A morphological study of the removed livers from patients receiving living donor liver transplantation for adult biliary atresia

Matsuura, Toshiharu; Kohashi, Kenichi; Yanagi, Yusuke; Saeki, Isamu; Hayashida, Miyeko; Aishima, Shinichi; Oda, Yoshinao; Taguchi, Tomoaki

doi:10.1007/s00383-012-3183-6

Cited by 15 publications

(12 citation statements)

References 12 publications

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“…Explants, especially in older children and adults, may show two distinct areas, central hypertrophic segments and atrophic lateral segments. The segments of the liver where bile drainage is well maintained may show regeneration, while other segments without sufficient bile drainage show atrophy [74]. Portal areas demonstrate prominent arterioles and attenuated veins.…”

Section: Liver Explantsmentioning

confidence: 99%

Biliary Atresia: pathology, Etiology and Pathogenesis

Vij¹,

Rela

2020

Future Sci. OA

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Biliary atresia is a progressive fibrosing obstructive cholangiopathy of the intrahepatic and extrahepatic biliary system, resulting in obstruction of bile flow and neonatal jaundice. Histopathological findings in liver biopsies include the expansion of the portal tracts, with edematous fibroplasia and bile ductular proliferation, with bile plugs in duct lumen. Lobular morphological features may include variable multinucleate giant cells, bilirubinostasis and hemopoiesis. The etiopathogenesis of biliary atresia is multifactorial and multiple pathomechanisms have been proposed. Experimental and clinical studies have suggested that viral infection initiates biliary epithelium destruction and release of antigens that trigger a Th1 immune response, which leads to further injury of the bile duct, resulting in inflammation and obstructive scarring of the biliary tree. It has also been postulated that biliary atresia is caused by a defect in the normal remodelling process. Genetic predisposition has also been proposed as a factor for the development of biliary atresia.

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Section: Liver Explantsmentioning

confidence: 99%

Biliary Atresia: pathology, Etiology and Pathogenesis

Vij¹,

Rela

2020

Future Sci. OA

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“…BA is the most common indication for a liver transplant at a paediatric liver transplant centre and has good outcomes which are comparable to the entire cohort [44][45][46]. Common histological findings include established cirrhosis with a central hypertrophic area and relative atrophy of the periphery of the liver [47]. In the hypertrophied area of the liver, the liver architecture can be near normal with bile ducts whereas the atrophic area usually shows severe fibrosis and only a few bile ducts remain [48].…”

Section: Biliary Atresiamentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

Gupta

Samyn

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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“…25 Explanted BA livers at the time of liver transplant often present with macroregenerative nodules in the central lobes while left lateral segments are degenerative and atrophic. 9 The segments of the liver where bile flow is well maintained are generally thought to culminate in regeneration, while other segments without sufficient bile drainage are degenerating with toxic bile acid in cholestatic condition. However, the mechanism of ongoing cirrhosis could be also secondary to immunemediated processes associated with maternal mircochimerism.…”

Section: The Secondary Insult By Maternal Cells As Effector Lymphocytmentioning

confidence: 99%

“…The liver explanted at the time of liver transplant macroscopically appears that fibrosis is heterogenous with a centrally located regenerative nodule which is an only functioning part with good bile drainage, while the other presumably undrained peripheral segments culminate in atrophic fibrous nodules. 9 However, even unicteric patients with abundant bile ducts in the perihilar nodule often require liver transplant with complications from portal hyptertension. 10 Therefore, it is of interest to investigate the mechanism of heterogenous fibrosis with ongoing portal hypertension in BA.…”

Section: Introductionmentioning

confidence: 99%