Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease

2009 ◽  
Vol 217 (3) ◽  
pp. 442-451 ◽  
Author(s):  
Mitsue Yasoshima ◽  
Yasunori Sato ◽  
Shinichi Furubo ◽  
Kazuo Kizawa ◽  
Takahiro Sanzen ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Matrix Proteins ◽  
Congenital Hepatic Fibrosis ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Intrahepatic Bile Ducts

scholarly journals Diffuse Oligosymptomatic Caroli’s Disease: Case Report

2020 ◽  
Vol 31 (2) ◽  
pp. 107-110
Author(s):  
Lemfadli Y ◽  
Bouchrit S ◽  
Ait Errami A ◽  
Oubaha S ◽  
Samlani Z ◽  
...  
Keyword(s):  
Case Report ◽  
Female Patient ◽  
Bile Ducts ◽  
Clinical Case ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Caroli Disease ◽  
Intrahepatic Bile Ducts ◽  
Disease Case

This article describes a case of Caroli’s disease in a 53-year-old female patient who complained nonspecificabdominal pain without cholestasis or cholangitis. Ultrasound and hepatic magnetic resonanceimaging showed segmental saccular dilations connected to intrahepatic bile ducts without hepaticfibrosis. This clinical case shows the possibility of having oligosymptomatic forms in the diffuse formsof Caroli disease, therefore the interest to consider this diagnosis in case of non-specific abdominalsigns and to request a hepatic ultrasound. Bangladesh J Medicine July 2020; 31(2) : 107-110

Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys

1986 ◽  
Vol 16 (4) ◽  
pp. 302-305 ◽  
Author(s):  
C. H. Davies ◽  
D. A. Stringer ◽  
H. Whyte ◽  
A. Daneman ◽  
K. Mancer
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidneys ◽  
Intrahepatic Bile Ducts

Congenital Dilatation of the Intrahepatic Bile Ducts (Caroli's Disease)

Radiology ◽  
1978 ◽  
Vol 127 (3) ◽  
pp. 746-746 ◽  
Author(s):  
Javier Lucaya ◽  
Jose Luis Gomez ◽  
Ceferino Molino ◽  
Juan Gonzalez Atienza
Keyword(s):  
Bile Ducts ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Intrahepatic Bile Ducts

scholarly journals Caroli's disease (clinical observation)

2021 ◽  
Vol 13 (3) ◽  
pp. 150-152
Author(s):  
G. G. Kuttykuzhanova ◽  
A. Zh. Tanirbergenova ◽  
S. Zh. Abdirazakova ◽  
Z. Zh. Urikbaeva
Keyword(s):  
Early Diagnosis ◽  
Rare Diseases ◽  
Medical Practice ◽  
Bile Ducts ◽  
Clinical Observation ◽  
Diagnostic Methods ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Intrahepatic Stones ◽  
Intrahepatic Bile Ducts

In their medical practice doctors deal with rare diseases which present difficulties in making a clinical diagnosis, in this case additional diagnostic methods are required. One of these diseases is Caroli's disease (syndrome), characterized by cystic enlargement of the intrahepatic bile ducts with frequent formation of intrahepatic stones. Taking into account the difficulties of early diagnosis, we present our own observation.

scholarly journals Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease) in two Golden retriever littermates : clinical communication

2006 ◽  
Vol 77 (4) ◽  
Author(s):  
R.D. Last ◽  
J.M. Hill ◽  
M. Roach ◽  
T. Kaldenberg
Keyword(s):  
Bile Ducts ◽  
Autosomal Recessive ◽  
Autosomal Recessive Inheritance ◽  
Golden Retriever ◽  
Portal Fibrosis ◽  
Clinical Communication ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Intrahepatic Bile Ducts ◽  
Collecting Ducts

Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease) described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.

scholarly journals Polycystic Kidney Rat Is a Novel Animal Model of Caroli’s Disease Associated with Congenital Hepatic Fibrosis

2001 ◽  
Vol 158 (5) ◽  
pp. 1605-1612 ◽  
Author(s):  
Takahiro Sanzen ◽  
Kenichi Harada ◽  
Mitsue Yasoshima ◽  
Yasuhito Kawamura ◽  
Masahiko Ishibashi ◽  
...  
Keyword(s):  
Animal Model ◽  
Hepatic Fibrosis ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidney ◽  
Caroli's Disease ◽  
Caroli’S Disease
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