Clinical profile and outcomes of hepatocellular carcinoma in primary Budd-Chiari syndrome

BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.

A Budd-Chiari Syndrome Due to C Protein Deficiency: A Case Report at YaoundéGeneral Hospital (Cameroon)

Primary Budd-Chiari syndrome (BCS) is a spontaneously fatal disease characterized by an obstruction of the hepatic venous outflow tract due to thrombosis or a primary disease of the venous wall. The primary form of BCS is extremely rare. This is a disease mainly affecting young adults of both sexes. Clinical manifestations are variable;they can be asymptomatic, acute, or subacute but mostly chronic. Several causes have been identified, such as myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and inherited thrombotic disorders. Data on primary BCS in Sub-Saharan Africa is rare as most publications available are case reports. In these reports, the causes are unknown with poor prognosis in most cases often leading to patient death. We herein present a case report of a male patient diagnosed with a primary BCS at Yaoundé General Hospital (Cameroon) caused by a Protein C deficiency who presented with ascites decompensating liver cirrhosis. Treatment was based on anticoagulants, diuretics and laxatives administration. Two years after the diagnosis, the patient is alive with clinical and paraclinical improvement.

ERAS管理模式联合预防性护理在Budd-Chiari综合征合并肝静脉血栓患者中的应用

目的探讨快速康复外科(ERAS)管理模式联合预防性护理在Budd-Chiari综合征(BCS)合并肝静脉血栓行介入治疗患者中的应用。方法选取2018年1月至2022年12月徐州医科大学附属医院收治的366例BCS合并肝静脉血栓患者,采用随机数字表法将其分成2组(各183例),即对照组(常规护理)与观察组(ERAS管理模式联合预防性护理)。比较2组干预后的效果。结果相比于对照组,观察组的各项健康相关认知水平优秀率均更高,并发症总计发生率更低(P均<0.05)。观察组在术中、术后即刻的应激指标均明显低于对照组(P均<0.05)。术后1周,观察组的门静脉血流动力学指标均优于对照组(P均<0.05)。结论对BCS合并肝静脉血栓行介入治疗患者实施ERAS管理模式联合预防性护理,有助于提高其健康相关认知水平,改善应激指标和门静脉血流动力学指标,减少并发症的发生。

Budd-Chiari syndrome:Etiology,pathogenesis and diagnosis

Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. Hypercoagulable state could be identified in 75% of the patients; more than one etiologic factor may play a role in 25% of the patients. Primary myeloproliferative diseases are the leading cause of the disease. Two of the hepatic veins must be blocked for clinically evident disease. Liver congestion and hypoxic damage of hepatocytes eventually result in predominantly centrilobular fibrosis. Doppler ultrasonography of the liver should be the initial diagnostic procedure. Hepatic venography is the reference procedure if required. Additionally liver biopsy may be helpful for differential diagnosis. The prognosis of the chronic form is acceptable compared to other chronic liver diseases.

Hepatocellular carcinoma in Budd-Chiari syndrome:A single center experience with long-term follow-up in South Korea

AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS between June 1988 and May 2008.The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or venography.The median follow-up period was 103 ± 156 [interquartile range(IQR)] mo.RESULTS:The median age of the patients was 47 ± 16(IQR) years.At diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class C.During the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 years.In patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 years.CONCLUSION:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea.The HVPG is expected to provide additional information for predicting HCC development in BCS patients.

Surgical treatment of Budd-Chiari syndrome:analysis of 221 cases

BACKGROUND:Budd-Chiari syndrome (B-CS) refers to post-hepatic portal hypertension and/or inferior vena cava hypertension caused by obstruction of blood flow at the portal cardinal hepatic vein.The treatments of B-CS include operations on pathological membrane lesions,shunting and combined operations.Studies have shown that China,Japan,India and South Africa have a high incidence of B-CS.In China,the Yellow River Basin in Henan,Shandong,Jiangsu and Anhui Provinces also have a high incidence,around 10 per 100 000.METHODS:The clinical data of 221 B-CS patients were analyzed retrospectively.We focused on pathological types,surgical methods,effectiveness and complications of treatment,and follow-up.RESULTS:Based on imaging findings such as color ultrasonography,angiography or magnetic resonance angiography,the 221 patients were divided into 3 types (five subtypes):type Ia (72 patients),type Ib (20),type II (72),type IIIa (33),and type IIIb (24).Surgical procedures included balloon membranotomy with or without stent (65 patients),improved splenopneumopexy (18),radical resection of membrane and thrombus (17),inferior vena cava bypass [29,with cavocaval transflow (13) and cavoatrial transflow (16)],mesocaval shunt (41),splenocaval shunt (25),splenoatrial shunt (12),splenojugular shunt (6),and combined methods (8).The complication rate was 9.05% (20/221) and the perioperative death rate was 2.26% (5/221).All of the patients were followed up from 6 months to 5 years.The success rate was 84.6% (187/221),and the recurrence rate was 8.9% (9/101) and 13.5% (13/96) after 1-and 5-year follow-up,respectively.CONCLUSION:The rational choice of surgical treatment based on B-CS pathological typing may increase the success rate and decrease the recurrence.

Transjugular intrahepatic portosystemic shunt for severe jaundice in patients with acute Budd-Chiari syndrome

AIM:To evaluate the feasibility of transjugular intrahepatic portosystemic shunt(TIPS)for severe jaundice secondary to acute Budd-Chiari syndrome(BCS).METHODS:From February 2009 to March 2013,37patients with severe jaundice secondary to acute BCS were treated.Sixteen patients without hepatic venule,hepatic veins(HV)obstruction underwent percutaneous angioplasty of the inferior vena cava(IVC)and/or HVs.Twenty-one patients with HV occlusion underwent TIPS.Serum bilirubin,liver function,demographic data and operative data of the two groups of patients were analyzed.RESULTS:Twenty-one patients underwent TIPS and the technical success rate was 100%,with no technical complications.Sixteen patients underwent recanalization of the IVC and/or HVs and the technical success rate was 100%.The mean procedure time for TIPS was 84.0±12.11 min and angioplasty was44.11±5.12 min(P<0.01).The mean portosystemic pressure in the TIPS group decreased significantly from 40.50±4.32 to 16.05±3.50 mm Hg(P<0.01).The mean portosystemic pressure gradient decreased significantly from 33.60±2.62 to 7.30±2.21 mm Hg(P<0.01).At 8 wk after the procedures,in the TIPS group,total bilirubin(TBIL)decreased significantly from 266.24±122.03 before surgery to 40.11±3.52μmol/L(P<0.01)and direct bilirubin(DBIL)decreased significantly from 194.22±69.82μmol/L to 29.82±3.10μmol/L(P<0.01).In the angioplasty group,bilirubin returned to the normal range,with TBIL decreased significantly from 258.22±72.71μmol/L to 13.33±3.54μmol/L(P<0.01)and DBIL from175.08±39.27 to 4.03±1.74μmol/L(P<0.01).Liver function improved faster than TBIL.After 2 wk,in the TIPS group,alanine aminotransferase(ALT)decreased significantly from 50.33±40.61 U/L to 28.67±7.02U/L(P<0.01)and aspartate aminotransferase(AST)from 49.46±34.33 U/L to 26.89±8.68 U/L(P<0.01).In the angioplasty group,ALT decreased significantly from 51.56±27.90 to 14.22±2.59μmol/L(P<0.01)and AST from 60.66±39.89μmol/L to 8.18±1.89μmol/L(P<0.01).After mean follow-up of 12.6 mo,there was no recurrence of jaundice in either group.CONCLUSION:Severe jaundice is not a contraindication for TIPS in patients with acute BCS and TIPS is appropriate for severe jaundice due to BCS.

Budd-Chiari syndrome in China: A 30-year retrospective study on survival from a single center

AIM To investigate 30-year treatment outcomes associated with Budd-Chiari syndrome(BCS) at a tertiary hospital in China.METHODS A total of 256 patients diagnosed with primary BCS at our tertiary hospital between November 1983 and September 2013 were followed and retrospectively studied. Cumulative survival rates and cumulative mortality rates of major causes were calculated by Kaplan-Meier analysis, and the independent predictors of survival were identified using a Cox regression model. RESULTS Thirty-four patients were untreated; however, 222 patients were treated by medicine, surgery, or interventional radiology. Forty-four patients were lost to follow-up; however, 212 patients were followed, 67 of whom died. The symptom remission rates of treated and untreated patients were 81.1%(107/132) and 46.2%(6/13), respectively(P = 0.009). The cumulative 1-, 5-, 10-, 20-, and 30-year survival rates of the treated patients were 93.5%, 81.6%, 75.2%, 64.7%, and 58.2%, respectively; however, the 1-, 5-, 10-, 20-, and 30-year survival rates of the untreated patients were 70.8%, 70.8%, 53.1%, 0%, and unavailable, respectively(P = 0.007). Independent predictors of survival for treated patients were gastroesophageal variceal bleeding(HR = 3.043, 95%CI: 1.363-6.791, P = 0.007) and restenosis(HR = 4.610, 95%CI: 1.916-11.091, P = 0.001). The cumulative 1-, 5-, 10-, 20-, and 30-year mortality rates for hepatocellular carcinoma were 0%, 2.6%, 3.5%, 8%, and 17.4%, respectively.CONCLUSION Long-term survival is satisfactory for treated Chinese patients with BCS. Hepatocellular carcinoma is a chronic complication and should be monitored with long-term follow-up.

Necessity and indications of invasive treatment for Budd-Chiari syndrome

BACKGROUND:The development of collaterals in Budd-Chiari syndrome has been described and these collaterals play an important role in the presentation of this disease.These collaterals are diagnostic and their use in management strategy has never been evaluated.This study aimed to investigate the indications,feasibility and necessity of invasive treatment for patients with Budd-Chiari syndrome and to determine whether such a strategy is necessary for optimal management.METHODS:Twenty-nine patients who had been treated at our unit were enrolled in this study.Based on physical and biochemical examination,and hemodynamic compensation by collaterals,18 patients underwent radiological intervention (group A),while the other 11 had no invasive treatment (group B).The related hemodynamic parameters were acquired when percutaneous angiography was performed.RESULTS:In group A,all patients underwent successfully inferior vena cava (IVC) balloon angioplasty with or without stenting.Four patients also underwent hepatic vein angioplasty.In these patients,the mean IVC pressure before and after treatment was statistically different (29.3±9.2 vs 15.1±4.6 mmHg,P<0.01).The mean IVC pressure was much lower in group B than in group A (12.9±2.4 vs 29.3±9.2 mmHg,P<0.01),but there was no difference from that of the patients after radiological treatment (12.9±2.4 vs 15.1±4.6 mmHg,P>0.05).Median follow-up was 32.3 months (mean 21.3 months;range 3-61 months).In the course of follow-up,the patients in group A survived with good systemic status except for re-stenosis in one patient who underwent re-canalization of the IVC.In group B,10 patients had good systemic status except one patient who had a meso-caval shunt because of deterioration.CONCLUSIONS:The rationale of 'early diagnosis and early treatment' is not suitable for all patients with Budd-Chiari syndrome.Satisfactory survival can be achieved in some patients without invasive treatment,who are completely compensated by rich collaterals.Nonetheless,a positive treatment procedure should be performed if the patient's situation worsens in the course of regular follow-up.

Etiology and portal vein thrombosis in Budd-Chiari syndrome

AIM: To research the etiology, portal vein thrombosis and other features of Budd-Chiari syndrome (BCS) patients prospectively. METHODS: A total of 75 patients (40 female, 35 male) who were diagnosed between January 2002 and July 2004 as having BCS were studied prospectively. Findings from on physical examination, ultrasonography, duplex ultrasonography and venography were analyzed. Hemogram and blood chemistry were studied at the time of diagnosis and on each hospital visit. Bone marrow examination and immune phenotyping were performed by a hematologist when necessary. Protein C, S, antithrombin Ⅲ, activated protein C resistance, and anticardiolipin antibodies, antinuclear antibodies, and anti ds-DNA were studied twice. The presence of ascite, esophageal varices, and portal thrombosis were evaluated at admission and on every visit. RESULTS: At least one etiological factor was determined in 54 (72%) of the patients. The etiology could not be defined in 21 (28%) patients. One etiological factor was found in 39, 2 factors in 14 and 3 factors in 1 patient. The most common cause was the web (16%), the second was Hydatid disease (11%), the third was Behcet’s disease (9%). Portal vein thrombosis was present in 11 patients and at least one etiology was identified in 9 of them (82%). CONCLUSION: Behcet’s disease and hydatid disease are more prominent etiological factors in Turkey than in other countries. Patients with web have an excellent response to treatment without signs of portal veinthrombosis while patients having thrombofilic factors more than one are prone to develop portal vein thrombosis with worse clinical outcome.

Budd-Chiari syndrome management:Lights and shadows

Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy.Anticoagulation and medical therapy should be the first line treatment.Revascularization or TIPS are indicated in case of no response to medical therapy.OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT.However,no clear indica-tion can actually be given about the timing of different treatments.Moreover,there is some concern about treatment of some subgroup of patients,especially regarding the risk of recurrence after liver transplanta-tion.The topic of this paper is to critically review the actual knowledge of BCS management.

Selection of treatment modalities for Budd-Chiari Syndrome in China:A preliminary survey of published literature

AIM:To evaluate the frequency of use of various treatment modalities for Budd-Chiari syndrome(BCS)in China by conducting a preliminary survey of relevant literature.METHODS:All papers regarding the treatment of BCS in Chinese patients were identified by searching PubMed,Chinese Scientific and Technological Journal,and China National Knowledge Infrastructure databases.Data regarding the number of BCS patients treated with different treatment modalities over time were collected.The proportions of BCS patients undergoing various treatment modalities were calculated.RESULTS:Overall,300 of 3005 papers initially retrieved were included.These papers included 23352BCS patients treated with different treatment modalities.The treatment modalities include surgery(n=8625),interventional treatment(n=13940),surgery combined with interventional treatment(n=363),medical therapy(n=277),other treatments(n=91),and no treatment(n=56).After 2005,the number of BCS patients treated with surgery was drastically decreased,but the number of BCS patients who underwent interventional treatment was almost maintained.Shunt surgery was the most common type of surgery(n=3610).Liver transplantation was rarely employed(n=2).Balloon angioplasty with or without stenting was the most common type of interventional treatment(n=13747).Transjugular intrahepatic portosystemic shunt was rarely employed(n=81).CONCLUSION:Selection of treatment modalities for BCS might be different between China and Western countries.Further work should be necessary to establish a unanimous therapeutic strategy for BCS in China.

INTERVENTIONAL OR SEMI-INTERVENTIONAL TREATMENT FOR BUDD-CHIARI SYNDROME

Objective. Report the results of interventional or semi-interventional techniques for 173 patients withBudd-Chiari syndrome.Method. This group included 120 males and 53 females. The pathologic lesions composed of localizedcomplete occlusion of inferior vena cava (IVC) (78), IVC stenosis (49), IVC membrane with a hole(37), membrane of hepatic vein (HV) (3), IVC thrombosis (4), IVC membrane with thrombosis (2)and IVC lesion with occlusion of HV (32). Treatment methods included that I: Percutaneous transinferiorvena cava angioplasty (PTA) (76); Ⅱ: IVC PTA with stent (59); Ⅲ: Percutaneous transhepatic veinrecanalization (3); Ⅳ: ⅣC thrombolysis through a catheter (4); V: Combined transcardiac and trans-femoral venous membranotomy and balloon dilation (22); Ⅵ: Ⅴ and stent (17); Ⅶ: Stenting duringradical surgery (3); Ⅷ: Additional operation after intervention (23).Results. The immediate technique success rate for intervention was 90.1%, for the semi-interventionwas 100%. The ⅣC pressure was reduced from 3 to 29 cmH2O. Complications occurred in 8 cases. Thedeath rate was 2.9%. A follow-up study showed the recurrence rates were 14.5% in ⅣC PTA group,1.7% in ⅣC PTA with stent, 18.2% in combined technique without stent and no recurrence was foundin other groups.Conclusion. The PTA is the first choice for localized lesions. When elastic recoil occurs, immediatestenting is suggested. The semi-interventional approach is advised for PTA failure and more complicatedcases. For those with both ⅣC lesion and occlusion of HV, the additional operation is needed after ⅣCintervention.

Budd-Chiari like syndrome in decompensated alcoholic steatohepatitis and liver cirrhosis

A rare case of pseudo-Budd-Chiari Syndrome in a patientwith decompensated alcoholic liver disease is reported.Although clinical and radiological findings suggestedBudd-Chiari Syndrome, the liver biopsy revealedmicronodular cirrhosis and absence of histological signsof hepatic outflow obstruction.

The application and efficacy of stent place for Budd-Chiari syndrome

Objective To evaluate the application value and efficacy on stent place for Budd-Chiari syndrome(BCS). Methods From January 1990 to May 2017, 2228 patients with BCS were admitted to our institution. The mean age was 43.3 years. Stents were placed in inferior vena cava(IVC), hepatic vein(HV), or both after balloon dilation. During follow-up period, the patency of stent was evaluated by ultrasound regularly and the clinic sign was surveyed by letter, telephone or clinic visit. The restenosis of stent were treated with balloon dilatation and thrombolysis to restore the its function. Results IVC type was diagnosed in 1492 cases, HV type in 510 cases, and mixed type in 226 cases. Eighteen patients aborted treatment because of economic reasons, advanced liver cancer, severe scoliosis, or both bilateral iliac veins and total IVC occlusion. Among the other 2210 cases who underwent endovascular therapy, stents were implanted into IVC in 339 cases, HV in 97 cases, mixed type in 64 cases. The rate of restenosis in IVC stent was 11.50%(39/339). After repeat angioplasty, the long-term patency rate reached to 98.12%. The incidence of HV occlusion caused by IVC stent was 12.09%(n = 41). Restenosis occurred in 47 cases(48.45%) after HV stent placement. However, the 5-year patency rate was 91.75%(89/97) after repeat dilatation and stent re-implantation. The incidence of IVC obstruction caused by HV was 3.33%(3 cases). Conclusion IVC stent placement appears to be an effective treatment for the cases of IVC segmental occlusion, and at the same time, the stent has the dual role of compression and fixation of thrombus and support of lumen. The HV and accessory hepatic vein obstruction could happen when the IVC stent crossed these veins ostium. The incidence of the stent restenosis in the HV was higher than that in the IVC.

Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins

BACKGROUND: Collateralized intraand extra-hepatic routes in patients with Budd-Chiari syndrome (BCS) were important. This study aimed to investigate the feasibility and clinical outcomes of the staged management of BCS based on the degree of compensation provided by intraor extra-hepatic collateral circulations. METHODS: A total of 103 adult patients with BCS caused by co-obstruction of the inferior vena cava (IVC) and main hepatic veins (MHVs) between March 2001 and October 2009 were enrolled in this study. Based on the pathological classification and degree of hemodynamic compensation by collateral circulations, treatment priority for IVC hypertension was determined in the first-stage treatment. Patients were deemed eligible for second-stage treatment when the first-stage treatment failed to relieve. RESULTS: Imaging results revealed that most patients had collateral circulations to different extents. Based on the degree of compensation provided by these collateral circulations, 74 patients underwent single-stage treatment for IVC hypertension, i.e., radiologic intervention (RI) for 61 patients and surgical procedures (SPs) for 13. One patient was treated for portal hypertension. Twenty-nine patients underwent second-stage treatment (25 underwent RI and SP, and 4 only SP). The general morbidity and mortality after all procedures were 8.3% and 1.5%, respectively. After a median follow-up of 35 months, 4 patients underwent second-stage treatment and 7 underwent recanalization of the IVC/MHVs. Two patients died of hepatocellular carcinoma and 1 died of graft obstruction. CONCLUSION: Staged management produces excellent outcomes for patients with BCS caused by co-obstruction of the IVC and MHVs.

Budd-Chiari syndrome in a patient with ulcerative colitis and no inherited coagulopathy

We report a case of 27 year old female patient who was admitted to the hospital with an acute flare up of ulcerative colitis.The patient presented with complaints of persistent abdominal pain and bloody diarrhea despite aggressive therapy for her ulcerative colitis.A CT scan of the abdomen on admission revealed hepatic vein thrombosis,suggesting a diagnosis of Budd-Chiari syndrome.Significantly,an associated thrombosis of the inferior mesenteric vein was also detected.Based on imaging data and clinical assessment,the patient was started on anticoagulation therapy and an extensive work-up for hypercoagulability was initiated.Up to the time of publication,no significant findings suggesting this patient has an underlying coagulation disorder have been found.Based on our search of PUBMED,this report is one of only five reported adult cases of Budd-Chiari Syndrome associated with ulcerative colitis in the English literature in living patients without evidence of a co-existing coagulation disorder.This case highlights the potential for thrombosis at unusual sites in ulcerative colitis patients even in the absence of classical coagulation abnormalities.In addition to the case presented,we provide a brief review of previously reported cases of Budd-Chiari Syndrome occurring in patients with inflammatory bowel disease.

Pentadecapeptide BPC 157 resolves suprahepatic occlusion of the inferior caval vein, Budd-Chiari syndrome model in ratsPentadecapeptide BPC 157 resolves suprahepatic occlusion of the inferior caval vein, Budd-Chiari syndrome model in rats

BACKGROUND Recently,as a possible therapy resolving solution,pentadecapeptide BPC 157 therapy,has been used in alleviating various vascular occlusion disturbances.BPC 157 was previously reviewed as novel mediator of Robert cytoprotection and endothelium protection in the stomach,and gut-brain axis,beneficial therapy in gastrointestinal tract,with particular reference to vascular recruitment,ulcerative colitis and tumor cachexia,and other tissues healing.Here we raised new hypothesis about BPC 157 therapy in the Budd-Chiari syndrome in rats,rapid bypassing of the suprahepatic inferior caval vein occlusion,and rats recovery with the active and effective pharmacotherapy treatment.AIM To investigate Budd-Chiari syndrome model(inferior caval vein suprahepatic occlusion)resolution,since BPC 157 resolves various rat vascular occlusion.METHODS We assessed the activated bypassing pathways between the inferior and superior caval veins and portocaval shunt,counteracted caval/portal hypertension,aortal hypotension,venous/arterial thrombosis,electrocardiogram disturbances,liver and gastrointestinal lesions(i.e.,stomach and duodenum hemorrhages,in particular,congestion).Rats with suprahepatic occlusion of the inferior vena cava by ligation were medicated at 1 min,15 min,24 h,or 48 h post-ligation.Medication consisted of 10μg/kg BPC 157,10 ng BPC 157 or 5 m L/kg saline,administered once as an abdominal bath or intragastric application.Gross and microscopic observations were made,in addition to assessments of electrical activity of the heart(electrocardiogram),portal and caval hypertension,aortal hypotension,thrombosis,hepatomegaly,splenomegaly and venography.Furthermore,levels of nitric oxide,malondialdehyde in the liver and serum enzymes were determined.RESULTS BPC 157 counteracted increased P wave amplitude,tachycardia and ST-elevation,i.e.,right heart failure from acute thrombotic coronary occlusion.The bypassing pathway of the inferior vena cava-azygos(hemiazygos)vein-superior vena cava and portocaval shunt occurred rapidly.Even with severe caval portal hypertension,BPC 157 antagonized portal and caval hypertension and aortal hypotension,and also reduced refractory ascites.Thrombosis of portal vein tributaries,inferior vena cava,and hepatic and coronary arteries was attenuated.In addition,there was reduced pathology of the lungs(severe capillary congestion)and liver(dilated central veins and terminal portal venules),decreased intestine hemorrhagic lesions(substantial capillary congestion,submucosal edema and architecture loss),and increased liver and spleen weight.During the period of ligation,nitric oxide-and malondialdehyde-levels in the liver remained within normal healthy values,and increases in serum enzymes were markedly reduced.CONCLUSION BPC 157 counteracts Budd Chiari syndrome in rats.

Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt

AIM:To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.METHODS:Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men,median age 40 years (range 17-66 years)].Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals.Management consisted of tests for underlying haematological,endocrinological,or hypercoagulative disorders parallel to initiation of specific treatment of BCS.RESULTS:BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders.Fortythree percents had symptoms for less than one week with ascites as the most prevalent finding.Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics.The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo),and none required subsequent liver transplantation.Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics.A total of 14 TIPS revisions were needed,mostly of uncovered stents.Two died during follow-up:One non-TIPS patient worsened after 6 mo and died in relation to transplantation,and one TIPS patient died 4 years after the TIPS-procedure,unrelated to BCS.CONCLUSION:In our BCS cohort TIPS-treated patients have near-complete survival,reduced need for diuretics and compared to historical data a reduced need for liver transplantation.

Imaging and interventions in Budd-Chiari syndrome

Budd-Chiari syndrome(BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension.Clinically,two forms of disease(acute and chronic) are recognized.Mostly the patients present with ascites,hepatomegaly,and portal hypertension.In acute disease the liver is enlarged with thrombosed hepatic veins(HV) and ascites,whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava(IVC),or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC.Due to advances in radiological interventional techniques and hardware,there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation.The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.