Background:Acute cholangitis is an infection due to the bile duct obstruction.Despite progress in treat-ment,acute cholangitis remains potentially fatal.Early diagnosis and treatment improve the patient out-comes.The ...Background:Acute cholangitis is an infection due to the bile duct obstruction.Despite progress in treat-ment,acute cholangitis remains potentially fatal.Early diagnosis and treatment improve the patient out-comes.The present study aimed to identify clinical and biological factors at admission associated with 30-day mortality in acute cholangitis,to build an efficient prognostic score based on these parameters and to study the performances of this new score.Methods:We enrolled all adult patients consecutively hospitalized for acute cholangitis between January 2017 and December 2021.We developed a score system named ProChol using variables significantly asso-ciated with 30-day mortality in multivariate logistic analysis and simplified this system(named sProChol)based on a simple points-based approach.Results:In total,528 patients were included,with an average age of 77±13 years,a male predominance(54.2%)and a majority of lithiasis etiology(66.5%).Mortality in 30 days was 11.9%.In multivariate logis-tic analysis,tumor etiology[adjusted odds ratio(aOR)=15.43,95%confidence interval(CI):5.90-40.40],stent obstruction(aOR=5.12,95%CI:2.02-12.99),hypoalbuminemia(aOR=3.50,95%CI:1.25-9.81),renal failure(aOR=6.51,95%CI:2.62-16.18),oxygen therapy(aOR=4.63,95%CI:1.02-20.92)and cu-rative anticoagulation(aOR=2.60,95%CI:1.23-5.52)were independently associated with the 30-day mortality while fever was a protective factor(aOR=0.37,95%CI:0.16-0.84).ProChol score using these 7 parameters and sProChol using the 3 robust factors(etiology,renal failure and anticoagulation)presented respectively an area under receiver operating characteristic(ROC)curves(AUC)of 0.81 and 0.77,higher than Tokyo(AUC=0.72)and Gravito-Soares et al.score(AUC=0.71).Patients with sProChol≥4 had a significantly higher risk of transfer to intensive care unit(13.3%vs.5.1%;P<0.001)and longer length of stay(P=0.0006).Conclusions:ProChol and sProChol constructed from simple clinico-biological parameters at admission,present interesting performances in predicting the 30-day mortality in acute cholangitis.展开更多
BACKGROUND Ursodeoxycholic acid(UDCA)is the first-line therapeutic agent for primary biliary cholangitis(PBC).However,a subset of patients exhibit a suboptimal response to UDCA,and reliable predictive biomarkers remai...BACKGROUND Ursodeoxycholic acid(UDCA)is the first-line therapeutic agent for primary biliary cholangitis(PBC).However,a subset of patients exhibit a suboptimal response to UDCA,and reliable predictive biomarkers remain elusive.Studies have implicated plasma microRNAs(miRNAs)in the pathophysiological pro-gression of PBC,with certain miRNAs demonstrating potential as diagnostic and disease progression biomarkers.However,biomarkers capable of predicting the therapeutic efficacy of UDCA have not yet been identified.AIM To investigate differentially expressed miRNAs in PBC patients with divergent UDCA treatment responses and to explore potential biomarkers that predict treatment response in PBC.METHODS Plasma samples from treatment-naive PBC patients receiving≥1 year of standard UDCA treatment were collected.Efficacy was evaluated using the Paris I criteria.Patient samples were divided into discovery group(n=10)and validation group(n=30),with further stratification of patients into drug-resistant and drug-sensitive(DS)cohorts.Next-generation sequencing and quantitative real-time polymerase chain reaction were used to screen,functionally analyze,and validate the pre-treatment miRNA profiles of the treatment groups.RESULTS Forty-nine miRNAs were differentially expressed between the two groups before UDCA treatment(N=40).MiR-22-5p and miR-126-3p were highly expressed in the DS group before treatment(P<0.001),whereas miR-7706 exhibited a low expression(P=0.017).Post-treatment,miR-126-3p maintained low expression in the drug-resistant group(P=0.003),but showed elevated levels in the DS group(P<0.001).Logistic regression analysis identified miR-126-3p expression(odds ratio=34.32,95%confidence interval:1.95-605.40,P=0.016)as a significant factor influencing UDCA treatment response,while miR-22-5p(P=0.990)and miR-7706(P=0.157)showed no significant association.MiR-126-3p levels were negatively correlated with total bilirubin(r=-0.356,P=0.005)and immuno-globulin G levels(r=-0.311,P=0.015).The area under the receiver operating characteristic curve was 0.891(P=0.0003,95%confidence interval:0.772-1.000)with a sensitivity of 82.4%and a specificity of 84.6%.CONCLUSION Plasma miRNA expression profiles are heterogenous in patients with PBC with differential responses to UDCA therapy.MiR-126-3p demonstrates predictive potential for a suboptimal response to UDCA in patients with PBC.展开更多
Primary biliary cholangitis(PBC)is an autoimmune disease characterized by the selective destruction of intrahepatic small bile ducts,primarily by infiltrating lymphocytes,and has limited therapeutic options.A growing ...Primary biliary cholangitis(PBC)is an autoimmune disease characterized by the selective destruction of intrahepatic small bile ducts,primarily by infiltrating lymphocytes,and has limited therapeutic options.A growing body of evidence suggests that nanoparticles encapsulating rapamycin(ImmTOR)can suppress autoreactive lymphocytes and reduce inflammatory cytokine levels in various autoimmune diseases.In a recent study,Yang et al investigated the therapeutic effects of ImmTOR in a mouse model of PBC.ImmTOR treatment reduced the expression and number of CD4+T cells,CD8+T cells,and B cells isolated from the liver and spleen,improved liver inflammation and enzyme levels,and was associated with a concomitant decrease in anti-mitochondrial antibody levels.In this editorial,we highlight the significance of these findings,focusing on the potential mechanisms by which ImmTOR suppresses hepatic autoreactive T cells and reduces anti-mitochondrial antibody levels,ultimately improving liver pa-thology,through pathways such as mammalian target of rapamycin inhibition and autophagy restoration.We also offer a perspective on future research di-rections for PBC in both animal models and in vitro studies.展开更多
BACKGROUND The diagnosis of primary biliary cholangitis(PBC)remains challenging,particularly in cases where anti-mitochondrial antibody(AMA),anti-mitochondrial E2 subunit antibody(AMA-M2),anti-glycoprotein 210(anti-gp...BACKGROUND The diagnosis of primary biliary cholangitis(PBC)remains challenging,particularly in cases where anti-mitochondrial antibody(AMA),anti-mitochondrial E2 subunit antibody(AMA-M2),anti-glycoprotein 210(anti-gp210),and anti-speckled protein 100(anti-Sp100)are all negative.In such instances,the condition is often confirmed through a liver needle biopsy.AIM To identify additional plasma biomarkers for non-invasive diagnostic methods of PBC.METHODS We utilized the Sengenics KREX^(TM)immunome protein array to identify potential biomarkers for the diagnosis of PBC.Subsequently,we validated the predictive capability of the RPL30 antibody through an ELISA and retrospectively analyzed its association with the clinical features of 17 autoantibody-negative PBC cases and 45 autoantibody-positive PBC cases.RESULTS In our study we observed that RPL30 demonstrated the highest fold-change difference in PBC,with a penetrance frequency of 40%and a penetrance fold change of 38.30147.The analysis of anti-RPL30 optical density values between patients with AMA/AMA-M2/anti-gp210/anti-Sp100-negative PBC(autoantibody-negative PBC)and healthy controls using a receiver operating characteristic curve yielded an area under the curve of 0.853.This analysis established an optimal cutoff value of 0.0708,achieving 100%specificity and 75%sensitivity.The combination of anti-RPL30 and other autoantibodies elevated the diagnosis rate of PBC from 61.29%to 79.00%(P=0.0489).Anti-RPL30 demonstrated a high positive rate in antibody-negative PBC cases,including AMA/AMAM2/anti-gp210/anti-Sp100-negative cases.Correlation analysis of anti-RPL30 optical density values with clinical data from patients with PBC revealed a positive association with both the international normalized ratio(P=0.008)and the Model for End-Stage Liver Disease score(P=0.003).CONCLUSION Our study highlighted the potential of anti-RPL30 as a promising biomarker for diagnosing PBC,particularly in autoantibody-negative cases.展开更多
The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immun...The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immunomodulatory and anti-inflammatory properties but with very high-water solubility and low permeability,is formulated into a paeoniflorin/phospholipid complex microemulsion(PAE-ME)to enhance its delivery in this study.It demonstrated the PAE-ME's macrophage-regulating ability to repolarize the pro-inflammatory M1 subtype to the anti-inflammatory M2 type and reduce inflammatory cytokine release.In a PSC-IBD mouse model,PAE-ME alleviated the symptoms and regulated bile acid balance.Given the close connection and crosstalk between the liver and intestine,PAE-ME yielded a synergistic therapeutic effect on both the liver and intestinal lesions.These findings suggest a promising translational approach for complex comorbidities by acting on the liver-gut axis.展开更多
Acute cholangitis(AC)is a heterogeneous disease with considerable variation in clinical presentation and high medical costs.Although the overall mortality rate is decreasing.However,the mortality in severe AC ranged f...Acute cholangitis(AC)is a heterogeneous disease with considerable variation in clinical presentation and high medical costs.Although the overall mortality rate is decreasing.However,the mortality in severe AC ranged from 10%-30%due to sepsis,multi-organ failure,and systemic inflammatory response syndrome.Assessing the predictors of poor outcomes,including mortality,intensive care unit(ICU)admission,and hospital stay,is vital for early and effective intervention.We assessed the predictors of mortality and ICU admission in patients with AC.We conducted a literature search in PubMed/MEDLINE,Google Scholar,and Cochrane Library for relevant articles.The keywords used were acute cholangitis,biliary cholangitis,predictors,mortality,ICU admission,and Hospital stay.In addition,we assessed the role of early vs late endoscopic retrograde cholangiopancreatography on the outcomes.Old age,end-organ failure,red cell distribution width to albumin ratio,neutrophil-to-lymphocyte ratio,platelet-lymphocyte ratio,the need for ventilator support,diabetes,electrolyte imbalance,procalcitonin-to-albumin ratio,C-reactive protein-to-albumin,Glasgow Coma Scale,and systolic hypertension are predictors of poor outcomes in AC of varying etiology,and concurrent acute pancreatitis was not associated with ICU admission.展开更多
In this article,the author comment on the article by Zang et al.Tumor necrosis factor alpha-induced protein 3(TNFAIP3)was examined in this study as a novel biomarker to predict the efficiency of ursodeoxycholic acid(U...In this article,the author comment on the article by Zang et al.Tumor necrosis factor alpha-induced protein 3(TNFAIP3)was examined in this study as a novel biomarker to predict the efficiency of ursodeoxycholic acid(UDCA)and thereby improved primary biliary cholangitis(PBC)treatment.Differentially expressed genes in PBC patients and healthy controls(HCs)were detected using microarray expression analysis.PBC patients and HCs were examined for predictive performance and associations between important genes and clinicopathological features using immunohistochemistry,logistic regression,and receiver operating characteristic curve methods.Thirteen genes linked to the development of PBC were detected by the bioinformatic research.TNFAIP3 was chosen for additional examination from these 13 genes.TNFAIP3 was shown to be more expressed in PBCs patients than in HCs using immunohistochemical method.TNFAIP3 and fatigue have a significant impact on UDCA in PBC patients in multivariate cox regression analysis.Additionally,there was a correlation between TNFAIP3 expression and splenomegaly,alkaline phosphatase,albumin,total bilirubin,and age.In conclusion,TNFAIP3 and fatigue have significant impact on UDCA in PBC.These findings provide a new view on PBC pathophysiology and suggest that TNFAIP3 may be a suitable biomarker or therapeutic target for the disease.展开更多
BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholan...BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholangiocarcinoma.Although PSC predominantly affects adults,the incidence in pediatric patients is rising.For individuals in the advanced stages of liver disease,liver transplantation(LT)is the sole curative treatment option.However,the recurrence of PSC in the transplanted liver,known as recurrent PSC(rPSC),remains a significant concern.AIM To identify the potential risk factors for the recurrence of PSC in pediatric patients after undergoing LT.METHODS A literature search was carried out across databases,including PubMed,Embase,Cochrane Library,and Scopus,covering studies published from 1990 through 2024.The Newcastle-Ottawa scale was utilized to assess the quality of the selected studies.Statistical analyses were conducted using RevMan 5.3 software,where the risk of recurrence was quantified using hazard ratios(HR)with 95%CI.RESULTS A total of nine reports with 2524 pediatric patients with PSC were included in this analysis.The findings revealed several important risk factors connected to the rPSC in pediatric patients who had received a liver transplant,including concurrent inflammatory bowel disease(IBD),elevated liver enzyme levels,and the presence of PSCautoimmune hepatitis(AIH)overlap syndrome(all P<0.05).No statistically significant association was found between acute allograft rejection,Epstein-Barr virus infection,and the risk of rPSC recurrence in the pediatric liver transplant recipients.CONCLUSION The present systematic review and meta-analysis have identified various risk factors associated with the recurrence of PSC in pediatric patients who underwent LT,including IBD,elevated liver enzyme levels,and PSC-AIH overlap syndrome.展开更多
IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cho...IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis(PSC),and the presence of segmental stenosis suggests cholangiocarcinoma(CC).IgG4-SC responds well to steroid therapy,whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention.Since IgG4-SC was first described,it has become a third distinct clinical entity of sclerosing cholangitis.The aim of this review was to introduce the diagnostic methods for IgG4-SC.IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical,serological,morphological,and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.When intrapancreatic stenosis is detected,pancreatic cancer or CC should be ruled out.If multiple intrahepatic stenoses are evident,PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining.Associated inflammatory bowel disease is suggestive of PSC.If stenosis is demonstrated in the hepatic hilar region,CC should be discriminated by ultrasonography,intraductal ultrasonography,bile duct biopsy,and a higher cutoff serum IgG4 level of 182 mg/dL.展开更多
AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteri...AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for"primary sclerosing cholangitis"in Pubmed."Clinical characteristics"were specified into five predefined subthemes:epidemiology of IBD in PSC,characteristics of IBD in PSC(i.e.,location,disease behavior),risk of colorectal cancer development,IBD recurrence and de novo disease after liver transplantation for PSC,and safety and complications after proctocolectomy with ileal pouchanal anastomosis.Papers were selected for inclusion based on their relevance to the subthemes,and were reviewed by two independent reviewers.Only full papers relevant to PSC-IBD were included.Additionally the references of recent reviews for PSC(<5 years old)were scrutinized for relevant articles.RESULTS:Initial literature search for PSC yielded 4704results.After careful review 65 papers,comprising a total of 11406 PSC-IBD patients,were selected and divided according to subtheme.Four manuscripts overlapped and were included in two subthemes.Prevalence of IBD in PSC shows a large variance,ranging from 46.5%to 98.7%with ulcerative colitis(UC)being the most common type(>75%).The highest IBD rates in PSC are found in papers reviewing both endoscopic and histological data for IBD diagnosis.Although IBD in PSC is found to be a quiescent disease,pancolitis occurs often,with rates varying from 35%to 95%.Both backwash ileitis and rectal sparing are observed infrequently.The development of dysplasia or colorectal carcinoma is increased in PSC-IBD;the cumulative 10 years risk varying between 0%and11%.Exacerbation of IBD is common after liver transplantation for PSC and de novo disease is seen in1.3%to 31.3%of PSC-IBD patients.The risk for development of pouchitis in PSC-IBD is found to besignificant,affecting 13.8%to 90%of the patients after proctocolectomy with ileo anal-pouch anastomosis.CONCLUSION:IBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn’s disease and therefore requires specialized management.展开更多
This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inf...This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD.IBDassociated with PSC(PSC-IBD)shows an increased incidence of pancolitis,mild symptoms,and colorectal malignancy.Although an increased incidence of pancolitis is a characteristic finding,some cases are endoscopically diagnosed as right-sided ulcerative colitis.Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon.The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition.The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)are similar to those of PSC.The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.展开更多
AIM To investigate the diagnostic value of abnormal serum carbohydrate antigen 199(CA199) level in acute cholangitis secondary to choledocholithiasis.METHODS In this retrospective cohort study, the clinical data of 72...AIM To investigate the diagnostic value of abnormal serum carbohydrate antigen 199(CA199) level in acute cholangitis secondary to choledocholithiasis.METHODS In this retrospective cohort study, the clinical data of 727 patients with choledocholithiasis admitted to the Third Affiliated Hospital of Zunyi Medical College from June 2011 to June 2017 were collected. Among these patients, 258 patients had secondary acute cholangitis and served as observation group, and the remaining 569 choledocholithiasis patients served as the control group. Serum liver function indexes and tumor markers were detected in both groups, and the receiver operating characteristic(ROC) curves were constructed for markers showing statistical significances. The cutoff value, sensitivity, and specificity of each marker were calculated according to the ROC curves. RESULTS The results of liver function tests showed no significant differences between the two groups(P > 0.05). Tumor markers including serum CA125, CA153, carcinoembryonic antigen, and alpha fetoprotein levels were also not significantly different(P > 0.05); however, the serum CA199 level was significantly higher in the observation group than in the control group(P < 0.05). The ROC curve analysis showed that the area under the curve was 0.885(95%CI: 0.841-0.929) for CA199, and the cutoff value of 52.5 kU/L had the highest diagnostic accuracy, with a sensitivity of 86.8% and a specificity of 81.6%.CONCLUSION Abnormally elevated serum CA199 level has an important value in the diagnosis of acute cholangitis secondary to choledocholithiasis. It may be a specific inflammatory marker for acute cholangitis.展开更多
AIM: To examine the safety of immediate endoscopic sphincterotomy(EST) in patients with acute suppurative cholangitis(ASC) caused by choledocholithiasis, as compared with elective EST.METHODS: Patients with ASC due to...AIM: To examine the safety of immediate endoscopic sphincterotomy(EST) in patients with acute suppurative cholangitis(ASC) caused by choledocholithiasis, as compared with elective EST.METHODS: Patients with ASC due to choledocholithiasis were allocated to two groups: Those who underwent EST immediately and those who underwent EBD followed by EST 1 wk later because they were under anticoagulant therapy, had a coagulopathy(international normalized ratio > 1.3, partial thromboplastin time greater than twice that of control), or had a platelet count < 50000 × 103/μL. One of four trainees [200-400 cases of endoscopic retrograde cholangiopancreatography(ERCP)] supervised by a specialist(> 10000 cases of ERCP) performed the procedures. The success and complication rates associated with EST in each group were examined.RESULTS: Of the 87 patients with ASC, 59 were in the immediate EST group and 28 in the elective EST group. EST was successful in all patients in both groups. There were no complications associated with EST in either group of patients, although white blood cell count, C-reactiveprotein, total bilirubin, and serum concentrations of liver enzymes just before EST were significantly higher in the immediate EST group than in the elective EST group.CONCLUSION: Immediate EST can be as safe as elective EST for patients with ASC associated with choledocholithiasis provided they are not under anticoagulant therapy, or do not have a coagulopathy or a platelet count < 50000 × 103/μL. Moreover, the procedure was safely performed by a trainee under the supervision of an experienced specialist.展开更多
To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Us...To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Using the PubMed database, a literature search was conducted for relevant articles in English from the past 10 years. Relevant studies investigating PSC as a risk factor for CRC in IBD in the context of incidence and prevalence, pathogenesis, prevention and prognosis were included in this review. Recent evidence increasingly points to PSC as a significant risk factor in the development of CRC in patients with concomitant IBD. PSC may be an important risk factor for CRC in different populations worldwide. The mechanism for this increase in risk is still unclear. The efficacy of UDCA as a chemopreventive agent remains controversial. Liver transplantation does not halt the development of CRC, although there is not enough evidence to suggest that it is associated with increased incidence of CRC. While routine colonoscopic surveillance should be performed in patients with concurrent PSC and IBD, more high-level evidence is required to support the benefits of the procedure. While many new developments have taken place in the last decade, the pathogenesis and optimal management of CRC development in IBD-PSC patients remain unclear.展开更多
AIM: To identify potential factors that can predict adverse short-term outcomes in patients with acute cholangitis undergoing endoscopic retrograde cholangiopancreatography(ERCP). METHODS: Retrospective analysis of co...AIM: To identify potential factors that can predict adverse short-term outcomes in patients with acute cholangitis undergoing endoscopic retrograde cholangiopancreatography(ERCP). METHODS: Retrospective analysis of consecutive patients admitted to our center for acute cholangitis and underwent ERCP from 2001 to 2012. Involvement of two or more organ systems was termed as organ failure(OF). Cardiovascular failure was defined based on a systolic blood pressure of < 90 mmHg despite fluid replacement and/or requiring vasopressor treatment; respiratory failure if the Pa02 /Fi02 ratio was < 300 mmHg and/or required mechanical ventilation; coagulopathy if the platelet count was < 80; and renal insufficiency if serum creatinine was > 1.9 mg/dL. Variables associated with short term adverse clinical outcomes defined as persistent OF and/or 30-d mortality was determined. RESULTS: A total of 172 patients(median age 62 years, 56.4% female) were included. The median door to ERCP time was 17 h. Bile duct stones were the most common etiology(n = 67, 39.2%). In multivariate analysis, factors that were independently associated with persistent OF and/or 30-d mortality included American Society of Anesthesiology(ASA) physical classification score > 3(OR = 7.70; 95%CI: 2.73-24.40), presence of systemic inflammatory response syndrome(OR = 3.67; 95%CI: 1.34-10.3) and door to ERCP time greater than 72 h(OR = 3.36; 95%CI: 1.12-10.20). Door to ERCP time greater than 72 h was also associated with 70% increase in the mean length of stay(P < 0.001). Every one point increase in the ASA physical classification and every 1 mg/dL increase in the preERCP bilirubin level was associated with a 34% and 2% increase in the mean length of hospital stay, respectively. Transfer status did not impact clinical outcomes. CONCLUSION: Higher ASA physical classification and delays in ERCP are associated with adverse clinical outcomes and prolonged length of hospital stay in patients with acute cholangitis undergoing ERCP.展开更多
Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively eval...Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.展开更多
Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of ...Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective.The prevalence of IBD,mainly ulcerative colitis in PSC patients is estimated to be 21%-80%,dependent on screening programs and nationality.PSC-associated colitis is likely to be extensive,characterized by rectal sparing,backwash ileitis,and generally mild symptoms.It is also more likely to progress to colorectal malignancy,making it imperative for clinicians to maintain a high level of suspicion when tackling PSC patients.There is no optimal surveillance strategy but current guidelines advocate that colonoscopy is necessary at the time of PSC diagnosis with annual endoscopic follow-up.Random biopsies have been criticized and a shift towards targeted biopsies using chromoendoscopy,laser endomicroscopy and narrow-band imaging has been noted.Techniques directed towards genetic mutations instead of histological abnormalities hold promise for easier,more accurate diagnosis of dysplastic lesions.Chemopreventive measures against colorectal cancer have been sought in these patients.Ursodeoxycholic acid seemed promising at first but subsequent studies yielded conflicting results showing anticarcinogenic effects in low doses(8-15 mg/kg per day) and carcinogenic properties in high doses(15-30 mg/kg per day).展开更多
Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilso...Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilson s disease presenting as pigmented gallstone pancreatitis have not been reported in the literature.In the present report,we describe a case of a 37-year-old man who was admitted with jaundice and abdomina pain.The patient was diagnosed with acute pancreatitis,cholangitis,and obstructive jaundice caused by pigmented gallstones that were detected during retrograde cholangiopancreatography.However,because of his long-term jaundice and the presence of pigmented gallstones,the patient underwent further evaluation for Wilson’s disease,which was subsequently confirmed.This patient’s unique presentation exemplifies the overlap in the clinical and laboratory parameters of Wilson’s disease and cholestasis,and the difficulties associated with their differentiation.It suggests thatWilson’s disease should be considered in patients with pancreatitis,cholangitis,and severe protracted jaundice caused by pigmented gallstones.展开更多
AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characte...AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characteristics of 34 patients with sclerosing cholangitis(SC) associated with AIP were compared with those of 4 patients with PSC.RESULTS:SC with AIP occurred predominantly in el-derly men.Obstructive jaundice was the most frequent initial symptom in SC with AIP.Only SC patients with AIP had elevated serum IgG4 levels,and sclerosing diseases were more frequent in these patients.SC pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected in the bile duct wall and the periportal area,as well as in the pancreas,of SC patients with AIP.CONCLUSION:SC with AIP is distinctly different from PSC.The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.展开更多
Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate...Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.展开更多
文摘Background:Acute cholangitis is an infection due to the bile duct obstruction.Despite progress in treat-ment,acute cholangitis remains potentially fatal.Early diagnosis and treatment improve the patient out-comes.The present study aimed to identify clinical and biological factors at admission associated with 30-day mortality in acute cholangitis,to build an efficient prognostic score based on these parameters and to study the performances of this new score.Methods:We enrolled all adult patients consecutively hospitalized for acute cholangitis between January 2017 and December 2021.We developed a score system named ProChol using variables significantly asso-ciated with 30-day mortality in multivariate logistic analysis and simplified this system(named sProChol)based on a simple points-based approach.Results:In total,528 patients were included,with an average age of 77±13 years,a male predominance(54.2%)and a majority of lithiasis etiology(66.5%).Mortality in 30 days was 11.9%.In multivariate logis-tic analysis,tumor etiology[adjusted odds ratio(aOR)=15.43,95%confidence interval(CI):5.90-40.40],stent obstruction(aOR=5.12,95%CI:2.02-12.99),hypoalbuminemia(aOR=3.50,95%CI:1.25-9.81),renal failure(aOR=6.51,95%CI:2.62-16.18),oxygen therapy(aOR=4.63,95%CI:1.02-20.92)and cu-rative anticoagulation(aOR=2.60,95%CI:1.23-5.52)were independently associated with the 30-day mortality while fever was a protective factor(aOR=0.37,95%CI:0.16-0.84).ProChol score using these 7 parameters and sProChol using the 3 robust factors(etiology,renal failure and anticoagulation)presented respectively an area under receiver operating characteristic(ROC)curves(AUC)of 0.81 and 0.77,higher than Tokyo(AUC=0.72)and Gravito-Soares et al.score(AUC=0.71).Patients with sProChol≥4 had a significantly higher risk of transfer to intensive care unit(13.3%vs.5.1%;P<0.001)and longer length of stay(P=0.0006).Conclusions:ProChol and sProChol constructed from simple clinico-biological parameters at admission,present interesting performances in predicting the 30-day mortality in acute cholangitis.
基金Supported by the National Key Research and Development Program of China,No.2019YFC0840704Beijing Municipal Science and Technology Program,No.Z201100005520047.
文摘BACKGROUND Ursodeoxycholic acid(UDCA)is the first-line therapeutic agent for primary biliary cholangitis(PBC).However,a subset of patients exhibit a suboptimal response to UDCA,and reliable predictive biomarkers remain elusive.Studies have implicated plasma microRNAs(miRNAs)in the pathophysiological pro-gression of PBC,with certain miRNAs demonstrating potential as diagnostic and disease progression biomarkers.However,biomarkers capable of predicting the therapeutic efficacy of UDCA have not yet been identified.AIM To investigate differentially expressed miRNAs in PBC patients with divergent UDCA treatment responses and to explore potential biomarkers that predict treatment response in PBC.METHODS Plasma samples from treatment-naive PBC patients receiving≥1 year of standard UDCA treatment were collected.Efficacy was evaluated using the Paris I criteria.Patient samples were divided into discovery group(n=10)and validation group(n=30),with further stratification of patients into drug-resistant and drug-sensitive(DS)cohorts.Next-generation sequencing and quantitative real-time polymerase chain reaction were used to screen,functionally analyze,and validate the pre-treatment miRNA profiles of the treatment groups.RESULTS Forty-nine miRNAs were differentially expressed between the two groups before UDCA treatment(N=40).MiR-22-5p and miR-126-3p were highly expressed in the DS group before treatment(P<0.001),whereas miR-7706 exhibited a low expression(P=0.017).Post-treatment,miR-126-3p maintained low expression in the drug-resistant group(P=0.003),but showed elevated levels in the DS group(P<0.001).Logistic regression analysis identified miR-126-3p expression(odds ratio=34.32,95%confidence interval:1.95-605.40,P=0.016)as a significant factor influencing UDCA treatment response,while miR-22-5p(P=0.990)and miR-7706(P=0.157)showed no significant association.MiR-126-3p levels were negatively correlated with total bilirubin(r=-0.356,P=0.005)and immuno-globulin G levels(r=-0.311,P=0.015).The area under the receiver operating characteristic curve was 0.891(P=0.0003,95%confidence interval:0.772-1.000)with a sensitivity of 82.4%and a specificity of 84.6%.CONCLUSION Plasma miRNA expression profiles are heterogenous in patients with PBC with differential responses to UDCA therapy.MiR-126-3p demonstrates predictive potential for a suboptimal response to UDCA in patients with PBC.
文摘Primary biliary cholangitis(PBC)is an autoimmune disease characterized by the selective destruction of intrahepatic small bile ducts,primarily by infiltrating lymphocytes,and has limited therapeutic options.A growing body of evidence suggests that nanoparticles encapsulating rapamycin(ImmTOR)can suppress autoreactive lymphocytes and reduce inflammatory cytokine levels in various autoimmune diseases.In a recent study,Yang et al investigated the therapeutic effects of ImmTOR in a mouse model of PBC.ImmTOR treatment reduced the expression and number of CD4+T cells,CD8+T cells,and B cells isolated from the liver and spleen,improved liver inflammation and enzyme levels,and was associated with a concomitant decrease in anti-mitochondrial antibody levels.In this editorial,we highlight the significance of these findings,focusing on the potential mechanisms by which ImmTOR suppresses hepatic autoreactive T cells and reduces anti-mitochondrial antibody levels,ultimately improving liver pa-thology,through pathways such as mammalian target of rapamycin inhibition and autophagy restoration.We also offer a perspective on future research di-rections for PBC in both animal models and in vitro studies.
基金Supported by National Natural Science Foundation of China,No.82172257Health Care Major Project of Guangzhou,No.202206080001Science and Technology Cooperation Program of Fujian Province,No.2021I0036。
文摘BACKGROUND The diagnosis of primary biliary cholangitis(PBC)remains challenging,particularly in cases where anti-mitochondrial antibody(AMA),anti-mitochondrial E2 subunit antibody(AMA-M2),anti-glycoprotein 210(anti-gp210),and anti-speckled protein 100(anti-Sp100)are all negative.In such instances,the condition is often confirmed through a liver needle biopsy.AIM To identify additional plasma biomarkers for non-invasive diagnostic methods of PBC.METHODS We utilized the Sengenics KREX^(TM)immunome protein array to identify potential biomarkers for the diagnosis of PBC.Subsequently,we validated the predictive capability of the RPL30 antibody through an ELISA and retrospectively analyzed its association with the clinical features of 17 autoantibody-negative PBC cases and 45 autoantibody-positive PBC cases.RESULTS In our study we observed that RPL30 demonstrated the highest fold-change difference in PBC,with a penetrance frequency of 40%and a penetrance fold change of 38.30147.The analysis of anti-RPL30 optical density values between patients with AMA/AMA-M2/anti-gp210/anti-Sp100-negative PBC(autoantibody-negative PBC)and healthy controls using a receiver operating characteristic curve yielded an area under the curve of 0.853.This analysis established an optimal cutoff value of 0.0708,achieving 100%specificity and 75%sensitivity.The combination of anti-RPL30 and other autoantibodies elevated the diagnosis rate of PBC from 61.29%to 79.00%(P=0.0489).Anti-RPL30 demonstrated a high positive rate in antibody-negative PBC cases,including AMA/AMAM2/anti-gp210/anti-Sp100-negative cases.Correlation analysis of anti-RPL30 optical density values with clinical data from patients with PBC revealed a positive association with both the international normalized ratio(P=0.008)and the Model for End-Stage Liver Disease score(P=0.003).CONCLUSION Our study highlighted the potential of anti-RPL30 as a promising biomarker for diagnosing PBC,particularly in autoantibody-negative cases.
基金National Key Research and Development Program of China(No.2022YFE0203600,China)National Natural Science Foundation of China(Nos.82341232,81925035)+3 种基金Department of Science and Technology of Guangdong Province(No.2021B0909050003)Chinese Academy of Sciences President's International Fellowship Initiative(No.2024VBB0004)the Scientific Innovation Group Project in Zhongshan(No.CXTD2022011)supported by grants from the Program of Shanghai Committee of Science and Technology,China(No.22S21902900)。
文摘The concurrence of primary sclerosing cholangitis(PSC)and inflammatory bowel disease(IBD)presents a therapeutic challenge,often necessitating liver transplantation in severe cases.Paeoniflorin(PAE),known for its immunomodulatory and anti-inflammatory properties but with very high-water solubility and low permeability,is formulated into a paeoniflorin/phospholipid complex microemulsion(PAE-ME)to enhance its delivery in this study.It demonstrated the PAE-ME's macrophage-regulating ability to repolarize the pro-inflammatory M1 subtype to the anti-inflammatory M2 type and reduce inflammatory cytokine release.In a PSC-IBD mouse model,PAE-ME alleviated the symptoms and regulated bile acid balance.Given the close connection and crosstalk between the liver and intestine,PAE-ME yielded a synergistic therapeutic effect on both the liver and intestinal lesions.These findings suggest a promising translational approach for complex comorbidities by acting on the liver-gut axis.
文摘Acute cholangitis(AC)is a heterogeneous disease with considerable variation in clinical presentation and high medical costs.Although the overall mortality rate is decreasing.However,the mortality in severe AC ranged from 10%-30%due to sepsis,multi-organ failure,and systemic inflammatory response syndrome.Assessing the predictors of poor outcomes,including mortality,intensive care unit(ICU)admission,and hospital stay,is vital for early and effective intervention.We assessed the predictors of mortality and ICU admission in patients with AC.We conducted a literature search in PubMed/MEDLINE,Google Scholar,and Cochrane Library for relevant articles.The keywords used were acute cholangitis,biliary cholangitis,predictors,mortality,ICU admission,and Hospital stay.In addition,we assessed the role of early vs late endoscopic retrograde cholangiopancreatography on the outcomes.Old age,end-organ failure,red cell distribution width to albumin ratio,neutrophil-to-lymphocyte ratio,platelet-lymphocyte ratio,the need for ventilator support,diabetes,electrolyte imbalance,procalcitonin-to-albumin ratio,C-reactive protein-to-albumin,Glasgow Coma Scale,and systolic hypertension are predictors of poor outcomes in AC of varying etiology,and concurrent acute pancreatitis was not associated with ICU admission.
文摘In this article,the author comment on the article by Zang et al.Tumor necrosis factor alpha-induced protein 3(TNFAIP3)was examined in this study as a novel biomarker to predict the efficiency of ursodeoxycholic acid(UDCA)and thereby improved primary biliary cholangitis(PBC)treatment.Differentially expressed genes in PBC patients and healthy controls(HCs)were detected using microarray expression analysis.PBC patients and HCs were examined for predictive performance and associations between important genes and clinicopathological features using immunohistochemistry,logistic regression,and receiver operating characteristic curve methods.Thirteen genes linked to the development of PBC were detected by the bioinformatic research.TNFAIP3 was chosen for additional examination from these 13 genes.TNFAIP3 was shown to be more expressed in PBCs patients than in HCs using immunohistochemical method.TNFAIP3 and fatigue have a significant impact on UDCA in PBC patients in multivariate cox regression analysis.Additionally,there was a correlation between TNFAIP3 expression and splenomegaly,alkaline phosphatase,albumin,total bilirubin,and age.In conclusion,TNFAIP3 and fatigue have significant impact on UDCA in PBC.These findings provide a new view on PBC pathophysiology and suggest that TNFAIP3 may be a suitable biomarker or therapeutic target for the disease.
文摘BACKGROUND Primary sclerosing cholangitis(PSC)is a long-term liver condition defined by the inflammation and scarring of the bile ducts,resulting in complications such as liver cirrhosis,portal hypertension,and cholangiocarcinoma.Although PSC predominantly affects adults,the incidence in pediatric patients is rising.For individuals in the advanced stages of liver disease,liver transplantation(LT)is the sole curative treatment option.However,the recurrence of PSC in the transplanted liver,known as recurrent PSC(rPSC),remains a significant concern.AIM To identify the potential risk factors for the recurrence of PSC in pediatric patients after undergoing LT.METHODS A literature search was carried out across databases,including PubMed,Embase,Cochrane Library,and Scopus,covering studies published from 1990 through 2024.The Newcastle-Ottawa scale was utilized to assess the quality of the selected studies.Statistical analyses were conducted using RevMan 5.3 software,where the risk of recurrence was quantified using hazard ratios(HR)with 95%CI.RESULTS A total of nine reports with 2524 pediatric patients with PSC were included in this analysis.The findings revealed several important risk factors connected to the rPSC in pediatric patients who had received a liver transplant,including concurrent inflammatory bowel disease(IBD),elevated liver enzyme levels,and the presence of PSCautoimmune hepatitis(AIH)overlap syndrome(all P<0.05).No statistically significant association was found between acute allograft rejection,Epstein-Barr virus infection,and the risk of rPSC recurrence in the pediatric liver transplant recipients.CONCLUSION The present systematic review and meta-analysis have identified various risk factors associated with the recurrence of PSC in pediatric patients who underwent LT,including IBD,elevated liver enzyme levels,and PSC-AIH overlap syndrome.
文摘IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis(PSC),and the presence of segmental stenosis suggests cholangiocarcinoma(CC).IgG4-SC responds well to steroid therapy,whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention.Since IgG4-SC was first described,it has become a third distinct clinical entity of sclerosing cholangitis.The aim of this review was to introduce the diagnostic methods for IgG4-SC.IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical,serological,morphological,and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.When intrapancreatic stenosis is detected,pancreatic cancer or CC should be ruled out.If multiple intrahepatic stenoses are evident,PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining.Associated inflammatory bowel disease is suggestive of PSC.If stenosis is demonstrated in the hepatic hilar region,CC should be discriminated by ultrasonography,intraductal ultrasonography,bile duct biopsy,and a higher cutoff serum IgG4 level of 182 mg/dL.
基金Supported by Partners Seeking a Cure and a VIDI grant from the Netherlands Organization for Scientific Research,No.016.136.308(to Weersma RK)
文摘AIM:To review the current literature for the specificclinical characteristics of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC).METHODS:A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for"primary sclerosing cholangitis"in Pubmed."Clinical characteristics"were specified into five predefined subthemes:epidemiology of IBD in PSC,characteristics of IBD in PSC(i.e.,location,disease behavior),risk of colorectal cancer development,IBD recurrence and de novo disease after liver transplantation for PSC,and safety and complications after proctocolectomy with ileal pouchanal anastomosis.Papers were selected for inclusion based on their relevance to the subthemes,and were reviewed by two independent reviewers.Only full papers relevant to PSC-IBD were included.Additionally the references of recent reviews for PSC(<5 years old)were scrutinized for relevant articles.RESULTS:Initial literature search for PSC yielded 4704results.After careful review 65 papers,comprising a total of 11406 PSC-IBD patients,were selected and divided according to subtheme.Four manuscripts overlapped and were included in two subthemes.Prevalence of IBD in PSC shows a large variance,ranging from 46.5%to 98.7%with ulcerative colitis(UC)being the most common type(>75%).The highest IBD rates in PSC are found in papers reviewing both endoscopic and histological data for IBD diagnosis.Although IBD in PSC is found to be a quiescent disease,pancolitis occurs often,with rates varying from 35%to 95%.Both backwash ileitis and rectal sparing are observed infrequently.The development of dysplasia or colorectal carcinoma is increased in PSC-IBD;the cumulative 10 years risk varying between 0%and11%.Exacerbation of IBD is common after liver transplantation for PSC and de novo disease is seen in1.3%to 31.3%of PSC-IBD patients.The risk for development of pouchitis in PSC-IBD is found to besignificant,affecting 13.8%to 90%of the patients after proctocolectomy with ileo anal-pouch anastomosis.CONCLUSION:IBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn’s disease and therefore requires specialized management.
文摘This is a review of the characteristic findings of inflammatory bowel disease(IBD)associated with primary sclerosing cholangitis(PSC)and their usefulness in the diagnosis of sclerosing cholangitis.PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD.IBDassociated with PSC(PSC-IBD)shows an increased incidence of pancolitis,mild symptoms,and colorectal malignancy.Although an increased incidence of pancolitis is a characteristic finding,some cases are endoscopically diagnosed as right-sided ulcerative colitis.Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon.The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition.The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)are similar to those of PSC.The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.
基金Supported by the Fund from the Guizhou Provincial Department of Health Science and Technology,No.GZWJKJ2014-2-151the Science and Technology Fund of Guizhou Province,No.QKHLH[2016]7421Zunyi Science and Technology Research and Development Fund,No.ZSKHS[2016]06
文摘AIM To investigate the diagnostic value of abnormal serum carbohydrate antigen 199(CA199) level in acute cholangitis secondary to choledocholithiasis.METHODS In this retrospective cohort study, the clinical data of 727 patients with choledocholithiasis admitted to the Third Affiliated Hospital of Zunyi Medical College from June 2011 to June 2017 were collected. Among these patients, 258 patients had secondary acute cholangitis and served as observation group, and the remaining 569 choledocholithiasis patients served as the control group. Serum liver function indexes and tumor markers were detected in both groups, and the receiver operating characteristic(ROC) curves were constructed for markers showing statistical significances. The cutoff value, sensitivity, and specificity of each marker were calculated according to the ROC curves. RESULTS The results of liver function tests showed no significant differences between the two groups(P > 0.05). Tumor markers including serum CA125, CA153, carcinoembryonic antigen, and alpha fetoprotein levels were also not significantly different(P > 0.05); however, the serum CA199 level was significantly higher in the observation group than in the control group(P < 0.05). The ROC curve analysis showed that the area under the curve was 0.885(95%CI: 0.841-0.929) for CA199, and the cutoff value of 52.5 kU/L had the highest diagnostic accuracy, with a sensitivity of 86.8% and a specificity of 81.6%.CONCLUSION Abnormally elevated serum CA199 level has an important value in the diagnosis of acute cholangitis secondary to choledocholithiasis. It may be a specific inflammatory marker for acute cholangitis.
文摘AIM: To examine the safety of immediate endoscopic sphincterotomy(EST) in patients with acute suppurative cholangitis(ASC) caused by choledocholithiasis, as compared with elective EST.METHODS: Patients with ASC due to choledocholithiasis were allocated to two groups: Those who underwent EST immediately and those who underwent EBD followed by EST 1 wk later because they were under anticoagulant therapy, had a coagulopathy(international normalized ratio > 1.3, partial thromboplastin time greater than twice that of control), or had a platelet count < 50000 × 103/μL. One of four trainees [200-400 cases of endoscopic retrograde cholangiopancreatography(ERCP)] supervised by a specialist(> 10000 cases of ERCP) performed the procedures. The success and complication rates associated with EST in each group were examined.RESULTS: Of the 87 patients with ASC, 59 were in the immediate EST group and 28 in the elective EST group. EST was successful in all patients in both groups. There were no complications associated with EST in either group of patients, although white blood cell count, C-reactiveprotein, total bilirubin, and serum concentrations of liver enzymes just before EST were significantly higher in the immediate EST group than in the elective EST group.CONCLUSION: Immediate EST can be as safe as elective EST for patients with ASC associated with choledocholithiasis provided they are not under anticoagulant therapy, or do not have a coagulopathy or a platelet count < 50000 × 103/μL. Moreover, the procedure was safely performed by a trainee under the supervision of an experienced specialist.
基金Supported by Career Development Fellowship of the National Health and Medical Research Council of Australia,to Leong R
文摘To examine and evaluate recent evidence regarding the epidemiology, pathogenesis and management of colorectal cancer(CRC) development in inflammatory bowel disease(IBD)-primary sclerosing cholangitis(PSC) patients. Using the PubMed database, a literature search was conducted for relevant articles in English from the past 10 years. Relevant studies investigating PSC as a risk factor for CRC in IBD in the context of incidence and prevalence, pathogenesis, prevention and prognosis were included in this review. Recent evidence increasingly points to PSC as a significant risk factor in the development of CRC in patients with concomitant IBD. PSC may be an important risk factor for CRC in different populations worldwide. The mechanism for this increase in risk is still unclear. The efficacy of UDCA as a chemopreventive agent remains controversial. Liver transplantation does not halt the development of CRC, although there is not enough evidence to suggest that it is associated with increased incidence of CRC. While routine colonoscopic surveillance should be performed in patients with concurrent PSC and IBD, more high-level evidence is required to support the benefits of the procedure. While many new developments have taken place in the last decade, the pathogenesis and optimal management of CRC development in IBD-PSC patients remain unclear.
基金Supported by The American College of Gastroenterology Grant to Navaneethan U
文摘AIM: To identify potential factors that can predict adverse short-term outcomes in patients with acute cholangitis undergoing endoscopic retrograde cholangiopancreatography(ERCP). METHODS: Retrospective analysis of consecutive patients admitted to our center for acute cholangitis and underwent ERCP from 2001 to 2012. Involvement of two or more organ systems was termed as organ failure(OF). Cardiovascular failure was defined based on a systolic blood pressure of < 90 mmHg despite fluid replacement and/or requiring vasopressor treatment; respiratory failure if the Pa02 /Fi02 ratio was < 300 mmHg and/or required mechanical ventilation; coagulopathy if the platelet count was < 80; and renal insufficiency if serum creatinine was > 1.9 mg/dL. Variables associated with short term adverse clinical outcomes defined as persistent OF and/or 30-d mortality was determined. RESULTS: A total of 172 patients(median age 62 years, 56.4% female) were included. The median door to ERCP time was 17 h. Bile duct stones were the most common etiology(n = 67, 39.2%). In multivariate analysis, factors that were independently associated with persistent OF and/or 30-d mortality included American Society of Anesthesiology(ASA) physical classification score > 3(OR = 7.70; 95%CI: 2.73-24.40), presence of systemic inflammatory response syndrome(OR = 3.67; 95%CI: 1.34-10.3) and door to ERCP time greater than 72 h(OR = 3.36; 95%CI: 1.12-10.20). Door to ERCP time greater than 72 h was also associated with 70% increase in the mean length of stay(P < 0.001). Every one point increase in the ASA physical classification and every 1 mg/dL increase in the preERCP bilirubin level was associated with a 34% and 2% increase in the mean length of hospital stay, respectively. Transfer status did not impact clinical outcomes. CONCLUSION: Higher ASA physical classification and delays in ERCP are associated with adverse clinical outcomes and prolonged length of hospital stay in patients with acute cholangitis undergoing ERCP.
文摘Sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.
文摘Patients with primary sclerosing cholangitis(PSC) complicated by inflammatory bowel disease(IBD) represent a distinct subset of patients with unique characteristics,which have serious clinical implications.The aim of this literature review was to shed light to the obscure clinical and molecular aspects of the two diseases combined utilizing current data available and putting issues of diagnosis and treatment into perspective.The prevalence of IBD,mainly ulcerative colitis in PSC patients is estimated to be 21%-80%,dependent on screening programs and nationality.PSC-associated colitis is likely to be extensive,characterized by rectal sparing,backwash ileitis,and generally mild symptoms.It is also more likely to progress to colorectal malignancy,making it imperative for clinicians to maintain a high level of suspicion when tackling PSC patients.There is no optimal surveillance strategy but current guidelines advocate that colonoscopy is necessary at the time of PSC diagnosis with annual endoscopic follow-up.Random biopsies have been criticized and a shift towards targeted biopsies using chromoendoscopy,laser endomicroscopy and narrow-band imaging has been noted.Techniques directed towards genetic mutations instead of histological abnormalities hold promise for easier,more accurate diagnosis of dysplastic lesions.Chemopreventive measures against colorectal cancer have been sought in these patients.Ursodeoxycholic acid seemed promising at first but subsequent studies yielded conflicting results showing anticarcinogenic effects in low doses(8-15 mg/kg per day) and carcinogenic properties in high doses(15-30 mg/kg per day).
文摘Wilson’s disease is a rare disorder of copper transport in hepatic cells,and may present as cholestatic liver disease;pancreatitis and cholangitis are rarely associated with Wilsons’s disease.Moreover,cases of Wilson s disease presenting as pigmented gallstone pancreatitis have not been reported in the literature.In the present report,we describe a case of a 37-year-old man who was admitted with jaundice and abdomina pain.The patient was diagnosed with acute pancreatitis,cholangitis,and obstructive jaundice caused by pigmented gallstones that were detected during retrograde cholangiopancreatography.However,because of his long-term jaundice and the presence of pigmented gallstones,the patient underwent further evaluation for Wilson’s disease,which was subsequently confirmed.This patient’s unique presentation exemplifies the overlap in the clinical and laboratory parameters of Wilson’s disease and cholestasis,and the difficulties associated with their differentiation.It suggests thatWilson’s disease should be considered in patients with pancreatitis,cholangitis,and severe protracted jaundice caused by pigmented gallstones.
文摘AIM:To clarify the characteristic features of biliary le-sions in patients with autoimmune pancreatitis(AIP) and compare them with those of primary sclerosing cholangitis(PSC) .METHODS:The clinicopathological characteristics of 34 patients with sclerosing cholangitis(SC) associated with AIP were compared with those of 4 patients with PSC.RESULTS:SC with AIP occurred predominantly in el-derly men.Obstructive jaundice was the most frequent initial symptom in SC with AIP.Only SC patients with AIP had elevated serum IgG4 levels,and sclerosing diseases were more frequent in these patients.SC pa-tients with AIP responded well to steroid therapy.Seg-mental stenosis of the lower bile duct was observed only in SC patients with AIP,but a beaded and pruned-tree appearance was detected only in PSC patients.Dense infi ltration of IgG4-positive plasma cells was de-tected in the bile duct wall and the periportal area,as well as in the pancreas,of SC patients with AIP.CONCLUSION:SC with AIP is distinctly different from PSC.The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.
基金Supported by Grants-in-Aid for Clinical Research from National Hospital Organization,Grants-in-Aid for Scientific Research from the Ministry of Health,Labour and Welfare of Japan
文摘Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.
