Developmental and Inherited Liver Disease

Quaglia, Alberto; Roberts, Eve A.; Torbenson, Michael

doi:10.1016/b978-0-7020-6697-9.00003-0

Cited by 8 publications

(20 citation statements)

References 2,140 publications

(2,299 reference statements)

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“…The periportal suppurative hepatitis found in one case might have been derived from a suppurative condition in the digestive tract. In addition, a cyst lined by a single layer of cuboidal epithelial cells was diagnosed as a solitary bile duct cyst [12].…”

Section: Discussionmentioning

confidence: 99%

Gross and histological lesions in the livers of sika deer with particular emphasis on fascioliasis

Matsuda

Kogame

Niki

et al. 2020

The Journal of Veterinary Medical Science

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We performed gross and histological examinations of the livers of sika deer (Cervus nippon yesoensis) in Hokkaido, Japan. Out of 1,381 deer slaughtered for venison production, thickening and dilation of the large intrahepatic bile ducts and Fasciola flukes in the duct lumens were detected in 621 deer (45.0%). Furthermore, 107 non-bile lesions (75 intrahepatic and 32 capsular lesions) were detected during gross examinations. Histologically, the bile duct lesions included chronic proliferative cholangitis, papillary hyperplasia, goblet cell and pyloric gland metaplasia, and periductal fibrosis. Many of the intrahepatic non-bile duct lesions (53/75, 71%) were considered to be Fasciola fluke migration-associated lesions, including two lesion types: necrosis, hemorrhage, and eosinophilic granuloma formation (29 lesions), and lymphoid tissue formation (24 lesions). Lymphoid tissue formation was considered to result from the persistent immune responses against dead Fasciola flukes. An epidermoid liver cyst was found incidentally, which has not been reported in the veterinary literature. In summary, this study demonstrated the predominance of fascioliasis-associated lesions in sika deer livers. The gross and histological lesions caused by Fasciola flukes in sika deer were similar to fascioliasis in other animals. Moreover, we described lymphoid tissue formation as a fascioliasis-associated lesion for the first time. The fact that bile duct lesions (45.0%) had a markedly higher prevalence than fascioliasis-associated parenchymal lesions (53/1,381, 3.8%) indicated that sika deer are a permissive host for fascioliasis. Our results provide information that will aid pathological examinations of sika deer.

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Section: Discussionmentioning

confidence: 99%

Gross and histological lesions in the livers of sika deer with particular emphasis on fascioliasis

Matsuda

Kogame

Niki

et al. 2020

The Journal of Veterinary Medical Science

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“…Large cell change is also reported. HCC is usually well or moderately differentiated in tyrosinemia[ 10 ]. The long-term outcome for LT recipients with HT-1 is excellent, and ranges from 85%-100% across series[ 11 ].…”

Section: Group Amentioning

confidence: 99%

“…It is a common metabolic disorder caused by the defect in the enzyme galactose 1-phosphate uridyl transferase (GALT). The incidence is 1 in 45000 births[ 10 ]. The metabolites galactitol and galactose-1-phosphate are hepatotoxic and infants present with ALF; nevertheless, there is always an underlying liver scarring[ 18 ].…”

Section: Group Amentioning

confidence: 99%

“…The diagnosis was classically suggested by the detection of urinary reducing sugars (nonglucose), which have no sensitivity or specificity, and may be positive with any cause of liver failure. GALT activity can be measured in erythrocytes, however the test must be done before the child receives blood transfusion[ 10 ]. Genetic assay is confirmatory.…”

Section: Group Amentioning

confidence: 99%

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Pediatric metabolic liver diseases: Evolving role of liver transplantation

Menon¹,

Vij²,

Sachan³

et al. 2021

WJT

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Metabolic liver diseases (MLD) are the second most common indication for liver transplantation (LT) in children. This is based on the fact that the majority of enzymes involved in various metabolic pathways are present within the liver and LT can cure or at least control the disease manifestation. LT is also performed in metabolic disorders for end-stage liver disease, its sequelae including hepatocellular cancer. It is also performed for preventing metabolic crisis’, arresting progression of neurological dysfunction with a potential to reverse symptoms in some cases and for preventing damage to end organs like kidneys as in the case of primary hyperoxalosis and methyl malonic acidemia. Pathological findings in explant liver with patients with metabolic disease include unremarkable liver to steatosis, cholestasis, inflammation, variable amount of fibrosis, and cirrhosis. The outcome of LT in metabolic disorders is excellent except for patients with mitochondrial disorders where significant extrahepatic involvement leads to poor outcomes and hence considered a contraindication for LT. A major advantage of LT is that in the post-operative period most patients can discontinue the special formula which they were having prior to the transplant and this increases their well-being and improves growth parameters. Auxiliary partial orthotopic LT has been described for patients with noncirrhotic MLD where a segmental graft is implanted in an orthotopic position after partial resection of the native liver. The retained native liver can be the potential target for future gene therapy when it becomes a clinical reality.

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“…Celgosivir, an α‐glucosidase inhibitor, has shown broad antiviral activity in vitro and hence, entered clinical development . Although α‐glucosidase appears to be a promising target, its inhibition is likely to affect processing of other indigenous proteins (serpins, polycystin 1/2, and fibrocystin) …”

Section: Host‐target Agentsmentioning

confidence: 99%

Evolution of efficacious pangenotypic hepatitis C virus therapies

Ashraf

Iman

Khalid

et al. 2018

Medicinal Research Reviews

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Hepatitis C compromises the quality of life of more than 350 million individuals worldwide. Over the last decade, therapeutic regimens for treating hepatitis C virus (HCV) infections have undergone rapid advancements. Initially, structure-based drug design was used to develop molecules that inhibit viral enzymes. Subsequently, establishment of cell-based replicon systems enabled investigations into various stages of HCV life cycle including its entry, replication, translation, and assembly, as well as role of host proteins. Collectively, these approaches have facilitated identification of important molecules that are deemed essential for HCV life cycle. The expanded set of putative virus and host-encoded targets has brought us one step closer to developing robust strategies for efficacious, pangenotypic, and well-tolerated medicines against HCV. Herein, we provide an overview of the development of various classes of virus and host-directed therapies that are currently in use along with others that are undergoing clinical evaluation.

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Developmental and Inherited Liver Disease

Cited by 8 publications

References 2,140 publications

Gross and histological lesions in the livers of sika deer with particular emphasis on fascioliasis

Gross and histological lesions in the livers of sika deer with particular emphasis on fascioliasis

Pediatric metabolic liver diseases: Evolving role of liver transplantation

Evolution of efficacious pangenotypic hepatitis C virus therapies

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