Extrahepatic Biliary Atresia in a 4-Week-Old Pug

Thiel, C.; Steinbach, Sarah; Schmidt, Martin J.; Amort, K.; Zahn, Nanette; Kramer, Martin; Moritz, Andreas

doi:10.1111/j.1532-950x.2014.12210.x

Cited by 3 publications

(1 citation statement)

References 32 publications

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“…This highlights the role of this organ in potential causes of the disease, including toxins, viruses, and genetic mutations. Animals with a gallbladder - such as sheep 62 , cows 62 , dogs 63 , 64 , monkeys 65 , and cats 66 have all been shown to develop symptoms similar to those seen in human BA, supporting the present hypothesis that the presence of a gallbladder may contribute to the condition’s onset. When examining the causes of biliary atresia in humans, it may be possible in the near future to consider the health of gallbladder wall progenitors as one of multiple contributing factors.…”

Section: Discussionsupporting

confidence: 74%

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia

Miyazaki,

Takami,

Uemura

et al. 2024

Commun Med

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Background Biliary atresia (BA) is an intractable disease of unknown cause that develops in the neonatal period. It causes jaundice and liver damage due to the destruction of extrahepatic biliary tracts,. We have found that heterozygous knockout mice of the SRY related HMG-box 17 (Sox17) gene, a master regulator of stem/progenitor cells in the gallbladder wall, exhibit a condition like BA. However, the precise contribution of hypoplastic gallbladder wall to the pathogenesis of hepatobiliary disease in Sox17 heterozygous embryos and human BA remains unclear. Methods We employed cholangiography and histological analyses in the mouse BA model. Furthermore, we conducted a retrospective analysis of human BA. Results We show that gallbladder wall hypoplasia causes abnormal multiple connections between the hilar hepatic bile ducts and the gallbladder-cystic duct in Sox17 heterozygous embryos. These multiple hilar extrahepatic ducts fuse with the developing intrahepatic duct walls and pull them out of the liver parenchyma, resulting in abnormal intrahepatic duct network and severe cholestasis. In human BA with gallbladder wall hypoplasia (i.e., abnormally reduced expression of SOX17), we also identify a strong association between reduced gallbladder width (a morphometric parameter indicating gallbladder wall hypoplasia) and severe liver injury at the time of the Kasai surgery, like the Sox17-mutant mouse model. Conclusions Together with the close correlation between gallbladder wall hypoplasia and liver damage in both mouse and human cases, these findings provide an insight into the critical role of SOX17-positive gallbladder walls in establishing functional bile duct networks in the hepatic hilus of neonates.

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Section: Discussionsupporting

confidence: 74%

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia

Miyazaki,

Takami,

Uemura

et al. 2024

Commun Med

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Translational Research in Biliary Atresia: News from Mice and Men

Madadi-Sanjani

Petersen

2019

Eur J Pediatr Surg

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Biliary atresia (BA) is a fibro-obliterative cholangiopathy of unknown etiology. While Kasai portoenterostomy achieves temporary biliary drainage in some cases, BA remains the most common indication for liver transplantation during childhood. During the last few decades, observations on BA, like cholestatic diseases in animals and the introduction of different animal models for BA, have not achieved the anticipated results, and we are still not able to translate the basic research to the patient's bedside. This article presents a review of the literature on available BA animal models and gives a glimpse of future developments.

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Canine hepatobiliary anatomy, physiology and congenital disorders

Jaffey

2021

J of Small Animal Practice

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The biliary system is an integral component of normal physiologic homeostasis and essential for survival. It acts as a conduit for the removal of detoxified and catabolised compounds as well as aids in fat digestion and absorption. Derangements in this system can have dangerous sequela that are associated with varying degrees of morbidity and mortality. Moreover, abnormalities in development of the biliary system can have varied and unpredictable changes on function and long‐term outcome. The aims of this article were to review canine hepatobiliary anatomy, physiology and cholestasis as well as summarise congenital biliary disorders including human corollaries.

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Extrahepatic Biliary Atresia in a 4-Week-Old Pug

Cited by 3 publications

References 32 publications

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia

Impact of gallbladder hypoplasia on hilar hepatic ducts in biliary atresia

Translational Research in Biliary Atresia: News from Mice and Men

Canine hepatobiliary anatomy, physiology and congenital disorders

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