Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cau...Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cause is haematopoietic stem cell transplantation （STC） and is also seen after solid organ transplantation. The incidence of veno occlusive disease （VOD） after STC ranges from 0 to 70%, but is decreasing. Survival is good when VOD is a mild form, but when it is severe and associated with an increase of hepatic venous pressure gradient 〉 20 mmHg, and mortality is about 90%. Prevention remains the best therapeutic strategy, by using non-myeloablative conditioning regimens before STC. Prophylactic administration of ursodeoxycholic add, being an antioxidant and antiapoptotic agent, can have some benefit in reducing overall mortality. Defibrotide, which has pro-fibrinolytic and antithrombotic properties, is the most effective therapy; decompression of the sinusoids by a b-ansjugular intrahepatic portosystemic shunt （TIPS） can be tried, especially to treat VOD after liver transplantation and when multiorgan failure （HOF） is not present. Liver transplantation can be the last option, but can not be considered a standard rescue therapy, because usually the concomitant presence of multiorgan failure contraindicates this procedure.展开更多
Cholangiocarcinoma(CC)is rare malignant tumors composed of cells that resemble those of the biliary tract.It is notoriously difficult to diagnose,and is associated with a high mortality.Traditionally,CC is divided int...Cholangiocarcinoma(CC)is rare malignant tumors composed of cells that resemble those of the biliary tract.It is notoriously difficult to diagnose,and is associated with a high mortality.Traditionally,CC is divided into intrahepatic and extraheaptic disease according to its location within the biliary tree.Intrahepatic cholangiocellular carcinoma(IH-CCC)or peripheral cholangiocellular carcinoma(CCC)appears within the second bifurcation of hepatic bile duct,and is the second most common primary liver cancer following hepatocellular carcinoma(HCC),IH-CCC or peripheral CCC often presents with advanced clinical features,and the cause for this cancer rise is still unclear.MRI,CT and PET provide useful diagnostic information in those patients.Surgical resection is the only chance for cure,with results depending on selected patients and careful surgical technique.Liver transplantation could offer long-term survival in selected patients when combined with chemotherapy.Chemotherapy,radiation therapy or combination therapies remain as the only treatment for inoperable patients.However,these are uniformly ineffective in patients' survival.展开更多
Living donor liver transplantation (LDLT) has gone through its formative years and established as a legitimate treatment when a deceased donor liver graft is not timely or simply not available at all. Nevertheless, LD...Living donor liver transplantation (LDLT) has gone through its formative years and established as a legitimate treatment when a deceased donor liver graft is not timely or simply not available at all. Nevertheless, LDLT is characterized by its technical complexity and ethical controversy. These are the consequences of a single organ having to serve two subjects, the donor and the recipient, instantaneously. The transplant community has a common ground on assuring donor safety while achieving predictable recipient success. With this background, a reflection of the development of LDLT may be appropriate to direct future research and patient-care efforts on this life-saving treatment alternative.展开更多
文摘Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cause is haematopoietic stem cell transplantation （STC） and is also seen after solid organ transplantation. The incidence of veno occlusive disease （VOD） after STC ranges from 0 to 70%, but is decreasing. Survival is good when VOD is a mild form, but when it is severe and associated with an increase of hepatic venous pressure gradient 〉 20 mmHg, and mortality is about 90%. Prevention remains the best therapeutic strategy, by using non-myeloablative conditioning regimens before STC. Prophylactic administration of ursodeoxycholic add, being an antioxidant and antiapoptotic agent, can have some benefit in reducing overall mortality. Defibrotide, which has pro-fibrinolytic and antithrombotic properties, is the most effective therapy; decompression of the sinusoids by a b-ansjugular intrahepatic portosystemic shunt （TIPS） can be tried, especially to treat VOD after liver transplantation and when multiorgan failure （HOF） is not present. Liver transplantation can be the last option, but can not be considered a standard rescue therapy, because usually the concomitant presence of multiorgan failure contraindicates this procedure.
文摘Cholangiocarcinoma(CC)is rare malignant tumors composed of cells that resemble those of the biliary tract.It is notoriously difficult to diagnose,and is associated with a high mortality.Traditionally,CC is divided into intrahepatic and extraheaptic disease according to its location within the biliary tree.Intrahepatic cholangiocellular carcinoma(IH-CCC)or peripheral cholangiocellular carcinoma(CCC)appears within the second bifurcation of hepatic bile duct,and is the second most common primary liver cancer following hepatocellular carcinoma(HCC),IH-CCC or peripheral CCC often presents with advanced clinical features,and the cause for this cancer rise is still unclear.MRI,CT and PET provide useful diagnostic information in those patients.Surgical resection is the only chance for cure,with results depending on selected patients and careful surgical technique.Liver transplantation could offer long-term survival in selected patients when combined with chemotherapy.Chemotherapy,radiation therapy or combination therapies remain as the only treatment for inoperable patients.However,these are uniformly ineffective in patients' survival.
文摘Living donor liver transplantation (LDLT) has gone through its formative years and established as a legitimate treatment when a deceased donor liver graft is not timely or simply not available at all. Nevertheless, LDLT is characterized by its technical complexity and ethical controversy. These are the consequences of a single organ having to serve two subjects, the donor and the recipient, instantaneously. The transplant community has a common ground on assuring donor safety while achieving predictable recipient success. With this background, a reflection of the development of LDLT may be appropriate to direct future research and patient-care efforts on this life-saving treatment alternative.
