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Inherited liver shunts in dogs elucidate pathways regulating embryonic development and clinical disorders of the portal vein.

van Steenbeek FG, van den Bossche L, Leegwater PA, Rothuizen J - Mamm. Genome (2011)

Bottom Line: Both subtypes result in nearly complete bypass of the liver by the portal blood flow.The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis.Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.

View Article: PubMed Central - PubMed

Affiliation: Faculty of Veterinary Medicine, Utrecht University, Yalelaan 104, PO Box 80154, 3508 TD Utrecht, The Netherlands. f.g.vansteenbeek@uu.nl

ABSTRACT
Congenital disorders of the hepatic portal vasculature are rare in man but occur frequently in certain dog breeds. In dogs, there are two main subtypes: intrahepatic portosystemic shunts, which are considered to stem from defective closure of the embryonic ductus venosus, and extrahepatic shunts, which connect the splanchnic vascular system with the vena cava or vena azygos. Both subtypes result in nearly complete bypass of the liver by the portal blood flow. In both subtypes the development of the smaller branches of the portal vein tree in the liver is impaired and terminal branches delivering portal blood to the liver lobules are often lacking. The clinical signs are due to poor liver growth, development, and function. Patency of the ductus venosus seems to be a digenic trait in Irish wolfhounds, whereas Cairn terriers with extrahepatic portosystemic shunts display a more complex inheritance. The genes involved in these disorders cannot be identified with the sporadic human cases, but in dogs, the genome-wide study of the extrahepatic form is at an advanced stage. The canine disease may lead to the identification of novel genes and pathways cooperating in growth and development of the hepatic portal vein tree. The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis. Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.

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Pedigree of test matings with Cairn terriers with extrahepatic shunt. An affected female (#9) was used three times, with her unaffected father (#1), with her affected son (#21), and with an unrelated affected male (#10). Squares represent males, circles represent females. Solid symbols are affected dogs and open symbols are healthy dogs (reprinted with permission from John Wiley and Sons; van Straten et al. 2005)
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Fig3: Pedigree of test matings with Cairn terriers with extrahepatic shunt. An affected female (#9) was used three times, with her unaffected father (#1), with her affected son (#21), and with an unrelated affected male (#10). Squares represent males, circles represent females. Solid symbols are affected dogs and open symbols are healthy dogs (reprinted with permission from John Wiley and Sons; van Straten et al. 2005)

Mentions: A pedigree analysis of affected Cairn terriers born in The Netherlands between 1990 and 2001 was performed to study the genetics of EHPSS (van Straten et al. 2005). A total of 6,367 pups were screened for shunts by measuring venous ammonia concentrations at an age of 6 weeks. Prevalences of 1.9–5.9% in three breeding lines were significantly higher than the prevalence in the entire population (0.58%), indicating a hereditary basis for this disease. Three test matings were performed (Fig. 3). A successfully operated on female was mated with her unaffected father, an affected son, and an unrelated affected male. Four of the 19 pups (21%) born from these matings were affected, providing further evidence that EHPSS is a genetic disorder. The mode of inheritance is most likely polygenic and there seems to be no sex effect (van Straten et al. 2005).Fig. 3


Inherited liver shunts in dogs elucidate pathways regulating embryonic development and clinical disorders of the portal vein.

van Steenbeek FG, van den Bossche L, Leegwater PA, Rothuizen J - Mamm. Genome (2011)

Pedigree of test matings with Cairn terriers with extrahepatic shunt. An affected female (#9) was used three times, with her unaffected father (#1), with her affected son (#21), and with an unrelated affected male (#10). Squares represent males, circles represent females. Solid symbols are affected dogs and open symbols are healthy dogs (reprinted with permission from John Wiley and Sons; van Straten et al. 2005)
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3275728&req=5

Fig3: Pedigree of test matings with Cairn terriers with extrahepatic shunt. An affected female (#9) was used three times, with her unaffected father (#1), with her affected son (#21), and with an unrelated affected male (#10). Squares represent males, circles represent females. Solid symbols are affected dogs and open symbols are healthy dogs (reprinted with permission from John Wiley and Sons; van Straten et al. 2005)
Mentions: A pedigree analysis of affected Cairn terriers born in The Netherlands between 1990 and 2001 was performed to study the genetics of EHPSS (van Straten et al. 2005). A total of 6,367 pups were screened for shunts by measuring venous ammonia concentrations at an age of 6 weeks. Prevalences of 1.9–5.9% in three breeding lines were significantly higher than the prevalence in the entire population (0.58%), indicating a hereditary basis for this disease. Three test matings were performed (Fig. 3). A successfully operated on female was mated with her unaffected father, an affected son, and an unrelated affected male. Four of the 19 pups (21%) born from these matings were affected, providing further evidence that EHPSS is a genetic disorder. The mode of inheritance is most likely polygenic and there seems to be no sex effect (van Straten et al. 2005).Fig. 3

Bottom Line: Both subtypes result in nearly complete bypass of the liver by the portal blood flow.The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis.Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.

View Article: PubMed Central - PubMed

Affiliation: Faculty of Veterinary Medicine, Utrecht University, Yalelaan 104, PO Box 80154, 3508 TD Utrecht, The Netherlands. f.g.vansteenbeek@uu.nl

ABSTRACT
Congenital disorders of the hepatic portal vasculature are rare in man but occur frequently in certain dog breeds. In dogs, there are two main subtypes: intrahepatic portosystemic shunts, which are considered to stem from defective closure of the embryonic ductus venosus, and extrahepatic shunts, which connect the splanchnic vascular system with the vena cava or vena azygos. Both subtypes result in nearly complete bypass of the liver by the portal blood flow. In both subtypes the development of the smaller branches of the portal vein tree in the liver is impaired and terminal branches delivering portal blood to the liver lobules are often lacking. The clinical signs are due to poor liver growth, development, and function. Patency of the ductus venosus seems to be a digenic trait in Irish wolfhounds, whereas Cairn terriers with extrahepatic portosystemic shunts display a more complex inheritance. The genes involved in these disorders cannot be identified with the sporadic human cases, but in dogs, the genome-wide study of the extrahepatic form is at an advanced stage. The canine disease may lead to the identification of novel genes and pathways cooperating in growth and development of the hepatic portal vein tree. The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis. Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.

Show MeSH
Related in: MedlinePlus