Ductal Plate Malformation in a Nonhuman Primate

Wallace, Sidney; Blanchard, Terrell W.; Rabin, Lionel; Dick, Edward J.; Allan, Jonathan S.; Hubbard, Gene B.

doi:10.1354/vp.46-1-84

Cited by 10 publications

(20 citation statements)

References 9 publications

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“…2,20,21 The present case had portal–portal bridging fibrosis, with marked increase in small or irregular bile duct profiles, minimal to absent inflammation, and lack of nodular regeneration or other histologic evidence of chronic hepatitis. These histologic criteria allow the diagnosis of a ductal plate malformation closely resembling CHF.…”

Section: Discussionmentioning

confidence: 60%

“…In this context, persistent type IV collagen immunoreactivity in the present report case supports the diagnosis of a ductal plate malformation. 2,18 …”

Section: Discussionmentioning

confidence: 99%

“…1 The condition is described in humans and some animal species, 2 characterized by the persistence of embryonic bile ducts, abnormal branching of the intrahepatic portal veins and progressive fibrosis of the portal tracts. 3 …”

Section: Introductionmentioning

confidence: 99%

“…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 …”

Section: Introductionmentioning

confidence: 99%

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

Guerra

Daniel

Cardoso

et al. 2015

Journal of Feline Medicine and Surgery Open Reports

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Case summaryWe describe the case of a 1-year-old male Persian cat diagnosed with congenital hepatic fibrosis (CHF) associated with renal polycystic disease and, for the first time, we have shown that there was no C >A mutation in exon 29 of PKD1 (polycystic kidney disease 1). The cat presented with a history of chronic weight loss, anorexia, vomiting, depression and lethargy, with profuse salivation and ascites on clinical examination. A mild elevation in liver-associated plasma enzymes suggested a hepatic disease. Owing to the cat’s deteriorating condition, it was euthanized. During necropsy, the liver was found to be enlarged, firm and reddish, and the kidney had multiple small cortical cysts. Immunohistochemistry revealed that bile duct cells and epithelial cells of renal cysts showed positive immunoreactivity to keratin 19. Collagen fibers surrounding bile ducts within portal areas demonstrated reactivity to type IV collagen antibody, confirming the congenital nature of the process. A diagnosis of ductal plate malformation consistent with CHF associated with polycystic kidney in a young Persian cat was made. Interestingly, genetic testing revealed a wild-type sequence at position 3284 in exon 29 of PKD1.Relevance and novel informationThe absence of the classic genetic mutation associated with the particular clinical presentation supports the hypothesis of a distinct etiopathogenesis among fibropolycystic diseases in domestic cats. Moreover, congenital hepatic fibrosis is a rare but important differential diagnosis for young Persian cats and their crosses with clinical signs of chronic end-stage liver disease.

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Section: Discussionmentioning

confidence: 60%

“…In this context, persistent type IV collagen immunoreactivity in the present report case supports the diagnosis of a ductal plate malformation. 2,18 …”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…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 …”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Guerra

Daniel

Cardoso

et al. 2015

Journal of Feline Medicine and Surgery Open Reports

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“…6,10 CHF has been described mostly in humans as well as sporadically reported in different species, including dogs, 4,11 cats, 2,18 calves, 3,9,14 and a nonhuman primate. 16 In horses, CHF has been described only in foals of the Swiss Franches-Montagnes (SFM) breed, and it consists of a lethal, monogenic autosomal recessive inherited disease linked to 2 mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene. 7,8,13 The objective of this work was to describe a CHF case in a Purebred Spanish Horse (PSH) foal and to test the 2 mutations in the PKHD1 gene associated with CHF, in both the affected PSH foal and in a healthy population of related PSHs (n ¼ 5).…”

mentioning

confidence: 99%

Congenital Hepatic Fibrosis in a Purebred Spanish Horse Foal: Pathology and Genetic Studies on PKHD1 Gene Mutations

et al. 2018

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A 1-month-old Purebred Spanish Horse (PSH) foal presented with progressive hepatic failure culminating in death. Hepatic lesions were consistent with congenital hepatic fibrosis (CHF). Genetic studies in the PKHD1 gene in the affected foal revealed that it was heterozygous for the 2 previously described single-nucleotide polymorphisms (SNPs) linked to CHF in Swiss Franches-Montagnes (SFM) horses. In addition, 2 novel mutations were detected, the foal being homozygous for one of them and heterozygous for the other. Genetic studies in a healthy PSH population ( n = 35) showed a 3-fold higher genotypic frequency for PKHD1 SNP g.49,630,834G>A and a 5-fold higher genotypic frequency for PKHD1 SNP g.49,597,760A>T compared with those reported for SFM horses. SNPs in the PKHD1 gene in CHF-affected SFM horses might not fully explain the CHF observed in the PSH. Other mutations in the PKHD1 gene could play a more important role in the PSH.

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An unusual presentation of a hepatic ductal malformation in a Boxer dog

Kaniok¹,

Gonzalez-Gasch²,

Alloway³

et al. 2021

Vet Record Case Reports

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An 11‐year‐old male neutered Boxer presented for evaluation of an external ear canal mass, postoperatively diagnosed as a benign inflammatory lesion. Preoperative staging of the ear mass identified a 10‐cm mass affecting the majority of the left lateral hepatic lobe, with inconclusive cytology. A left lateral liver lobectomy was performed. Histopathology revealed the hepatic parenchyma was largely effaced and replaced by a myriad of small calibre tubules lined by well‐differentiated biliary epithelium (cytokeratin19 and cytokeratin7 positive (CK19+, CK7+) supported by abundant fibrosis. Small cords and islands of hepatocytes (hepatocyte paraffin1 positive) (HepPar1+) were entrapped throughout these bile ducts. Analysis of the proliferation index via antigen KI‐67 (Ki67) immunohistochemistry confirmed lack of proliferative activity. Histological and immunohistochemical features were consistent with a ductal plate malformation (DPM). This is the first time a DPM of this size has been described on a computed tomography (CT) scan.

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Ductal Plate Malformation in a Nonhuman Primate

Cited by 10 publications

References 9 publications

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

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

Congenital Hepatic Fibrosis in a Purebred Spanish Horse Foal: Pathology and Genetic Studies on PKHD1 Gene Mutations

An unusual presentation of a hepatic ductal malformation in a Boxer dog

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