Cholangiocarcinoma(CCA) is a malignant tumour that arises from biliary epithelium at any portion of the biliary tree.CCA is currently classified as intra-hepatic or extra-hepatic CCA(EH-CCA).Recent evidences suggest t...Cholangiocarcinoma(CCA) is a malignant tumour that arises from biliary epithelium at any portion of the biliary tree.CCA is currently classified as intra-hepatic or extra-hepatic CCA(EH-CCA).Recent evidences suggest that intra-hepatic CCA(IH-CCA) and EH-CCA are biologically different cancers,giving further support to a number of recent epidemiological studies showing large differences in terms of incidence,mortality and risk factors.The purpose of this manuscript is to review recent literature dealing with the descriptive epidemiology and risk factors of CCA with a special effort to compare IH-with EH-CCA.展开更多
The liver has a central role in regulating inflammation by its capacity to secrete a number of proteins that control both local and systemic inflammatory responses. Chronic inflammation or an exaggerated inflammatory ...The liver has a central role in regulating inflammation by its capacity to secrete a number of proteins that control both local and systemic inflammatory responses. Chronic inflammation or an exaggerated inflammatory response can produce detrimental effects on target organs. Chronic hepatitis C virus(HCV) infection causes liver inflammation by complex and not yet well-understood molecular pathways, including direct viral effects and indirect mechanisms involving cytokine pathways,oxidative stress and steatosis induction. An increasing body of evidence recognizes the inflammatory response in chronic hepatitis C as pathogenically linked to the development of both liver-limited injury(fibrosis, cirrhosis and hepatocellular carcinoma) and extrahepatic HCV-related diseases(lymphoproliferative disease,atherosclerosis, cardiovascular and brain disease). Defining the complex mechanisms of HCV-induced inflammation could be crucial to determine the global impact of infection, to estimate progression of the disease, and to explore novel therapeutic approaches to avert HCVrelated diseases. This review focuses on HCV-related clinical conditions as a result of chronic liver and systemic inflammatory states.展开更多
Background:Whether the extra-hepatic bile duct(EHBD)should be routinely resected for gallbladder carcinoma(GBC)remains controversial.The current study aimed to determine the clinical impact of combined EHBD resection ...Background:Whether the extra-hepatic bile duct(EHBD)should be routinely resected for gallbladder carcinoma(GBC)remains controversial.The current study aimed to determine the clinical impact of combined EHBD resection during curative surgery for advanced GBC.Methods:In total,213 patients who underwent curative surgery for T2,T3 or T4 GBC were enrolled.The clinicopathological features were compared between the patients treated with EHBD resection and those without EHBD resection.Meanwhile,univariable and multivariable Cox-proportional hazards regression models were used to identify risk factors for overall survival(OS).Results:Among the 213 patients identified,87(40.8%)underwent combined EHBD resection.Compared with patients without EHBD resection,patients with EHBD resection suffered more post-operative complications(33.3%vs.21.4%,P=0.046).However,the median OS of the EHBD resection group was longer than that of the non-EHBD resection group(25 vs.11 months,P=0.008).Subgroup analyses were also performed according to tumor(T)category and lymph-node metastasis.The median OS was significantly longer in the EHBD resection group than in the non-EHBD resection group for patients with T3 lesion(15 vs.7 months,P=0.002),T4 lesion(11 vs.6 months,P=0.021)or lymph-node metastasis(12 vs.7 months,P<0.001).No survival benefit of EHBD resection was observed in GBC patients with T2 lesion or without lymph-node metastasis.T category,lymph-node metastasis,margin status,pre-operative CA19-9 level and EHBD resection were identified as independent prognostic factors for OS of patients with advanced GBC(all P values<0.05).Conclusions EHBD resection can independently affect the OS in advanced GBC.For GBC patients with T3 lesion,T4 lesion and lymph-node metastasis,combined EHBD resection is justified and may improve OS.展开更多
BACKGROUND Primary liver cancer is one of the most common malignant tumours,while primary splenic lymphoma is a rare malignancy.Thus,cases of hepatocellular carcinoma(HCC)combined with splenic lymphoma are extremely r...BACKGROUND Primary liver cancer is one of the most common malignant tumours,while primary splenic lymphoma is a rare malignancy.Thus,cases of hepatocellular carcinoma(HCC)combined with splenic lymphoma are extremely rare.CASE SUMMARY We present a 62-year-old woman who was admitted to the Interventional Radiology Department with a lump in the spleen and liver as well as multiple enlarged lymph nodes visible by ultrasound.Contrast-enhanced computed of the abdomen revealed a circular,low-density,shallow mass(approximately 2.6 cm in diameter)in the left intrahepatic lobe and multiple round,low-density shadows in the spleen with clear boundaries(maximum diameter 7.6 cm).Based on the characteristic clinical symptoms and explicit radiological findings,the clinical diagnosis was HCC with metastasis to the liver portal,retroperitoneal lymph nodes,and spleen.After transcatheter arterial chemoembolization and sequential radiofrequency ablation,the-fetoprotein level returned to the normal range,and the hepatitis B cirrhosis improved.In addition,splenic tumour biopsy confirmed the diagnosis of primary malignant lymphoma,which went into remission after chemotherapy.CONCLUSION HCC with primary splenic non-Hodgkin lymphoma is extremely rare and easily misdiagnosed.Better understanding would facilitate early diagnosis,treatment and prognosis.展开更多
Since its identification in 1989, hepatitis C virus(HCV) has emerged as a worldwide health problem with roughly 185 million chronic infections, representing individuals at high risk of developing cirrhosis and liver c...Since its identification in 1989, hepatitis C virus(HCV) has emerged as a worldwide health problem with roughly 185 million chronic infections, representing individuals at high risk of developing cirrhosis and liver cancer. In addition to being a frequent cause of morbidity and mortality due to liver disease, HCV has emerged as an important trigger of lymphoproliferative disorders, owing to its lymphotropism, and of a wide spectrum of extrahepatic manifestations(HCV-EHMs) affecting different organ systems. The most frequently observed HCV-EHMs include mixed cryoglobulinemia and cryoglobulinemic vasculitis, B-cell non-Hodgkin's lymphoma, nephropathies, thyreopathies, type 2 diabetes mellitus, cardiovascular diseases, and several neurological conditions. In addition, neuropsychiatric disorders and neurocognitive dysfunction are reported in nearly 50% of patients with chronic HCV infection, which are independent of the severity of liver disease or HCV replication rates. Fatigue, sleep disturbance, depression and reduced quality of life are commonly associated with neurocognitive alterations in patients with non-cirrhotic chronic HCV infection, regardless of the stage of liver fibrosis and the infecting genotype. These manifestations, which are the topic of this review, typically occur in the absence of structural brain damage or signal abnormalities on conventional brain magnetic resonance imaging(MRI), although metabolic and microstructural changes can be detected by in vivo proton magnetic resonance spectroscopy, perfusion-weighted and diffusion tensorMRI, and neurophysiological tests of cognitive processing. Several lines of evidence, including comparative and longitudinal neuropsychological assessments in patients achieving spontaneous or treatment-induced viral clearance, support a major pathogenic role for HCV in neuropsychiatric and neurocognitive disorders.展开更多
Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in su...Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease. Further work up for the cause of recent hypertension included a contrast computerized tomography of abdomen, which revealed concomitant pathologies of chronic liver disease and multiple aneurysms in bilateral kidneys. This case illustrates the unusual presentation of extrahepatic manifestation of viral hepatitis in the form of PAN of kidneys. PAN as an independent entity may be missed in specialized clinics evaluating liver pathologies, due to its insidious onset, atypical clinical symptoms and multi-systemic manifestations. The knowledge of extrahepatic, renal and vascular manifestations of hepatitis B unrelated to liver disease should be considered by physicians at the time of diagnosis and management of patients with HBV.展开更多
We have read with interest the Letter to the Editor by Drs.Zhuang and Zhong,who presented the clinical data of 68 patients with Wilson’s disease(WD)who were admitted to the hospital before and during the coronavirus ...We have read with interest the Letter to the Editor by Drs.Zhuang and Zhong,who presented the clinical data of 68 patients with Wilson’s disease(WD)who were admitted to the hospital before and during the coronavirus disease 2019(COVID-19)pandemic,and appreciated their findings on hepatic and some extrahepatic manifestations.Nevertheless,given the strong impact of the pandemic on patients with neurological and psychiatric disorders,we would have expected a worsening of the psychiatric and/or neurological impairments in these patients.In contrast,according to the authors,these manifestations remained,somewhat unexpectedly,unchanged.This finding is in contrast with most of the current literature that highlights not only an increased incidence of mental health disorders in the general population but also an exacerbation of neurological and psychiatric symptoms in patients with chronic diseases,especially in those with pre-existing neuropsychiatric disorders,such as WD.Although the study was mainly focused on the hepatic features of WD patients taking anti-copper treatment,a generic and cumulative definition of neurological and psychiatric manifestations,as in this study,does not allow for further considerations.Future studies during and after the pandemic are necessary to clarify the real impact,either direct or indirect,of the COVID-19 pandemic on the neurological and psychiatric symptoms of WD patients.展开更多
Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a signific...Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a significant number of extrahepatic manifestations (EHMs).Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime.The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders,including lymphomas and immune-complex deposit diseases causing local and/or systemic complications.Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement,mainly skin,kidney,peripheral nerves,and salivary glands,and less frequently causes widespread vasculitis and malignant lymphoma.MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance,i.e.>6%.Severe disease requires immunosuppressive or plasma exchange therapy.HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%,much higher than that in general population.Therefore,all patients with PCT should be screened for HCV.The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands.Skin manifestations due to PCT usually respond to anti-HCV treatment together with reducing skin sun exposure,avoiding triggers,having routine phlebotomy (especially for people with chronic iron overload states),and using chloroquine.Lichen planus (LP),which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population.The prevalence of HCV in patients with LP varies based on geographic location.We review here the basic pathophysiology,clinical features,and management of dermatologic manifestations of HCV.展开更多
Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in childr...Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.展开更多
文摘Cholangiocarcinoma(CCA) is a malignant tumour that arises from biliary epithelium at any portion of the biliary tree.CCA is currently classified as intra-hepatic or extra-hepatic CCA(EH-CCA).Recent evidences suggest that intra-hepatic CCA(IH-CCA) and EH-CCA are biologically different cancers,giving further support to a number of recent epidemiological studies showing large differences in terms of incidence,mortality and risk factors.The purpose of this manuscript is to review recent literature dealing with the descriptive epidemiology and risk factors of CCA with a special effort to compare IH-with EH-CCA.
文摘The liver has a central role in regulating inflammation by its capacity to secrete a number of proteins that control both local and systemic inflammatory responses. Chronic inflammation or an exaggerated inflammatory response can produce detrimental effects on target organs. Chronic hepatitis C virus(HCV) infection causes liver inflammation by complex and not yet well-understood molecular pathways, including direct viral effects and indirect mechanisms involving cytokine pathways,oxidative stress and steatosis induction. An increasing body of evidence recognizes the inflammatory response in chronic hepatitis C as pathogenically linked to the development of both liver-limited injury(fibrosis, cirrhosis and hepatocellular carcinoma) and extrahepatic HCV-related diseases(lymphoproliferative disease,atherosclerosis, cardiovascular and brain disease). Defining the complex mechanisms of HCV-induced inflammation could be crucial to determine the global impact of infection, to estimate progression of the disease, and to explore novel therapeutic approaches to avert HCVrelated diseases. This review focuses on HCV-related clinical conditions as a result of chronic liver and systemic inflammatory states.
基金supported by the grant from the Science&Technology Support Project of Sichuan Province(No.2018JY0019).
文摘Background:Whether the extra-hepatic bile duct(EHBD)should be routinely resected for gallbladder carcinoma(GBC)remains controversial.The current study aimed to determine the clinical impact of combined EHBD resection during curative surgery for advanced GBC.Methods:In total,213 patients who underwent curative surgery for T2,T3 or T4 GBC were enrolled.The clinicopathological features were compared between the patients treated with EHBD resection and those without EHBD resection.Meanwhile,univariable and multivariable Cox-proportional hazards regression models were used to identify risk factors for overall survival(OS).Results:Among the 213 patients identified,87(40.8%)underwent combined EHBD resection.Compared with patients without EHBD resection,patients with EHBD resection suffered more post-operative complications(33.3%vs.21.4%,P=0.046).However,the median OS of the EHBD resection group was longer than that of the non-EHBD resection group(25 vs.11 months,P=0.008).Subgroup analyses were also performed according to tumor(T)category and lymph-node metastasis.The median OS was significantly longer in the EHBD resection group than in the non-EHBD resection group for patients with T3 lesion(15 vs.7 months,P=0.002),T4 lesion(11 vs.6 months,P=0.021)or lymph-node metastasis(12 vs.7 months,P<0.001).No survival benefit of EHBD resection was observed in GBC patients with T2 lesion or without lymph-node metastasis.T category,lymph-node metastasis,margin status,pre-operative CA19-9 level and EHBD resection were identified as independent prognostic factors for OS of patients with advanced GBC(all P values<0.05).Conclusions EHBD resection can independently affect the OS in advanced GBC.For GBC patients with T3 lesion,T4 lesion and lymph-node metastasis,combined EHBD resection is justified and may improve OS.
基金Supported by the Medical and Health Technology Plan of Zhejiang Province,No.2020KY1086 and No.2022ZH086。
文摘BACKGROUND Primary liver cancer is one of the most common malignant tumours,while primary splenic lymphoma is a rare malignancy.Thus,cases of hepatocellular carcinoma(HCC)combined with splenic lymphoma are extremely rare.CASE SUMMARY We present a 62-year-old woman who was admitted to the Interventional Radiology Department with a lump in the spleen and liver as well as multiple enlarged lymph nodes visible by ultrasound.Contrast-enhanced computed of the abdomen revealed a circular,low-density,shallow mass(approximately 2.6 cm in diameter)in the left intrahepatic lobe and multiple round,low-density shadows in the spleen with clear boundaries(maximum diameter 7.6 cm).Based on the characteristic clinical symptoms and explicit radiological findings,the clinical diagnosis was HCC with metastasis to the liver portal,retroperitoneal lymph nodes,and spleen.After transcatheter arterial chemoembolization and sequential radiofrequency ablation,the-fetoprotein level returned to the normal range,and the hepatitis B cirrhosis improved.In addition,splenic tumour biopsy confirmed the diagnosis of primary malignant lymphoma,which went into remission after chemotherapy.CONCLUSION HCC with primary splenic non-Hodgkin lymphoma is extremely rare and easily misdiagnosed.Better understanding would facilitate early diagnosis,treatment and prognosis.
基金Supported by Ministero della Salute,Italy,No.RF-2011-02347955 to Monaco S
文摘Since its identification in 1989, hepatitis C virus(HCV) has emerged as a worldwide health problem with roughly 185 million chronic infections, representing individuals at high risk of developing cirrhosis and liver cancer. In addition to being a frequent cause of morbidity and mortality due to liver disease, HCV has emerged as an important trigger of lymphoproliferative disorders, owing to its lymphotropism, and of a wide spectrum of extrahepatic manifestations(HCV-EHMs) affecting different organ systems. The most frequently observed HCV-EHMs include mixed cryoglobulinemia and cryoglobulinemic vasculitis, B-cell non-Hodgkin's lymphoma, nephropathies, thyreopathies, type 2 diabetes mellitus, cardiovascular diseases, and several neurological conditions. In addition, neuropsychiatric disorders and neurocognitive dysfunction are reported in nearly 50% of patients with chronic HCV infection, which are independent of the severity of liver disease or HCV replication rates. Fatigue, sleep disturbance, depression and reduced quality of life are commonly associated with neurocognitive alterations in patients with non-cirrhotic chronic HCV infection, regardless of the stage of liver fibrosis and the infecting genotype. These manifestations, which are the topic of this review, typically occur in the absence of structural brain damage or signal abnormalities on conventional brain magnetic resonance imaging(MRI), although metabolic and microstructural changes can be detected by in vivo proton magnetic resonance spectroscopy, perfusion-weighted and diffusion tensorMRI, and neurophysiological tests of cognitive processing. Several lines of evidence, including comparative and longitudinal neuropsychological assessments in patients achieving spontaneous or treatment-induced viral clearance, support a major pathogenic role for HCV in neuropsychiatric and neurocognitive disorders.
文摘Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease. Further work up for the cause of recent hypertension included a contrast computerized tomography of abdomen, which revealed concomitant pathologies of chronic liver disease and multiple aneurysms in bilateral kidneys. This case illustrates the unusual presentation of extrahepatic manifestation of viral hepatitis in the form of PAN of kidneys. PAN as an independent entity may be missed in specialized clinics evaluating liver pathologies, due to its insidious onset, atypical clinical symptoms and multi-systemic manifestations. The knowledge of extrahepatic, renal and vascular manifestations of hepatitis B unrelated to liver disease should be considered by physicians at the time of diagnosis and management of patients with HBV.
文摘We have read with interest the Letter to the Editor by Drs.Zhuang and Zhong,who presented the clinical data of 68 patients with Wilson’s disease(WD)who were admitted to the hospital before and during the coronavirus disease 2019(COVID-19)pandemic,and appreciated their findings on hepatic and some extrahepatic manifestations.Nevertheless,given the strong impact of the pandemic on patients with neurological and psychiatric disorders,we would have expected a worsening of the psychiatric and/or neurological impairments in these patients.In contrast,according to the authors,these manifestations remained,somewhat unexpectedly,unchanged.This finding is in contrast with most of the current literature that highlights not only an increased incidence of mental health disorders in the general population but also an exacerbation of neurological and psychiatric symptoms in patients with chronic diseases,especially in those with pre-existing neuropsychiatric disorders,such as WD.Although the study was mainly focused on the hepatic features of WD patients taking anti-copper treatment,a generic and cumulative definition of neurological and psychiatric manifestations,as in this study,does not allow for further considerations.Future studies during and after the pandemic are necessary to clarify the real impact,either direct or indirect,of the COVID-19 pandemic on the neurological and psychiatric symptoms of WD patients.
文摘Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a significant number of extrahepatic manifestations (EHMs).Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime.The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders,including lymphomas and immune-complex deposit diseases causing local and/or systemic complications.Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement,mainly skin,kidney,peripheral nerves,and salivary glands,and less frequently causes widespread vasculitis and malignant lymphoma.MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance,i.e.>6%.Severe disease requires immunosuppressive or plasma exchange therapy.HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%,much higher than that in general population.Therefore,all patients with PCT should be screened for HCV.The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands.Skin manifestations due to PCT usually respond to anti-HCV treatment together with reducing skin sun exposure,avoiding triggers,having routine phlebotomy (especially for people with chronic iron overload states),and using chloroquine.Lichen planus (LP),which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population.The prevalence of HCV in patients with LP varies based on geographic location.We review here the basic pathophysiology,clinical features,and management of dermatologic manifestations of HCV.
文摘Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.
