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Transanal eco-Doppler evaluation after hemorrhoidal artery embolization

World Journal of Gastroenterology 2024年第17期30卷 2332-2342页

作者： Roberta Tutino Tommaso Stecca Fabrizio Farneti Marco Massani Giulio Aniello Santoro Dipartimento Chirurgia generale e Specialistica AOU Cittàdella Salute e della Scienza di TorinoTorino 10126Italy division of general Surgery 1 Treviso Regional HospitalAULSS 2 Marca TrevigianaTreviso 31100Italy division of radiology Treviso Regional HospitalAULSS 2 Marca TrevigianaTreviso 31100Italy Tertiary Referral Pelvic Floor Center Division of General Surgery 2AULSS 2 Marca TrevigianaTreviso 31100Italy

BACKGROUND Hemorrhoidal artery embolization(Emborrhoid)is a novel method for the treatment of severe hemorrhoidal *** having a technical success rate of 93%-100%,the clinical success ranges between 63%and 94%,with a rebleeding rate of 13.6%.AIM To evaluate the effectiveness of this procedure in reducing hemorrhoidal flow and hemorrhoidal *** This prospective observational pilot study was conducted at division of general Surgery 1 and Tertiary Referral Pelvic Floor Center,Treviso Regional Hospital,*** a 2 months period(February-March 2022),consecutive patients with hemorrhoidal bleeding scores(HBSs)≥4,Goligher scores of II or III,failure of non-operative management,and a candidate for Emborrhoid were *** ultrasound with eco-Doppler was performed preoperatively and 1 month after the *** primary endpoint was to quantify the changes in arterial hemorrhoidal flow after *** secondary endpoint was to evaluate the correlation between the flow changes and the *** Eleven patients underwent *** overall pretreatment mean systolic peak(MSP)was 14.66 cm/*** highest MSP values were found in the anterior left lateral(17.82 cm/s at 1 o’clock and 15.88 cm/s at 3 o’clock)and in the posterior right lateral(14.62 cm/s at 7 o’clock and 16.71 cm/s at 9 o’clock)quadrants of the anal *** treatment,the overall MSP values were significantly reduced(P=0.008)although the correlation between MSP and HBS changes was weak(P=0.570).A statistical difference was found between distal embolization compared with proximal embolization(P=0.047).However,the coil landing zone was not related to symptoms improvement(P=1.000).A significant difference in MSP changes was also reported between patients with type 1 and type 2 superior rectal artery(SRA)anatomy(P=0.040).No relationship between hemorrhoidal grades(P=1.000),SRA anatomy(P=1.000)and treatment outcomes was *** The preliminary findings of this pilo

关键词： Hemorrhoidal artery embolization Hemorrhoidal embolization Hemorrhoidal vascularization Transanal eco-Doppler Transanal ultrasound

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Extravasated contrast volumetric assessment on computed tomography angiography in gastrointestinal bleeding:A useful predictor of positive angiographic findings

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World Journal of radiology 2024年第5期16卷 115-127页

作者： Laura Maria Cacioppa Chiara Floridi Alessandra Bruno NicolòRossini Tommaso Valeri Alessandra Borgheresi Riccardo Inchingolo Francesco Cortese Giacomo Novelli Alessandro Felicioli Mario Torresi Pietro Boscarato Letizia Ottaviani Andrea Giovagnoni division of Interventional radiology Department of Radiological SciencesUniversity Politecnica Delle MarcheAncona 60126Italy Department of Clinical Special and Dental SciencesUniversity Politecnica Delle MarcheAncona 60126Italy division of radiology Department of Radiological SciencesUniversity Hospital"Azienda Ospedaliero Universitaria Delle Marche"Ancona 60126Italy Interventional radiology Unit "F Miulli"Regional General HospitalAcquaviva delle Fonti 70021Italy Department of radiology IRCCS INRCAAncona 60126Italy

BACKGROUND Gastrointestinal bleeding(GIB)is a severe and potentially life-threatening condition,especially in cases of delayed *** tomography angiography(CTA)plays a pivotal role in the early identification of upper and lower GIB and in the prompt treatment of the *** To determine whether a volumetric estimation of the extravasated contrast at CTA in GIB may be a predictor of subsequent positive angiographic *** In this retrospective single-centre study,35 patients(22 men;median age 69 years;range 16-92 years)admitted to our institution for active GIB detected at CTA and further submitted to catheter angiography between January 2018 and February 2022 were ***-three(65.7%)patients underwent endoscopy before *** volumetry was evaluated in both arterial and venous phases via a semi-automated dedicated *** rate was obtained from volume change between the two phases and standardised for unit *** were divided into two groups,according to the angiographic signs and their concordance with *** Upper bleeding accounted for 42.9%and lower GIB for 57.1%.Mean haemoglobin value at the admission was 7.7 g/dL.A concordance between positive CTA and direct angiographic bleeding signs was found in 19(54.3%)*** no significant differences in terms of bleeding volume in the arterial phase(0.55 mL vs 0.33 mL,P=0.35),a statistically significant volume increase in the venous phase was identified in the group of patients with positive angiography(2.06 mL vs 0.9 mL,P=0.02).In the latter patient group,a significant increase in bleeding rate was also detected(2.18 mL/min vs 0.19 mL/min,P=0.02).CONCLUSION In GIB of any origin,extravasated contrast volumetric analysis at CTA could be a predictor of positive angiography and may help in avoiding further unnecessary procedures.

关键词： Gastrointestinal haemorrhage Computed tomography angiography Volumetric analysis Computer-assisted image interpretation Therapeutic embolization Transcatheter arterial embolization

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Cellular angiofibroma arising from the rectocutaneous fistula in an adult: A case report

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World Journal of Clinical Cases 2024年第10期12卷 1778-1784页

作者： Hao-En Chen Yu-Yang Lu Ruei-Yu Su Hong-Hau Wang Chao-Yang Chen Je-Ming Hu Jung-Cheng Kang Kuan-Hsun Lin Ta-Wei Pu Department of Surgery Taichung Armed Forces General HospitalTaichung 411Taiwan Department of Surgery Tri-Service General HospitalNational Defense Medical CenterTaipei 114Taiwan Department of pediatrics Tri-Service General HospitalNational Defense Medical CenterTaipei 114Taiwan Department of Pathology Tri-Service General HospitalNational Defense Medical CenterTaipei 105Taiwan Department of Pathology and Laboratory Medicine Taoyuan Armed Forces General HospitalTaoyuan 32551Taiwan Department of radiology Tri-Service General Hospital Songshan BranchNational Defense Medical CenterTaipei 105Taiwan division of Colon and Rectal Surgery Department of SurgeryTri-Service General HospitalNational Defense Medical CenterTaipei 114Taiwan division of Colon and Rectal Surgery Department of SurgeryTaiwan Adventist HospitalTaipei 105Taiwan division of Thoracic Surgery Department of SurgeryTri-Service General HospitalNational Defense Medical CenterTaipei 114Taiwan division of Colon and Rectal Surgery Department of SurgeryTri-Service General Hospital Songshan BranchNational Defense Medical CenterTaipei 105Taiwan

BACKGROUND Rectocutaneous fistulae are *** infection originates within the anal glands and subsequently extends into adjacent regions,ultimately resulting in fistula *** angiofibroma(CAF),also known as an angiomy ofibroblastoma-like tumor,is a rare benign soft tissue neoplasm predominantly observed in the scrotum,perineum,and inguinal area in males and in the vulva in *** describe the first documented case CAF that developed within a rectocutaneous fistula and manifested as a perineal *** SUMMARY In the outpatient setting,a 52-year-old male patient presented with a 2-year history of a growing perineal mass,accompanied by throbbing pain and minor scrotal *** examination revealed a soft,well-defined,non-tender mass at the left buttock that extended towards the perineum,without a visible *** initial assessment identified a soft tissue tumor,and the laboratory data were within normal *** and pelvic computed tomography(CT)revealed swelling of the abscess cavity that was linked to a rectal cutaneous fistula,with a track-like lesion measuring 6 cm×0.7 cm in the left perineal region and attached to the left *** examination found no significant inner orifices.A left medial gluteal incision revealed a thick-walled mass,which was excised along with the extending tract,and curettage was *** examination confirmed CAF *** patient achieved total resolution during follow-up assessments and did not require additional *** CT imaging supports perineal lesion diagnosis and *** angiofibromas,even with a cutaneous fistula,can be excised transperineally.

关键词： Angiofibroma Perineal mass Rectocutaneous fistula Anorectal fistula Anal fistula Case report

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Diseases of bile duct in children

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World Journal of Gastroenterology 2024年第9期30卷 1043-1072页

作者： Sutha Eiamkulbutr Chomchanat Tubjareon Anapat Sanpavat Teerasak Phewplung Nimmita Srisan Palittiya Sintusek Department of pediatrics King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of pediatrics Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of Pathology Chulalongkorn UniversityBangkok 10330Thailand Department of radiology Chulalongkorn UniversityBangkok 10330Thailand Department of Surgery Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Center of Excellence in Thai pediatric Gastroenterology Hepatology and ImmunologyDivision of GastroenterologyDepartment of PediatricsKing Chulalongkorn Memorial HospitalChulalongkorn UniversityBangkok 10330Thailand

Several diseases originate from bile duct *** studies on these diseases,certain etiologies of some of them still cannot be *** most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical *** diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to *** majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial *** cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings *** article provides a concise review of bile duct diseases,with interesting cases.

关键词： Bile duct Cholestasis Biliary atresia Biliary hypoplasia Biliary imaging Inspissated bile syndrome Choledochal cyst

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Treatment of SMA type 1 infants using a single-dose AAV9-mediated gene therapy via intrathecal injection of GC101: An open-label, single-arm study

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中华医学杂志(英文版) 2024年第16期137卷 2024 Jul 9页

作者： Ma Xiuwei Zhuang Lu Ma Wenhao Li Jun Wang Xiaodong Li Zhongqiu Jiang Xinyang Wang Yongxia Du Ying Zhang Yingqian He Fang Zhu Zhiming Du Shaopeng Xu Juan Gu Ruijie Zhang Yanping Zhang Shan Li Ting Yang Xiao Zhang Sheng Zhu Lina Li Qiuping Dong Xiaoyan Wu Xiaobing Feng Zhichun Department of Neurodevelopment Faculty of Pediatrics The Seventh Medical Center of Chinese PLA General Hospital Beijing China National Engineering Laboratory for Birth Defects Prevention and Control of Key Technology Beijing China Research and Development division Genecradle Therapeutics Inc. Beijing China Medical division Genecradle Therapeutics Inc. Beijing China Department of Pharmacy Medical Supplies Center of Chinese PLA General Hospital Beijing China Beijing Key Laboratory of pediatric Organ Failure Beijing China

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A novel homozygous missense variant in ARSK causes MPS X,a new subtype of mucopolysaccharidosis

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Genes & Diseases 2024年第3期11卷 87-90页

作者： Miao Sun Cornelia K.Kaminsky Philip Deppe Mai-Brittllse Frederic M.Vaz Barbara Plecko Torben Lubke Linda M.Randolph division of Genomic Medicine Department of Pathology and Laboratory MedicineChildren's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA Department of radiology Children's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA Department of Chemistry BiochemistryBielefeld UniversityBielefeld 33615Germany Amsterdam UMC Location University of Amsterdam Department of Clinical Chemistry and PediatricsLaboratory Genetic Metabolic DiseasesEmma Children's HospitalMeibergdreef 9Amsterdam 1100 DEthe Netherlands Amsterdam Gastroenterology Endocrinology Metabolism Inborn Errors of MetabolismAmsterdam 1105 BKthe Netherlands Core Facility Metabolomics Amsterdam UMC Location University of AmsterdamAmsterdam 1100DDthe Netherlands Department of pediatrics Division of General PediatricsMedical Universityof GrazGraz8036Austria division of Medical Genetics Department of PediatricsChildren's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA

Mucopolysaccharidoses(MPS)are a group of rare inborn errors of metabolism caused by defective lysosomal enzymes which prevent cells from degrading and recycling certain carbohydrates and fats,resulting in the storage of glycosaminoglycans in cells throughout the *** leads to multisystem abnormalities involving bone,connective tissues,brain,blood,spinal cord,skin,and other tissues.

关键词： metabolism doses polys

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World Journal of Clinical Cases 2024年第1期12卷 176-179页

作者： Hsin-Chia Angela Lin Kam-Fai Lee Tzu Hao Huang division of general Surgery Department of SurgeryChang Gung Memorial HospitalChiayi 613Taiwan Department of Anatomic Pathology Chang Gung Memorial HospitalChiayi 613Taiwan

BACKGROUND Gastric IgG4-related disease(IgG4-RD)is rarely encountered in clinical practice,and especially more so among pediatric *** our knowledge,this is the first report of IgG4-RD presenting as a calcifying gastric mass in a *** describe how this entity was difficult to differentiate from a gastrointestinal stromal tumor(GIST)imaging-based ***,this case highlights the importance of considering IgG4-RD in the differential diagnosis of gastric tumor before performing surgical resection,especially to distinguish it from malignancy to avoid unnecessary *** SUMMARY The patient suffered from epigastric pain for several *** and computed tomography scan revealed a submucosal *** diagnoses included GIST,leiomyoma,teratoma,and mucinous ***,laparoscopic proximal gastrectomy allowed for the definitive diagnosis of IgG4-related stomach *** We emphasize the importance of considering IgG4-RD in the differential diagnosis of gastric submucosal tumors before performing surgical resection.

关键词： IgG4-related disease Gastrointestinal stromal tumor Child pediatric Case report

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Prevalence and risk factors for impaired renal function among Asian patients with nonalcoholic fatty liver disease

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Hepatobiliary & Pancreatic Diseases International 2024年第3期23卷 241-248页

作者： Chao Sun George Boon-Bee Goh Wan-Cheng Chow Wah-Kheong Chan Grace Lai-Hung Wong Wai-Kay Seto Yi-Hsiang Huang Han-Chieh Lin I-Cheng Lee Hye Won Lee Seung Up Kim Vincent Wai-Sun Wong Jian-Gao Fan Center for Fatty Liver Disease Department of GastroenterologyXinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Key Lab of Pediatric Gastroenterology and NutritionShanghai 200092China Department of Gastroenterology&Hepatology Singapore General HospitalSingaporeSingapore Gastroenterology and Hepatology Unit Department of MedicineFaculty of MedicineUniversity of MalayaKuala LumpurMalaysia Department of Medicine and Therapeutics and State Key Laboratory of Digestive Disease The Chinese University of Hong KongHong KongChina Department of Medicine The University of Hong KongHong KongChina Institute of Clinical Medicine National Yang Ming Chiao Tung University School of MedicineTaipeiTaiwanChina division of Gastroenterology and Hepatology Taipei Veterans General HospitalTaipeiTaiwanChina Faculty of Medicine National Yang Ming Chiao Tung University School of MedicineTaipeiTaiwanChina Department of Internal Medicine Yonsei University College of MedicineSeoulKorea

Background:Nonalcoholic fatty liver disease(NAFLD)is associated with impaired renal function,and both diseases often occur alongside other metabolic ***,the prevalence and risk factors for impaired renal function in patients with NAFLD remain *** objective of this study was to identify the prevalence and risk factors for renal impairment in NAFLD ***:All adults aged 18-70 years with ultrasound-diagnosed NAFLD and transient elastography examination from eight Asian centers were enrolled in this prospective *** fibrosis and cirrhosis were assessed by FibroScan-aspartate aminotransferase(FAST),Agile 3+and Agile 4 *** renal function and chronic kidney disease(CKD)were defined by an estimated glomerular filtration rate(eGFR)with value of<90 mL/min/1.73 m^(2) and<60 mL/min/1.73 m^(2),respectively,as estimated by the CKD-Epidemiology Collaboration(CKD-EPI)***:Among 529 included NAFLD patients,the prevalence rates of impaired renal function and CKD were 37.4%and 4.9%,*** multivariate analysis,a moderate-high risk of advanced liver fibrosis and cirrhosis according to Agile 3+and Agile 4 scores were independent risk factors for CKD(P<0.05).Furthermore,increased fasting plasma glucose(FPG)and blood pressure were significantly associated with impaired renal function after controlling for the other components of metabolic syndrome(P<0.05).Compared with patients with normoglycemia,those with prediabetes[FPG≥5.6 mmol/L or hemoglobin A1c(HbA1c)≥5.7%]were more likely to have impaired renal function(P<0.05).Conclusions:Agile 3+and Agile 4 are reliable for identifying NAFLD patients with high risk of *** glycemic control in the prediabetic stage might have a potential renoprotective role in these patients.

关键词： Nonalcoholic fatty liver disease Impaired renal function Agile 3+ Agile 4 Metabolic syndrome

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Fetal lung growth predicts the risk for early-life respiratory infections and childhood asthma

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World Journal of pediatrics 2024年第5期20卷 481-495页

作者： Dimitra E.Zazara Olympia Giannou Steven Schepanski Mirja Pagenkemper Anastasios D.Giannou Maike Pincus Ioannis Belios Stefan Bonn Ania C.Muntau Kurt Hecher Anke Diemert Petra Clara Arck division for Experimental Feto-Maternal Medicine Department of Obstetrics and Fetal MedicineUniversity Medical Center Hamburg-Eppendorf(UKE)Martinistraße 5220251HamburgGermany University Children’s Hospital UKEHamburgGermany Computer Engineering and Informatics Department Polytechnic SchoolUniversity of PatrasPatrasGreece Institute of Developmental Neurophysiology Center for Molecular Neurobiology Hamburg(ZMNH)UKEHamburgGermany Department of Obstetrics and Fetal Medicine UKEHamburgGermany Department of general Visceral and Thoracic SurgeryUKEHamburgGermany Section of Molecular Immunology and Gastroenterology Ⅰ.Department of MedicineUKEHamburgGermany pediatrics and pediatric Pneumology Practice BerlinGermany Institute of Medical Systems Biology ZMNHUKEHamburgGermany Hamburg Center for Translational Immunology UKEHamburgGermany

Background Early-life respiratory infections and asthma are major health burdens during *** predicting an increased risk for early-life respiratory diseases are ***,we identified the predictive value of ultrasound-monitored fetal lung growth for the risk of early-life respiratory infections and *** Fetal lung size was serially assessed at standardized time points by transabdominal ultrasound in pregnant women participating in a pregnancy *** between fetal lung growth and respiratory infections in infancy or early-onset asthma at five years were ***-learning models relying on extreme gradient boosting regressor or classifier algorithms were developed to predict respiratory infection or asthma risk based on fetal lung *** model development and validation,study participants were randomly divided into a training and a testing group,respectively,by the employed *** Enhanced fetal lung growth throughout pregnancy predicted a lower early-life respiratory infection *** sex was associated with a higher risk for respiratory infections in *** lung growth could also predict the risk of asthma at five years of *** designed three machine-learning models to predict the risk and number of infections in infancy as well as the risk of early-onset *** models’R2 values were 0.92,0.90 and 0.93,respectively,underscoring a high accuracy and agreement between the actual and predicted *** variables included known risk factors and novel predictors,such as ultrasound-monitored fetal lung *** Sonographic monitoring of fetal lung growth allows to predict the risk for early-life respiratory infections and asthma.

关键词： Asthma Child risk Early-life respiratory infections Prenatal lung development Sexual dimorphism

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Transjugular intrahepatic portosystemic shunt vs conservative treatment for recurrent ascites:A propensity score matched comparison

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World Journal of Gastroenterology 2022年第41期28卷 5944-5956页

作者： Martin Philipp Theresia Blattmann Jörn Bienert Kristian Fischer Luisa Hausberg Jens-Christian Kröger Thomas Heller Marc-AndréWeber Georg Lamprecht Department of Medicine Division of Gastroenterology and EndocrinologyRostock University Medical CenterRostock 18057Germany Institute of Diagnostic and Interventional radiology Pediatric Radiology and NeuroradiologyRostock University Medical CentreRostock 18057Germany

BACKGROUND Transjugular intrahepatic portosystemic shunt(TIPS) placement is an effective intervention for recurrent tense ascites. Some studies show an increased risk of acute on chronic liver failure(ACLF) associated with TIPS placement. It is not clear whether ACLF in this context is a consequence of TIPS or of the pre-existing liver *** To better understand the risks of TIPS in this challenging setting and to compare them with those of conservative *** Two hundred and fourteen patients undergoing their first TIPS placement for recurrent tense ascites at our tertiary-care center between 2007 and 2017 were identified(TIPS group). Three hundred and ninety-eight patients of the same time interval with liver cirrhosis and recurrent tense ascites not undergoing TIPS placement(No TIPS group) were analyzed as a control group. TIPS indication,diagnosis of recurrent ascites, further diagnoses and clinical findings were obtained from a database search and patient records. The in-hospital mortality and ACLF incidence of both groups were compared using 1:1 propensity score matching and multivariate logistic *** After propensity score matching, the TIPS and No TIPS groups were comparable in terms of laboratory values and ACLF incidence at hospital admission. There was no detectable difference in mortality(TIPS: 11/214, No TIPS 13/214). During the hospital stay, ACLF occurred more frequently in the TIPS group than in the No TIPS group(TIPS: 70/214, No TIPS: 57/214, P = 0.04). This effect was confined to patients with severely impaired liver function at hospital admission as indicated by a significant interaction term of Child score and TIPS placement in multivariate logistic regression. The TIPS group had a lower ACLF incidence at Child scores < 8 points and a higher ACLF incidence at ≥ 11 points. No significant difference was found between groups in patients with Child scores of 8 to 10 *** TIPS placement for recurrent tense as

关键词： Liver cirrhosis Ascites Transjugular intrahepatic portosystemic shunt Acute on chronic liver failure Mortality Propensity score

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