scholarly journals Caroli Disease (Clinical Observation)

2019 ◽  
Vol 28 (6) ◽  
pp. 77-83
Author(s):  
I. G. Nikitin ◽  
A. A. Karabinenko ◽  
A. E. Nikitin ◽  
E. I. Dedov ◽  
D. G. Zhukova ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Bile Ducts ◽  
Abdominal Cavity ◽  
Unknown Origin ◽  
Symptomatic Therapy ◽  
Biliary Cirrhosis ◽  
Caroli Disease ◽  
Intrahepatic Bile Ducts ◽  
The Moment

The incidence of Caroli disease is estimated to be 1 case per 1 million people; as a result, there are very few available clinical observations. At the moment, the treatment of Caroli disease is limited to symptomatic therapy and the prevention of complications, as well as to corrective surgery and liver transplantation.Aim. The aim of this observation is to describe one case of a rare congenital liver disease – Caroli disease – characterised by a segmented non-obstructive fibrocystic dilation of the intrahepatic bile ducts.Key findings. A 21-year-old woman was hospitalised with the signs of a systemic inflammatory reaction, hepatosplenomegaly, jaundice, portal hypertension, hepatocellular insufficiency, as well as with the manifestations of cytolytic and cholestatic syndromes. At the age of 8, she was diagnosed with a cyst of the bile ducts, which was treated with cystoenteroanastomosis. At the age of 20, in connection with high portal hypertension, portocaval shunting was performed and a mesenteric-caval anastomosis was applied. During the present hospitalisation, an expansion of the intrahepatic bile ducts was revealed by ultrasound and MSCT of the abdominal cavity with contrast, which made it possible to diagnose Caroli disease.Conclusion. A case of Caroli disease is described, which resulted in continuously recurrent cholangitis and biliary cirrhosis. This state required liver transplantation. Caroli disease should be included in differential diagnosis in patients suffering from the fever of unknown origin and cholestasis syndrome. An early diagnosis of Caroli disease is important for preventing complications, improving the quality of life and increasing the life expectancy of patients suffering from this rare disease.

scholarly journals Primary biliary cirrhosis and myopathy: an uncommon association

1999 ◽  
Vol 54 (5) ◽  
pp. 165-168 ◽  
Author(s):  
Bruno Cupertino Migueletto ◽  
Abrahão Elias Hallack Neto ◽  
Elaine Zamora Domingues ◽  
Pedro Paulo Neves de Castro ◽  
Hartmut Stocker ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Bile Ducts ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Muscular Weakness ◽  
Muscular Diseases ◽  
Histological Features ◽  
Intrahepatic Bile Ducts ◽  
Organ Systems

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

Vascular plexus around intrahepatic bile ducts in normal livers and portal hypertension

1989 ◽  
Vol 8 (2) ◽  
pp. 139-149 ◽  
Author(s):  
Tadashi Terada ◽  
Fumio Ishida ◽  
Yasuni Nakanuma
Keyword(s):  
Portal Hypertension ◽  
Bile Ducts ◽  
Vascular Plexus ◽  
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

RhGH Attenuates Ischemia Injury of Intrahepatic Bile Ducts Relating to Liver Transplantation

2011 ◽  
Vol 171 (1) ◽  
pp. 300-310 ◽  
Author(s):  
Zheng Wang ◽  
Jie Zhou ◽  
Jianhua Lin ◽  
Yu Wang ◽  
Yixiong Lin ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Bile Ducts ◽  
Intrahepatic Bile Ducts

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.

Primary biliary cirrhosis

2010 ◽  
pp. 2464-2468
Author(s):  
M.F. Bassendine ◽  
D.E.J. Jones
Keyword(s):  
Liver Disease ◽  
Epithelial Cells ◽  
Primary Biliary Cirrhosis ◽  
Case History ◽  
Bile Ducts ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Blood Tests ◽  
Intrahepatic Bile Ducts ◽  
Immune Mediated

Case History—A 60 yr old woman presenting with abnormal liver blood tests. Primary biliary cirrhosis is a chronic, cholestatic liver disease in which the biliary epithelial cells lining the small intrahepatic bile ducts are the target for immune-mediated damage leading to progressive ductopenia. The cause is unknown, but presumed to be autoimmune....

scholarly journals PATHOMORPHOLOGICAL STRUCTURE OF INTRAHEPATIC DUCTS AND CHOLEDOCH IN PATIENTS WITH ACUTE AND CHRONIC CHOLANGITIS.

2019 ◽  
pp. 36-40
Author(s):  
O. V. Maloshtan ◽  
A. O. Nekliudov ◽  
R. M. Smachylo ◽  
A. M. Tyschenko ◽  
М. О. Кlyosova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Bile Ducts ◽  
Inflammatory Process ◽  
Acute Cholangitis ◽  
Clinical Manifestations ◽  
Control Group ◽  
Biliary Cirrhosis ◽  
Intrahepatic Bile Ducts ◽  
Morphological Status ◽  
Acute Cholestasis

Abstract. The morphological status of bile duct cells was studied in patients with acute and chronic cholangitis at different terms of the disease.All patients (32) were divided into 4 groups: up to 5 days, 12-14 days, 28-30 days and 10-12 months. The control group included 12 patients without clinical manifestations of choledocholithiasis. Acute cholestasis is registered in the intrahepatic bile ducts, the maximal severity of which is on first two weeks. In the period of 28-30 days the residual phenomena of acute cholangitis and the growth of connective tissue in the duct walls and around them are registered. In patients with cholangitis, the largest changes in the wall of the choledochus are recorded in 3-5 days from the onset of the disease in the form of edema-desquamative and epithelium necrosis. On 12-14 days marked "attenuation" and demarcation of the inflammatory process, and on 28-30 there’s the appearance of connective tissue in the wall of the choledoch. Patients operated on for chronic recurrent cholangitis for 1 year are documented on the background of the development of choledoch stricture, cholestasis, cholangiosclerosis, and biliary cirrhosis, as a complication.

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