Results 11 to 20 of about 1,160,128 (185)

Revisiting Caroli Syndrome in a Tanzanian Patient. [PDF]

Cureus, 2020
Caroli disease and Caroli syndrome are two rare congenital diseases of the intrahepatic bile ducts. Caroli syndrome is characterized by the saccular dilatation of intrahepatic bile ducts associated with congenital hepatic fibrosis. It is rarely diagnosed in childhood.
Wambura C, Sharma M, Surani S.
europepmc   +5 more sources

Caroli′s syndrome in a post renal transplant patient: Case report and review of the literature

The Saudi Journal of Gastroenterology, 2012
Caroli′s syndrome is characterized by bile duct ectasia in association with hepatic fibrosis. It is usually transmitted in an autosomal recessive fashion and has been well documented to be associated with autosomal recessive polycystic kidney disease and
Muhammad Z Bawany, Osama Alaradi, Ali Nawras   +2 more
doaj   +2 more sources

Hepatobiliary langerhans cell histiocytosis mimicking Caroli syndrome and recurring in a liver allograft [PDF]

Frontiers in Medicine
Langerhans cell histiocytosis (LCH) involving the hepatobiliary system can progress to sclerosing cholangitis and cirrhosis and may require liver transplantation (LT).
Tingting Hu, Chao Wang, Chengjia Liu, Jianzhong Sun   +3 more
doaj   +2 more sources

Clinical outcomes of liver transplantation in Caroli syndrome: a retrospective analysis [PDF]

Orphanet Journal of Rare Diseases
Objective To evaluate the clinical outcomes of liver transplantation (LT) in patients with Caroli syndrome. Methods We retrospectively analyzed clinical data from 20 patients diagnosed with Caroli syndrome who underwent LT at Beijing Friendship Hospital,
Fei Hou, Hao-Feng Xiong, Ying Liu, Shuang Zhao, Yi-Zhi Zhang, Qian Kang, Jing-Yi Liu, Lin Wei, Wei Qu, Zhi-Gui Zeng, Zhi-Jun Zhu, Li-Ying Sun   +11 more
doaj   +2 more sources

A Challenging Case of Hepatoblastoma Concomitant with Autosomal Recessive Polycystic Kidney Disease and Caroli Syndrome—Review of the Literature [PDF]

Frontiers in Pediatrics, 2017
We report a rare case of an 18-month-old female with autosomal recessive polycystic kidney disease, Caroli syndrome, and pure fetal type hepatoblastoma. The liver tumor was surgically resected with no chemotherapy given.
Nevil Kadakia, Steven J. Lobritto, Nadia Ovchinsky, Helen E. Remotti, Darrell J. Yamashiro, Darrell J. Yamashiro, Darrell J. Yamashiro, Jean C. Emond, Mercedes Martinez   +8 more
doaj   +2 more sources

Clinical outcomes in Caroli disease and Caroli syndrome: a longitudinal observational cohort study [PDF]

Scientific Reports
Caroli’s disease (CD) and Caroli’s syndrome (CS) are rare congenital fibropolycystic liver disorders. While CS is known to be associated with more severe hepatic complications than isolated CD, direct longitudinal comparisons between the two entities ...
Amr Shaaban Hanafy, Eslam Kamal Fahmy, Rania Naguib, Moaz Abulfaraj, Ahmed F. Omar, Hend Naguib, Mohamed Mahmoud Abdelrahman, Hany A. Elkattawy   +7 more
doaj   +2 more sources

Portal hypertension: An uncommon presentation of Caroli's syndrome [PDF]

Clinical Case Reports (discontinued), 2020
AbstractPortal hypertension is not a classical presentation of Caroli's syndrome. However, some young children can present with overt signs and symptoms indicative of advanced disease state despite the improvement in imaging technology which has decreased its diagnostic age. High index of clinical suspicion can help in timely diagnosis and management.
Suraj Shrestha, Akash Raut
exaly   +4 more sources

Biallelic WDR19 Variants: Systematic Analysis of Genotype-Phenotype Correlations. [PDF]

Hum Mutat
Ciliopathies represent a diverse group of inherited disorders resulting from dysfunctional cilia. A rare subset is associated with biallelic variants in WDR19, with fewer than 100 affected individuals reported worldwide. This study is aimed at reviewing the clinical and molecular spectrum of WDR19‐associated ciliopathies, with a particular focus on ...
Webster Z, Woods L, Dalziel R, Drake K, de Valles-Ibáñez G, Poke G.   +5 more
europepmc   +2 more sources

Caroli disease associated with autosomal recessive polycystic kidney disease: CT imaging features of a case report [PDF]

Radiology Case Reports
Caroli disease (CD) is a rare congenital hepatobiliary disorder characterized by multifocal segmental dilatation of the intrahepatic bile ducts, which may involve the entire liver or only a specific region.
Mohammad Tahir Aien, MD, Hashmatullah Osmani, MD, Mohammad Iqbal Haqyar, MD   +2 more
doaj   +2 more sources

Stroke after percutaneous transhepatic variceal obliteration of esophageal varix in Caroli syndrome [PDF]

Korean Journal of Pediatrics, 2013
Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation ...
Yoo Min Lee, Yoon Lee, Yon Ho Choe
doaj   +2 more sources