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Perigastric extraskeletal Ewing's sarcoma: A case report

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World Journal of Gastroenterology 2009年第2期15卷 245-247页

作者： Radoje B Colovic Nikica M Grubor Marjan T Micev Slavko V Matic Henry Dushan Edward Atkinson Stojan M Latincic institute for digestive diseases Clinical Center of Serbia Koste Todorovica 6 Belgrade 11000 Serbia Imperial College School of Medicine St Mary's Hospital Praed Street London W2 1NY United Kingdom

Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specifi c enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

关键词： Stomach Extraskeletal Ewing's sarcoma

Nonalcoholic fatty liver disease aggravates acute pancreatitis through bacterial translocation and cholesterol metabolic dysregulation in the liver and pancreas in mice

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Hepatobiliary & Pancreatic diseases International 2023年第5期22卷 504-511页

作者： Tian-Yu Lin Yi-Fan Zhang Yang Wang Yun Liu Jun Xu Yu-Lan Liu Department of Gastroenterology Peking University People’s HospitalBeijing 100044China clinical center of Immune-Mediated digestive diseases Peking University People’s HospitalBeijing 100044China Central Laboratory&institute of clinical Molecular Biology Peking University People’s HospitalBeijing 100044China

Background:Nonalcoholic fatty liver disease(NAFLD)is an independent risk factor for severe acute pancreatitis(AP).The underlying mechanism remains *** sought to determine how bacterial translocation and cholesterol metabolism in the liver and pancreas affect the severity of AP in NAFLD ***:C57BL/6N mice were fed on a high-fat diet(HFD)to generate the NAFLD model,and mice in the control group were provided with a normal diet(ND).After being anesthetized with ketamine/xylazine,mice got a retrograde infusion of taurocholic acid sodium into the pancreatic duct to induce AP,and sham operation(SO)was used as *** amylase and Schmidt’s pathological score system were used to evaluate AP *** loads,total cholesterol level,and cholesterol metabolic-associated molecules[low-density lipoprotein receptor(LDLR)and ATP-binding cassette transporter A1(ABCA1)]were analyzed in the liver and ***:Compared with the ND-AP group,mice in the HFD-AP group had severer pancreatitis,manifested with higher serum amylase levels and higher AP pathologic scores,especially the inflammation and hemorrhage *** with the HFD-SO group and ND-AP group,bacterial loads in the liver and pancreas were significantly higher in the HFD-AP *** in the HFD-AP group showed a decreased LDLR expression and an increased ABCA1 expression in the pancreas,although there was no significant difference in pancreas total cholesterol between the HFD-AP group and the ND-AP ***:NAFLD aggravates AP via increasing bacterial translocation in the liver and pancreas and affecting pancreas cholesterol metabolism in mice.

关键词： Nonalcoholic fatty liver disease Acute pancreatitis Bacterial translocation Cholesterol metabolism

Prognostic Factors Related to In-hospital Death in Children with Biliary Atresia:Analysis of a Nationwide Inpatient Database

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Journal of clinical and Translational Hepatology 2023年第2期11卷 416-424页

作者： Yu Shi Yi-Zhou Jiang Guang-Peng Zhou Ying Shi Lan-Xia Gan Yuan-Yuan Kong Hai-Bo Wang Zhi-Jun Zhu Li-Ying Sun Liver Transplantation center Beijing Friendship HospitalCapital Medical UniversityBeijingChina National clinical Research center for digestive diseases Beijing Friendship HospitalCapital Medical UniversityBeijingChina Department of Critical Liver diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityBeijingChina clinical center for Pediatric Liver Transplantation Capital Medical UniversityBeijingChina National clinical Research center for Cardiovascular diseases Fuwai Hospital Chinese Academy of Medical SciencesShenzhenGuangdong ProvinceChina China Standard Medical Information Research center ShenzhenGuangdongChina clinical Trial Unit Precision Medicine InstituteFirst Affiliated Hospital of Sun Yat-Sen UniversityGuangzhouChina

Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to *** study aimed to identify the possible risk factors affecting in-hospital death in BA patients in ***:We collected data from the Hospital Quality Monitoring System,a national inpatient *** patients aged up to 2 years old with a diagnosis of BA were *** subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no *** regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital ***:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of *** proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years *** signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital *** no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe ***,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.

关键词： Biliary atresia In-hospital death Liver transplantation Kasai portoenterostomy

TL1A Promotes Fibrogenesis in Colonic Fibroblasts via the TGF-β1/Smad3 Signaling Pathway

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Current Medical Science 2024年第3期44卷 519-528页

作者： Jia SONG Dong-lei SUN Chen-yang LI Yu-xin LUO Qian LIU Yue YAO Hong ZHANG Ting-ting YANG Mei SONG Xin-li BAI Xiao-lan ZHANG Department of Gastroenterology The Second Hospital of Hebei Medical UniversityHebei Key Laboratory of GastroenterologyHebei Institute of GastroenterologyHebei Clinical Research Center for Digestive DiseasesShijiazhuang050000China Department of Pediatrics The Second Hospital of Hebei Medical UniversityShijiazhuang050000China

Objective Intestinal fibrosis is a refractory complication of inflammatory bowel disease(IBD).Tumor necrosis factor ligand-related molecule-1A(TL1A)is important for IBD-related intestinal fibrosis in a dextran sodium sulfate(DSS)-induced experimental colitis *** study aimed to explore the effects of TL1A on human colonic *** A trinitrobenzene sulfonic acid(TNBS)-induced experimental colitis model of LCK-CD2-TL1A-GFP transgenic(Tg)or wild-type(WT)mice was established to determine the effect and mechanism of TL1A on intestinal *** human colonic fibroblast CCD-18Co cell line was treated concurrently with TL1A and human peripheral blood mononuclear cell(PBMC)*** proliferation and activation of CCD-18Co cells were detected by BrdU assays,flow cytometry,immunocytochemistry and Western *** metabolism was tested by Western blotting and real-time quantitative polymerase chain reaction(RT-qPCR).Results The level of collagen metabolism in the TNBS+ethyl alcohol(EtOH)/Tg group was greater than that in the TNBS+EtOH/WT *** growth factor-β1(TGF-β1)and p-Smad3 in the TNBS+EtOH/Tg group were upregulated as compared with those in the TNBS+EtOH/WT *** proliferation of CCD-18Co cells was promoted by the addition of human PBMC supernatant supplemented with 20 ng/mL TL1A,and the addition of human PBMC supernatant and TL1A increased CCD-18Co proliferation by 24.4%at 24 ***1A promoted cell activation and increased the levels of COL1A2,COL3A1,and TIMP-1 in CCD-18Co *** of CCD-18Co cells with TL1A increased the expression of TGF-β1 and *** TL1A promotes TGF-β1-mediated intestinal fibroblast activation,proliferation,and collagen deposition and is likely related to an increase in the TGF-β1/Smad3 signaling pathway.

关键词： tumor necrosis factor ligand-related molecule-1A fibrosis inflammatory bowel disease myofibroblasts transforming growth factor-β1

Cystic lymphangioma of the pancreas

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World Journal of Gastroenterology 2008年第44期14卷 6873-6875页

作者： Radoje B Colovic Nikica M Grubor Marjan T Micev Henry Dushan E Atkinson Vitomir I Rankovic Mihajlo M Jagodic institute for digestive diseases Clinical Center of SerbiaFirst Surgical ClinicKoste Todorovica 6Belgrade11000Serbia Imperial College School of Medicine St Mary's HospitalPraed StreetLondon W21NYUnited Kingdom

Lymphangioma of the pancreas is an extremely rare benign tumour of lymphatic origin, with fewer than 60 published cases. Histologically, it is polycystic, with the cysts separated by thin septa and lined with endothelial cells. Though congenital, it can affect all age groups, and occurs more frequently in females. Patients usually present with epigastric pain and an associated palpable mass. Complete excision is curative, even though, depending on the tumour location, surgery may be simple or involve extensive pancreatic resection and anastomoses. The authors present a 49-year-old woman in whom a polycystic septated mass, 35 mm× 35 mm in size, was discovered by ultrasonography （US） in the body of the pancreas during investigations for epigastric pain and nausea. At surgery, a well circumscribed polycystic tumor was completely excised, with preservation of the pancreatic duct. The postoperative recovery was uneventful. Histology confirmed a microcystic lymphangioma of the pancreas. Immunohistochemistry showed cystic endothelial cells reactivity to factor Ⅷ -RA （＋＋）, CD31 （＋＋＋） and CD34 （-）. Postoperatively, abdominal pain disappeared and the patient remained symptomfree for 12 mo until now. Although extremely rare, lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of a pancreatic cystic lesion, especially in women.

关键词： Pancreas Cystic lymphangioma Localsurgical excision

Intraoperative endoscopy in obstructive hypopharyngeal carcinoma

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World Journal of Gastroenterology 2006年第28期12卷 4561-4564页

作者： Predrag Pesko Milos Bjelovic Predrag Sabljak Dejan Stojakov Ebrahimi Keramatollah Dejan Velickovic Bratislav Spica Branka Nenadic Aleksandra Djuric-Stefanovic Djordjije Saranovic Vera Todorovic Department of Esophagogastric Surgery First Surgical University HospitalClinical Center of SerbiaBelgradeSerbia and Montenegro institute of Anesthesiology First Surgical University HospitalClinical Center of SerbiaBelgradeSerbia and Montenegro Department of Radiology First Surgical University HospitalClinical Center of SerbiaBelgradeSerbia and Montenegro institute of Medical Investigations BelgradeSerbia and Montenegro

AIM： To demonstrate the necessity of intraoperative endoscopy in the diagnosis of secondary primary tumors of the upper digestive tract in patients with obstructive hypopharyngeal carcinoma. METHODS： Thirty-one patients with hypopharyngeal squamous cell carcinoma had been operated, with radical intent, at our Institution in the period between 1978 and 2004. Due to obstructive tumor mass, in 7 （22.6%） patients, preoperative endoscopic evaluation of the esophagus and stomach could not be performed. In those patients, intraoperative endoscopy, made through an incision in the cervical esophagus, was standard diagnostic method for examination of the esophagus and stomach. RESULTS： We found synchronous foregut carcinomas in 3 patients （9.7%）. In two patients, synchronous carcinomas had been detected during preoperative endoscopic evaluation, and in one （with obstructive carcinoma） using intraoperative endoscopy. In this case, preoperative barium swallow and CT scan did not reveal the existence of second primary tumor within esophagus, despite the fact that small, but T2 carcinoma, was present. CONCLUSION： It is reasonable to use intraoperative endoscopy as a selective screening test in patients with obstructive hypopharyngeal carcinoma.

关键词： Hypopharynx Neoplasms Squamous cell Endoscopy Multiple primary Synchronous tumors

Vanishing bile duct syndrome in human immunodeficiency virus infected adults:A report of two cases

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World Journal of Gastroenterology 2013年第1期19卷 115-121页

作者： Ana Paula Oppenheimer Christopher Koh Mary McLaughlin John C Williamson Thomas D Norton Jennifer Laudadio Theo Heller David E Kleiner Kevin P High Caryn G Morse Section of Infectious diseases Department of MedicineWake Forest School of MedicineWinston-SalemNC 27109-7205United States Liver diseases Branch National Institute of DiabetesDigestive and Kidney DiseasesNational Institutes of Health Clinical CenterBethesdaMD 20892-1403United States Laboratory of Immunoregulation National Institute of Allergy and Infectious DiseasesNational Institutes of Health Clinical CenterBethesdaMD 20892-1403United States Division of Infectious diseases and Immunology Department of MedicineNew York University School of MedicineNew YorkNY 10016United States Laboratory of Pathology Wake Forest School of MedicineWinston-SalemNC 27109-7205United States Laboratory of Pathology National Cancer InstituteNational Institutes of Health Clinical CenterBethesdaMD 20892-1403United States Department of Critical Care Medicine National Institutes of Health Clinical CenterBethesdaMD 20892-1403United States

Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to *** in association with medications,autoimmune disorders,cancer,transplantation,and infections,the specific mechanisms of disease are not *** date,only 4 cases of VBDS have been reported in human immunodeficiency virus(HIV) infected *** report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated *** includes hyperbilirubinemia,normal liver imaging,and negative viral and autoimmune hepatitis *** HIV-infected subjects,VBDS occurred at a range of CD4+ T-cell counts,in some cases following initiation or change in antiretroviral *** was associated with two cases;nevirapine,antibiotics,and viral co-infection were suggested as etiologies in the other *** HIV-positive patients with progressive cholestasis,early identification of VBDS and referral for transplantation may improve outcomes.

关键词： Human immunodeficiency virus Antiretroviral therapy Vanishing bile duct syndrome Ductopenia Liver biopsy

Five years of fecal microbiota transplantation-an update of the Israeli experience

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World Journal of Gastroenterology 2018年第47期24卷 5403-5414页

作者： Sharon A Greenberg Ilan Youngster Nathaniel A Cohen Dan M Livovsky Jacob Strahilevitz Eran Israeli Ehud Melzer Kalman Paz Naomi Fliss-Isakov Nitsan Maharshak Department of Gastroenterology and Liver diseases Tel Aviv Sourasky Medical Centeraffiliated with the Sackler Faculty of MedicineTel Aviv UniversityTel Aviv 6423906Israel Bacteriotherapy Clinic Tel Aviv Sourasky Medical Centeraffiliated with the Sackler Faculty of MedicineTel Aviv UniversityTel Aviv 6423906Israel Assaf Harofe Medical center Zerifin 70300Israel digestive diseases institute Shaare Zedek Medical CenterJerusalem 91031Israel Department of clinical Microbiology and Infectious diseases Hadassah-Hebrew UniversityJerusalem 91120Israel Department of Gastroenterology and Liver diseases Hadassah-Hebrew UniversityJerusalem 91120Israel Gastrointestinal and Liver diseases institute Kaplan Medical CenterRehovot 76100Israel

AIM To evaluate and describe the efficacy of fecal microbiota transplantation(FMT) for Clostridium difficile infection(CDI) in a national Israeli *** All patients who received FMT for recurrent(recurrence within 8 wk of the previous treatment) or refractory CDI from 2013 through 2017 in all the five medical centers in Israel currently performing FMT were included. Stool donors were screened according to the Israeli Ministry of Health guidelines. clinical and laboratory data of patients were collected from patients' medical files, and they included indications for FMT, risk factors for CDI and disease severity. Primary outcome was FMT success(at least 2 mo free of CDI-related diarrhea post-FMT). Secondary outcomes included initial response to FMT(cessation of diarrhea within 7 d) and recurrence at 6 *** There were 111 FMTs for CDI, with a median age of 70 years [interquartile range(IQR): 53-82], and 42%(47) males. Fifty patients(45%) were treated via the lower gastrointestinal(LGI, represented only by colonoscopy) route, 37(33%) via capsules, and 24(22%) via the upper gastrointestinal(UGI) route. The overall success rate was 87.4%(97 patients), with no significant difference between routes of administration(P = 0.338). In the univariant analysis, FMT success correlated with milder disease(P = 0.01), ambulatory setting(P < 0.05) and lower Charlson comorbidity score(P < 0.05). In the multivariant analysis, only severe CDI [odd ratio(OR) = 0.14, P < 0.05] and inpatient FMT(OR = 0.19, P < 0.05) were each independently inversely related to FMT success. There were 35(32%) patients younger than 60 years of age, and 14(40%) of them had a background of inflammatory bowel *** FMT is a safe and effective treatment for CDI, with capsules emerging as a successful and well-tolerated route. Severe CDI is less likely to respond to FMT.

关键词： Clostridium difficile infection Capsules Israel Fecal microbiota transplantation

Effect of liver transplantation with primary hyperoxaluria type 1:Five case reports and review of literature

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World Journal of clinical Cases 2023年第5期11卷 1068-1076页

作者： Xin-Yue Wang Zhi-Gui Zeng Zhi-Jun Zhu Lin Wei Wei Qu Ying Liu Yu-Le Tan Jun Wang Hai-Ming Zhang Wen Shi Li-Ying Sun Department of Critical Liver diseases Liver Research CenterBeijing Friendship HospitalCapital Medical UniversityBeijing 101100China Liver Transplantation center Beijing Friendship HospitalCapital Medical UniversityBeijing 101100China clinical center for Pediatric Liver Transplantation Capital Medical UniversityBeijing 101100China National clinical Research center for digestive diseases Beijing Friendship HospitalCapital Medical UniversityBeijing 101100China Beijing clinical Research institute Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND Primary hyperoxaluria type 1(PH1)is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase,resulting in increased endogenous oxalate deposition and end-stage renal *** transplantation is the only effective ***,its approach and timing remain *** SUMMARY We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant center of the Beijing Friendship Hospital from March 2017 to December *** cohort included 4 males and 1 *** median age at onset was 4.0 years(range:1.0-5.0),age at diagnosis was 12.2 years(range:6.7-23.5),age at liver transplantation(LT)was 12.2 years(range:7.0-25.1),and the follow-up time was 26.3 mo(range:12.8-40.1).All patients had delayed diagnosis,and 3patients had progressed to end-stage renal disease by the time they were *** patients received preemptive LT;their estimated glomerular filtration rate was maintained at>120 mL/min/1.73 m2,indicating a better *** patients received sequential liver and kidney *** transplantation,serum and urinary oxalate decreased,and liver function *** the last follow-up,the estimated glomerular filtration rates of the latter 3 patients were 179,52 and 21 mL/min/1.73 *** Different transplantation strategies should be adopted for patients based on their renal function ***-LT offers a good therapeutic approach for PH1.

关键词： Primary hyperoxaluria type 1 Liver transplantation Combined liver and kidney transplantation Sequential liver and kidney transplantation Renal calculi End-stage renal disease Case reports