Ductal remnants in extrahepatic biliary atresia: A histopathologic study with clinical correlation

Chandra, Roma; Altman, R. Peter

doi:10.1016/s0022-3476(78)80495-8

Cited by 98 publications

(34 citation statements)

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“…3,[36][37][38][39][40][41][42][43][44][45] In most cases, the biliary remnant from patients with BA has marked inflammation of the bile duct, extended segments in which there is damage to and necrosis of bile duct epithelium, and at least 1 segment in which the bile duct is obliterated. We did not see inflammation, necrosis, or obliteration of the extrahepatic biliary system in the inv mouse.…”

Section: Discussionmentioning

confidence: 99%

“…3,[36][37][38][39][40][41][42][43][44][45] In fact, Landing hypothesized a number of years ago that BA, choledochal cyst, and idiopathic neonatal hepatitis represented different manifestations of a single disease process termed ''infantile obstructive cholangiopathy.'' 46 In this hypothesis, injury could occur to a varying degree in the liver alone, bile duct alone, or both; injury to the bile duct epithelium could result in duct obliteration or weakening of the duct wall with subsequent dilatation and cyst formation or both.…”

Section: Discussionmentioning

confidence: 99%

“…However, multiple accessory duct structures have also been described in the biliary remnant taken from human infants with BA. [36][37][38][39][40][41][42][43] Again, the comparison of biliary remnants from inv mice to human infants with BA is not ideal, because the former are probably seen much earlier in the evolution of the lesion and without any inflammatory changes, and the latter are almost always at end-stage with marked inflammation and obliteration. There are rare patients with other anomalies of the extrahepatic bile ducts, including stenosis and duplication, [53][54][55][56][57][58][59] and even several patients with such anomalies in association with situs inversus.…”

Section: Discussionmentioning

confidence: 99%

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Anomalous development of the hepatobiliary system in theinv mouse

et al. 1999

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Extrahepatic biliary atresia (BA) is a devastating disease of the neonate in which the hepatic and/or common bile duct is obliterated or interrupted. Infants and children with this diagnosis constitute 50% to 60% of the pediatric population that undergoes orthotopic liver transplantation. However, there is still very little known about the etiology and pathogenesis of BA. Several recent studies have demonstrated that anomalies of situs determination are more commonly associated with BA than previously recognized. In this study, we examined the pathogenesis of jaundice in the inv mouse, a transgenic mouse in which a recessive deletion of the inversin gene results in situs inversus and jaundice. The results show that these mice have cholestasis with conjugated hyperbilirubinemia, failure to excrete technetium-labeled mebrofenin from the liver into the small intestine, lack of continuity between the extrahepatic biliary tree and the small intestine as demonstrated by Trypan blue cholangiography, and a liver histological picture indicative of extrahepatic biliary obstruction with negligible inflammation/necrosis within the hepatic parenchyma. Lectin histochemical staining of biliary epithelial cells in serial sections suggests the presence of several different anomalies in the architecture of the extrahepatic biliary system. These results suggest that the inversin gene plays an essential role in the morphogenesis of the hepatobiliary system and raise the possibility that alterations in the human orthologue of inversin account for some of the cases of BA in which there are also anomalies of situs determination. (HEPATOLOGY 1999;30:372-378.)

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Anomalous development of the hepatobiliary system in theinv mouse

et al. 1999

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“…Abnormalities in each segment will depend on the type of biliary atresia and extent of extrabiliary obliteration, which can be included in the surgical pathology report. Early studies by Chandra and Altman, 98 analyzing bile duct remnants at the level of the portal plate, have claimed significant correlation between residual bile duct size greater than 150 mm and successful post-Kasai bile drainage and overall patient outcome. While some subsequent studies 18,94,99 have confirmed a more favorable outcome in cases showing bile duct sizes greater than 150 to 200 mm at the portal plate, others 100,101 have found no significant correlation.…”

Section: Kasai Specimenmentioning

confidence: 99%

Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management

Moreira¹,

Cabral²,

Cowles³

et al. 2012

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Biliary atresia is an inflammatory cholangiopathy of infancy that results in progressive fibrosis and obliteration of bile ducts and represents the main indication for liver transplant in young children. In spite of extensive investigation, its etiology has remained poorly understood. Timely surgical intervention (Kasai procedure) may result in significant benefit to these patients and represents the final goal of an accurate diagnostic evaluation. Objective.—To present an overview of biliary atresia, including clinical and surgical approaches to this disease, with emphasis on the histopathologic evaluation. Data Sources.—Review of relevant literature indexed in PubMed (US National Library of Medicine). Conclusion.—A well-coordinated multidisciplinary approach is required in the assessment of suspected cases of biliary atresia. Pathologic examination of biopsy specimens is an integral part of the diagnostic algorithm and, therefore, plays a pivotal role in the diagnostic evaluation of this disease.

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“…In infants with "noncorrectable" forms, portal dissection is undertaken with placement of a Roux-en-Y anastomosis of jejunum to the undersurface of the liver capsule (2). The magnitude of postoperative bile flow and resolution of jaundice are dependent on age at operation and ductal remnant size (20,21). Most patients have failure of establishment of postoperative bile flow and pursue a course of progressive liver failure eventuating in death, or transplantation in the first 2 y of life.…”

Section: -Oh Vitamin Dmentioning

confidence: 99%

Bone Disease in Chronic Childhood Cholestasis II. Better Absorption of 25-OH Vitamin D than Vitamin D in Extrahepatic Biliary Atresia

1990

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ABSTRACT. Infants with extrahepatic biliary atresia (EHBA) commonly develop rickets in infancy, whereas long-term survivors with EHBA commonly develop osteopenia with increasing age. We evaluated baseline vitamin D (Dz and D3), 25-OH vitamin D2 and D,, 1,25(0H)2 vitamin D, bone mineral content, and vitamin D2 and 25-OH vitamin D3 absorption in six infants and children (age 4-22 mo) with EHBA whose portoenterostomy failed to produce bile flow (group 1) and five infants and children (age 10112 to 8-4/12 y) with EHBA whose portoenterostomy repair led to good postoperative bile flow (group 2). Baseline serum vitamin Dz and DJ were undetectable in all subjects in group 1 despite supplements of 2500-5000 IU/ day, whereas all group 2 subjects given supplements (doses 400-5000 IU/d) had measurable levels. Baseline serum 25-OH vitamin D was less than 15 ng/mL in five of six (three with rickets) in group 1, whereas only one in group 2 had concentrations less than 15 ng/mL. A significantly blunted rise of vitamin D2 above baseline and reduced area under the absorption curve after 1000 IU/kg vitamin DZ were found in group 1 patients compared to group 2 (both p < 0.01), and five pediatric controls (both p < 0.01). The peak change and area under the absorption curve for serum 25-OH vitamin D3 from baseline after 10 pg/kg 25-OH vitamin D3 were significantly reduced for group 1 (both at least p < 0.05) and group 2 compared to controls (both p < 0.05). Malabsorption of supplemental doses of vitamin D compromises their efficacy to prevent rickets in infants with EHBA with poor bile flow after portoenterostomy. Despite severe malabsorption of vitamin D, we suggest that oral 25-OH vitamin D may be absorbed sufficiently to prevent rickets. (Pediatr Res 27:26-31, 1990) Abbreviations EHBA, extrahepatic biliary atresia PTH, parathyroid hormone EHBA accounts for approximately 25% of cases of neonatal cholestasis (1). Operable forms with atresia limited to the distal portions of the biliary tract account for 10-15% of the cases. In

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Ductal remnants in extrahepatic biliary atresia: A histopathologic study with clinical correlation

Cited by 98 publications

References 5 publications

Anomalous development of the hepatobiliary system in theinv mouse

Anomalous development of the hepatobiliary system in theinv mouse

Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management

Bone Disease in Chronic Childhood Cholestasis II. Better Absorption of 25-OH Vitamin D than Vitamin D in Extrahepatic Biliary Atresia

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