BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury...BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury and bleeding in rural areas in China. This study was undertaken to better understand VOD and its relations to the effect of Gymura segetum. METHODS: Between 2000 and 2002,two patients were admitted to our department because of VOD. Before admission, both of them had been injured and taken oral decoction of patent drug Gymura segetum. We analyzed the clinical manifestations, diagnosis and therapy of the two patients. RESULTS: Pyrrolizidine in Panax notginseng was proved to induce VOD. The diagnosis of VOD depended on hepatic biopsy. CONCLUSION: Gymura segetum can induce VOD. More attention should be paid to its unsuscepted side effects.展开更多
Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is co...Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is commonly given to transplant recipients from the start of conditioning through the early weeks of transplant. However, high quality evidence from randomized controlled trials is scarce with small sample sizes and the trials yielded conflicting results. Although various treatment options for hepatic VOD are available, most have not undergone stringent evaluation with randomized controlled trial and therefore it remains uncertain which treatment offers real benefit. It remains controversial whether VOD prophylaxis should be given, which prophylactic therapy should be given, who should receive prophylaxis, and what treatment should be offered once VOD is established.展开更多
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
AIM: To analyze the association between plasma bilirubin levels and veno-occlusive disease(VOD) in non-adult patients undergoing hematopoietic stem cell transplantation(HSCT) during cyclosporine therapy.METHODS: A tot...AIM: To analyze the association between plasma bilirubin levels and veno-occlusive disease(VOD) in non-adult patients undergoing hematopoietic stem cell transplantation(HSCT) during cyclosporine therapy.METHODS: A total of 123 patients taking cyclosporinewere evaluated using an electronic medical system at the Seoul National University Children's Hospital from the years 2004 through 2011. Patients were grouped by age and analyzed for incidence and type of adverse drug reactions(ADRs) including VOD. RESULTS: The HSCT patients were divided into three age groups: G#1 ≥ 18; 9 ≤ G#2 ≤ 17; and G#3 ≤ 8 years of age). The majority of transplant donor types were cord blood transplantations. Most prevalent ADRs represented acute graft-vs-host disease(a GVHD) and VOD. Although the incidences of a GVHD did not vary among the groups, the higher frequency ratios of VOD in G#3 suggested that an age of 8 or younger is a risk factor for developing VOD in HSCT patients. After cyclosporine therapy, the trough plasma concentrations of cyclosporine were lower in G#3 than in G#1, indicative of its increased clearance. Moreover, in G#3 only, a maximal total bilirubin level(BILmax) of ≥ 1.4 mg/d L correlated with VOD incidence after cyclosporine therapy. CONCLUSION: HSCT patients 8 years of age or younger are more at risk for developing VOD, diagnosed as hyperbilirubinemia, tender hepatomegaly, and ascites/weight gain after cyclosporine therapy, which may be represented by a criterion of plasma BILmax being ≥ 1.4 mg/d L, suggestive of more sensitive VOD indication in this age group.展开更多
The Hepatic sinusoidal obstruction syndrome (HSOS), also known as veno-occlusive disease (VOD), is a well-known complication of haematopoietic stem cell transplantation, of the treatment of Wilms tumor and rhabdomyosa...The Hepatic sinusoidal obstruction syndrome (HSOS), also known as veno-occlusive disease (VOD), is a well-known complication of haematopoietic stem cell transplantation, of the treatment of Wilms tumor and rhabdomyosarcoma and maintenance therapy of acute lymphoblastic leukemias. Its occurrence is rare in other cancers of the child. We report the observation of a 7-year-old girl with Burkitt’s lymphoma who developed a severe HSOS during her second induction treatment with dexamethasone, cisplatin, cytosine arabinoside. The evolution was fatal. This observation shows that the diagnosis of HSOS should not be excluded in the absence of the risk factors usually described.展开更多
We report a panel of severe inflammatory and vascular intraocular disorders occurring during interferon-alpha （IFN-α） treatment in eight hepatitis C virus （HCV）- infected patients. These events include three case...We report a panel of severe inflammatory and vascular intraocular disorders occurring during interferon-alpha （IFN-α） treatment in eight hepatitis C virus （HCV）- infected patients. These events include three cases of Vogt-Koyanagi-Harada like （VKH） disease （an association of panuveitis, retinal detachment, ear and meningeal detachment and skin and hair changes）, two cases of central retinal vein occlusion, one case of central retinal artery occlusion, one case of severe hypertensive retinopathy and one case of bilateral ischemic optic neuropathy with severe visual impairment. Rare as they are, such severe ophthalmological complications require a close follow-up of HCV-infected patients under IFN-α breabnent with ophthalmological monitoring if any ocular manifestation occurs.展开更多
Background Budd-Chiari syndrome with hepatic vein occlusion （HVBCS） can induce severe portal hypertension and liver damage. We retrospectively analyzed hepatic CT features of HVBCS and evaluated the usefulness of tr...Background Budd-Chiari syndrome with hepatic vein occlusion （HVBCS） can induce severe portal hypertension and liver damage. We retrospectively analyzed hepatic CT features of HVBCS and evaluated the usefulness of triphasic enhancement of CT examinations and CT angiography （CTA） in its diagnosis. Methods Twenty-five cases with HVBCS, confirmed by digital subtraction angiography （DSA）, received a triphasic enhancement CT scan within one week before DSA. The CTA images of the relevant blood vessels were reconstructed with maximum intensity projection, volume rendering and oblique reformat techniques. Results Compared with DSA, the detection rate of transverse CT and CTA images for abnormal hepatic vein were 81.7% （58/71） and 95.8% （68/71） （X^2=7.044, P=-0.008）, for membranous obstruction were 47.4% （9/19） and 84.2% （16/19） respectively （X^2 =5.729, P=-0.017 ）, for segmental obstruction were 88.0% （22/25） and 100% （25/25） respectively （X^2=1.418, P=-0.234）. The detection rates for hepatic vein stenosis were 100% with each method. Diffuse hepatomegaly was found in all 6 cases in acute phase and 3 of 19 cases in chronic phase who had severe obstruction of three hepatic veins without patent intrahepatic collaterals. The other 16 cases in chronic phase had hepatatrophia to different extents related to the obstructed hepatic vein. All in acute phase and 15 in chronic phase presented typical patchy enhancement initially in caudate lobe and perihilar areas and enlarged with time delay. In all cases, parenchyma areas with atrophy, necrosis and congestion demonstrated lower and later enhancement. In all the parts, which had normal enhancement at least one patent outflow hepatic vein, accessory hepatic vein or collateral vessel was detected.Conclusion Dynamic enhancement CT examination by multislice spiral CT not only could improve the diagnosis of HVBCS by CTA technique, but also could noninvasively provide anatomical information and reveal damage to the hepatic parenchyma.展开更多
文摘BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury and bleeding in rural areas in China. This study was undertaken to better understand VOD and its relations to the effect of Gymura segetum. METHODS: Between 2000 and 2002,two patients were admitted to our department because of VOD. Before admission, both of them had been injured and taken oral decoction of patent drug Gymura segetum. We analyzed the clinical manifestations, diagnosis and therapy of the two patients. RESULTS: Pyrrolizidine in Panax notginseng was proved to induce VOD. The diagnosis of VOD depended on hepatic biopsy. CONCLUSION: Gymura segetum can induce VOD. More attention should be paid to its unsuscepted side effects.
文摘Hepatic veno-occlusive disease(VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is commonly given to transplant recipients from the start of conditioning through the early weeks of transplant. However, high quality evidence from randomized controlled trials is scarce with small sample sizes and the trials yielded conflicting results. Although various treatment options for hepatic VOD are available, most have not undergone stringent evaluation with randomized controlled trial and therefore it remains uncertain which treatment offers real benefit. It remains controversial whether VOD prophylaxis should be given, which prophylactic therapy should be given, who should receive prophylaxis, and what treatment should be offered once VOD is established.
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.
基金Supported by The Education and Research Encouragement Fund of Seoul National University Hospital
文摘AIM: To analyze the association between plasma bilirubin levels and veno-occlusive disease(VOD) in non-adult patients undergoing hematopoietic stem cell transplantation(HSCT) during cyclosporine therapy.METHODS: A total of 123 patients taking cyclosporinewere evaluated using an electronic medical system at the Seoul National University Children's Hospital from the years 2004 through 2011. Patients were grouped by age and analyzed for incidence and type of adverse drug reactions(ADRs) including VOD. RESULTS: The HSCT patients were divided into three age groups: G#1 ≥ 18; 9 ≤ G#2 ≤ 17; and G#3 ≤ 8 years of age). The majority of transplant donor types were cord blood transplantations. Most prevalent ADRs represented acute graft-vs-host disease(a GVHD) and VOD. Although the incidences of a GVHD did not vary among the groups, the higher frequency ratios of VOD in G#3 suggested that an age of 8 or younger is a risk factor for developing VOD in HSCT patients. After cyclosporine therapy, the trough plasma concentrations of cyclosporine were lower in G#3 than in G#1, indicative of its increased clearance. Moreover, in G#3 only, a maximal total bilirubin level(BILmax) of ≥ 1.4 mg/d L correlated with VOD incidence after cyclosporine therapy. CONCLUSION: HSCT patients 8 years of age or younger are more at risk for developing VOD, diagnosed as hyperbilirubinemia, tender hepatomegaly, and ascites/weight gain after cyclosporine therapy, which may be represented by a criterion of plasma BILmax being ≥ 1.4 mg/d L, suggestive of more sensitive VOD indication in this age group.
文摘The Hepatic sinusoidal obstruction syndrome (HSOS), also known as veno-occlusive disease (VOD), is a well-known complication of haematopoietic stem cell transplantation, of the treatment of Wilms tumor and rhabdomyosarcoma and maintenance therapy of acute lymphoblastic leukemias. Its occurrence is rare in other cancers of the child. We report the observation of a 7-year-old girl with Burkitt’s lymphoma who developed a severe HSOS during her second induction treatment with dexamethasone, cisplatin, cytosine arabinoside. The evolution was fatal. This observation shows that the diagnosis of HSOS should not be excluded in the absence of the risk factors usually described.
文摘We report a panel of severe inflammatory and vascular intraocular disorders occurring during interferon-alpha （IFN-α） treatment in eight hepatitis C virus （HCV）- infected patients. These events include three cases of Vogt-Koyanagi-Harada like （VKH） disease （an association of panuveitis, retinal detachment, ear and meningeal detachment and skin and hair changes）, two cases of central retinal vein occlusion, one case of central retinal artery occlusion, one case of severe hypertensive retinopathy and one case of bilateral ischemic optic neuropathy with severe visual impairment. Rare as they are, such severe ophthalmological complications require a close follow-up of HCV-infected patients under IFN-α breabnent with ophthalmological monitoring if any ocular manifestation occurs.
文摘Background Budd-Chiari syndrome with hepatic vein occlusion （HVBCS） can induce severe portal hypertension and liver damage. We retrospectively analyzed hepatic CT features of HVBCS and evaluated the usefulness of triphasic enhancement of CT examinations and CT angiography （CTA） in its diagnosis. Methods Twenty-five cases with HVBCS, confirmed by digital subtraction angiography （DSA）, received a triphasic enhancement CT scan within one week before DSA. The CTA images of the relevant blood vessels were reconstructed with maximum intensity projection, volume rendering and oblique reformat techniques. Results Compared with DSA, the detection rate of transverse CT and CTA images for abnormal hepatic vein were 81.7% （58/71） and 95.8% （68/71） （X^2=7.044, P=-0.008）, for membranous obstruction were 47.4% （9/19） and 84.2% （16/19） respectively （X^2 =5.729, P=-0.017 ）, for segmental obstruction were 88.0% （22/25） and 100% （25/25） respectively （X^2=1.418, P=-0.234）. The detection rates for hepatic vein stenosis were 100% with each method. Diffuse hepatomegaly was found in all 6 cases in acute phase and 3 of 19 cases in chronic phase who had severe obstruction of three hepatic veins without patent intrahepatic collaterals. The other 16 cases in chronic phase had hepatatrophia to different extents related to the obstructed hepatic vein. All in acute phase and 15 in chronic phase presented typical patchy enhancement initially in caudate lobe and perihilar areas and enlarged with time delay. In all cases, parenchyma areas with atrophy, necrosis and congestion demonstrated lower and later enhancement. In all the parts, which had normal enhancement at least one patent outflow hepatic vein, accessory hepatic vein or collateral vessel was detected.Conclusion Dynamic enhancement CT examination by multislice spiral CT not only could improve the diagnosis of HVBCS by CTA technique, but also could noninvasively provide anatomical information and reveal damage to the hepatic parenchyma.
