Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease [PDF]
Radiology Case Reports, 2019 Caroli syndrome, which is characterized by saccular and fusiform dilatation of the biliary ducts, is usually observed in association with autosomal recessive polycystic kidney disease (ARPKD).
Amanda Rivas +4 more
doaj +5 more sources
Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome [PDF]
Case Reports in Pediatrics, 2017 Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that ...
W. De Jesus-Rojas +5 more
doaj +7 more sources
A Rare Diagnosis of Caroli Syndrome in a Young Patient [PDF]
Clinical Case ReportsCaroli syndrome is a rare but serious congenital disorder associated with portal hypertension and polycystic kidney disease. Early diagnosis via imaging, particularly MRCP, is crucial to prevent life‐threatening complications such as cholangitis and ...
Elaheh Karimzadeh‐Soureshjani +4 more
doaj +4 more sources
Prenatal diagnosis and molecular characterization of PKHD1 variants in two Chinese fetuses with Caroli disease/syndrome [PDF]
Frontiers in GeneticsBackgroundCaroli disease (CD) and Caroli syndrome (CS) are rare inherited disorders characterized by dilatation of intrahepatic bile ducts, caused by PKHD1 pathogenic variants.
Hui Huang +6 more
doaj +4 more sources
Caroli syndrome associated with atrial septal defect and polydactyly: a case report [PDF]
Journal of Medical Case Reports, 2023 Introduction Caroli disease is multifocal segmental dilatation of the large intrahepatic bile ducts that connect to the main duct. It is considered a rare disease with an incidence rate of 1 in 1,000,000 births.
Ali Ghassa, Lina Khouri
doaj +2 more sources
Caroli syndrome in a dog [PDF]
Ciência Rural, 2022 : Caroli syndrome is characterized by a combination of intrahepatic biliary ductal ectasia and congenital ductal fibrosis due to the failure of involution of ductal plates and large intrahepatic ducts.
Bianca Santana de Cecco +7 more
doaj +5 more sources
Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study [PDF]
BMC Gastroenterology, 2020 Background Caroli syndrome (CS) is a rare congenital disorder without pathognomonic clinical symptoms or laboratory findings; therefore, the diagnosis is often delayed.
Wen Shi +5 more
doaj +2 more sources
Rare variants in PKHD1 associated with Caroli syndrome: Two case reports [PDF]
Molecular Genetics & Genomic Medicine, 2022 Background Caroli disease (CD, OMIM #600643) is a rare autosomal recessive disorder characterized by polycystic segmental dilatation of the intrahepatic bile ducts and extreme variability in age of onset and clinical manifestations.
Carola Giacobbe +5 more
doaj +2 more sources
Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law? [PDF]
Medical Sciences, 2019 Caroli syndrome, characterized by saccular dilatation of intrahepatic ducts and congenital hepatic fibrosis, is without therapy in part due to its ultra-rare prevalence and the apparent lack of availability of a suitable experimental model. While the PCK
Bharvi M. Chavre +8 more
doaj +2 more sources
CAROLI DISEASE AND CAROLI SYNDROME [PDF]
Medical Journal Armed Forces India, 1999 Caroli syndrome consists of Caroli disease and congenital hepatic fibrosis [1]. It is an uncommon congenital disorder of the intrahepatic bile ducts. The disease is presumably of autosomal recessive hereditary character [2]. A classical case history of the Caroli syndrome is presented. Non surgical management with endoprosthesis is discussed.
Man Mohan Harjai, Kk Maudar, K K Maudar
exaly +3 more sources