ductopenia
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vanishing bile duct diseases, paucity of interlobular biliary ducts, ductopenic pattern, loss of interlobular bile ducts, bile duct loss, vanishing bile duct syndromes, hepatic ductopenia
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
- 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
- familial hemophagocytic lymphohistiocytosis (15906086)
- Langerhans cell histiocytosis
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)
- zonisamide (#17122520 #)
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