Autoimmune hepatitis-primary biliary cholangitis overlap syndrome complicated by various autoimmune diseases:A case report

BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.

Clinical issues and challenges in imaging of gastrointestinal diseases:A minireview and our experience

Imaging techniques play a crucial role in the modern era of medicine,particularly in gastroenterology.Nowadays,various non-invasive and invasive imaging modalities are being routinely employed to evaluate different gastrointestinal(GI)diseases.However,many instrumental as well as clinical issues are arising in the area of modern GI imaging.This minireview article aims to briefly overview the clinical issues and challenges encountered in imaging GI diseases while highlighting our experience in the field.We also summarize the advances in clinically available diagnostic methods for evaluating different diseases of the GI tract and demonstrate our experience in the area.In conclusion,almost all imaging techniques used in imaging GI diseases can also raise many challenges that necessitate careful consideration and profound expertise in this field.

Epidemiology of biliary tract cancer in China:A narrative review

Biliary tract cancer(BTC)is a group of rare malignancies that affect the gallbladder and bile ducts.Although rare,BTC is becoming a significant public health burden in China,particularly among males and older individuals.The increasing trends in BTC incidence and mortality in China are influenced by various demographic,environmental,and lifestyle factors.In this review,we examine available epidemiological data on the incidence,mortality,prognosis,and trends of different BTC subtypes in China.We also discuss the challenges and opportunities for improving the prevention,diagnosis,and management of BTC in China,and identify areas for further research and intervention.The article aims to provide a better understanding of the epidemiological features of BTC in China and to inform public health strategies and clinical practice.

Nab-paclitaxel plus capecitabine as first-line treatment for advanced biliary tract cancers:An open-label,non-randomized,phase II clinical trial

BACKGROUND Biliary tract cancers(BTCs)are a heterogeneous group of tumors with high malignancy,poor prognosis,and limited treatment options.AIM To explore the efficacy and safety of nab-paclitaxel plus capecitabine as first-line treatment for advanced and metastatic BTCs.METHODS This open-label,non-randomized,double-center,phase II clinical trial recruited systemic therapy-naive patients with unresectable or metastatic BTCs between April 2019 and June 2022 at Beijing Cancer Hospital and the First Hospital of China Medical University.Eligible patients were administered nab-paclitaxel(150 mg/m^(2),day 1)and capecitabine(2000 mg/m^(2),twice daily,days 1-7)in 14-day cycles until experiencing intolerable toxicity or disease progression.The primary outcome was the objective response rate(ORR).The secondary outcomes included the disease control rate(DCR),overall survival(OS),progression-free survival(PFS),and safety.RESULTS A total of 44 patients successfully completed the trial,with a median age of 64.00 years(interquartile range,35.00-76.00),and 26(59.09%)were females.Tumor response assessment was impeded for one patient due to premature demise from tumor hemorrhage.Among the remaining 43 patients undergoing at least one imaging assessment,the ORR was 23.26%[95%confidence interval(CI):11.80%-38.60%],and the DCR was 69.77%(95%CI:53.90%-82.80%).The median OS was 14.1 months(95%CI:8.3-19.9),and the median PFS was 4.4 months(95%CI:2.5-6.3).A total of 41 patients(93.18%)experienced at least one adverse event(AE),with 10 patients(22.73%)encountering grade≥3 AEs,and the most frequent AEs of any grade were alopecia(79.50%),leukopenia(54.55%),neutropenia(52.27%),and liver dysfunction(40.91%),and no treatment-related deaths were documented.CONCLUSION Nab-paclitaxel plus capecitabine may be an effective and safe first-line treatment strategy for patients with advanced or metastatic BTCs.

Clinical and socioeconomic determinants of survival in biliary tract adenocarcinomas

BACKGROUND Despite advances in detection and treatments,biliary tract cancers continue to have poor survival outcomes.Currently,there is limited data investigating the significance of socioeconomic status,race/ethnicity,and environmental factors in biliary tract cancer survival.Data from the Surveillance,Epidemiology,and End Results database for biliary and gallbladder adenocarcinomas were extracted from 1975 to 2016.Socioe-conomic data included smoking,poverty level,education,adjusted household income,and percentage of foreign-born persons and urban population.Survival was calculated with Cox proportional hazards models for death in the 5-year period following diagnosis.RESULTS Our study included 15883 gallbladder,11466 intrahepatic biliary,12869 extrahepatic biliary and 7268 ampulla of Vater adenocarcinoma cases.When analyzing county-specific demographics,patients from counties with higher incomes were associated with higher survival rates[hazard ratio(HR)=0.97,P<0.05].Similarly,counties with a higher percentage of patients with a college level education and counties with a higher urban population had higher 5-year survival rates(HR=0.96,P=0.002 and HR=0.97,P=0.004,respectively).CONCLUSION Worse survival outcomes were observed in lower income counties while higher income and education level were associated with higher 5-year overall survival among gallbladder and biliary malignancies.

Occult pancreaticobiliary reflux is a pathogenic factor of some benign biliary diseases and gallbladder cancer

Background:Pancreaticobiliary maljunction(PBM)is a well-known high-risk factor for biliary malignant tumors because of constant pancreaticobiliary reflux(PBR).However,the impact of occult pancreaticobiliary reflux(OPR),which is characterized by high bile amylase levels in individuals with anatomically normal pancreaticobiliary junction,on biliary diseases remains unclear.The aim of this study was to assess the correlation between OPR and biliary diseases.Methods:We enrolled 94 consecutive patients with normal pancreaticobiliary junction and primary biliary diseases confirmed by magnetic resonance cholangiopancreatography.We prospectively collected patients’bile samples and measured bile amylase levels.We investigated the incidence of OPR and the difference in bile amylase levels among these patients and assessed the correlation between high bile amylase levels(HBAL)and benign or malignant biliary diseases,as well as the OPR risk factors.Results:The incidence of OPR was 36.6%in patients with benign biliary diseases,26.7%in those with cholangiocarcinoma and 62.5%in those with gallbladder cancer.The median bile amylase level tended to be higher in patients with gallbladder cancer than in those with benign biliary diseases,but there was no significant difference(165.5 IU/L vs.23.0 IU/L,P=0.212).The prevalence of an HBAL with bile amylase levels of 1000-7500 IU/L was similar in patients with gallbladder cancer and benign biliary diseases.However,the incidence of HBAL with bile amylase levels greater than 7500 IU/L was significantly higher in patients with gallbladder cancer than in those with benign biliary diseases(37.5%vs.4.2%,P=0.012).Multivariate logistic regression analysis revealed that choledocholithiasis was an independent risk factor for OPR.Conclusions:OPR can occur in benign and malignant biliary diseases,and it may be a pathogenic factor for some benign biliary diseases and a high-risk factor for gallbladder cancer.There is a correlation between choledocholithiasis and OPR.

Association between gut microbiota and hepatocellular carcinoma and biliary tract cancer:A mendelian randomization study

BACKGROUND An increasing number of studies have begun to discuss the relationship between gut microbiota and diseases,yet there is currently a lack of corresponding articles describing the association between gut microbiota and hepatocellular carcinoma(HCC)and biliary tract cancer(BTC).This study aims to explore the relationship between them using Mendelian randomization(MR)analysis method.AIM To assess the relationship between gut microbiota and HCC and BTC.METHODS We obtained Genome-wide association study(GWAS)data for the gut microbiome from the intestinal microbiota genomic library(MiBioGen,https://mibiogen.gcc.rug.nl/).Additionally,we accessed data pertaining to HCC and BTC from the IEU open GWAS platform(https://gwas.mrcieu.ac.uk/).Our analysis employed fundamental instrumental variable analysis methods,including inverse-variance weighted,MR and Egger.To ensure the dependability of the results,we subjected the results to tests for multiple biases and heterogeneity.RESULTS During our investigation,we discovered 11 gut microbiota linked to an increased risk to BTC and HCC.The former included the genus Eubacterium hallii group(P=0.017),Candidatus Soleaferrea(P=0.034),Flavonifractor(P=0.021),Lachnospiraceae FCS020(P=0.034),the order Victivallales(P=0.018),and the class Lentisphaeria(P=0.0.18).The latter included the genus Desulfovibrio(P=0.042),Oscillibacter(P=0.023),the family Coriobacteriaceae(P=0.048),the order Coriobacteriales(P=0.048),and the class Coriobacteriia(P=0.048).Furthermore,in BTC,we observed 2 protective gut microbiota namely the genus Dorea(P=0.041)and Lachnospiraceae ND3007 group(P=0.045).All results showed no evidence of multiplicity or heterogeneity.CONCLUSION This study explores a causal link between gut microbiota and HCC and BTC.These insights may enhance the mechanistic knowledge of microbiota-related HCC and BTC pathways,potentially informing therapeutic strategies.

Impact of open hepatectomy on postoperative bile leakage in patients with biliary tract cancer

BACKGROUND Bile leakage is a common and serious complication of open hepatectomy for the treatment of biliary tract cancer.AIM To evaluate the incidence,risk factors,and management of bile leakage after open hepatectomy in patients with biliary tract cancer.METHODS We retrospectively analyzed 120 patients who underwent open hepatectomy for biliary tract cancer from February 2018 to February 2023.Bile leak was defined as bile drainage from the surgical site or drain or the presence of a biloma on imaging.The incidence,severity,timing,location,and treatment of the bile leaks were recorded.The risk factors for bile leakage were analyzed using univariate and multivariate logistic regression analyses.RESULTS The incidence of bile leak was 16.7%(20/120),and most cases were grade A(75%,15/20)according to the International Study Group of Liver Surgery classification.The median time of onset was 5 d(range,1-14 d),and the median duration was 7 d(range,2-28 d).The most common location of bile leakage was the cut surface of the liver(70%,14/20),followed by the anastomosis site(25%,5/20)and the cystic duct stump(5%,1/20).Most bile leaks were treated conservatively with drainage,antibiotics,and nutritional support(85%,17/20),whereas some required endoscopic retrograde cholangiopancreatography with stenting(10%,2/20)or percutaneous transhepatic cholangiography with drainage(5%,1/20).Risk factors for bile leakage include male sex,hepatocellular carcinoma,major hepatectomy,blood loss,and blood transfusion.CONCLUSION Bile leakage is a frequent complication of open hepatectomy for biliary tract cancer.However,most cases are mild and can be conservatively managed.Male sex,hepatocellular carcinoma,major hepatectomy,blood loss,and blood transfusion were associated with an increased risk of bile leak.

Transmembrane serine protease 4 expression in the prognosis of radical resection for biliary tract cancer

BACKGROUND Recent advancements in biliary tract cancer(BTC)treatment have expanded beyond surgery to include adjuvant therapy,yet the prognosis remains poor.Identifying prognostic biomarkers could enhance the assessment of patients who have undergone radical resection for BTC.AIM To determine transmembrane serine protease 4(TMPRSS4)utility as a prognostic biomarker of radical resection for BTC.METHODS Medical records of patients who underwent radical resection for BTC,excluding intrahepatic cholangiocarcinoma,were retrospectively reviewed.The associations between TMPRSS4 expression and clinicopathological factors,overall survival,and recurrence-free survival were analyzed.RESULTS Among the 85 patients undergoing radical resection for BTC,46(54%)were TMPRSS4-positive.The TMPRSS4-positive group exhibited significantly higher preoperative carbohydrate antigen 19-9(CA19-9)values and greater lymphatic invasion than the TMPRSS4-negative group(P=0.019 and 0.039,respectively).Postoperative overall survival and recurrence-free survival were significantly worse in the TMPRSS4-positive group(median survival time:25.3 months vs not reached,P<0.001;median survival time:28.7 months vs not reached,P=0.043,respectively).Multivariate overall survival analysis indicated TMPRSS4 positivity,pT3/T4,and resection status R1 were independently associated with poor prognosis(P=0.032,0.035 and 0.030,respectively).TMPRSS4 positivity correlated with preoperative CA19-9 values≥37 U/mL and pathological tumor size≥30 mm(P=0.016 and 0.038,respectively).CONCLUSION TMPRSS4 is a potential prognostic biomarker of radical resection for BTC.

Primary biliary cholangitis presenting with granulomatous lung disease misdiagnosed as lung cancer:A case report

BACKGROUND There are few cases of pulmonary granulomatous changes secondary to primary biliary cirrhosis(PBC).No case of granulomatous lung disease secondary to PBC misdiagnosed as lung cancer had been reported.CASE SUMMARY A middle-aged woman presented with lung nodules and was misdiagnosed with lung cancer by positron emission tomography/computed tomography.She underwent left lobectomy,and the pathology of the nodules showed granulomatous inflammation,which was then treated with antibiotics.However,a new nodule appeared.Further investigation with lung biopsy and liver serology led to the diagnosis of PBC,and chest computed tomography indicated significant reduction in the pulmonary nodule by treatment with methylprednisolone and ursodeoxycholic acid.CONCLUSION Diagnosis of pulmonary nodules requires integrating various clinical data to avoid unnecessary pulmonary lobectomy.

Analysis of sleep quality,disease uncertainty,and psychological tolerance in patients undergoing chemotherapy for digestive tract malignancies

BACKGROUND Colorectal cancer is the second leading cause of cancer-related deaths among digestive tract malignancies,following gastric cancer.Sleep is of great significance for maintaining human health.The incidence of sleep disorders in patients with cancer is approximately twice that observed in the general population.Lack of sleep can prolong hospital stays,increase the likelihood of infection,and increase mortality rates.Therefore,studying the factors related to sleep quality is significant for improving the quality of life of patients with malignant tumors of the digestive tract.AIM To investigate the relationships among sleep quality,disease uncertainty,and psychological resilience in patients undergoing chemotherapy for digestive tract malignancies.METHODS A total of 131 patients with malignant digestive tract tumors who were treated at Hefei BOE Hospital between April 2021 and September 2022 were selected as research participants.Based on their Pittsburgh Sleep Quality Index(PSQI)scores,participants were divided into either the sleep disorder group(PSQI score>7)or the normal sleep group(PSQI score≤7).The clinical data—together with the Mishel Uncertainty in Illness Scale for Adults(MUIS-A)and Connor-Davidson Resilience Scale(CD-RISC)scores—were compared.RESULTS In this study,78(59.54%)patients with digestive tract malignancies developed sleep disorders after chemotherapy.Sleep disorder incidence was higher in patients with colorectal cancer than in those with gastric and esophageal cancers(P<0.05).The total MUIS-A score and those for each item in the sleep disorder group were higher than those in the normal sleep group.The total CD-RISC score and those for each item in the sleep disorder group were lower than those in the normal sleep group(P<0.05).The PSQI scores of patients with malignant digestive tract tumors were positively correlated with the scores for lack of disease information,disease uncertainty,and unpredictability in the MUIS-A and negatively correlated with the scores for tenacity,self-improvement,and optimism in the CD-RISC(P<0.05).CONCLUSION Patients undergoing chemotherapy for digestive tract malignancies are prone to sleep problems related to disease uncertainty and psychological resilience.Therefore,interventions can be implemented to improve their sleep quality.

Current status of the biliary tract malformation

The choledochal cyst(CC)can be better termed as biliary tract malformation because of the close association of embryology and etiology in the causation of CC.Contrary to Babbitt's postulation of reflux,damage and dilatation,reflux was not demonstrable as the causative factor in all varieties of CC.High pressure in the biliary system,otherwise termed ductal hypertension,is put forth as an alternative to explain the evolution of CC.The forme fruste type,which does not find a place in the standard classification,typifies the ductal hypertension hypothesis.Hence a closer,in-depth review would be able to highlight this apt terminology of biliary tract malformation.

Applications of gastric peroral endoscopic myotomy in the treatment of upper gastrointestinal tract disease

Gastric peroral endoscopic myotomy(G-POME)is an emerging minimally invasive endoscopic technique involving the establishment of a submucosal tun-nel around the pyloric sphincter.In 2013,Khashab et al used G-POME for the first time in the treatment of gastroparesis with enhanced therapeutic efficacy,prov-iding a new direction for the treatment of gastroparesis.With the recent and rapid development of G-POME therapy technology,progress has been made in the treatment of gastroparesis and other upper digestive tract diseases,such as congenital hypertrophic pyloric stenosis and gastric sleeve stricture,with G-POME.This article reviews the research progress and future prospects of G-POME for the treatment of upper digestive tract gastrointestinal diseases.

Global, regional, and national burden of gallbladder and biliary diseases from 1990 to 2019

BACKGROUND Gallbladder and biliary diseases(GABDs)are a major public health issue.AIM To analysis the cause-specific incidence,prevalence,and years lived with disability(YLDs)and its temporal trends of GABDs at the global,regional,and national level.Data on GABD were available from the Global Burden of Disease study 2019.METHODS The estimated annual percentage change(EAPC)was used to quantify temporal trend in GABD age-standardized incidence rates(ASIRs),age-standardized prevalence rate(ASPR),and age-standardized YLD rate(ASYR)by region,sex.We analyzed the relationship between the GABD burden and country development level using the human development index(HDI).RESULTS In 2019,the incident cases of GABD were 52003772,with an ASIR of 63432/100000 population.Globally,the number of incident cases and ASIR of GABD increased 97%and 58.9%between 1990 and 2019.Although,the ASPR and ASYR decreased from 1990 to 2019,the number of prevalent and YLDs cases increased.The highest ASIR was observed in Italy,and the highest ASPR and ASYR was observed in United Kingdom.The highest burden of GABD was found in low-SDI region,and the burden in female was significantly higher than males.A generally negative correlation(ρ=-0.24,P<0.05)of GABD with the EAPC and human development index(HDI)(in 2021)were observed for ASIR.What’s more,no correlation in ASPR(ρ=-0.06,P=0.39)and ASYR(ρ=-0.07,P=0.36)of GABD with the EAPC and HDI(in 2021)were observed,respectively.CONCLUSION GABD remain a major global public health challenge;however,the burden of GABD varies geographically.Globally,the number of incident cases and ASIR of GABD increased between 1990 and 2019.The results of our study provide insight into the global disease burden of GABD and may assist policymakers in formulating effective policies to mitigate modifiable risk factors.

Reoperation for benign biliary tract diseases in 149 cases: causes and prevention

BACKGROUND: Failure to diagnose and treat benign bi- liary tract disease relatively common surgical disease may cause serious consequences. Since the introduction of B- mode ultrasonography, CT, or MRI early and accurate diagnosis of the disease has been possible. In clinical prac- tice, however, these methods have not been adequately used. Inappropriate surgical procedures can also lead to bile duct injury or stenosis after injury, residual cholecystitis, stenosis after cholangiojejunostomy, or stenosis of the Od- di' s sphincter. But improvement of the diagnosis and treat- ment of benign biliary tract disease remains a great chal- lenge to clinicians. METHODS: A total of 149 patients with benign biliary tract disease who had received reoperation from June 1988 to June 2001 were analyzed retrospectively. Among them 95 patients (63.76%) received operation twice and 38 (25.5%) underwent 3 operations. Sixteen patients (10.74%) needed 4 or more operations. The procedures for the first opera- tion included cholecystectomy (71 patients, 47.65%), cho- lecystectomy with exploration of the common bile duct (42, 28.19%), cholangiojejunostomy (21, 14.1%), and la- paroscopic cholecystectomy (15, 10.06%). RESULTS: The causes for reoperation included residual and recurrent bile duct stones in 53 patients (35.57%), bile duct injury or stenosis after injury in 41 (27.52%), residual cho- lecystitis with or without stones in 28 (18.8%), stenosis af- ter cholangiojejunostomy in 17 (11.41%), stenosis of the Oddi's sphincter in 5 (5.35%), and others in 5 (5.35%). Four patients (2.68%) died after operation. CONCLUSIONS: To prevent reoperation for benign biliary tract diseases, the following measures should be taken to in- crease preoperative diagnostic rate, to understand condi- tions of the biliary tract by using imaging techniques and cholangiography, to examine comprehensively and careful- ly with choledochoscopy, cholangiography and B-mode ul-trasonography intraoperatively, to choose appropriate ope- rative procedures to decrease the rate of residual stones, and to decide the time for the first repair according to inju- ry type of the bile duct. Roux-en-Y hepaticojejunostomy with cholangioplasty is the best operation for the recon- struction of the biliary tract.

Current status of peroral cholangioscopy in biliary tract diseases

Peroral cholangioscopy(POC) is an important tool for the management of a selected group of biliary diseases. Because of its direct visualization, POC allows targeted diagnostic and therapeutic procedures. POC can be performed using a dedicated cholangioscope that is advanced through the accessory channel of a duodenoscope or via the insertion of a small-diameter endoscope directly into the bile duct. POC was first described in the 1970 s, but the use of earlier generation devices was substantially limited by the cumbersome equipment setup and high repair costs. For nearly ten years, several technical improvements, including the single-operator system, high-quality images, the development of dedicated accessories and the increased size of the working channel, have led to increased diagnostic accuracy, thus assisting in the differentiation of benign and malignant intraductal lesions, targeting biopsies and the precise delineation of intraductal tumor spread before surgery. Furthermore, lithotripsy of difficult bile duct stones, ablative therapies for biliary malignancies and direct biliary drainage can be performed under POC control. Recent developments of new types of conventional POCs allow feasible, safe and effective procedures at reasonable costs. In the current review, we provide an updated overview of POC, focusing our attention on the main current clinical applications and on areas for future research.

Prognostic relevance of circulating CK19 mRNA in advanced malignant biliary tract diseases

AIM: To determine the role of circulating tumor cells (CTCs) in prediction of the overall survival of patients with advanced malignant biliary tract obstruction. METHODS: We investigated the prognostic value of CTCs by examining two markers, cytokeratin (CK) 19 and human telomerase reverse transcriptase (hTERT) mRNA, in 40 patients diagnosed with advanced malig- nant biliary tract diseases. Quantitative real-time re- verse transcription polymerase chain reaction was used to detect CK19 and hTERT mRNA in the peripheral blood of these patients. Overall survival was analyzed using the Kaplan-Meier method and Cox regression modeling.RESULTS: Positive CK19 and hTERT mRNA expression was detected in 45% and 60%, respectively, of the 40 patients. Univariable analysis indicated that positive CK19 mRNA expression was significantly associated with worse overall survival (P = 0.009). Multivariable analysis determined that positive CK19 mRNA expres- sion, patient's age and serum bilirubin were each inde- pendently associated with overall survival. CONCLUSION: CK19 mRNA expression levels in pe- ripheral blood appear to provide a valuable marker to predict the overall survival of patients with advanced malignant biliary tract obstruction.

Gastrointestinal Diseases Classification Using Deep Transfer Learning and Features Optimization

Gastrointestinal diseases like ulcers, polyps’, and bleeding areincreasing rapidly in the world over the last decade. On average 0.7 millioncases are reported worldwide every year. The main cause of gastrointestinaldiseases is a Helicobacter Pylori (H. Pylori) bacterium that presents in morethan 50% of people around the globe. Many researchers have proposeddifferent methods for gastrointestinal disease using computer vision techniques.Few of them focused on the detection process and the rest of themperformed classification. The major challenges that they faced are the similarityof infected and healthy regions that misleads the correct classificationaccuracy. In this work, we proposed a technique based on Mask Recurrent-Convolutional Neural Network (R-CNN) and fine-tuned pre-trainedResNet-50 and ResNet-152 networks for feature extraction. Initially, the region ofinterest is detected using Mask R-CNN which is later utilized for the trainingof fine-tuned models through transfer learning. Features are extracted fromfine-tuned models that are later fused using a serial approach. Moreover, anImproved Ant Colony Optimization (ACO) algorithm has also opted for thebest feature selection from the fused feature vector. The best-selected featuresare finally classified using machine learning techniques. The experimentalprocess was conducted on the publicly available dataset and obtained animproved accuracy of 96.43%. In comparison with state-of-the-art techniques,it is observed that the proposed accuracy is improved.

Endoscopic retrograde cholangiopancreatography in pancreatic and biliary tract disease in Korean children

AIM:To assess the indications,findings,therapeutic procedures,safety,and complications of endoscopic retrograde cholangiopancreatography(ERCP) performed in Korean children.METHODS:The demographic characteristics,indications for ERCP,findings,therapeutic procedures,and complications of 122 pediatric patients who underwent 245 ERCPs in the Asan Medical Center between June 1994 and March 2008 were investigated.RESULTS:The mean age of the 122 patients was 8.0 ± 4.2 years.Indications were biliary pathology in 78(64.0%),pancreatic pathology in 43(35.2%),and chronic abdominal pain in one.Biliary indications included choledochal cysts in 40,choledocholithiasis in 24,suspected sclerosing cholangitis in 8,trauma in 2,and other conditions in 4.Pancreatic indications includedacute pancreatitis in 7,acute recurrent pancreatitis in 11,chronic pancreatitis in 20,trauma in 3,and pancreatic mass in 2.Of the 245 ERCPs,success rate was 98.4% and 190(77.6%) were for therapeutic purposes,including endoscopic nasal drainage(51.8%),biliary sphincterotomy(38.0%),pancreatic sphincterotomy(23.3%),stent insertion(15.1%),stone extraction(18.8%),and balloon dilatation(11.0%).Complications were postERCP pancreatitis in 16(6.5%),ileus in 23(9.4%),hemorrhage in 2(0.8%),perforation in 2(0.8%),sepsis in 1(0.4%),and impacted basket in 1(0.4%).There were no procedure-related deaths,and most complications improved under supportive care.CONCLUSION:This study showed that there is a high incidence of choledochal cyst and diagnostic and therapeutic ERCP for the management of various biliary and pancreatic diseases was safe and effective in Korean children.

Connective tissue diseases in primary biliary cirrhosis:A population-based cohort study

AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.