We present a female patient with continuous melena, diagnosed with rectal variceal bleeding. She had a history of esophageal varices, which were treated with endoscopic therapy. Five years after the treatment of esoph...We present a female patient with continuous melena, diagnosed with rectal variceal bleeding. She had a history of esophageal varices, which were treated with endoscopic therapy. Five years after the treatment of esophageal varices, continuous melena occurred. Since colonoscopy showed that the melena was caused by giant rectal varices, we thought that they were not suitable to receive endoscopic treatment. We chose the modified percutaneous transhepatic obliteration with sclerosant, which is one of the interventional radiology techniques but a new clinical procedure for rectal varices. After the patient received this therapy, her condition of rectal varices was markedly improved.展开更多
Objective:To investigate the clinical efficacy and safety of ultrasound-guided local anesthesia for endovenous laser combined with sclerotherapy in the treatment of varicose great saphenous veins.Methods:A total of 53...Objective:To investigate the clinical efficacy and safety of ultrasound-guided local anesthesia for endovenous laser combined with sclerotherapy in the treatment of varicose great saphenous veins.Methods:A total of 53 patients with varicose great saphenous veins admitted to our hospital from December 2023 to June 2025 were selected and divided into a traditional surgery group(18 cases)and a laser combined with sclerotherapy group(35 cases)according to the surgical method.The venous clinical severity score(VCSS),chronic venous insufficiency quality of life questionnaire(CIVIQ)score,visual analog scale(VAS)score for pain,complication rate,surgical time,treatment cost,recovery time,and patient satisfaction were compared between the two groups at 1 week,1 month,and 3 months postoperatively.Results:The VCSS scores of the laser group at each postoperative time point were lower than those of the traditional group,and the CIVIQ scores were higher than those of the traditional group(all p<0.05).The incidence of complications in the laser group(8.57%),the VAS score at 24 hours postoperatively,the duration of pain,and the utilization rate of analgesic medications were all significantly lower than those in the conventional group(all p<0.05).The laser group also demonstrated shorter operative and recovery times compared to the conventional group,along with higher patient satisfaction,albeit at a higher treatment cost(p<0.05).Conclusion:Endovenous laser combined with sclerotherapy under ultrasound guidance for the treatment of great saphenous vein varicosis offers advantages such as minimal trauma,rapid recovery,mild pain,and fewer complications,demonstrating significant clinical efficacy and good safety,thus possessing high clinical application value.展开更多
Cholangiocarcinoma(CCA)is a rare type of cancer which arises from the bile duct epithelium and carries a poor prognosis.One of the main risk factors in the Western world is primary sclerosing cholangitis.Surgical rese...Cholangiocarcinoma(CCA)is a rare type of cancer which arises from the bile duct epithelium and carries a poor prognosis.One of the main risk factors in the Western world is primary sclerosing cholangitis.Surgical resection has traditionally been the only curative treatment but can only be offered to patients with early disease,excluding those with locally advanced disease.Despite initial poor outcomes,liver transplantation(LT)has evolved as a viable treatment for a select group of patients with CCAs that are deemed unresectable.This review aims to explore the evolution of the role of LT in patients with CCA.展开更多
BACKGROUND Sclerosing encapsulating peritonitis(SEP),also known as abdominal cocoon syndrome,is rare in children.The etiology of primary SEP is believed to be associated with retrograde menstruation or viral peritonit...BACKGROUND Sclerosing encapsulating peritonitis(SEP),also known as abdominal cocoon syndrome,is rare in children.The etiology of primary SEP is believed to be associated with retrograde menstruation or viral peritonitis in young adolescent girls,whereas secondary SEP refers to SEP caused by other factors such as surgery,peritoneal dialysis,or tuberculosis.Secondary SEP is rare and lack diagnostic specificity in children.Given the potential to cause acute intestinal obstruction,surgical intervention is often required.CASE SUMMARY We report the case of a 10-year-old girl with secondary SEP who was admitted to our center with acute bowel obstruction.CONCLUSION In this report,we emphasized the imaging manifestations,diagnosis,and operative management of the case.Although postoperative SEP in children is rare,the long-term prognosis is favorable when accompanied with accurate diagnosis,appropriate perioperative management,and timely follow-up.展开更多
This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is str...This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is strongly associated with ulcerative colitis(UC).Endoscopic evaluation of UC is feasible and reliable in routine clinical practice,and the Mayo endoscopic subscore(MES)is one of the most commonly used endoscopic evaluation measures for UC.Patients with PSCUC are at higher risk of developing cancer and biliary tract cancer.Endoscopic scoring alone appears unreliable,and histopathological evaluation is essential to accurately assess and make effective therapeutic decisions for PSC-UC.Therefore,we aimed to discuss the accuracy of MES in patients with UC and PSC-UC and to explore the consistency between MES and the Nancy histological index.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
BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs inc...BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs include abdominal pain,an abdominal mass,menstrual abnormalities,and infertility.Infrequently,patients will experience androgen-related manifestations of masculinization,such as increased hair,acne,or a low voice.Diagnosis must be confirmed by immunohistochemical analysis of the tissue as clinical symptoms and imaging studies are unreliable.CASE SUMMARY A 14-year-old female presented with amenorrhea.After a thorough medical examination,endocrine and tumor markers analysis,and imaging,a pelvic mass was discovered.The patient also exhibited endocrine dysfunction but was not positive for any tumor markers.The patient underwent surgery to remove the ovarian tumor.Immunohistochemical analysis of the resected specimen indicated an OSST.During the postoperative follow-up,the patient had attained menarche.CONCLUSION This case’s clinical manifestation of endocrine dysfunction due to OSST provides new insights that will assist clinicians in the diagnosis and treatment of this common tumor.展开更多
BACKGROUND Autoimmune liver disease(AILD)recurrence is common after liver transplantation(LT).While several risk factors for recurrence have been identified,their combined predictive value has yet to be thoroughly inv...BACKGROUND Autoimmune liver disease(AILD)recurrence is common after liver transplantation(LT).While several risk factors for recurrence have been identified,their combined predictive value has yet to be thoroughly investigated.AIM To evaluate the combined predictive value of clinical and laboratory risk factors for AILD recurrence after LT.METHODS This retrospective cohort study included 79 patients with AILD who underwent LT at a single liver transplant center.We compared clinical and laboratory variables between patients with and without recurrent disease and assessed the predictive performance of these factors using four logistic regression models and their corresponding area underthe receiver operating characteristic curve (AUC).RESULTSRecurrent AILD occurred in 26.58% of patients (95%CI: 17-38), the median time to recurrence was 28 months(interquartile range: 16-38). Patients with recurrent AILD had significantly higher pre-transplant Child-Pugh scores[11.61 ± 1.16 vs 10.58 ± 1.96 points;odds ratio (OR) = 1.43, 95%CI: 1.03-2.00;P = 0.032] and model for end-stage liverdisease score (MELD) (22.76 ± 5.47 vs 18.81 ± 7.24 points;OR = 1.08, 95%CI: 1.01-1.16;P = 0.032), compared to thosewithout recurrence. Additionally, baseline alanine aminotransferase (ALT) > 2 times the upper limit of normal(ULN) was significantly associated with recurrence (31% vs 57.1%;OR = 2.96, 95%CI: 1.06-8.28;P = 0.038). Ourmodels, incorporating several risk variables, demonstrated moderate predictive ability for AILD recurrence. TheAUCs were as follows: (1) Model 1 (AUC = 0.75, 95%CI: 0.58-0.87);(2) Model 2 (AUC = 0.74, 95%CI: 0.59-0.90);(3)Model 3 (AUC = 0.72, 95%CI: 0.58-0.88);and (4) Model 4 (AUC = 0.63, 95%CI: 0.40-0.76), with no statisticallysignificant difference between the models (P = 0.488).CONCLUSIONHigher pre-transplant Child-Pugh and MELD scores, as well as ALT > 2 ULN, were associated with an increasedrisk of AILD recurrence.展开更多
Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary chola...Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome.After thoroughly reviewing their work,we offer insights that primarily relate to their study design to enhance the medical community’s understanding of this complex 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
To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of ...To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.展开更多
Polidocanol is the most widely used Sclerotherapy in European and American countries. It is affordable, easy to use and apply, and has high efficiency. The purpose of this paper is to give an overview of the recent ba...Polidocanol is the most widely used Sclerotherapy in European and American countries. It is affordable, easy to use and apply, and has high efficiency. The purpose of this paper is to give an overview of the recent basics of the Sclerotherapy, chemistry and formulation, clinical applications, and side-effects and complications of Polidocanol, and to discuss the Polidocanol as the safety and efficacy of Sclerotherapy.展开更多
Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. ...Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.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
Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseas-es(AiLD),namely autoimmune hepatitis types 1 and 2(AIH-1 and 2),primary biliary cirrhosis(PBC),and the s...Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseas-es(AiLD),namely autoimmune hepatitis types 1 and 2(AIH-1 and 2),primary biliary cirrhosis(PBC),and the sclerosing cholangitis variants in adults and children.AIH-1 is specified by anti-nuclear antibody(ANA) and smooth muscle antibody(SMA).AIH-2 is specified by antibody to liver kidney microsomal antigen type-1(anti-LKM1) and anti-liver cytosol type 1(anti-LC1).SMA,ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation.PBC is specified by antimitochondrial antibodies(AMA) react-ing with enzymes of the 2-oxo-acid dehydrogenase complexes(chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly react-ing with nuclear pore gp210 and nuclear body sp100.Sclerosing cholangitis presents as at least two variants,first the classical primary sclerosing cholangitis(PSC) mostly affecting adult men wherein the only(and non-specific) reactivity is an atypical perinuclear antineutro-phil cytoplasmic antibody(p-ANCA),also termed peri-nuclear anti-neutrophil nuclear antibodies(p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis(ASC) with serological features resembling those of type 1 AIH.Liver diagnostic serol-ogy is a fast-expanding area of investigation as new purified and recombinant autoantigens,and automatedtechnologies such as ELISAs and bead assays,become available to complement(or even compete with) tradi-tional immunofluorescence procedures.We survey for the first time global trends in quality assurance impact-ing as it does on(1) manufacturers/purveyors of kits and reagents,(2) diagnostic service laboratories that fulfill clinicians' requirements,and(3) the end-user,the physician providing patient care,who must properly interpret test results in the overall clinical context.展开更多
The three major immune disorders of the liver are autoimmune hepatitis(AIH),primary biliary cirrhosis(PBC) and primary sclerosing cholangitis(PSC).Variant forms of these diseases are generally called overlap syndromes...The three major immune disorders of the liver are autoimmune hepatitis(AIH),primary biliary cirrhosis(PBC) and primary sclerosing cholangitis(PSC).Variant forms of these diseases are generally called overlap syndromes,although there has been no standardised definition.Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC.The AIH-PBC overlap syndrome is the most common form,affecting almost 10% of adults with AIH or PBC.Single cases of AIH and autoimmune cholangitis(AMA-negative PBC) overlap syndrome have also been reported.The AIH-PSC overlap syndrome is predominantly found in children,adolescents and young adults with AIH or PSC.Interestingly,transitions from one autoimmune to another have also been reported in a minority of patients,especially transitions from PBC to AIH-PBC overlap syndrome.Overlap syndromes show a progressive course towards liver cirrhosis and liver failure without treatment.Therapy for overlap syndromes is empiric,since controlled trials are not available in these rare disorders.Anticholestatic therapy with ursodeoxycholic acid is usually combined with immunosuppressive therapy with corticosteroids and/or azathioprine in both AIH-PBC and AIH-PSC overlap syndromes.In end-stage disease,liver transplantation is the treatment of choice.展开更多
The most appropriate treatment for Klatskin tumor(KT)with a curative intention is multimodal therapy based on achieving resection with tumour-free margins(R0resections)combined with other types of neoadjuvant or adjuv...The most appropriate treatment for Klatskin tumor(KT)with a curative intention is multimodal therapy based on achieving resection with tumour-free margins(R0resections)combined with other types of neoadjuvant or adjuvant treatment(the most important factor affecting KT survival is the possibility of R0 resections,achieving 5-year survival rate of 40%-50%).Thirty to forty percent of patients with KT are inoperable and present a 5-year survival rate of 0%.In irresectable non-disseminated KT patients,using liver transplantation without neoadjuvant treatment,the 5-year survival rate increase to 38%,reaching 50%survival in early stage.In selected cases,with liver transplantation and neoadjuvant treatment(chemotherapy and radiotherapy),the actuarial survival rate is 65%at 5 years and 59%at 10 years.In conclusion,correct staging,neoadjuvant treatment,living donor and priority on the liver transplant waiting list may lead to improved results.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
文摘We present a female patient with continuous melena, diagnosed with rectal variceal bleeding. She had a history of esophageal varices, which were treated with endoscopic therapy. Five years after the treatment of esophageal varices, continuous melena occurred. Since colonoscopy showed that the melena was caused by giant rectal varices, we thought that they were not suitable to receive endoscopic treatment. We chose the modified percutaneous transhepatic obliteration with sclerosant, which is one of the interventional radiology techniques but a new clinical procedure for rectal varices. After the patient received this therapy, her condition of rectal varices was markedly improved.
文摘Objective:To investigate the clinical efficacy and safety of ultrasound-guided local anesthesia for endovenous laser combined with sclerotherapy in the treatment of varicose great saphenous veins.Methods:A total of 53 patients with varicose great saphenous veins admitted to our hospital from December 2023 to June 2025 were selected and divided into a traditional surgery group(18 cases)and a laser combined with sclerotherapy group(35 cases)according to the surgical method.The venous clinical severity score(VCSS),chronic venous insufficiency quality of life questionnaire(CIVIQ)score,visual analog scale(VAS)score for pain,complication rate,surgical time,treatment cost,recovery time,and patient satisfaction were compared between the two groups at 1 week,1 month,and 3 months postoperatively.Results:The VCSS scores of the laser group at each postoperative time point were lower than those of the traditional group,and the CIVIQ scores were higher than those of the traditional group(all p<0.05).The incidence of complications in the laser group(8.57%),the VAS score at 24 hours postoperatively,the duration of pain,and the utilization rate of analgesic medications were all significantly lower than those in the conventional group(all p<0.05).The laser group also demonstrated shorter operative and recovery times compared to the conventional group,along with higher patient satisfaction,albeit at a higher treatment cost(p<0.05).Conclusion:Endovenous laser combined with sclerotherapy under ultrasound guidance for the treatment of great saphenous vein varicosis offers advantages such as minimal trauma,rapid recovery,mild pain,and fewer complications,demonstrating significant clinical efficacy and good safety,thus possessing high clinical application value.
文摘Cholangiocarcinoma(CCA)is a rare type of cancer which arises from the bile duct epithelium and carries a poor prognosis.One of the main risk factors in the Western world is primary sclerosing cholangitis.Surgical resection has traditionally been the only curative treatment but can only be offered to patients with early disease,excluding those with locally advanced disease.Despite initial poor outcomes,liver transplantation(LT)has evolved as a viable treatment for a select group of patients with CCAs that are deemed unresectable.This review aims to explore the evolution of the role of LT in patients with CCA.
文摘BACKGROUND Sclerosing encapsulating peritonitis(SEP),also known as abdominal cocoon syndrome,is rare in children.The etiology of primary SEP is believed to be associated with retrograde menstruation or viral peritonitis in young adolescent girls,whereas secondary SEP refers to SEP caused by other factors such as surgery,peritoneal dialysis,or tuberculosis.Secondary SEP is rare and lack diagnostic specificity in children.Given the potential to cause acute intestinal obstruction,surgical intervention is often required.CASE SUMMARY We report the case of a 10-year-old girl with secondary SEP who was admitted to our center with acute bowel obstruction.CONCLUSION In this report,we emphasized the imaging manifestations,diagnosis,and operative management of the case.Although postoperative SEP in children is rare,the long-term prognosis is favorable when accompanied with accurate diagnosis,appropriate perioperative management,and timely follow-up.
文摘This article relates to the discussion of a recent study published by Wohl et al.Primary sclerosing cholangitis(PSC)is a chronic inflammatory liver disease that affects the intra-and extrahepatic bile ducts and is strongly associated with ulcerative colitis(UC).Endoscopic evaluation of UC is feasible and reliable in routine clinical practice,and the Mayo endoscopic subscore(MES)is one of the most commonly used endoscopic evaluation measures for UC.Patients with PSCUC are at higher risk of developing cancer and biliary tract cancer.Endoscopic scoring alone appears unreliable,and histopathological evaluation is essential to accurately assess and make effective therapeutic decisions for PSC-UC.Therefore,we aimed to discuss the accuracy of MES in patients with UC and PSC-UC and to explore the consistency between MES and the Nancy histological index.
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BACKGROUND Ovarian sclerosing stromal tumors(OSSTs)are found most commonly in females at 20-30 years of age.They can also occur at any point during pre-puberty,puberty,or menopause.Clinical manifestations of OSSTs include abdominal pain,an abdominal mass,menstrual abnormalities,and infertility.Infrequently,patients will experience androgen-related manifestations of masculinization,such as increased hair,acne,or a low voice.Diagnosis must be confirmed by immunohistochemical analysis of the tissue as clinical symptoms and imaging studies are unreliable.CASE SUMMARY A 14-year-old female presented with amenorrhea.After a thorough medical examination,endocrine and tumor markers analysis,and imaging,a pelvic mass was discovered.The patient also exhibited endocrine dysfunction but was not positive for any tumor markers.The patient underwent surgery to remove the ovarian tumor.Immunohistochemical analysis of the resected specimen indicated an OSST.During the postoperative follow-up,the patient had attained menarche.CONCLUSION This case’s clinical manifestation of endocrine dysfunction due to OSST provides new insights that will assist clinicians in the diagnosis and treatment of this common tumor.
文摘BACKGROUND Autoimmune liver disease(AILD)recurrence is common after liver transplantation(LT).While several risk factors for recurrence have been identified,their combined predictive value has yet to be thoroughly investigated.AIM To evaluate the combined predictive value of clinical and laboratory risk factors for AILD recurrence after LT.METHODS This retrospective cohort study included 79 patients with AILD who underwent LT at a single liver transplant center.We compared clinical and laboratory variables between patients with and without recurrent disease and assessed the predictive performance of these factors using four logistic regression models and their corresponding area underthe receiver operating characteristic curve (AUC).RESULTSRecurrent AILD occurred in 26.58% of patients (95%CI: 17-38), the median time to recurrence was 28 months(interquartile range: 16-38). Patients with recurrent AILD had significantly higher pre-transplant Child-Pugh scores[11.61 ± 1.16 vs 10.58 ± 1.96 points;odds ratio (OR) = 1.43, 95%CI: 1.03-2.00;P = 0.032] and model for end-stage liverdisease score (MELD) (22.76 ± 5.47 vs 18.81 ± 7.24 points;OR = 1.08, 95%CI: 1.01-1.16;P = 0.032), compared to thosewithout recurrence. Additionally, baseline alanine aminotransferase (ALT) > 2 times the upper limit of normal(ULN) was significantly associated with recurrence (31% vs 57.1%;OR = 2.96, 95%CI: 1.06-8.28;P = 0.038). Ourmodels, incorporating several risk variables, demonstrated moderate predictive ability for AILD recurrence. TheAUCs were as follows: (1) Model 1 (AUC = 0.75, 95%CI: 0.58-0.87);(2) Model 2 (AUC = 0.74, 95%CI: 0.59-0.90);(3)Model 3 (AUC = 0.72, 95%CI: 0.58-0.88);and (4) Model 4 (AUC = 0.63, 95%CI: 0.40-0.76), with no statisticallysignificant difference between the models (P = 0.488).CONCLUSIONHigher pre-transplant Child-Pugh and MELD scores, as well as ALT > 2 ULN, were associated with an increasedrisk of AILD recurrence.
基金Supported by The Key Research and Development Project of the Science and Technology Department of Sichuan Province,China,No.2023YFS0280The High-Level Research Initiation Fund of The First Affiliated Hospital of Chengdu Medical College,China,No.CYFY-GQ43.
文摘Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome.After thoroughly reviewing their work,we offer insights that primarily relate to their study design to enhance the medical community’s understanding of this complex 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.
基金supported by grants from the National Natural Science Foundation of China(No.82273082)Chongqing Technical Innovation and Application Development Key Project(No.cstc2021jscx-cylhX0005)。
文摘To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.
文摘Polidocanol is the most widely used Sclerotherapy in European and American countries. It is affordable, easy to use and apply, and has high efficiency. The purpose of this paper is to give an overview of the recent basics of the Sclerotherapy, chemistry and formulation, clinical applications, and side-effects and complications of Polidocanol, and to discuss the Polidocanol as the safety and efficacy of Sclerotherapy.
文摘Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.
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.
文摘Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseas-es(AiLD),namely autoimmune hepatitis types 1 and 2(AIH-1 and 2),primary biliary cirrhosis(PBC),and the sclerosing cholangitis variants in adults and children.AIH-1 is specified by anti-nuclear antibody(ANA) and smooth muscle antibody(SMA).AIH-2 is specified by antibody to liver kidney microsomal antigen type-1(anti-LKM1) and anti-liver cytosol type 1(anti-LC1).SMA,ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation.PBC is specified by antimitochondrial antibodies(AMA) react-ing with enzymes of the 2-oxo-acid dehydrogenase complexes(chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly react-ing with nuclear pore gp210 and nuclear body sp100.Sclerosing cholangitis presents as at least two variants,first the classical primary sclerosing cholangitis(PSC) mostly affecting adult men wherein the only(and non-specific) reactivity is an atypical perinuclear antineutro-phil cytoplasmic antibody(p-ANCA),also termed peri-nuclear anti-neutrophil nuclear antibodies(p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis(ASC) with serological features resembling those of type 1 AIH.Liver diagnostic serol-ogy is a fast-expanding area of investigation as new purified and recombinant autoantigens,and automatedtechnologies such as ELISAs and bead assays,become available to complement(or even compete with) tradi-tional immunofluorescence procedures.We survey for the first time global trends in quality assurance impact-ing as it does on(1) manufacturers/purveyors of kits and reagents,(2) diagnostic service laboratories that fulfill clinicians' requirements,and(3) the end-user,the physician providing patient care,who must properly interpret test results in the overall clinical context.
文摘The three major immune disorders of the liver are autoimmune hepatitis(AIH),primary biliary cirrhosis(PBC) and primary sclerosing cholangitis(PSC).Variant forms of these diseases are generally called overlap syndromes,although there has been no standardised definition.Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC.The AIH-PBC overlap syndrome is the most common form,affecting almost 10% of adults with AIH or PBC.Single cases of AIH and autoimmune cholangitis(AMA-negative PBC) overlap syndrome have also been reported.The AIH-PSC overlap syndrome is predominantly found in children,adolescents and young adults with AIH or PSC.Interestingly,transitions from one autoimmune to another have also been reported in a minority of patients,especially transitions from PBC to AIH-PBC overlap syndrome.Overlap syndromes show a progressive course towards liver cirrhosis and liver failure without treatment.Therapy for overlap syndromes is empiric,since controlled trials are not available in these rare disorders.Anticholestatic therapy with ursodeoxycholic acid is usually combined with immunosuppressive therapy with corticosteroids and/or azathioprine in both AIH-PBC and AIH-PSC overlap syndromes.In end-stage disease,liver transplantation is the treatment of choice.
文摘The most appropriate treatment for Klatskin tumor(KT)with a curative intention is multimodal therapy based on achieving resection with tumour-free margins(R0resections)combined with other types of neoadjuvant or adjuvant treatment(the most important factor affecting KT survival is the possibility of R0 resections,achieving 5-year survival rate of 40%-50%).Thirty to forty percent of patients with KT are inoperable and present a 5-year survival rate of 0%.In irresectable non-disseminated KT patients,using liver transplantation without neoadjuvant treatment,the 5-year survival rate increase to 38%,reaching 50%survival in early stage.In selected cases,with liver transplantation and neoadjuvant treatment(chemotherapy and radiotherapy),the actuarial survival rate is 65%at 5 years and 59%at 10 years.In conclusion,correct staging,neoadjuvant treatment,living donor and priority on the liver transplant waiting list may lead to improved results.
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.
