Aetiology of biliary atresia: what is actually known?

Claus Petersen; Mark Davenport

doi:10.1186/1750-1172-8-128

Aetiology of biliary atresia: what is actually known?

Claus Petersen^*, Mark Davenport

^*Corresponding author for this work

Hannover Medical School

Research output: Contribution to journal › Literature review › peer-review

95 Citations (Scopus)

Abstract

Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.

Original language	English
Article number	128
Number of pages	13
Journal	Orphanet Journal of Rare Diseases
Volume	8
DOIs	https://doi.org/10.1186/1750-1172-8-128
Publication status	Published - 29 Aug 2013

Keywords

Biliary atresia
Neonatal cholestasis
Animal models
Etiology
Neonatal immunology
REGULATORY T-CELLS
SPLENIC MALFORMATION SYNDROME
REOVIRUS TYPE-3 INFECTION
BILE-DUCT INJURY
MURINE MODEL
EPITHELIAL INJURY
DIFFERENTIAL EXPRESSION
INFLAMMATORY RESPONSE
HEPATOTROPIC VIRUSES
NEONATAL CHOLESTASIS

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@article{e3beb2d36ba546ea9e1ed7d8e6b92a89,

title = "Aetiology of biliary atresia: what is actually known?",

abstract = "Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.",

keywords = "Biliary atresia, Neonatal cholestasis, Animal models, Etiology, Neonatal immunology, REGULATORY T-CELLS, SPLENIC MALFORMATION SYNDROME, REOVIRUS TYPE-3 INFECTION, BILE-DUCT INJURY, MURINE MODEL, EPITHELIAL INJURY, DIFFERENTIAL EXPRESSION, INFLAMMATORY RESPONSE, HEPATOTROPIC VIRUSES, NEONATAL CHOLESTASIS",

author = "Claus Petersen and Mark Davenport",

year = "2013",

month = aug,

day = "29",

doi = "10.1186/1750-1172-8-128",

language = "English",

volume = "8",

journal = "Orphanet Journal of Rare Diseases",

issn = "1750-1172",

publisher = "BioMed Central",

}

TY - JOUR

T1 - Aetiology of biliary atresia

T2 - what is actually known?

AU - Petersen, Claus

AU - Davenport, Mark

PY - 2013/8/29

Y1 - 2013/8/29

N2 - Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.

AB - Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.

KW - Biliary atresia

KW - Neonatal cholestasis

KW - Animal models

KW - Etiology

KW - Neonatal immunology

KW - REGULATORY T-CELLS

KW - SPLENIC MALFORMATION SYNDROME

KW - REOVIRUS TYPE-3 INFECTION

KW - BILE-DUCT INJURY

KW - MURINE MODEL

KW - EPITHELIAL INJURY

KW - DIFFERENTIAL EXPRESSION

KW - INFLAMMATORY RESPONSE

KW - HEPATOTROPIC VIRUSES

KW - NEONATAL CHOLESTASIS

U2 - 10.1186/1750-1172-8-128

DO - 10.1186/1750-1172-8-128

M3 - Literature review

SN - 1750-1172

VL - 8

JO - Orphanet Journal of Rare Diseases

JF - Orphanet Journal of Rare Diseases

M1 - 128

ER -

Aetiology of biliary atresia: what is actually known?

Abstract

Keywords

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