The onecut transcription factor HNF6 is required for normal development of the biliary tract

Development ◽  
2002 ◽  
Vol 129 (8) ◽  
pp. 1819-1828 ◽  
Author(s):  
Frédéric Clotman ◽  
Vincent J. Lannoy ◽  
Michael Reber ◽  
Silvia Cereghini ◽  
David Cassiman ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Biliary Tract ◽  
Bile Ducts ◽  
Normal Development ◽  
Intrahepatic Bile Duct ◽  
Liver Development ◽  
Prenatal Period ◽  
Intrahepatic Bile Ducts ◽  
Extrahepatic Bile Ducts ◽  
Duct Development

During liver development, hepatoblasts differentiate into hepatocytes or biliary epithelial cells (BEC). The BEC delineate the intrahepatic and extrahepatic bile ducts, and the gallbladder. The transcription factors that control the development of the biliary tract are unknown. Previous work has shown that the onecut transcription factor HNF6 is expressed in hepatoblasts and in the gallbladder primordium. We now show that HNF6 is also expressed in the BEC of the developing intrahepatic bile ducts, and investigate its involvement in biliary tract development by analyzing the phenotype of Hnf6–/– mice. In these mice, the gallbladder was absent, the extrahepatic bile ducts were abnormal and the development of the intrahepatic bile ducts was perturbed in the prenatal period. The morphology of the intrahepatic bile ducts was identical to that seen in mice whose Hnf1β gene has been conditionally inactivated in the liver. HNF1β expression was downregulated in the intrahepatic bile ducts of Hnf6–/– mice during development. Furthermore, we found that HNF6 can stimulate the Hnf1β promoter. We conclude that HNF6 is essential for differentiation and morphogenesis of the biliary tract and that intrahepatic bile duct development is controlled by a HNF6→HNF1β cascade.

scholarly journals For the 30th anniversary of laparoscopic cholecystectomy: problems remain

2019 ◽  
Vol 100 (3) ◽  
pp. 537-541
Author(s):  
I V Fedorov ◽  
A N Chugunov ◽  
L E Slavin ◽  
D A Slavin ◽  
V I Fedorov
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Bile Ducts ◽  
Perioperative Complications ◽  
Open Cholecystectomy ◽  
Intrahepatic Bile Ducts ◽  
Negative Effect ◽  
Iatrogenic Damage ◽  
Extrahepatic Bile Ducts ◽  
Legal Costs

The review describes perioperative complications of laparoscopic cholecystectomy. Over the past 30 years, laparoscopy has become the «gold standard» for cholecystectomy and one of the most frequently performed procedures in abdominal surgery. Nevertheless, despite the advantages of the method, it has an «Achilles heel» - the frequency of iatrogenic damage to the extrahepatic bile ducts is 3-5 times higher than with an open cholecystectomy. This complication has a negative effect on the survival of patients after surgery, leads to deterioration in the quality of life and is a major source of legal costs in many countries. In general, the total range for any damage to the biliary tract during laparoscopic cholecystectomy is 0.32-0.52%, while the complication rate and mortality rate are 1.6-5.3% and 0.08-0.14%, respectively. Patients who have undergone a complete intersection of the hepaticoholedochus, become «bile cripples» for life. Recurrent cholangitis, strictures of anastomoses with a possible outcome in liver cirrhosis are quite likely in later periods after damage to the intrahepatic bile ducts. Technological efforts to improve the results of laparoscopic cholecystectomy reside. These include the routine use of intraoperative cholangiography, infrared fluorescent cholangiography, etc. Nevertheless, despite the growing number of methods designed to reduce these complications, evidence of their effectiveness remains limited. The most important factors ensuring the safety of laparoscopic cholecystectomy are recognized: understanding of anatomy, adequate exposure when using electrosurgery, psychological readiness to invite a senior colleague in time for help, the ability to recognize a situation that requires conversion and rejection of laparoscopy.

scholarly journals Hepatocholescintigraphy in diagnosis of functional state of biliodigestive anastomoses

2019 ◽  
Vol 24 (1) ◽  
pp. 53-60
Author(s):  
Yu. V. Kulezneva ◽  
L. V. Bondar ◽  
E. A. Vasina ◽  
M. B. Boboeva ◽  
P. M. Cholak ◽  
...  
Keyword(s):  
Biliary Tract ◽  
Functional State ◽  
Bile Flow ◽  
Bile Ducts ◽  
Diagnostic Value ◽  
Bowel Anastomosis ◽  
Biliodigestive Anastomosis ◽  
Intrahepatic Bile Ducts ◽  
Highly Effective ◽  
Flow Through

Aim. To identify diagnostic value of hepatocholescintigraphy for evaluation of functional state of biliodigestive anastomoses (BDA).Material and methods. 99mTc-IDA hepatocholescintigraphy was applied in 52 patients to evaluate function of biliodigestive anastomoses.Results. Scintigraphic signs of “bowel-anastomosis” and/or “BDA-intrahepatic bile ducts” reflux were revealed in 14 (27%) cases. Obstruction of biliodigestive anastomosis occurred in 3 (5.7%) patients. Normal bile flow through BDA was observed in 10 (19.2%) patients, impaired passage – in 21 (40.3%) cases. Tracer delay in intrahepatic bile ducts was noted in 21 (25.0%) cases, signs of adhesive process near deferent bowel – in 3 (3.8%) cases.Conclusion. Hepatocholescintigraphy is highly effective method to assess biliary tract in patients after Roux-en-Y biliodigestive anastomoses formation.

scholarly journals Dynamic three‐dimensional morphogenesis of intrahepatic bile ducts in mouse liver development

Hepatology ◽  
2015 ◽  
Vol 61 (3) ◽  
pp. 1003-1011 ◽  
Author(s):  
Yasuo Takashima ◽  
Maiko Terada ◽  
Masuyo Kawabata ◽  
Atsushi Suzuki
Keyword(s):  
Mouse Liver ◽  
Bile Ducts ◽  
Three Dimensional ◽  
Liver Development ◽  
Intrahepatic Bile Ducts

scholarly journals Recent advances in understanding cholangiocarcinoma

2020 ◽  
Vol 9 ◽  
Author(s):  
Pedro Luiz Serrano Uson Junior ◽  
Mansi Arora ◽  
James M Bogenberger ◽  
Mitesh J Borad
Keyword(s):  
Bile Ducts ◽  
Recent Progress ◽  
Advanced Disease ◽  
Personalized Therapy ◽  
Intrahepatic Bile Ducts ◽  
Recent Advances ◽  
Extrahepatic Bile Ducts ◽  
Molecular Landscape ◽  
Definition Of

The definition of cholangiocarcinoma (CCA) encompasses all tumors originating in the epithelium of the bile ducts, including the intrahepatic bile ducts (ICCA) and extrahepatic bile ducts (ECCA). The incidence of ICCA and ECCA has increased in the last few decades, and molecular advances in both entities have brought understanding of their differences and allowed treatment advances aimed at personalized therapy. In this review, we discuss recent progress in the molecular landscape of CCAs, emerging treatment biomarker-guided strategies, and future insights into the management of advanced disease.

scholarly journals A Rare Inflammatory Myofibroblastic Tumor of Pancreas in a Child With Cholestasis Presentation

2020 ◽  
Author(s):  
Sanaz Mehrabani ◽  
Javad Shokri-Shirvani
Keyword(s):  
Bile Ducts ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Eosinophil Infiltration ◽  
Unknown Etiology ◽  
Intrahepatic Bile Ducts ◽  
Chief Complaints ◽  
Head Of The Pancreas ◽  
Inflammatory Myofibroblastic Tumors ◽  
Extrahepatic Bile Ducts ◽  
The Common

Inflammatory myofibroblastic tumors (IMTs) are rare with unknown etiology. As pancreas involvement is rare in IMTs, here, we report a case of a girl with IMT, referred to our hospital. A 4-year-old girl presented with chief complaints of generalized itching and jaundice. Abdominopelvic computed tomography (CT) scans with contrast showed a homogeneous isodense mass lesion in the head of the pancreas with a compressive effect on the distal part of the common bile duct (CBD). Dilatation of intrahepatic bile ducts and CBD (8 mm) was observed. Magnetic resonance cholangiopancreatography (MRCP) examination showed a dilated gallbladder without stones and intrahepatic/extrahepatic bile ducts. The CBD was dilated, and a mass was found in the head of the pancreas. Immune-histochemical studies revealed spindle myofibroblastic tissues with lymphoplasmacytic and eosinophil infiltration. All of them were compatible with pancreatic IMTs. The surgery improved the symptoms. The IMTs of the pancreas can have symptoms like pancreatic cancer. The careful evaluation by imaging and pathology is recommended.

ATRESIA OF THE INTRAHEPATIC BILE DUCTS

PEDIATRICS ◽  
1951 ◽  
Vol 8 (5) ◽  
pp. 628-647
Author(s):  
EDWARD H. AHRENS ◽  
RUTH C. HARRIS ◽  
H. EDWARD MACMAHON
Keyword(s):  
Bile Ducts ◽  
Serum Lipids ◽  
Biliary Cirrhosis ◽  
Case Histories ◽  
Course Of Disease ◽  
Biliary Epithelium ◽  
Intrahepatic Bile Ducts ◽  
Extrahepatic Bile Ducts ◽  
Embryologic Development ◽  
Secondary Biliary Cirrhosis

Case histories of four patients with atresia of the intrahepatic interlobular bile ducts are reviewed. Three of the four also had anomalies of the extrahepatic bile ducts. All had secondary biliary cirrhosis. The course of disease was distinguished by a relatively long life span, all patients surviving more than three years and one remaining alive at five years. Generalized skin xanthomatosis occurred in all patients, together with a marked and characteristic elevation of the serum lipids. There was no indication on postmortem examination that intrahepatic bile ducts had developed and then become obliterated. The evidence presented in this study suggests that absence of interlobular bile ducts is a developmental anomaly, and that the embryologic development of biliary epithelium is normally independent of the growth of hepatic trabeculae.

scholarly journals Intrahepatic Transposition of Bile Ducts

ISRN Surgery ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Jasmin Delić ◽  
Admedina Savković ◽  
Eldar Isaković ◽  
Sergije Marković ◽  
Alma Bajtarevic ◽  
...  
Keyword(s):  
Bile Duct ◽  
Bile Ducts ◽  
Anatomical Variation ◽  
Intrahepatic Bile Duct ◽  
Main Research ◽  
Intrahepatic Bile Ducts ◽  
Anatomical Terminology ◽  
Segment Viii ◽  
Safe Performance

Objective. To describe the intrahepatic bile duct transposition (anatomical variation occurring in intrahepatic ducts) and to determine the frequency of this variation. Material and Methods. The researches were performed randomly on 100 livers of adults, both sexes. Main research methods were anatomical macrodissection. As a criterion for determination of variations in some parts of bile tree, we used the classification of Segmentatio hepatis according to Couinaud (1957) according to Terminologia Anatomica, Thieme Stuugart: Federative Committee on Anatomical Terminology, 1988. Results. Intrahepatic transposition of bile ducts was found in two cases (2%), out of total examined cases (100): right-left transposition (right segmental bile duct, originating from the segment VIII, joins the left liver duct-ductus hepaticus sinister) and left-right intrahepatic transposition (left segmental bile duct originating from the segment IV ends in right liver duct-ductus hepaticus dexter). Conclusion. Safety and success in liver transplantation to great extent depends on knowledge of anatomy and some common embryological anomalies in bile tree. Variations in bile tree were found in 24–43% of cases, out of which 1–22% are the variations of intrahepatic bile ducts. Therefore, good knowledge on ductal anatomy enables good planning, safe performance of therapeutic and operative procedures, and decreases the risk of intraoperative and postoperative complications.

Biliary Tract Cancer

2017 ◽  
Author(s):  
Jill Koshiol ◽  
Catterina Ferreccio ◽  
Susan S. Devesa ◽  
Juan Carlos Roa ◽  
Joseph F. Fraumeni
Keyword(s):  
United States ◽  
Gallbladder Cancer ◽  
Biliary Tract ◽  
Biliary Tract Cancer ◽  
Bile Ducts ◽  
The United States ◽  
Ampulla Of Vater ◽  
Biliary Tract Cancers ◽  
Tract Cancer ◽  
Extrahepatic Bile Ducts

Biliary tract cancers encompass tumors of the gallbladder, extrahepatic bile ducts, and ampulla of Vater. In the United States, biliary tract cancer is the fifth most common malignant neoplasm of the digestive tract, accounting for about 3,700 deaths per year. The gallbladder is the primary subsite for 40% of biliary tract cancers, followed by the extrahepatic bile ducts (33%), ampulla of Vater (20%), and unspecified subsite (8%). Gallbladder cancer occurs twice as often in women than men, while other biliary tumors are more common in men. Risk of gallbladder cancer is elevated in Amerindians, including the Pima Indians in the United States and the Mapuches in Chile, and in certain Hispanic populations. While a significant fraction of these tumors are related to underlying gallstones (cholelithiasis), information on other risk factors is limited, due to the rarity of the tumors, the often rapidly fatal course, and small number of epidemiologic studies.

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