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ductopenia

Wednesday 4 June 2003

Etiology

I. Associated forms of bile duct paucity

- metabolic and genetic disorders

  • alpha1-antitrypsin deficiency
  • inborn errors of bile acid metabolism
  • peroxisomal diseases (cerebrohepatorenal syndrome, Zellweger syndrome)
  • coprostanic acidemia

- genetic diseases

  • chromosomal diseases: 45X (Turner syndrome), trisomy 17, trisomy 18, trisomy 21
  • Ellis-van Creveld syndrome (12100513)
  • Byler disease
  • cystic fibrosis

- inflammatory and dysimmune disorders

  • auto-immune sclerosing cholangitis
  • hepatic GVHD
  • chronic hepatic allograft rejection
  • hepatic sarcoidosis

- bilairy atresia (late)
- Hodgkin lymphoma (11241447)
- histiocytic hepatic infiltration and histiocytic portal infiltration

- panhypopituitarism
- drug-induced vanishing bile-duct syndrome

  • antibiotic-associated vanishing bile-duct syndrome

- fetal infections

  • fetal rubella (rubella fetopathy)
  • fetal CMV infection (CMV fetopathy)
  • fetal syphilis

- miscellaneous

  • prune belly sequence
  • congenital pancreatic hypoplasia associated with paucity of bile ducts and renal microcysts (15214468)
  • leprechaunism (9339119)
  • PFIC (progressive familial intrahepatic cholestasis)

- drug-induced ductopenia (drug-induced vanishing bile duct syndrome)

II. idiopathic forms

A. syndromic paucity of interlobular bile ducts (Alagille’s syndrome)

B. nonsyndromic paucity of interlobular bile ducts

  • familial idiopathic childhood ductopenia
  • familial idiopathic adulthood ductopenia

References

- Desmet VJ, van Eyken P, Roskams T. Histopathology of vanishing bile duct diseases. Adv Clin Path. 1998 Apr;2(2):87-99. PMID: 10358336

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