Pathogenesis of Extrahepatic Bile Duct Atresia (EHBA)

Schweizer, Paul; Petersen, Michael Mørk; Drews, Ulrich; Bachmann, Christian; Schweizer, Michael T.

doi:10.1055/s-2005-865788

Cited by 5 publications

(6 citation statements)

References 23 publications

(28 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Tubular structures bounded by four or more hepatic cells with microvilli have been considered as the earliest trace of bile canaliculi, and some authors consider them to be developing cholangioles and small bile ducts [17]. We found that CK-positive cells, endodermal hepatoblast precursor cells, appeared first in periportal hepatocytes around the lobules, and not, as Schweizer described, around the branch of portal vein [16]. Bile ductular cell proliferation is commonly seen in hepatobiliary diseases and is thought to originate from a proliferation of preexisting interlobular bile ducts and ductules or from stem cell transformation of the periportal hepatocytes [10,14,19,21,22], and this was also seen in our BA, ICC and CON groups.…”

supporting

confidence: 47%

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

2007

View full text Add to dashboard Cite

show abstract

supporting

confidence: 47%

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

2007

View full text Add to dashboard Cite

show abstract

“…The keys to understanding the pathogenesis of BA lie on the knowledge of the response of the bile ducts, as well as of the liver parenchyma to a total extrahepatic bile duct obstruction (9). BDL in rats leads to ductular proliferation and portal fibrosis similar to the pathological findings of BA.…”

mentioning

confidence: 99%

Bile duct ligation in neonatal rats: Is it a valid experimental model for biliary atresia studies?

Gibelli

Tannuri

Mello

et al. 2009

Pediatric Transplantation

View full text Add to dashboard Cite

BA is the most important disease requiring liver transplantation in children. Common BDL in rats is a classic experimental model to study biliary obstruction. The response of the neonatal animal to BDL has yet to be completely understood and few reports have focused on the behavioral differences of the liver between neonatal and adult animals. Ninety newborn Wistar rats aged six days, weighing 8.0-13.9 g, and 90 adult Wistar rats weighing 199.7-357.0 g, were submitted to BDL. After surgery, they were randomly divided and killed on the 3rd, 5th, 7th, 14th, 21st and 28th day post-BDL. Hepatic biopsies were obtained and the following were measured: (i) semiquantification of the bile ductule proliferation and inflammatory infiltrate by HE stain, (ii) quantification of portal and periportal fibrosis with the Sirius-red stain. Although the initial response of ductule proliferation and inflammatory infiltrate were less intense in the newborn animal, the portal and periportal fibrosis were higher when compared with adult animals (p < 0.0491). These findings may contribute to the understanding of the pathophysiology of BA.

show abstract

“…62 ,The variety of clinical presentations support the notion that the proposed mechanisms are not mutually exclusive but may play roles individually or in combination in certain patients .…”

Section: Etiologymentioning

confidence: 79%

Revival of Landing's Concept of Infantile Obstructive Cholangiopathy A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon!

Sharma

Gupta

2014

J. paediatr. surg. Bangladesh

View full text Add to dashboard Cite

:The concept of infantile obstructive cholangiopathy started in 1974 by Landing and remained till early 1980s. However, the concept has not been used much since then as nothing new has developed over the last 40 years sufficient enough to change the course of the disease or identify its etiology. This review article is an attempt to consolidate evidence on review of literature pointing towards a common etiopathological process leading to the spectrum of anomalies constituting neonatal cholestasis. The importance of early referral of any case with neonatal jaundice in a full term neonate, preferably at 2 weeks age is highlighted. This will help to identify and treat more cases within the correctable range and prevent the disease from progressing to a life threatening situation especially in developing countries where the resources for liver transplantation are meagre. Introduction:Newborn babies are at increased propensity for developing hyperbilirubinemia due to increased bilirubin production or its decreased excretion. The increased production is attributed to a greater red cell mass per kg as compared to adults and a shorter life span of red blood cells. The decreased excretion is attributed to defective uptake of bilirubin due to hepatic immaturity and decreased ligandin, defective conjugation due to decreased uridine diphospho glucuronyl transferase (UDPG-T) activity, decreased hepatic excretion of bilirubin and increased enterohepatic circulation due to higher levels of glucuronidase Review Article REVIVAL OF LANDING'S CONCEPT OF INFANTILE Physiological Jaundice:The physiologic jaundice seen in newborn babies appears on the third day of life and usually lasts till the tenth day in full term babies. It appears slightly earlier in preterm babies and may last upto two weeks. However, the serum bilirubin levels do not exceed 12 mg % in full term babies and 15 mg % in preterm babies. The normal range of total and conjugated bilirubin are <1 mg/dl and <0.25 mg/dl respectively, although jaundice can only be appreciated in newborn babies only when the total bilirubin is greater than 5 mg/dL Physiological jaundice may be accentuated by immaturity, birth asphyxia, acidosis, hypothermia, hypoglycemia, septicemia, blood group incompatibilities, drugs like salicylates, gentamycin, sulpha drugs, cephalhematoma, polycythemia, hypothyroidism intrauterine infections and breast milk jaundice. At high levels, unconjugated bilirubin can cross the blood-brain barrier and cause brain injury (kernicterus). The usual therapy for unconjugated jaundice is phototherapy and occasionally exchange transfusion. Neonatal Cholestasis :Infants with the persistence of jaundice beyond the physiologic limits, in the absence of an apparent medical cause, need to be investigated without much delay. The causes for Conjugated Hyperbilirubinemia (neonatal cholestasis) include obstructive and nonobstructive disorders. These are broadly called extrahepatic biliary obstruction and neonatal hepatitis. Cholestasis thus results from either to s...

show abstract

Pathogenesis of Extrahepatic Bile Duct Atresia (EHBA)

Abstract: There is no finding in Biliary Atresia which cannot be completely explained as the result of an intrinsic developmental error, probably due to disturbances or interruption of epithelium/mesoderm interaction during embryogenesis.

Cited by 5 publications

References 23 publications

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

Bile duct ligation in neonatal rats: Is it a valid experimental model for biliary atresia studies?

Revival of Landing's Concept of Infantile Obstructive Cholangiopathy A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon!

Contact Info

Product

Resources

About

Pathogenesis of Extrahepatic Bile Duct Atresia (EHBA)

Abstract: There is no finding in Biliary Atresia which cannot be completely explained as the result of an intrinsic developmental error, probably due to disturbances or interruption of epithelium/mesoderm interaction during embryogenesis.

Cited by 5 publications

References 23 publications

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

Hepatic Fibrosis with Choledochal Cyst in Infants and Children - An Immunohistochemical Assessment

Bile duct ligation in neonatal rats: Is it a valid experimental model for biliary atresia studies?

Revival of Landing's Concept of Infantile Obstructive Cholangiopathy  A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon!

Contact Info

Product

Resources

About

Revival of Landing's Concept of Infantile Obstructive Cholangiopathy A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon!