIM.235555) : delta4-3-oxosteroid-5beta-reductase deficiency (AKR1D1 - MIM.604741).
    • CBAS3 (MIM.603711) : microsomal oxysterol 7-alpha-hydroxylase (CYP7B1 - 603711)
    • CBAS4 (MIM.214950) : alpha-methylacyl-CoA racemase gene (AMACR; MIM-604489)
  • bile anomalies
    • cystic fibrosis

References

- Bove KE, Heubi JE, Balistreri WF, Setchell KD. Bile acid synthetic defects and liver disease: a comprehensive review. Pediatr Dev Pathol. 2004 Jul-Aug;7(4):315-34. PMID: 15383928

- Knisely AS. Progressive familial intrahepatic cholestasis: an update. Pediatr Dev Pathol. 2004 Jul-Aug;7(4):309-14. PMID: 15383927

- Knisely AS. Progressive familial intrahepatic cholestasis: a personal perspective. Pediatr Dev Pathol. 2000 Mar-Apr;3(2):113-25. PMID: 10679031

- Jevon GP, Dimmick JE. Histopathologic approach to metabolic liver disease: Part 1. Pediatr Dev Pathol. 1998 May-Jun;1(3):179-99. PMID: 10463278


- Histopathological diagnosis of intra- and extrahepatic neonatal cholestasis, Braz J Med Biol Res, July 1998, Volume 31(7) 911-919