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Severe chronic diarrhea and maculopapular rash:A case report

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World Journal of Gastroenterology 2011年第34期17卷 3948-3952页

作者： Alessandra Elvevi Federica Grifoni Federica branchi Umberto Gianelli Dario Conte Gastrointestinal Unit 2 Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico and University of Milan Federica Grifoni Hematology Unit Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico and University of Milan Umberto Gianelli Department of Pathology Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico and University of Milan

Systemic mastocytosis (SM) is a heterogeneous disease of the bone marrow characterized by abnormal growth, accumulation and activation of clonal mast cells (MCs). We report a case of SM with multiorgan involvement. A 30-year-old man presented with diarrhea, flushing, maculopapular rash with itching and weight loss. The upper and lower gastrointestinal endoscopies showed macroscopic involvement of stomach and duodenum; mucosal samples from stomach, duodenum, colon and distal ileum showed mucosal inf iltration by large, spindleshaped MCs with abnormal surface molecule expression (CD2 and CD25), a picture fully consistent with SM, according to the World Health Organization diagnostic criteria. A computed tomography scan showed diffuse lymphadenopathy, hepatosplenomegaly and diffuse small bowel involvement. Bone marrow aspirate and biopsy were diagnostic for SM; serum tryptase levels were increased (209 ng/mL, normal values < 20 ng/mL). The conclusive diagnosis was smouldering SM. There wereno therapeutic indications except for treatment of symptoms. The patient was strictly followed up because of the risk of aggressive evolution.

关键词： Mast cells Systemic mastocytosis Bone marrow Tryptase

Non-invasive evaluation of intestinal disorders: The role of elastographic techniques

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World Journal of Gastroenterology 2017年第16期23卷 2832-2840页

作者： Federica branchi Flavio Caprioli Stefania Orlando Dario Conte Mirella Fraquelli Gastroenterology and Endoscopy Unit Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico20122 MilanItaly Department of Pathophysiology and Transplantation Universitàdegli Studi di Milano20122 MilanItaly

Over the recent years the non-invasive techniques for the evaluation of the small bowel have been playing a major role in the management of chronic intestinal diseases, such as inflammatory bowel diseases(IBD). The diagnostic performances of magnetic resonance imaging, computed tomography and ultrasound in the field of small bowel disorders, have been assessed and established for more than two decades. Newer sonographic techniques, such as strain elastography and shear wave elastography, have been put forward for the assessment of disease activity and characterization of IBD-related damage in the setting of Crohn's disease and other gastrointestinal disorders. The data from the preliminary research and clinical studies have shown promising results as regards the ability of elastographic techniques to differentiate inflammatory from fibrotic tissue. The distinction between IBD activity(inflammation) and IBD-related damage(fibrosis) is currently considered crucial for the assessment and management of patients. Moreover, all the elastographic techniques are currently being considered in the setting of other intestinal disorders(e.g., rectal tumors, appendicitis). The aim of this paper is to offer both a comprehensive narrative review of the non-invasive techniques available for the assessment of small-bowel disorders, with particular emphasis on inflammatory bowel diseases, and a summary of the current evidence on the use of elastographic techniques in this setting.

关键词： elastography Fibrosis Inflammatory bowel disease Bowel wall Stricture

Non-invasive assessment of liver fibrosis in chronic hepatitis B

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World Journal of Gastroenterology 2014年第40期20卷 14568-14580页

作者： Federica branchi Clara Benedetta Conti Alessandra Baccarin Pietro Lampertico Dario Conte Mirella Fraquelli Second Division of Gastroenterology IRCCS Fondazione Cà Granda Ospedale Maggiore Policlinico Università degli Studi di Milano 20122 Milan Italy First Division of Gastroenterology IRCCS Fondazione Cà Granda Ospedale Maggiore Policlinico Università degli Studi di Milano 20122 Milan Italy

The goal of this review is to provide a comprehensive picture of the role,clinical applications and future perspectives of the most widely used non-invasive techniques for the evaluation of hepatitis B virus(HBV)*** the past decade many non-invasive methods have been developed to reduce the need for liver biopsy in staging fibrosis and to overcome whenever possible its limitations,mainly:invasiveness,costs,low reproducibility,poor acceptance by *** techniques conceived to assess liver stiffness,in particular transient elastography,and the most commonly used biological markers will be assessed against their respective role and limitations in staging hepatic *** evidence highlights that both liver stiffness and some bio-chemical markers correlatewith survival and major clinical end-points such as liver decompensation,development of hepatocellular carcinoma and portal *** the non-invasive techniques here discussed can play a major role in the management of patients with chronic HBV-related *** their prognostic value,transient elastography and some bio-chemical markers can be used to better categorize patients with advanced fibrosis and cirrhosis and assign them to different classes of risk for clinically relevant *** recent data indicates that the combined measurements of liver and spleen stiffness enable the reliable prediction of portal hypertension and esophageal varices development.

关键词： Liver fibrosis Cirrhosis Hepatitis B virus Transient elastography Non invasive markers

可适性居住建筑——基于用户需求的空间高效利用

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建筑学报 2013年第1期 36-39页

作者： branchi Pablo Emilio 魏力恺西班牙ACR集团西班牙纳瓦拉公立大学天津大学建筑学院天津300072

首先分析近年来社会和人口统计数字的一系列变化,这些变化显示出因房屋空间剩余而导致资源不充分利用的现象,并将促使人们对居住建筑产生新的使用方式;进而介绍了西班牙ACORDE项目提出的一种在开放建筑框架下最大限度提高空间灵活性和... 详细信息

首先分析近年来社会和人口统计数字的一系列变化,这些变化显示出因房屋空间剩余而导致资源不充分利用的现象,并将促使人们对居住建筑产生新的使用方式;进而介绍了西班牙ACORDE项目提出的一种在开放建筑框架下最大限度提高空间灵活性和可适性的新理念。指出该项目希望尽可能提高空间灵活度,通过植入设备将各功能单元分隔成随意连接或隔断的独立空间,从而允许用户根据未来需求实现建筑空间的可适性变化。

关键词： ACORDE项目居住建筑可适性开放建筑建筑生命周期节能

Esophagogastric junction outflow obstruction successfully treated with laparoscopic Heller myotomy and Dor fundoplication: First case report in the literature

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World Journal of Gastrointestinal Surgery 2019年第2期11卷 112-116页

作者： Pedro F Pereira Andre RP Rosa Leonardo A Mesquita Marcelle J Anzolch Rafael N branchi Augusto L Giongo Francisco C Paix?o Marcio F Chedid Cleber DP Kruel Division of Gastrointestinal Surgery and Liver Transplantation Hospital de Clinicas de Porto AlegreFederal University of Rio Grande do Sul

BACKGROUND Esophagogastric junction outflow obstruction(EGJOO) is a rare syndrome,characterized by an elevation of the integrated relaxation pressure of the lower esophageal sphincter, not accompanied by alterations in esophageal motility that may lead to the criteria for achalasia. We were unable to find any prior report of the combination of Heller myotomy with anterior partial fundoplication(Dor) as the treatment for EGJOO. We herein report a case of EGJOO treated with laparoscopic Heller myotomy combined with Dor *** SUMMARY A 26-year-old man presented with a 3-year history of solid dysphagia and a 30-kg weight loss. He was treated with oral nifedipine, isosorbide, and omeprazole,without resolution of symptoms. An upper gastrointestinal series(barium swallow) revealed a "bird's beak" sign. Esophagogastroduodenoscopy was positive for Los Angeles grade A peptic esophagitis. High-resolution esophageal manometry was compatible with EGJOO. Esophageal pH monitoring showed pathological acid reflux both in orthostatic and decubitus position. An 8-cm laparoscopic Heller myotomy combined with an anterior 220° Dor fundoplication was performed. Solid diet was introduced on postoperative day 2, and the patient was discharged home the same day. At 17-mo follow-up, he reported no symptoms. Barium swallow was compatible with complete radiologic *** esophageal manometry and upper endoscopy showed normal findings 9 mo after the *** Surgical treatment with Heller myotomy and Dor fundoplication is a potential treatment option for EGJOO refractory to medical treatment.

关键词： Esophagogastric junction outflow obstruction Heller myotomy Partial fundoplication Dor fundoplication Case report

Diagnosis of gluten related disorders: Celiac disease, wheat allergy and non-celiac gluten sensitivity

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World Journal of Gastroenterology 2015年第23期21卷 7110-7119页

作者： Luca Elli Federica branchi Carolina Tomba Danilo Villalta Lorenzo Norsa Francesca Ferretti Leda Roncoroni Maria Teresa Bardella Center for the Prevention and Diagnosis of Celiac Disease Gastroenterology and Endoscopy Unit Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico Department of Pathophysiology and Transplantation Università degli Studi di Milano Allergology and Clinical Immunology Unit AOSanta Maria degli Angeli Department of Biomedical Surgical and Dental Sciences Università degli Studi di Milano

Cereal crops and cereal consumption have had a vital role in Mankind's history. In the recent years gluten ingestion has been linked with a range of clinical disorders. Gluten-related disorders have gradually emerged as an epidemiologically relevant phenomenon with an estimated global prevalence around 5%. Celiac disease, wheat allergy and non-celiac gluten sensitivity represent different gluten-related disorders. Similar clinical manifestations can be observed in these disorders, yet there are peculiar pathogenetic pathways involved in their development. Celiac disease and wheat allergy have been extensively studied, while non-celiac gluten sensitivity is a relatively novel clinical entity, believed to be closely related to other gastrointestinal functional syndromes. The diagnosis of celiac disease and wheat allergy is based on a combination of findings from the patient's clinical history and specific tests, including serology and duodenal biopsies in case of celiac disease, or laboratory and functional assays for wheat allergy. On the other hand, non-celiac gluten sensitivity is still mainly a diagnosis of exclusion, in the absence of clear-cut diagnostic criteria. A multimodal pragmatic approach combining findings from the clinical history, symptoms, serological and histological tests is required in order to reach an accurate diagnosis. A thorough knowledge of the differences and overlap in clinical presentation among gluten-related disorders, and between them and other gastrointestinal disorders, will help clinicians in the process of differential diagnosis.

关键词： Celiac disease Gluten sensitivity Wheatsensitivity Allergy Gluten-free diet

Role of capsule endoscopy in suspected celiac disease: A European multi-centre study

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World Journal of Gastroenterology 2017年第4期23卷 703-711页

作者： Marisol Luján-Sanchis Enrique Pérez-Cuadrado-Robles Javier Garcia-Lledo José-Francisco Juanmartinena Fernandez Luca Elli Victoria-Alejandra Jiménez-García Juan Egea-Valenzuela Julio Valle-Munoz Cristina Carretero-Ribón Ignacio Fernández-Urién-Sainz Antonio López-Higueras Noelia Alonso-Lazaro Mileidis Sanjuan-Acosta Francisco Sanchez-Ceballos Bruno Rosa Santiago González-Vázquez Federica branchi Lucía Ruano-Díaz César Prieto-de-Frías Vicente Pons-Beltrán Pilar Borque-Barrera Begona González-Suárez Sofía Xavier Federico Argüelles-Arias Juan-Manuel Herrerías-Gutiérrez Enrique Pérez-Cuadrado-Martínez Javier Sempere-García-Argüelles Digestive Diseases Unit General University Hospital of Valencia Small Bowel Unit Hospital Morales Meseguer Digestive Diseases Unit General University Gregorio Maranón Unit of Gastroenterology and Endoscopy Complejo Hospitalario de Navarra Center for prevention and Diagnosis of Celiac Disease Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico Unit of Gastroenterology and Endoscopy University Hospital Virgen Macarena Gastroenterology department Hospital Virgen de la Arrixaca Department of Gastroenterology Complejo Hospitalario de Toledo Department of Gastroenterology University of Navarra Clinic1 Endoscopy Digestive Unit Digestive Diseases AreaUniversitari i Politècnic La Fe Hospital1 Digestive Diseases Unit University Hospital Nuestra Senora de Candelaria1 Digestive Diseases Unit Clinical Hospital San Carlos1 Digestive Diseases Unit Hospital da Senhora da Oliveira-Guimaraes1 Endoscopy Digestive Unit Hospital Clinic de Barcelona

AIMTo analyze the diagnostic yield (DY), therapeutic impact (TI) and safety of capsule endoscopy (CE).METHODSThis is a multi-centre, observational, analytical, retrospective study. A total of 163 patients with suspicion of celiac disease (CD) (mean age = 46.4 ± 17.3 years, 68.1% women) who underwent CE from 2003 to 2015 were included. Patients were divided into four groups: seronegative CD with atrophy (Group-I, n = 19), seropositive CD without atrophy (Group-II, n = 39), contraindication to gastroscopy (Group-III, n = 6), seronegative CD without atrophy, but with a compatible context (Group-IV, n = 99). DY, TI and the safety of CE were *** overall DY was 54% and the final diagnosis was villous atrophy (n = 65, 39.9%), complicated CD (n = 12, 7.4%) and other enteropathies (n = 11, 6.8%; 8 Crohn’s). DY for groups I to IV was 73.7%, 69.2%, 50% and 44.4%, respectively. Atrophy was located in duodenum in 24 cases (36.9%), diffuse in 19 (29.2%), jejunal in 11 (16.9%), and patchy in 10 cases (15.4%). Factors associated with a greater DY were positive serology (68.3% vs 49.2%, P = 0.034) and older age (P = 0.008). On the other hand, neither sex nor clinical presentation, family background, positive histology or HLA status were associated with DY. CE results changed the therapeutic approach in 71.8% of the cases. Atrophy was associated with a greater TI (92.3% vs 45.3%, P < 0.001) and 81.9% of the patients responded to diet. There was one case of capsule retention (0.6%). Agreement between CE findings and subsequent histology was 100% for diagnosing normal/other conditions, 70% for suspected CD and 50% for complicated *** has a high DY in cases of suspicion of CD and it leads to changes in the clinical course of the disease. CE is safe procedure with a high degree of concordance with histology and it helps in the differential diagnosis of CD.

关键词： Capsule endoscopy Celiac disease Anti-transglutaminase antibodies Gluten-free diet Non-celiac gluten sensitivity