BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or ...BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.展开更多
Background:Minimal hepatic encephalopathy(MHE)is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurOphysiolOgic tests.We aimed to diagnose MHE in children wi...Background:Minimal hepatic encephalopathy(MHE)is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurOphysiolOgic tests.We aimed to diagnose MHE in children with extrahepatic portal vein obstruction fEHPV01 and to evaluate the el№ct of lactulose on MHE.Methods:A prospective study was carried out on 30 patients with EHPVO(21 males;mean age 10+2.5 years).The study was carried out in the Pediatric Hepatology Unit.Cairo University Pediatric Hospital,Cairo,Egypt,between 2011 and 2013.AII patients were subjected to clinical and Iaboratory assessment.neuropsychmetric testin2 using the arabic version of Wechsler intelligence tests.neurophysiological testing by visual electroencephalogram and P300 event related potentials(ERP).展开更多
AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies condu...AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.展开更多
A variety of hepatic and biliary tract disorders may complicate the clinical course of celiac disease. Some of these have been hypothesized to share common genetic factors or have a common immunopathogenesis, such as ...A variety of hepatic and biliary tract disorders may complicate the clinical course of celiac disease. Some of these have been hypothesized to share common genetic factors or have a common immunopathogenesis, such as primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune forms of hepatitis or cholangitis. Other hepatic changes in celiac disease may be associated with malnutrition resulting from impaired nutrient absorption, including hepatic steatosis. In addition, celiac disease may be associated with rare hepatic complications, such as hepatic T-cell lymphoma. Finally, pancreatic exocrine function may be impaired in celiac disease and represent a cause of treatment failure.展开更多
Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in children.Variceal bleed and splenomegaly are their predominant presentation.Laboratory features show cytopenias(hypersplenism)...Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in children.Variceal bleed and splenomegaly are their predominant presentation.Laboratory features show cytopenias(hypersplenism)and preserved hepatic synthetic functions.Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases.After variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO.Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate.Shunt surgeries prevent rebleed,improve growth and QoL.Non-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade,massive splenomegaly and patent portal vein are discriminating features of NCPF.Shunt surgery is required in severe cases when endotherapy is insufficient for the varices.Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly.Ductal plate malformation forms the histological hallmark of CHF.CHF is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy.Liver transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s syndrome.Combined liver-kidney transplantation is indicated when both liver and renal issues are present.展开更多
文摘BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.
文摘Background:Minimal hepatic encephalopathy(MHE)is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurOphysiolOgic tests.We aimed to diagnose MHE in children with extrahepatic portal vein obstruction fEHPV01 and to evaluate the el№ct of lactulose on MHE.Methods:A prospective study was carried out on 30 patients with EHPVO(21 males;mean age 10+2.5 years).The study was carried out in the Pediatric Hepatology Unit.Cairo University Pediatric Hospital,Cairo,Egypt,between 2011 and 2013.AII patients were subjected to clinical and Iaboratory assessment.neuropsychmetric testin2 using the arabic version of Wechsler intelligence tests.neurophysiological testing by visual electroencephalogram and P300 event related potentials(ERP).
文摘AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
文摘A variety of hepatic and biliary tract disorders may complicate the clinical course of celiac disease. Some of these have been hypothesized to share common genetic factors or have a common immunopathogenesis, such as primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune forms of hepatitis or cholangitis. Other hepatic changes in celiac disease may be associated with malnutrition resulting from impaired nutrient absorption, including hepatic steatosis. In addition, celiac disease may be associated with rare hepatic complications, such as hepatic T-cell lymphoma. Finally, pancreatic exocrine function may be impaired in celiac disease and represent a cause of treatment failure.
文摘Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in children.Variceal bleed and splenomegaly are their predominant presentation.Laboratory features show cytopenias(hypersplenism)and preserved hepatic synthetic functions.Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases.After variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO.Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate.Shunt surgeries prevent rebleed,improve growth and QoL.Non-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade,massive splenomegaly and patent portal vein are discriminating features of NCPF.Shunt surgery is required in severe cases when endotherapy is insufficient for the varices.Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly.Ductal plate malformation forms the histological hallmark of CHF.CHF is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy.Liver transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s syndrome.Combined liver-kidney transplantation is indicated when both liver and renal issues are present.
