1.Primary sclerosing cholangitis:A review and update
H.Tabibian JAMES ; L.Bowlus CHRISTOPHER
Liver Research 2017;1(4):221-230
Primary sclerosing cholangitis(PSC)is a rare,chronic,cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibro-obliterative inflammation of the bile ducts.In a clinically significant proportion of patients,PSC pro-gresses to cirrhosis,end-stage liver disease,and/or hepatobiliary cancer,though the disease course can be highly variable.Despite clinical trials of numerous pharmacotherapies over several decades,safe and effective medical therapy remains to be established.Liver transplantation is an option for select patients with severe complications of PSC,and its outcomes are generally favorable.Periodic surveillance testing for pre-as well as post-transplant patients is a cornerstone of preventive care and health maintenance.Here we provide an overview of PSC,including its epidemiology,etiopathogenesis,clinical features,associated disorders,surveillance,and emerging potential therapies.
2.Hemobilia:Etiology,diagnosis,and treatment
Berry RANI ; Han JAMES ; A.Kardashian ANI ; F.LaRusso NICHOLAS ; H.Tabibian JAMES
Liver Research 2018;2(4):200-208
Hemobilia refers to bleeding from and/or into the biliary tract and is an uncommon but important cause of gastrointestinal hemorrhage.Reports of hemobilia date back to the 1600s,but due to its relative rarity and challenges in diagnosis,only in recent decades has hemobilia been more critically studied.The majority of cases of hemobilia are iatrogenic and caused by invasive procedures involving the liver,pancreas,bile ducts and/or the hepatopancreatobiliary vasculature,with trauma and malignancy rep-resenting the two other leading causes.A classic triad of right upper quadrant pain,jaundice,and overt upper gastrointestinal bleeding has been described(i.e.Quincke's triad),but this is present in only 25%-30%of patients with hemobilia.Therefore,prompt diagnosis depends critically on having a high index of suspicion,which may be based on a patient's clinical presentation and having recently undergone(peri-)biliary instrumentation or other predisposing factors.The treatment of hemobilia depends on its severity and suspected source and ranges from supportive care to advanced endoscopic,interventional radiologic,or surgical intervention.Here we provide a clinical overview and update regarding the eti-ology,diagnosis,and treatment of hemobilia geared for specialists and subspecialists alike.
3.Biliary endoscopy in the management of primary sclerosing cholangitis and its complications
M.Fung BRIAN ; H.Tabibian JAMES
Liver Research 2019;3(2):106-117
Primary sclerosing cholangitis(PSC)is a chronic,idiopathic,cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts.It can affect individuals of all age groups and gender,has no established pharmacotherapy,and is associated with a variety of neoplastic(e.g.cholangiocarcinoma)and non-neoplastic(e.g.dominant strictures)hepatobiliary com-plications.Given these considerations,endoscopy plays a major role in the care of patients with PSC.In this review,we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC.Where evidence is limited,we suggest pragmatic approaches based on currently available data and expert opinion.
4.Preventative care in cholestatic liver disease:Pearls for the specialist and subspecialist
Malik ADNAN ; A.Kardashian ANI ; Zakharia KAIS ; L.Bowlus CHRISTOPHER ; H.Tabibian JAMES
Liver Research 2019;3(2):118-127
Cholestatic liver diseases(CLDs)encompass a variety of disorders of abnormal bile formation and/or flow.CLDs often lead to progressive hepatic insult and injury and following the development of cirrhosis and associated complications.Many such complications are clinically silent until they manifest with severe sequelae,including but not limited to life-altering symptoms,metabolic disturbances,cirrhosis,and hepatobiliary diseases as well as other malignancies.Primary sclerosing cholangitis(PSC)and primary biliary cholangitis(PBC)are the most common CLDs,and both relate to mutual as well as unique complications.This review provides an overview of PSC and PBC,with a focus on preventive measures aimed to reduce the incidence and severity of disease-related complications.