Diseases of the Biliary Tree in Infancy and Childhood

Russo, Pierre; Rand, Elizabeth B.; Haber, Barbara

doi:10.1007/978-1-4419-9066-2_9

Cited by 4 publications

(1 citation statement)

References 286 publications

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“…The etiology of BA is unknown, but it is accepted that BA is a common clinical phenotype that results from different prenatal and/or perinatal insults to the biliary tree of susceptible subjects [1][2][3]. Two forms of BA are generally recognized, although more may exist.…”

Section: Introductionmentioning

confidence: 99%

Does “Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

et al. 2005

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We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.

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Section: Introductionmentioning

confidence: 99%

Does “Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

et al. 2005

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Fehlbildungen und Lebererkrankungen im Kindesalter

Tischoff

2020

Pathologie

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Cholestase-assoziierte Lebererkrankungen im Neugeborenen- und Säuglingsalter

Knöpfle

Adam

2008

Pathologe

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Cholestasis in neonates and infants frequently confronts pediatricians and pathologists with diagnostic problems. A specific feature of the liver in neonates is the ability to react to different causative factors with a non-specific hepatitis-like picture, the so-called neonatal hepatitis. A diagnostic discrimination of the various diseases is histologically only possible with close attention to typical morphologic features. Thus, extrahepatic biliary obstructions, such as atresia or stenosis of the hepatic duct or choledochal cysts present with portal bile duct proliferation and signs of bile retention in the neoducts. In Alagille syndrome (arteriohepatic dysplasia), however, paucity of intrahepatic bile ducts is an important diagnostic feature. Metabolic disorders, such as fructosemia and galactosemia are additionally associated with steatosis. Knowledge of the clinical course and laboratory and imaging data are necessary to make the definitive diagnosis in synopsis with the morphologic findings and requires a close co-operation between the pediatrician and the pathologist.

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Diseases of the Biliary Tree in Infancy and Childhood

Cited by 4 publications

References 286 publications

Does “Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

Does “Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

Fehlbildungen und Lebererkrankungen im Kindesalter

Cholestase-assoziierte Lebererkrankungen im Neugeborenen- und Säuglingsalter

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