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Crohn's disease and Takayasu's arteritis: An uncommon association

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World Journal of Gastroenterology 2013年第35期19卷 5933-5935页

作者： Andrea Taddio Massimo Maschio Stefano Martelossi Egidio Barbi Alessandro Ventura institute of child health IRCCS "Burlo Garofolo" Trieste and University of Trieste institute of child health IRCCS "Burlo Garofolo"

Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same *** finding has suggested the possibility that both diseases could share some common etiologic *** describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory *** and histological features from colonic specimens were consistent with *** with steroids and azathioprine was started,however disease flared every time steroids were *** year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and *** physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic *** associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo *** discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.

关键词： Crohn’s disease Takayasu arteritis Intractable inflammatory bowel disease children Treatment

Genetics of inflammatory bowel disease from multifactorial to monogenic forms

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World Journal of Gastroenterology 2015年第43期21卷 12296-12310页

作者： Anna Monica Bianco Martina Girardelli Alberto Tommasini institute for Maternal and child health-IRCCS "burlo garofolo"

Inflammatory bowel disease(IBD) is a group of chronic multifactorial disorders. According to a recent study,the number of IBD association loci is increased to 201,of which 37 and 27 loci contribute specifically to the development of Crohn's disease and ulcerative colitis respectively. Some IBD associated genes are involved in innate immunity,in the autophagy and in the inflammatory response such as NOD2,ATG16L1 and IL23 R,while other are implicated in immune mediated disease(STAT3) and in susceptibility to mycobacterium infection(IL12B). In case of early onset of IBD(VEO-IBD) within the 6th year of age,the disease may be caused by mutations in genes responsible for severe monogenic disorders such as the primary immunodeficiency diseases. In this review we discuss how these monogenic disorders through different immune mechanisms can similarly be responsible of VEO-IBD phenotype. Moreover we would highlight how the identification of pathogenic genes by Next Generation Sequencing technologies can allow to obtain a rapid diagnosis and to apply specific therapies.

关键词： Inflammatory bowel disease Primary immunodeficienc

Ages of celiac disease: From changing environment to improved diagnostics

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World Journal of Gastroenterology 2011年第32期17卷 3665-3671页

作者： Alberto Tommasini Tarcisio Not Alessandro Ventura Laboratory of Immunopathology Institute for Maternal and Child Health Scientific Institute for Research and Care Burlo Garofolo and University of Trieste Department of Pediatrics Institute for Maternal and Child Health Scientific Institute for Research and Care Burlo Garofolo and University of Trieste

From the time of Gee's landmark writings, the recent history of celiac disease (CD) can be divided into many ages, each driven by a diagnostic advance and a deeper knowledge of disease pathogenesis. At the same time, these advances were paralleled by the identification of new clinical patterns associated with CD and by a continuous redefinition of the prevalence of the disease in population. In the beginning, CD was considered a chronic indigestion, even if the causative food was not known; later, the disease was proven to depend on an intolerance to wheat gliadin, leading to typical mucosal changes in the gut and to a malabsorption syndrome. This knowledge led to curing the disease with a gluten-free diet. After the identification of antibodies to gluten (AGA) in the serum of patients and the identification of gluten-specific lymphocytes in the mucosa, CD was described as an immune disorder, resembling a chronic "gluten infection". The use of serological testing for AGA allowed identification of the higher prevalence of this disorder, revealing atypical patterns of presentation. More recently, the characterization of autoantibodies to endomysium and to transglutaminase shifted the attention to a complex autoimmune pathogenesis and to the increased risk of developing autoimmune disorders in untreated CD. New diagnostic assays, based on molecular technologies, will introduce new changes, with the promise of better defining the spectrum of gluten reactivity and the real burden of gluten related-disorders in the population. Herein, we describe the different periods of CD experience, and further developments for the next celiac age will be proposed.

关键词： Antibodies Autoimmunity Celiac disease Diagnostics History Intestinal mucosa

Is autophagy an elective strategy to protect neurons from dysregulated cholesterol metabolism?

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Neural Regeneration Research 2019年第4期14卷 582-587页

作者： Elisa Piscianz Liza Vecchi Brumatti Alberto Tommasini Annalisa Marcuzzi Department of Medicine Surgery and Health Sciences University of Trieste institute for Maternal and child health-IRCCS "burlo garofolo"

The balance of autophagy, apoptosis and necroptosis is crucial to determine the outcome of the cellular response to cholesterol dysregulation. Cholesterol plays a major role in regulating the properties of cell membranes, especially as regards their fluidity, and the regulation of its biosynthesis influences the shape and functions of these membranes. Whilst dietary cholesterol can easily be distributed to most organs, the central nervous system, whose membranes are particularly rich in cholesterol, mainly relies on de novo synthesis. For this reason, defects in the biosynthesis of cholesterol can variably affect the development of central nervous system. Moreover, defective synthesis of cholesterol and its intermediates may reflect both on structural cell anomalies and on the response to inflammatory stimuli. Examples of such disorders include mevalonate kinase deficiency, and Smith-Lemli-Opitz syndrome, due to deficiency in biosynthetic enzymes, and type C Niemann-Pick syndrome, due to altered cholesterol trafficking across cell compartments. Autophagy, as a crucial pathway dedicated to the degradation of cytosolic proteins and organelles, plays an essential role in the maintenance of homeostasis and in the turnover of the cytoplasmic material especially in the presence of imbalances such as those resulting from alteration of cholesterol metabolism. Manipulating the process of autophagy can offer possible strategies for improving neuronal cell viability and function in these genetic disorders.

关键词： cholesterol inflammation apoptosis autophagy neurons inherited disease necroptosis neuronal dysfunction

Pediatric stricturing Crohn's disease

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World Journal of Gastroenterology 2024年第12期30卷 1651-1654页

作者： Alessandro Boscarelli Matteo Bramuzzo Department of Pediatric Surgery and Urology Institute for Maternal and Child Health-IRCCS“Burlo Garofolo”Trieste 34137Italy Department of Gastrointestinal Endoscopy and Nutrition Institute for Maternal and Child Health-IRCCS"Burlo Garofolo"Trieste 34137Italy

Crohn’s disease(CD)is a chronic inflammatory disease of the digestive *** incidence of pediatric CD is increasing and is currently 2.5-11.4 per 100000 ***,approximately 25%of children with CD develop stricturing CD(SCD)that requires *** stricturing diseases refractory to pharmacological management frequently require non-pharmacological ***-pharmacological therapeutic strategies include endoscopic balloon dilatation,stricturoplasty,and surgical resection of the strictured ***,strictures tend to recur postoperatively regardless of treatment *** lifetime risk of surgery in patients with childhood SCD remains at 50%-90%.Thus,new and emerging strategies,advanced diagnostic tools,and minimally invasive approaches are under investigation to improve the outcomes and overall quality of life of pediatric patients with SCD.

关键词： Stricturing Crohn’s disease Pediatrics Insights Future perspectives

Altered pattern of tumor necrosis factor-alpha production in peripheral blood monocytes from Crohn's disease

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World Journal of Gastroenterology 2016年第41期22卷 9117-9126页

作者： Claudia Loganes Alessia Pin Samuele Naviglio Martina Girardelli Anna Monica Bianco Stefano Martelossi Alberto Tommasini Elisa Piscianz Department of Medicine Surgery and Health Sciences University of Trieste Department of Pediatrics Institute for Maternal and Child Health IRCCS "Burlo Garofolo"

AIM To evaluate the inflammatory state in Crohn's disease(CD) patients and correlate it with genetic background and microbial *** By means of flow cytometry, production of tumor necrosis factor-alpha(TNF-α) was measured in peripheral blood monocytes from patients suffering from CD, ulcerative colitis(UC) and in healthy subjects after stimulation of the NOD2 and TLR pathways. CD patients were genotyped for the three most common NOD2 variants(R702W, G908 R and L1007Pfs*2) and basal production of TNF-α was correlated to NOD2 genotype. Also, production of TNF-α was correlated to plasmatic levels of LPS Binding Protein(LBP), soluble(s) CD14 and to the activity state of the *** The patients with CD were characterized by a significantly higher monocyte basal expression of TNF-αcompared with healthy subjects and UC patients, and after stimulation with Pam3CSK4(ligand of TLR2/1) and MDP-L18(ligand of NOD2) this difference was maintained, while other microbial stimuli(LPS, ligand of TLR4 and Poly I:C, ligand of TLR3) induced massive activation in CD monocytes as well as in UC and in healthy control cells. There was no significant difference in the production of TNF- α between patients who carried CD-associated heterozygous or homozygous variants in NOD2 and patients with wild type NOD2 genotype. Although serum LBP levels have been shown to correlate positively with the state of activity of the disease, TNF-α production did not show a clear correlation with either LBP or s CD14 levels in plasma. Moreover, no clear correlation was seen between TNF-α production and activity indices in either CD or *** Peripheral monocytes from CD express higher basal and stimulated TNF-α than controls, regardless of NOD2 genotype and without a clear correlation with disease activity.

关键词： Crohn’s disease Ulcerative colitis Tumor necrosis factor-α NOD2 variants Toll like receptors Dysbiosis Activity index LPS-binding protein

Molecular mechMolecular mechanism of glucocorticoid resistance in inflammatory bowel disease

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World Journal of Gastroenterology 2011年第9期17卷 1095-1108页

作者： Sara De Iudicibus Raffaella Franca Stefano Martelossi Alessandro Ventura Giuliana Decorti Department of Reproduction and Development University of Trieste institute for Maternal and child health IRCCS burlo garofolo Department of Life Sciences University of Trieste

Natural and synthetic glucocorticoids (GCs) are widely employed in a number of inflammatory, autoimmune and neoplastic diseases, and, despite the introduction of novel therapies, remain the first-line treatment for inducing remission in moderate to severe active Crohn’s disease and ulcerative colitis. Despite their extensive therapeutic use and the proven effectiveness, considerable clinical evidence of wide inter-individual differences in GC efficacy among patients has been reported, in particular when these agents are used in inflammatory diseases. In recent years, a detailed knowledge of the GC mechanism of action and of the genetic variants affecting GC activity at the molecular level has arisen from several studies. GCs interact with their cytoplasmic receptor, and are able to repress inflammatory gene expression through several distinct mechanisms. The glucocorticoid receptor (GR) is therefore crucial for the effects of these agents: mutations in the GR gene (NR3C1, nuclear receptor subfamily 3, group C, member 1) are the primary cause of a rare, inherited form of GC resistance; in addition, several polymorphisms of this gene have been described and associated with GC response and ***, the GR is not self-standing in the cell and the receptor-mediated functions are the result of a complex interplay of GR and many other cellular partners. The latter comprise several chaperonins of the large cooperative hetero-oligomeric complex that binds the hormonefree GR in the cytosol, and several factors involved in the transcriptional machinery and chromatin remodeling, that are critical for the hormonal control of target genes transcription in the nucleus. Furthermore, variants in the principal effectors of GCs (e.g. cytokines and their regulators) have also to be taken into account for a comprehensive evaluation of the variability in GC response. Polymorphisms in genes involved in the transport and/or metabolism of these hormones have also been suggested as

关键词： Glucocorticoids Inflammatory bowel disease Pharmacogenomics

Association between orofacial granulomatosis and Crohn's disease in children:Systematic review

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World Journal of Gastroenterology 2014年第23期20卷 7497-7504页

作者： Marzia Lazzerini Matteo Bramuzzo Alessro Ventura institute for Maternal and child health IRCCS Burlo Garofolo34137 TriesteItaly University of Trieste 34137 TriesteItaly

AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.

关键词： Orofacial granulomatosis Melkersson-Rosenthal syndrome Cheilitis granulomatosa Crohn’ s disease children Systematic review

Ex vivo response to mucosal bacteria and muramyl dipeptide in inflammatory bowel disease

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World Journal of Gastroenterology 2016年第44期22卷 9734-9743页

作者： Claudia Loganes Erica Valencic Alessia Pin Elisa Marini Stefano Martelossi Samuele Naviglio Luigina De Leo Tarcisio Not Lorenzo Monasta Alberto Tommasini Annalisa Marcuzzi Department of Medicine Surgery and Health Sciences University of Trieste Strada di Fiume institute for Maternal and child health - IRCCS "burlo garofolo"

AIM To evaluate how mucosal bacteria impact on the spontaneous and muramyl dipeptide(MDP)-induced inflammation in Crohn's disease(CD) and ulcerative colitis(UC).METHODS Colonic mucosal biopsies were collected from children with active or remissive CD, UC and controls. Two tissue samples were taken from inflamed mucosal segments(in patients with active disease) or from noninflamed mucosa [in patients in remission or in healthy controls(HC)]. Experiments were performed in the presence or absence of antibiotics, to assess whether the disease-associated microbiota can modulate the cytokine response ex vivo. For this purpose, each specimen was half-cut to compare spontaneous and MDP-induced inflammation in the presence of live bacteria(LB) or antibiotics. After 24 h of culture, an array of 17 cytokines was assessed in supernatants. Statistical analyses were performed to find significant differences in single cytokines or in patterns of cytokine response in the different groups. RESULTS We demonstrated that subjects with CD display a spontaneous production of inflammatory cytokines including granulocyte-colony stimulating factor(G-CSF), interleukin(IL) 6, IL8, IL10 and IL12, that was not significantly influenced by the addition of antibiotics. UC specimens also displayed a trend of increased spontaneous secretion of several cytokines, which however was not significant due to broader variability among patients. After the addition of antibiotics, spontaneous IL8 secretion was significantly higher in UC than in controls. In HC, a trend towards the weakening of spontaneous IL8 production was observed in the presence of live mucosal bacteria with respect to the presence of antibiotics. In contrast, in the presence of LB UC showed an increasing trend of spontaneous IL8 production, while MDP stimulation resulted in lower IL8 production in the presence of antibiotics. We also showed that subjects with CD seem to have a lowered production of IL8 in response to MDP in the presence

关键词： Gut-microbiota Crohn’s disease Ulcerative colitis Cytokines Inflammation