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Vet Pathol 40:693-694 (2003)
© 2003 American College of Veterinary Pathologists
BRIEF COMMUNICATIONS AND CASE REPORTS

Bile Duct Dysplasia and Congenital Hepatic Fibrosis Associated with Polycystic Kidney (Caroli Syndrome) in a Rat

G. Bettini, L. Mandrioli and M. Morini

Abstract

Hepatic fibrosis with bile duct ectasia and hyperplasia associated with polycystic kidney disease, analogous to Caroli syndrome in humans, was observed in a rat used as a control in a subchronic toxicity study. Light microscopy of liver sections showed multiple cystic and segmental saccular dilatations and hyperplasia of the intrahepatic bile ducts associated with overgrowth of portal connective tissue; the kidneys had diffuse cystic dilatation of cortical renal tubules. The lesions resembled those of human cases of the fibropolycystic disease termed as Caroli syndrome, which is thought to be the result of a pathologic developmental process known as ductal plate malformation. Recently, an animal model of Caroli syndrome has been described in mutant rats from a colony that constantly showed renal and hepatic cysts and an autosomal recessive mode of inheritance. The finding in our case of identical hepatorenal lesions suggests that the same mutation has occurred incidentally in a standard colony.

Key words: Caroli disease; congenital hepatic fibrosis; polycystic kidney; rats.

Request reprints from Prof. G. Bettini, Dipartimento di Sanità Pubblica Veterinaria e Patologia Animale, Servizio di Anatomia Patologica, Via Tolara di Sopra 50, 40064 Ozzano Emilia (Bologna), Italy. E-mail: bettini{at}vet.unibo.it.




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S. Wallace, T. Blanchard, L. Rabin, E. Dick, J. Allan, and G. Hubbard
Ductal Plate Malformation in a Nonhuman Primate
Vet. Pathol., January 1, 2009; 46(1): 84 - 87.
[Abstract] [Full Text] [PDF]


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