Congenital Hepatic Fibrosis in 5 Dogs

Abstract

Congenital hepatic fibrosis is a disorder of biliary system development histologically characterized by diffuse periportal to bridging fibrosis with numerous small often-irregular bile ducts and reduction in the number of portal vein branches. The condition results from abnormal development of the ductal plate, the embryonic precursor to the interlobular bile ducts. It has rarely been reported in veterinary species, and it has never been reported in dogs. This article describes 5 cases of a ductal plate malformation in dogs consistent with congenital hepatic fibrosis. On light microscopy, all 5 livers had severe bridging fibrosis with a marked increase in the number of small bile ducts, which often had irregular, dilated profiles reminiscent of the developing ductal plate. In addition, 80% (4 of 5) of cases lacked typical portal vein profiles. Cytokeratin 7 and proliferating cell nuclear antigen immunohistochemistry was performed on the 3 cases for which paraffin-embedded tissue was available. The bile duct profiles were strongly positive for cytokeratin 7 in all 3 cases, and they were negative for proliferating cell nuclear antigen or only had rare positive cells. All 5 dogs presented with clinical signs of portal hypertension. Congenital hepatic fibrosis should be considered in the differential diagnosis in young dogs that present with portal hypertension and lesions that may have been interpreted as bridging biliary hyperplasia or extrahepatic biliary obstruction.

Congenital hepatic fibrosis and polycystic kidney disease not linked to C >A mutation in exon 29 of PKD1 in a Persian cat

“…CHF can be distinguished from chronic extrahepatic biliary obstruction by the typical lack of inflammation or evidence of cholestasis, but in some cases, the history and clinical and imaging data will need to be evaluated carefully. 4 …”

“…2 In veterinary medicine, CHF has been described in dogs, Swiss Freiberger foals, bovine fetuses and calves, and in an African green monkeys. 2,4–7 …”

Ductal Plate Malformation in the Liver of Boxer Dogs

“…The number of biopsies from different liver lobes and relative size of these biopsy specimens are provided in Supplemental Table S1. Six juvenile Boxers (3,5,21,180,270, and 360 days of age) and 10 adult non-Boxer dogs (4,4,5,5,8,8,9,9,10, and 14 years of age) were used as controls for histologic evaluations. The clinical histories of control dogs were reviewed to verify that reasons for tissue sample evaluation were unrelated to liver disease.…”

“…57 Genetic mechanisms causing DPM implicate mutations affecting structure and function of primary cilia. 5,17,24,31,37,56,57 The abnormal bile ducts may reflect a differentiation defect, defective maturation of primitive ductal structures, or abnormal duct expansion or proliferation. 57 Primary cilia are solitary nonmotile organelles located on the apical surface of most mammalian cells.…”

Avaliação morfológica e molecular pós-tratamento com os fatores hepatotróficos, da cirrose hepática induzida em ratas pela tioacetamida: análise a curto e longo prazo

“…É considerado um processo irreversível, mas há estudos sugerindo que a regressão e até mesmo a reversão da cirrose são possíveis (GARCIA-BAÑUELOS et al, 2002;SARIN, 2007;SATO et al, 2008;SOBREVALS et al, 2010 Na clínica veterinária, as injúrias hepáticas crônicas são comuns em cães e podem culminar em cirrose (WATSON, 2004;BROWN et al, 2010). As causas dessa patologia abrangem inflamações do trato gastrintestinal, uso prolongado de corticóides, anticonvulsivos e imunoterapia para Corynebacterium parvum e também infecções virais (adenovírus canino tipo I).…”