Acute liver failure (ALF) is a devastating clinical syndrome characterised by progressive encephalopathy, coagulopathy, and circulatory dysfunction, which commonly leads to multiorgan failure and death. Central to the...Acute liver failure (ALF) is a devastating clinical syndrome characterised by progressive encephalopathy, coagulopathy, and circulatory dysfunction, which commonly leads to multiorgan failure and death. Central to the pathogenesis of ALF is activation of the immune system with mobilisation of cellular effectors and massive production of cytokines. As key components of the innate immune system, monocytes and macrophages are postulated to play a central role in the initiation, progression and resolution of ALF. ALF in humans follows a rapidly progressive clinical course that poses inherent difficulties in delineating the role of these pivotal immune cells. Therefore, a number of experimental models have been used to study the pathogenesis of ALF. Here we consider the evidence from experimental and human studies of ALF on the role of monocytes and macrophages in acute hepatic injury and the ensuing extrahepatic manifestations, including functional monocyte deactivation and multiple organ failure.展开更多
Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a ...Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1(complete) shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.展开更多
Intrahepatic portosystemic shunts(IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis.It has been described in both children and adults and may be asymptomatic or be associated with a...Intrahepatic portosystemic shunts(IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis.It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications.When recognized,early intervention to occlude the shunt will reverse the associated complications.Literature review reports of surgical and radiological occlusion of the shunt,but due to its rarity,a standard therapeutic protocol has not been established.A case of a 38-year-old woman with abdominal pain and low grade encephalopathy,diagnosed with an IPSS and treated by right hepatectomy was reported.展开更多
According to the most recent WHO classification of hepatocellular adenomas,a small percentage of inflammatory hepatocellular adenomas presents with mutation in the β-catenin gene and are at higher risk of malignant t...According to the most recent WHO classification of hepatocellular adenomas,a small percentage of inflammatory hepatocellular adenomas presents with mutation in the β-catenin gene and are at higher risk of malignant transformation. It has been recognized that adenoma-like hepatocellular neoplasms with focal atypia,or in unusual clinical context present with similar cytogenetic and immunohistochemistry characteristics to well-differentiated hepatocellular carcinomas. We report a case of a well-differentiated hepatocellular neoplasm with Dubin-Johnson-like pigment displaying histological features overlapping with a β-catenin mutated inflammatory adenoma and a well-differentiated hepatocellular carcinoma in a non-cirrhotic liver. The patient was a 48-year-old woman,who was asymptomatic,and had a clinical history of intra-uterine exposure to diethylstilbestrol,previous cancers and past oral contraceptive use. The recently proposed term "well-differentiated hepatocellular neoplasm of uncertain malignant potential" should be applied in such cases to highlight the different pathogenesis and risk of malignancy compared to the typical adenomas,and to suggest a careful and customized clinical management.展开更多
文摘Acute liver failure (ALF) is a devastating clinical syndrome characterised by progressive encephalopathy, coagulopathy, and circulatory dysfunction, which commonly leads to multiorgan failure and death. Central to the pathogenesis of ALF is activation of the immune system with mobilisation of cellular effectors and massive production of cytokines. As key components of the innate immune system, monocytes and macrophages are postulated to play a central role in the initiation, progression and resolution of ALF. ALF in humans follows a rapidly progressive clinical course that poses inherent difficulties in delineating the role of these pivotal immune cells. Therefore, a number of experimental models have been used to study the pathogenesis of ALF. Here we consider the evidence from experimental and human studies of ALF on the role of monocytes and macrophages in acute hepatic injury and the ensuing extrahepatic manifestations, including functional monocyte deactivation and multiple organ failure.
文摘Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1(complete) shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.
文摘Intrahepatic portosystemic shunts(IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis.It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications.When recognized,early intervention to occlude the shunt will reverse the associated complications.Literature review reports of surgical and radiological occlusion of the shunt,but due to its rarity,a standard therapeutic protocol has not been established.A case of a 38-year-old woman with abdominal pain and low grade encephalopathy,diagnosed with an IPSS and treated by right hepatectomy was reported.
文摘According to the most recent WHO classification of hepatocellular adenomas,a small percentage of inflammatory hepatocellular adenomas presents with mutation in the β-catenin gene and are at higher risk of malignant transformation. It has been recognized that adenoma-like hepatocellular neoplasms with focal atypia,or in unusual clinical context present with similar cytogenetic and immunohistochemistry characteristics to well-differentiated hepatocellular carcinomas. We report a case of a well-differentiated hepatocellular neoplasm with Dubin-Johnson-like pigment displaying histological features overlapping with a β-catenin mutated inflammatory adenoma and a well-differentiated hepatocellular carcinoma in a non-cirrhotic liver. The patient was a 48-year-old woman,who was asymptomatic,and had a clinical history of intra-uterine exposure to diethylstilbestrol,previous cancers and past oral contraceptive use. The recently proposed term "well-differentiated hepatocellular neoplasm of uncertain malignant potential" should be applied in such cases to highlight the different pathogenesis and risk of malignancy compared to the typical adenomas,and to suggest a careful and customized clinical management.
