Mortality and health care burden of Budd Chiari syndrome in the United States:A nationwide analysis(1998-2017)

The Budd Chiari syndrome(BCS)is a rare and potentially fatal disease,but there is a paucity of data on the in-hospital mortality as well its economic burden on the health care system.AIM To evaluate trends in mortality,length of hospital stays and resource utilization among inpatients with BCS.METHODS Data on all adult patients with a diagnosis of BCS were extracted from the National Inpatient Sample(NIS)from 1998 to 2017.To make inferences regarding the national estimates for the total number of BCS discharges across the study period,sample weights were applied to each admission per recommendations from the NIS.RESULTS During the study period,there were 3591(8.73%)in-patient deaths.The overall inhospital mortality rates among BCS patients decreased from 18%in 1998 to 8%in 2017;the mortality decreased by 4.41%(P<0.0001)every year.On multivariate analysis,older age,higher comorbidity score,acute liver failure,acute kidney injury,acute respiratory failure,hepatic encephalopathy,hepatorenal syndrome,inferior vena cava thrombosis,intestinal infarct,sepsis/septic shock and cancer were associated increased risk of mortality.The average of length of stay was 8.8 d and it consistently decreased by 2.04%(95%CI:-2.67%,-1.41%,P<0.001)from 12.7 d in 1998 to 7.6 d in 2017.The average total charges after adjusted for Medical Care Consumers Price Index to 2017 dollars during the time period was$94440 and the annual percentage change increased by 1.15%(95%CI:0.35%,1.96%,P=0.005)from$95515 in 1998 to$103850 in 2017.CONCLUSION The in-hospital mortality rate for patients admitted with BCS in the United States has reduced between 1998 and 2017 and this may a reflection of better management of these patients.

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.

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.

Percutaneous drainage in the treatment of intrahepatic pancreatic pseudocyst with Budd-Chiari syndrome:A case report

BACKGROUND Intrahepatic pancreatic pseudocyst(IHPP)is an extremely rare complication of acute pancreatitis,with only a few cases previously described in the literature.To the best of our knowledge,IHPP with Budd-Chiari syndrome(BCS)has not yet been described.CASE SUMMARY A 35-year-old male presented with abdominal pain,vomiting and anorexia,followed by severe swelling of the lower body after 4 d.The morphological assessment(using computed tomography revealed the presence of a huge cyst of 18.28 cm×10.34 cm under the liver capsule accompanied by a large amount of ascites.Percutaneous puncture allowed us to detect a high level of amylase in the collection,confirming the diagnosis of IHPP.The cyst was treated by percu-taneous drainage,producing complete resolution of the cyst.CONCLUSION IHPP can be treated with percutaneous drainage,endoscopic drainage,surgery or even conservative treatment,depending on the specific condition.We recommend percutaneous drainage as the first choice of treatment when IHPP with secondary BCS.

Ultrasound liver elastography beyond liver fibrosis assessment

Several guidelines have indicated that liver stiffness(LS)assessed by means of shear wave elastography(SWE)can safely replace liver biopsy in several clinical scenarios,particularly in patients with chronic viral hepatitis.However,an increase of LS may be due to some other clinical conditions not related to fibrosis,such as liver inflammation,acute hepatitis,obstructive cholestasis,liver congestion,infiltrative liver diseases.This review analyzes the role that SWE can play in cases of liver congestion due to right-sided heart failure,congenital heart diseases or valvular diseases.In patients with heart failure LS seems directly influenced by central venous pressure and can be used as a prognostic marker to predict cardiac events.The potential role of LS in evaluating liver disease beyond the stage of liver fibrosis has been investigated also in the hepatic sinusoidal obstruction syndrome(SOS)and in the Budd-Chiari syndrome.In the hepatic SOS,an increase of LS is observed some days before the clinical manifestations;therefore,it could allow an early diagnosis to timely start an effective treatment.Moreover,it has been reported that patients that were successfully treated showed a LS decrease,that reached pre-transplantation value within two to four weeks.It has been reported that,in patients with Budd-Chiari syndrome,LS values can be used to monitor short and long-term outcome after angioplasty.

Risk factors of thrombosis in abdominal veins

AIM: To estimate the prevalence of inherited and acquired thrombophilic risk factors in patients with abdominal venous thrombosis and to compare the risk factor profiles between Budd-Chiari syndromes (BCS) and splanchnic vein thrombosis (SVT). METHODS: In this retrospective study, 36 patients with abdominal venous thrombosis were studied. The patients were divided into Budd-Chiari group (hepatic vein, IVC thrombosis) and splanchnic venous thrombosis group (portal, splenic, superior mesenteric veins) based on the veins involved. Hereditary and acquired thrombophilic risk factors were evaluated in all patients. RESULTS: Twenty patients had SVT, 14 had BCS, and 2 had mixed venous thrombosis. Ten patients (28%) had hereditary and 10 patients (28%) acquired thrombophilic risk factors. The acquired risk factors were significantly more common in the SVT group (SVT vs BCS: 45% vs 7%, χ2 = 5.7, P = 0.02) while hereditary risk factors did not show significant differences between the two groups (SVT vs BCS: 25% vs 36%, χ2 = 0.46, P = 0.7). Multiple risk factors were present in one (7%) patient with BCS and in 3 patients (15%) with SVT. No risk factors were identified in 57% of patients with BCS and in 45% of patients with SVT. CONCLUSION: Hereditary and acquired risk factors play an important role in the etiopathogenesis of abdominal venous thrombosis. Acquired risk factorsare significantly more common in SVT patients while hereditary factors are similar in both groups.

FunMod： A Cytoscape Functional Modules in Networks Plugin for Identifying Undirected Protein-Protein

The characterization of the interacting behaviors of complex biological systems is a pri- mary objective in protein protein network analysis and computational biology. In this paper we present FunMod, an innovative Cytoscape version 2.8 plugin that is able to mine undirected pro- rein-protein networks and to infer sub-networks of interacting proteins intimately correlated with relevant biological pathways. This plugin may enable the discovery of new pathways involved in dis- eases. In order to describe the role of each protein within the relevant biological pathways, FunMod computes and scores three topological features of the identified sub-networks. By integrating the results from biological pathway clustering and topological network analysis, FunMod proved to be useful for the data interpretation and the generation of new hypotheses in two case studies.