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.

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.

Budd-Chiari syndrome in myeloproliferative neoplasms:A review of literature

Myeloproliferative neoplasms(MPNs)are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs.Classical,Philadelphia-negative MPNs,i.e.,polycythemia vera,essential thrombocythemia and primary myelofibrosis,exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites,e.g.,portal,splanchnic or hepatic veins,the placenta or cerebral sinuses.The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury,stasis,elevated leukocyte adhesion,integrins,neutrophil extracellular traps,somatic mutations(e.g.,the V617F point mutation in the JAK2 gene),microparticles,circulating endothelial cells,and other factors,to name a few.Herein,we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs,with a particular focus on its epidemiology,pathogenesis,histopathology,risk factors,classification,clinical presentation,diagnosis,and management.

Fracture of inferior vena cava stent after endovascular treatment for Budd-Chiari syndrome:A case series and literature review

Budd-Chiari syndrome(BCS)is a rare condition characterized by hepatic venous outflow obstruction.Balloon angioplasty,with or without stenting,is the recommended first-line treatment modality in Asian countries.As a supplement to balloon angioplasty,expandable metallic Z-stent deployment can effectively improve long-term inferior vena cava(IVC)patency.Although stent placement is a standard and frequently performed treatment,very few IVC stent-related complications,such as stent fractures,have been reported.Here we present a case series and a comprehensive review of IVC stent fractures in patients with BCS.The most common characteristic of IVC stent fractures is a protrusion of the proximal segment of the IVC stent into the right atrium and its systolic and diastolic movements along with heart rhythms.Accurate stent deployment,large-diameter balloon dilation,patient breath-holding training,preferential selection of a triple stent,and the use of an internal jugular vein approach to stent deployment may ensure precise stent localization and avoid postoperative complications.

Budd-Chiari syndrome in children:Challenges and outcome

Budd-Chiari syndrome(BCS)is an uncommon disease of the liver,characterised by obstruction of the hepatic venous outflow tract.The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe.Compared to adults with BCS,children have primary BCS as the predominant etiology,earlier clinical presentation,and hence better treatment outcome.Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS,though work-up for the same is often unyielding in children.Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield.In recent years,advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS.Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response.Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness.This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.

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.

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.

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome:A comprehensive review

Budd-Chiari syndrome(BCS)is a relatively rare clinical condition with a wide range of symptomatology,caused by the obstruction of the hepatic venous outflow.If left untreated,it has got an high mortality rate.Its management is based on a step-wise approach,depending on the clinical presentation,and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques,such as transjugular intrahepatic portosystemic shunt(TIPS).TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients,including those awaiting orthotopic liver transplantation.In this review the pathophysiology,diagnosis and treatment options of BCS are presented,with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS.Moreover,unresolved issues and future research will be discussed.

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.

Large regenerative nodules in a patient with Budd-Chiari syndrome after TIPS positioning while on the liver transplantation list diagnosed by Gd-EOB-DTPA MRI

BACKGROUND:Large regenerative nodules (LRNs) are hyperplastic benign nodules most commonly associated with Budd-Chiari syndrome (BCS),caused by outflow obstruction of the hepatic veins or vena cava.To our knowledge,no cases of LRNs arising in BCS after transjugular intrahepatic portosystemic shunt (TIPS) positioning and detected by GdEOB-DTPA MRI have been reported in the literature.METHODS:A 58-year-old woman with BCS,on the liver transplantation (LT) list,underwent a follow-up enhanced MRI.Two years earlier,a TIPS had been placed.In 2008,recurrent hepaticoencephalopathy resistant to medical treatment fulfilled the LT criteria for BCS treated with TIPS and the patient was therefore added to the LT list.CT performed before TIPS had not detected any hepatic lesions.CT performed six months after TIPS showed its complete patency but documented two indeterminate hypervascular liver lesions.RESULTS:MRI performed with Gd-EOB-DTPA revealed additional hypervascular lesions with uptake and retention of the medium in the hepatobiliary phase,thus reflecting a benign behavior of hepatocellular composition.These MRI features were related to LRNs as confirmed by histopathologic analysis.CONCLUSIONS:Gd-EOB-DTPA-enhanced MRI is potentially superior to standard imaging using gadolinium chelates or spiral CT,especially for the differential diagnosis of hypervascular lesions.Gd-EOB-DTPA MRI may become the imaging method of choice for evaluating LT list patients with BCS after TIPS placement.

Treatment of membranous Budd-Chiari syndrome: analysis of 480 cases

BACKGROUND: Budd-Chiari syndrome (BCS) presents a kind of disease resulted from the occlusion of hepatic vein and/or the intrahepatic inferior vena cava. Its different pathological types were proposed. According to our expe- rience , the membranous type takes a large part of it, and we tried to explore the best treatment of membranous BCS through the analysis of 480 cases retrospectively. METHOD: The operative results of 480 patients with mem- branous BCS were analysed retrospectively. RESULTS: Patients after Kimura's finger rupture, inter- ventional treatment and membrane resection were followed up with rates of 84.62%, 86.55%, and 87.37%, respective- ly. The effective rates of the three methods were 61.4%, 91.7%, and 90.4%, respectively, and the recurrence rates of the disease after the 3 procedures were 38.6%, 8.3% and 9.6%, respectively. The long-term effects of interventional treatment and resection were significantly better than those of Kimura' s finger rupture (P <0.05). CONCLUSION: Balloon dilatation is the choice for mem- branous BCS. Patients with extensive lesion, thick mem- brane or recurrence after percutaneous transhepatic angio- graphy should undergo membrane resection.

Management of severe Budd-Chiari syndrome:report of 147 cases

BACKGROUND:Budd-Chiari syndrome (BCS) is an un- common disorder caused by the obstruction of hepatic ve- nous outflow and/or the inferior vena cava. Major thera- peutic approaches include operation and radiological inter- vention. This study was conducted to investigate the treat- ment of severe BCS. METHODS: The clinical data of 147 patients with severe BCS who had been treated at our hospital from November 1994 to December 2003 were retrospectively analyzed. RESULTS: One hundred twenty-one patients with BCS un- derwent surgery, including mesocaval C type shunt with ar- tificial graft (82 patients), splenojugular shunt (37), meso- jugular shunt (2), percutaneous transhepatic recanalization and dilatation and/or stent placement of the main hepatic vein (MHV) (12), and combined percutaneous transhepa- tic angioplasty (PTA) and stent placement of the inferior vena cava and mesocaval shunt (14). Follow-up for 6-108 months showed excellent results in 102 patients (69.4%), good results in 40 (27.2% ), and 5 deaths. CONCLUSION: Good results could be obtained in most of patients with BCS after different surgical treatments accord- ing to the pathological changes of the IVC and MHV.

Review of Budd-Chiari Syndrome

This study aims to report the Budd-Chiari syndrome clinical research status and progress that has occurred in over nearly 30 years in China, and emphasize the value of imaging in facilitating the diagnosis of Budd-Chiari syndrome based on more than 2500 cases. Findings on ultrasonography, computed tomography, magnetic resonance imaging, and digital subtraction angiography images are used to propose new Budd-Chiari syndrome types and subtypes. The new subtype classification presented here has important value for guiding interventional treatment.This study also proposes a new concept of anatomical and functional obstruction of hepatic vein that stresses the compensatory value of accessory hepatic vein and azygos vein and describes the risk of manipulation of the communication branch of inferior vena cava obstruction in interventional therapy.

Experts consensus on Chinese nomenclature of Budd-Chiari syndrome

In China,Budd-Chiari syndrome has been transliterated into six names according to the pronunciation of the letters.To standardize and unify the Chinese names of the disease,multi-disciplinary experts suggest translating Budd-Chiari syndrome into hepatic vein inferior venal cava obstruction syndrome as its Chinese name after reaching a consensus through discussion.

Novel roles of lipopolysaccharide and TLR4/NF-κB signaling pathway in inflammatory response to liver injury in Budd-Chiari syndrome

BACKGROUND Budd-Chiari syndrome(BCS)is an uncommon disorder characterized by obstruction of hepatic venous outflow.To date,the exact mechanism underlying hepatic injury derived from the hepatic venous outflow obstruction in BCS remains largely unknown.AIM To assess the role of NF-κB-mediated inflammation in BCS-induced liver injury in humans and rats.METHODS A total of 180 rats were randomly assigned into nine groups,including four BCS model groups(1,3,6 and 12 wk),four sham-operated groups(1,3,6 and 12 wk),and a control group.Lipopolysaccharide(LPS)levels in each group were detected by the Tachypleus Amebocyte Lysate assay.The mRNA and protein levels of TLR4,NF-κB,tumor necrosis factor(TNF)-α,interleukin(IL)-2 and interferon(IFN)-γwere quantified.In addition,60 patients with BCS and 30 healthy controls were enrolled,and their blood samples were analyzed.RESULTS Hepatic and plasma LPS levels were significantly increased in rats.The mRNA and protein expression levels of TLR4,NF-κB and inflammatory cytokines(TNF-α,IL-2 and IFN-γ)in liver tissues were significantly higher in the BCS model groups compared with the other two groups.In addition,the model groups(1,3,6 and 12 wk after BCS induction)showed significant differences in the levels of LPS,TLR4,NF-κB,TNF-α,IL-2 and IFN-γ.Notably,there was a significant correlation between the LPS concentrations and mRNA and protein levels of TLR4,NF-κB and inflammatory cytokines.Importantly,it was revealed that the levels of LPS,TLR4,NF-κB and inflammatory cytokines were significantly greater in chronic BCS patients than healthy controls and acute BCS patients.CONCLUSION LPS level is markedly elevated in BCS,in turn activating the TLR4/NF-κB signaling pathway,leading to induction of inflammatory cytokines(TNF-α,IL-2 and IFN-γ)in response to BCS-induced liver injury.

Outcomes of pregnancy in patients with known Budd-Chiari syndrome

AIM To analyse the risk of pregnancy(a prothrombotic state) in patients with Budd-Chiari Syndrome(BCS). METHODS Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015. RESULTS Out of 53 females with BCS, 7 women had 16 pregnancies. Median age at diagnosis of BCS in these women was 25 years(range 21-34 years). At least one causal factor for BCS was identified in 6 women(86%). Six women had undergone radiological decompressive treatment. All patients had anticoagulation. Six fetuses were lost before 20 wk gestation in 2 women. There were 9 deliveries over 32 wk gestation and one delivery at 27 wk. All infants did well. Seven babies were born by emergency caesarean section. There were no cases of thrombosis. Two patients had notable vaginal(PV) bleeding in 3 pregnancies. None of the patients had variceal haemorrhage. Two patients were diagnosed with pulmonary hypertension, one during pregnancyand the other in the post-partum period. There was no maternal mortality.CONCLUSION Maternal outcomes in patients with treated BCS are favourable and fetal outcomes beyond 20 wk gestation are good. There has been increased rate of caesarean section. Pulmonary hypertension is an important finding that needs further validation. These patients should be managed in centers experienced in treating high-risk pregnancies.

Restenosis after recanalization for Budd-Chiari syndrome: Management and long-term results of 60 patients

BACKGROUND Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction.For Asian Budd-Chiari syndrome patients,the major treatment modality is recanalization(percutaneous transluminal angioplasty with or without stent implantation).The cumulative 1-,5-,and 10-year primary patency rates and survival rates are reported to be excellent or satisfactory,but the long-term outcome of patients with restenosis(the most common complication after recanalization)is unknown.AIM To explore the treatment strategy for restenosis in patients with Budd-Chiari syndrome after interventional therapy and to evaluate the long-term follow-up results.METHODS The clinical data and follow-up results of 60 patients with restenosis after interventional therapy from November 1983 to December 2013 were retrospectively analyzed.RESULTS Sixty patients with restenosis were retrospectively divided into a percutaneous transluminal angioplasty(PTA)group(40 patients)and a PTA+stent group(20 patients)according to the primary recanalization method.For the patients with restenosis in the PTA group,13 refused treatment,and 27 received further treatment;among these patients,five had a second restenosis,two had a third restenosis,and one had a fourth restenosis.For the patients with restenosis in the PTA+stent group,nine refused treatment,ten received PTA alone,and the other received PTA+stent implantation.Among the patients who received further treatment,five had a second restenosis,three had a third restenosis,and one had a fourth restenosis.The 1-,5-,10-,20-,and 25-year cumulative survival rates of the 38 patients who received further treatment after restenosis were 100%,78.3%,78.3%,70.5%,and 70.5%,respectively;however,for the 22 patients who refused treatment,the survival rates were 72.7%,45.9%,30.6%,10.2%,and unavailable,respectively(P<0.001).CONCLUSION Long-term follow-up after interventional therapy is very important.Active treatment for patients with restenosis can improve prognosis,and minimally invasive treatment strategies for restenosis allows to obtain satisfactory results.

Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis:A case report and review of the literature

BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.

Gastric pentadecapeptide BPC 157 in cytoprotection to resolve major vessel occlusion disturbances,ischemia-reperfusion injury following Pringle maneuver,and Budd-Chiari syndrome

The stable gastric pentadecapeptide BPC 157 counteracts various venous occlusion-induced syndromes.Summarized are all these arguments,in the Robert’s cytoprotection concept terms,to substantiate the resolution of different major vessel occlusion disturbances,in particular ischemia-reperfusion injury following the Pringle maneuver and Budd-Chiari syndrome,which was obtained by BPC 157 therapy.Conceptually,there is new point(bypassed occluded or ruptured vessel,the equation endothelium maintenance→epithelium maintenance=blood vessel recruitment and activation towards defect or bypassing vessel occlusion),the recruitment of collateral blood vessels to compensate for vessel occlusion and reestablish blood flow.In this paper,we summarize the evidence of the native cytoprotective gastric pentadecapeptide BPC 157,which is stable in the human gastric juice,is a membrane stabilizer and counteracts gut-leaky syndrome.As a particular target,it is distinctive from the standard peptide growth factors,with particular molecular pathways involved,controlling VEGF and NO pathways.In the early 1990s,BPC 157 appeared as a late outbreak of the Robert’s and Szabo’s cytoprotection-organoprotection concept,epithelium,endothelium protection as previous theoretical/practical breakthrough in the 1980s,and brain-gut axis and gut-brain axis.As the time went on,with its reported effects,it is likely most useful theory practical implementation and justification.Meantime,several reviews suggest that BPC 157,which does not have a lethal dose(LD1),has profound cytoprotective activity,used to be demonstrated in ulcerative colitis and invented to multiple sclerosis trials.Likely,it may bring the theory to practical application,starting with the initial argument,no degradation in human gastric juice for more than 24 h,and thereby,the therapeutic effectiveness(including therapeutic per-oral regimen)and pleiotropic beneficial effects.

Budd-Chiari syndrome secondary to caval recurrence of renal cell carcinoma

BACKGROUND:Renal cell carcinoma(RCC)involves the inferior vena cava(IVC)in a minority of patients.Less commonly,it presents with Budd-Chiari syndrome.If untreated, the condition progresses towards liver failure and death.METHOD:We report a case of Budd-Chiari syndrome due to infiltration of the IVC and right atrium by recurrence of RCC 7 years after successful treatment by primary resection.RESULTS:Surgery was performed with a combined abdominal and thoracic approach with cardio-pulmonary by-pass and cardioplegia.The tumor was removed and a cadaveric iliac vein graft used to re-establish venous continuity between the right atrium and hepatic veins.CONCLUSIONS:Although it is a complex and high-risk procedure,aggressive surgery performed by an experienced team with liver transplant and cardiothoracic skills may enable resection of apparently advanced caval tumors.The case is discussed in the light of the current literature.