Inherited Congenital Extrahepatic Portosystemic Shunts in Cairn Terriers

Straten, Giora van; Leegwater, P.A.J.; Vries, M. de; Brom, W. E. van den; Rothuizen, J.

doi:10.1892/0891-6640(2005)19[321:icepsi]2.0.co;2

Cited by 22 publications

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“…4,9,10,13,16 Although congenital PSS are acknowledged to have a familial link in some breeds of dog, no such genetic predispositions have been conclusively demonstrated in other species to date. 5,11,15,17,18 In the current study, congenital portal vein hypoperfusion suggestive of a hepatic portosystemic shunt is described in 2 juvenile goats sired by the same buck. To the authors' knowledge, this is the first report that suggests a hereditary basis for PSS in the goat.…”

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confidence: 62%

“…Although little can be formally concluded from the limited number of cases examined, what can be stated is that the distribution pattern of clinically affected animals in the current report is not inconsistent with those of PSS and HMD (PPVH) in some breeds of dog for which an autosomal and polygenic mode of inheritance has been suggested. 2,14,17,18 In conclusion, HE that results from congenital portal vein hypoperfusion, and perhaps even congenital PSS, should be considered as a differential diagnosis in young goats that present with chronic, periodic CNS deficits and may, in fact, remain a viable diagnosis even in cases where multiple, interrelated goat kids are affected. …”

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confidence: 98%

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Hepatic Encephalopathy in Two Goat Kids with Common Paternity

et al. 2008

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Abstract. Two juvenile, intact, female mixed-breed goats from a common sire were presented for periodic neurologic deficits, seizures, and a generalized loss of body condition that occurred over a 4-6-week period. On physical examination, both goats were thin, obtunded, blind, and ataxic. Laboratory diagnostics revealed increased serum bile acids (95 mmol/l; reference interval: 0-50 mmol/l) in one of the goats. Both goats exhibited progressive physical and mental deterioration, and were eventually euthanized. Upon necropsy, no significant macroscopic lesions were noted. Microscopic examination, however, demonstrated hepatocellular atrophy and anomalies in the hepatic microvasculature, including duplication of hepatic arteries, small-to-indistinct portal veins, and oval cell hyperplasia. In addition, spongiform change was microscopically identified throughout the parenchyma of the brain, most notably within the white matter and along the junction of gray and white matter. The diagnosis of congenital portal vein hypoperfusion (suggestive of a portosystemic shunt) with resultant hepatic encephalopathy was proposed in each case based on the characteristic microscopic lesions in conjunction with the signalment and history of the goats. The observation that the affected kids were sired by the same buck suggests a hereditary basis for the condition in these animals as well.

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confidence: 62%

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confidence: 98%

Hepatic Encephalopathy in Two Goat Kids with Common Paternity

et al. 2008

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“…20 Canine breed studies, however, support a familial digenic, tri-allelic trait of PSSs in Irish Wolfhounds, whereas affected Cairn Terriers displayed a polygenic autosomal inheritance pattern. 20–22 None of the patient’s progeny had reported clinical signs attributed to PSSs at the time of writing the manuscript; however, these cats have not been actively investigated. Moreover, the queen only showed clinical signs during perigestational periods and was otherwise well between.…”

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confidence: 99%

Peripartum clinical manifestations of a mesentericorenocaval shunt in a Burmese cat

Hoon

Siow

Jenkins

et al. 2020

Journal of Feline Medicine and Surgery Open Reports

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Case summary A 3-year-old entire female Burmese cat was presented for investigation of intermittent lethargy during gestation followed by persistent hypersalivation and ataxia postpartum. The cat had queened three litters in total, with clinical signs worsening during the most recent lactation period. Mild anaemia (26%), hypoglycaemia (2.4 mmol/l; reference interval [RI] 3.9–8.3 mmol/l) and increased postprandial serum bile acids (74 µmol/l; RI <25 µmol/l) were identified on initial bloodwork. Multiphase contrast-enhanced CT identified a mesentericorenocaval portosystemic shunt; this was attenuated surgically with an ameroid constrictor. Clinical signs resolved after surgery. Follow-up 3 months postoperatively revealed normal pre- and postprandial serum bile acids (2 µmol/l and 3 µmol/l, respectively) with repeat CT identifying evidence of shunt attenuation. The cat continued to be healthy and free of clinical signs 12 months postoperatively. Relevance and novel information Mesentericorenocaval portosystemic shunt morphology has not been previously reported in the cat and should be considered as a differential diagnosis for cats presenting with peripartum onset of malaise, ptyalism or ataxia.

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“…The litter was identified using previously published methods. 1,4,5 Both parents were unaffected, but the 6 offspring included 1 affected male, 2 affected females, and 3 unaffected males. All 3 affected dogs were treated successfully by surgical attenuation of the shunt 6 at an age of 3-4 months.…”

Section: Parentsmentioning

confidence: 99%

“…C ongenital portosystemic shunts are diagnosed mainly in purebred dogs and found with high incidence in Cairn Terriers, 1 Yorkshire Terriers, 2 Dachshounds, Miniature Schnauzers, Golden Retrievers, and Labrador Retrievers. 3 Intrahepatic portosystemic shunt (IHPSS) in Irish Wolfhounds has been presumed to be hereditary, based on its overrepresentation in the breed and the familial distribution.…”

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Evidence of Inheritance of Intrahepatic Portosystemic Shunts in Irish Wolfhounds

Steenbeek¹,

Leegwater²,

Sluijs³

et al. 2009

Veterinary Internal Medicne

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Background: The etiogenesis of congenital portosystemic shunt in dogs is not understood. In Irish Wolfhounds, intrahepatic portosystemic shunt (IHPSS) is thought to be hereditary, but the mode of inheritance is unknown.Objectives: To document the genetic background and investigate the potential mode of inheritance of IHPSS in Irish Wolfhounds.Animals: Three mature, privately owned, affected siblings and their progeny produced in 2 litters. Methods: Prospective, observational study. Two test matings of 1 affected sire with 2 of his affected sisters were used to determine the inheritance pattern. Affection status was determined by measuring venous blood ammonia concentrations, detection of the shunt by ultrasonography and confirmation during surgical attenuation of the intrahepatic shunting vessel.Results: In 1 litter of 5 pups all had an IHPSS. In the other litter 5 of 11 pups were affected. Both left-and right-sided shunts occurred in both litters. No sex predisposition was evident among affected dogs.Conclusions and Clinical Importance: Our results show that IHPSS in Irish Wolfhounds is a familial disorder that is likely genetic. It is unlikely that the mode of inheritance is monogenic. A digenic, triallelic trait could explain the observed occurrence of IHPSS but other modes of inheritance cannot be excluded.

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Inherited Congenital Extrahepatic Portosystemic Shunts in Cairn Terriers

Cited by 22 publications

References 0 publications

Hepatic Encephalopathy in Two Goat Kids with Common Paternity

Hepatic Encephalopathy in Two Goat Kids with Common Paternity

Peripartum clinical manifestations of a mesentericorenocaval shunt in a Burmese cat

Evidence of Inheritance of Intrahepatic Portosystemic Shunts in Irish Wolfhounds

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