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Cancer of the uncinate process of the pancreas:surgical anatomy and clinicopathological features

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Hepatobiliary & Pancreatic Diseases International 2009年第6期8卷 569-574页

作者： Adrian W. O'Sullivan Nigel Heaton Mohamed Rela institute of liver studies King's College Hospital National Health Service(NHS) Foundation Trust

BACKGROUND:The clinicopathological features of uncinate process pancreatic cancer(UPPC) are poorly *** the anatomy of the uncinate process and its division during surgery are central to pancreaticoduodenectomy for *** set out to describe the embryology and anatomy of the uncinate process and the clinicopathological features of *** SOURCES:All published case series of UPPC were reviewed and included in this ***:The true incidence of UPPC is difficult to quantify,with the reported incidence ranging from 2.5% to 10.7% of pancreatic *** are 5 published series of UPPC including 117 patients,72 males and 45 females,aged from 45-53 years to 61-84 *** median survival was 5 or 5.5 months in 3 of the series,12.1 months in another based only on potentially resectable lesions and 17 months in another based only on resected ***:The number of reported series of UPPC is limited,with vague symptoms as the predominant presenting features of the *** prognosis is poor with synchronous venous resection demonstrating a survival advantage.

关键词： pancreatic neoplasms uncinate process anatomy embryology surgery

Update on Autoimmune Hepatitis

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Journal of Clinical and Translational Hepatology 2015年第1期3卷 42-52页

作者： Rodrigo Liberal Diego Vergani Giorgina Mieli-Vergani paediatric liver GI & Nutrition Centre and Institute of Liver Studies King's College London School of Medicine at King's College Hospital London UK

Autoimmune hepatitis (AIH),a liver disorder affecting both children and adults,is characterized by inflammatory liver histology,elevated transaminase levels,circulating nonorgan-specific autoantibodies,and increased levels of immunoglobulin G,in the absence of a known *** types of AIH are recognized according to seropositivity:smooth muscle antibody and/or antinuclear antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or liver cytosol type 1 define AIH type *** type 1 affects both adults and children,while AIH type 2 is mainly a paediatric disease,though it does occasionally affects young *** should be considered during the diagnostic workup of any patient with increased liver enzyme *** is exquisitely responsive to immunosuppressive treatment with prednisolone with or without azathioprine,with symptom free longterm survival for the majority of *** those who do not respond to standard treatment,or who are difficult-totreat,mycophenolate mofetil and,in the absence of a response,calcineurin inhibitors should be tried in addition to *** pathogenesis of AIH is not fully understood,although there is mounting evidence that genetic susceptibility,molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune *** damage is thought to be mediated primarily by CD4 T-cells,although recent studies support the involvement of diverse populations,including Th17 cells.A deeper understanding of the pathogenesis of AIH is likely to contribute to the development of novel treatments,such as the adoptive transfer of autologous expanded antigenspecific regulatory T-cells,which ultimately aim at restoring tolerance to liver-derived antigens.

关键词： Autoimmune hepatitis Autoantibodies Immunogenetics Regulatory T cells Immunosuppresion

Diagnosis in bile acid-CoA:Amino acid N-acyltransferase deficiency

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World Journal of Gastroenterology 2012年第25期18卷 3322-3326页

作者： Nedim Hadzi Laura N Bull Peter T Clayton AS Knisely paediatric liver service and institute of liver studies King's College HospitalLondon SE5 9RSUnited Kingdom liver Center Laboratory University of California San FranciscoSan FranciscoCA 94110United States Biochemistry Research Group Clinical and Molecular Genetics UnitUniversity College London Institute of Child HealthLondon WC1 N1EHUnited Kingdom institute of liver studies King's College HospitalLondon SE5 9RSUnited Kingdom

Cholate-CoA ligase （,EEL） and bile acid-CoA： amino acid N-acyltransferase （BAAT） sequentially mediate bile-acid amidation. Defects can cause intrahepatic cholestasis. Distinction has required gene sequencing. We assessed potential clinical utility of immunostaining of liver for CCL and BAAT. Using commercially available antibodies against BAAT and CCL, we immunostained liver from an infant with jaundice, deficiency of amidated bile acids, and transcription-terminating mutation in BAAT. CCL was normally expressed. BAAT expression was not de- tected. Immunostaining may facilitate diagnosis in bile- acid amidation defects.

关键词： Amidation Bile acid-CoA Amino acid N-ac- yltransferase Cholate-CoA ligase, Cholestasis Conjuga-tion Electrospray ionisation-mass spectroscopy Immu-nohistochemistry liver Neonatal hepatitis SIC27A5 Transmission electron microscopy

Pancreatoblastoma:A rare indication for liver transplantation in children

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Hepatobiliary & Pancreatic Diseases International 2020年第5期19卷 499-501页

作者： Maria Mercadal-Hally Sucheta Vaidya Hector Vilca-Melendez Nigel Heaton Anil Dhawan Tassos Grammatikopoulos paediatric liver GINutrition Centre and MowatLabsKing’s College Hospital NHS Foundation TrustDenmark HillLondon SE59RSUK Department of paediatric Oncology The Royal Marsden HospitalSuttonLondon SE59RSUK institute of liver studies King’s College Hospital NHS Foundation TrustDenmark HillLondon SE59RSUK

Pancreatoblastoma(PB)is one of the exocrine pancreatic tumors and the most common malignant pancreatic tumor in young *** can present with abdominal distension,pain,fatigue,vomiting and failure to *** pancreatic tumors usually affect patients between 1 and 8 years,with a median age of 5 *** is a slight preponderance in males and those of Asian descent[1].PB is considered to be embryonic in origin with moderately raised serum alpha-fetoprotein(AFP)levels[2].Axial imaging is necessary to assess other organ involvement,a common complication in adult patients,but diagnosis is confirmed on tissue histology[3].Complete surgical resection is the treatment of choice,if *** for neoadjuvant systemic chemotherapy include large tumors that involve adjacent major blood vessels or other organs and metastatic disease[1].Herein we report a pediatric case of PB with liver metastases who underwent liver transplantation for metastatic liver disease.

关键词： organs diagnosis chemotherapy

Autoimmmune hepatitis

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Cellular & Molecular Immunology 2022年第2期19卷 158-176页

作者： Benedetta Terziroli Beretta-Piccoli Giorgina Mieli-Vergani Diego Vergani Epatocentro Ticino&Facoltàdi Scienze Biomediche Universitàdella Svizzera ItalianaLuganoSwitzerland institute for Research in Biomedicine BellinzonaSwitzerland. King’s College London Faculty of Life Sciences&Medicine at King’s College Hospital LondonUK. paediatric liver GI and Nutrition CentreMowatLabsKing’s College HospitalLondonUK. institute of liver studies MowatLabsKing’s College HospitalLondonUK.

Autoimmune hepatitis(AIH)is a T-cell mediated,inflammatory liver disease affecting all ages and characterized by female preponderance,elevated serum transaminase and immunoglobulin G levels,positive circulating autoantibodies,and presence of interface hepatitis at liver *** type 1,affecting both adults and children,is defined by positive anti-nuclear and/or antismooth muscle antibodies,while type 2 AIH,affecting mostly children,is defined by positive anti-liver-kidney microsomal type 1 and/or anti-liver cytosol type 1 *** the autoantigens of type 2 AIH are well defined,being the cytochrome P4502D6(CYP2D6)and the formiminotransferase cyclodeaminase(FTCD),in type 1 AIH they remain to be ***-1 predisposition is conferred by possession of the MHC class II HLA DRB1*03 at all ages,while DRB1*04 predisposes to late onset disease;AIH-2 is associated with possession of DRB1*07 and DRB1**** majority of patients responds well to standard immunosuppressive treatment,based on steroid and azathioprine;second-and third-line drugs should be considered in case of intolerance or insufficient *** review offers a comprehensive overview of pathophysiological and clinical aspects of AIH.

关键词： Autoimmune Hepatitis Immunopathophysiology Treatment Genetic Predisposition

Torsion of spleen and portal hypertension: Pathophysiology and clinical implications

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World Journal of Hepatology 2021年第7期13卷 774-780页

作者： Ashish Kumar Jha Sameer Bhagwat Vishwa Mohan Dayal Arya Suchismita Department of Gastroenterology Indira Gandhi Institute of Medical SciencesPatna 800014India Department of paediatric Hepatology Institute of Liver and Biliary SciencesDelhi 110070India

The displacement of spleen from its normal location to other places is known as wandering spleen(WS)and is a rare *** repeated torsion of WS is due to the presence of long pedicle and absence/laxity of anchoring ligaments.A WS is an extremely rare cause of left-sided portal hypertension(PHT)and severe gastric variceal ***-sided PHT usually occurs as a result of splenic vein occlusion caused by splenic torsion,extrinsic compression of the splenic pedicle by enlarged spleen,and splenic vein *** is a paucity of data on WSrelated PHT,and these data are mostly in the form of case *** this review,we have analyzed the data of 20 reported cases of WS-related *** mechanisms of pathogenesis,clinico-demographic profile,and clinical implications are described in this *** majority of patients were diagnosed in the second to third decade of life(mean age:26 years),with a strong female preponderance(M:F=1:9).Eleven of the 20 WS patients with left-sided PHT presented with abdominal pain and *** 6 of the 11 patients,varices were detected incidentally on preoperative imaging studies or discovered ***,pre-operative search for varices is required in patients with splenic torsion.

关键词： Wandering spleen Splenic torsion Left-sided portal hypertension Gastric variceal bleeding Splenectomy

Autoimmune paediatric liver disease

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World Journal of Gastroenterology 2008年第21期14卷 3360-3367页

作者： Giorgina Mieli-Vergani Diego Vergani institute of liver studies King's College London School of Medicine at King's College HospitalLondon SE5 9RSUnited Kingdom

liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver *** is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody(SMA/ANA,type 1) or liver kidney microsomal antibody(LKM1,type 2).There is a female predominance in ***1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity,presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both *** most common type of paediatric sclerosing cholangitis is *** clinical,biochemical,immunological,and histological presentation of ASC is often indistinguishable from that of AIH type *** both,there are high IgG,non-organ specific autoantibodies,and interface *** is made by *** with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters,and decreased inflammatory activity on follow up liver ***,the cholangiopathy can *** may be evolution from AIH to ASC over the years,despite *** novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies,hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and *** classical AIH,it responds to treatment with prednisolone and *** novo AIH postliver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and *** this condition is a distinct entity or a form of atypical rejection

关键词： Autoimmune hepatitis Autoimmunesclerosing cholangitis liver transplant Children

Unveiling hidden outcomes in malignant gastric outlet obstruction research–insights from a"Pancreas 2000"review

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World Journal of Gastrointestinal Endoscopy 2024年第8期16卷 451-461页

作者： Filipe Vilas-Boas Giacomo Emanuele Maria Rizzo Charles De Ponthaud Stuart Robinson Sebastien Gaujoux Gabriele Capurso Giuseppe Vanella Bahadır Bozkırlı Department of Gastroenterology São João University HospitalPorto 4200Portugal Endoscopy service Department of Diagnostic and Therapeutic ServicesScientific Institute for ResearchHospitalization and Healthcare-The Mediterranean Institute for Transplantation and Highly Specialized TherapiesPalermo 90127SiciliaItaly Department of Digestive Hepato-Biliary and Pancreatic Surgery and Liver TransplantationAP-HP Pitié-SalpêtriereSorbonne UniversitéParis 75013Île-de-FranceFrance Department of Hepatobiliary Pancreatic and Transplant SurgeryFreeman HospitalNewcastle NE77DNNewcastle upon TyneUnited Kingdom Division of Pancreato-Biliary Endoscopy and Endosonography Pancreas Translational and Clinical Research CenterIRCCS San Raffaele Scientific InstituteVita-Salute San Raffaele UniversityMilano 20132Italy Department of General Surgery HPB-UnitAcıbadem Maslak HospitalIstanbul 34398Türkiye

Malignant gastric outlet obstruction(mGOO)is a major condition affecting patients with periampullary tumors,including pancreatic *** current treatment options include surgical gastroenterostomy,endoscopic stenting and more recently EUS-guided *** studies comparing the outcomes of the three procedures focus on technical success,clinical success and ***“occult”outcomes relevant to the patient’s viewpoints and perspective may ultimately impact on cancer-related and overall survival,such as body mass composition,nutritional biomarkers,chemotherapy tolerance and patient-reported quality of *** aim of this review is to provide an overview of potential key outcomes that should be explored in future comparative research around mGOO treatment options.

关键词： Malignant gastric outlet obstruction Endoscopic ultrasound-guided gastroenterostomy Patient-reported outcomes Body composition Nutrition Quality of life

Autoimmune liver serology:Current diagnostic and clinical challenges

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World Journal of Gastroenterology 2008年第21期14卷 3374-3387页

作者： Dimitrios P Bogdanos Diego Vergani Pietro Invernizzi Ian R Mackay institute of liver studies King's College London School of Medicine at King's College HospitalDenmark Hill CampusLondon SE5 9RSUnited Kingdom institute of liver studies King’s College London School of Medicine at King’s College HospitalDenmark Hill CampusLondon SE5 9RSUnited Kingdom Division of Internal Medicine and liver Unit San Paolo Hospital School of MedicineUniversity of Mi-lanMilan 20142Italy Department of Biochemistry and Molecular Bi-ology Monash UniversityClaytonVictoria 3800Australia

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 ***-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 *** 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 *** 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 *** 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 *** 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.

关键词： Autoantigen Autoimmune hepatitis Auto-antibody Primary biliary cirrhosis Primary sclerosing cholangitis liver disease