Disease relevance of Caroli Disease

• The regenerative process was evaluated in terms of liver size, function, and histology in 28 adults who had major hepatic resection: hepatocellular carcinoma (HCC) in 21, secondary liver cancer from colorectum in four, carcinoma of the gallbladder in one, Klatskin tumor in one, and Caroli's disease in one [1].
• Forty-seven ARPKD and 14 pedigrees with congenital hepatic fibrosis and/or Caroli's disease, were screened for PKHD1 mutations [2].
• Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor [3].
• Therefore, a high index of suspicion for the diagnosis of Caroli's disease is warranted especially in patients with ARPKD or ADPKD [4].
• Case report: chenodeoxycholic acid therapy of intrahepatic radiolucent gallstones in a patient with Caroli's syndrome [5].

High impact information on Caroli Disease

• We treated twelve patients who had Caroli's syndrome and intrahepatic stones with ursodeoxycholic acid (UDCA), 10-20 mg/kg daily [6].
• Activation of the MEK5/ERK5 cascade is responsible for biliary dysgenesis in a rat model of Caroli's disease [7].
• Autosomal dominant polycystic kidney disease with anticipation and Caroli's disease associated with a PKD1 mutation. Rapid communication [8].
• Role of PTC and ERCP in diagnostic imaging of the hepatobiliary tree: Caroli's disease in two siblings [9].
• Caroli's disease was complicated by a Budd-Chiari-like syndrome as a result of enlarged lymph nodes that compressed the vena cava [10].

Chemical compound and disease context of Caroli Disease

• Diagnosis of Caroli's disease by technetium-99m DISIDA cholescintigraphy. Report of three cases [11].
• A patient with the Caroli syndrome and multiple intrahepatic and extrahepatic cholesterol stones [12].
• A case of Caroli's disease is presented and the findings of various imaging modalities are discussed with emphasis on the role of Technetium 99mTc diethyl-iminodiacetic acid scan in reaching the final diagnosis [13].

Anatomical context of Caroli Disease

• Case of the day. Case 5. Congenital dilatation of intrahepatic bile ducts (? caroli disease) [14].

Gene context of Caroli Disease

• These results suggest that the activation of the MEK5-ERK5 cascade plays a pivotal role in the biliary dysgenesis of PCK rats, and also provide insights into the pathogenesis of Caroli's disease with CHF [7].

References