15 results on '"Erasmo Miele"'
Search Results
2. Functional Gastrointestinal Disorders in Mediterranean Countries According to Rome IV Criteria
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Caterina Strisciuglio, Sabrina Cenni, Maria Rosaria Serra, Pasquale Dolce, Sanja Kolacek, Sara Sila, Ivana Trivic, Michal Rozenfeld Bar Lev, Raanan Shamir, Aco Kostovski, Alexandra Papadopoulou, Elefthería Roma, Christina Katsagoni, Danijela Jojkic-Pavkov, Silvia Salvatore, Licia Pensabene, Elena Scarpato, Erasmo Miele, Annamaria Staiano, Strisciuglio, C., Cenni, S., Serra, M. R., Dolce, P., Kolacek, S., Sila, S., Trivic, I., Lev, M. R. B., Shamir, R., Kostovski, A., Papadopoulou, A., Roma, E., Katsagoni, C., Jojkic-Pavkov, D., Salvatore, S., Pensabene, L., Scarpato, E., Miele, E., and Staiano, A.
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Rome IV criteria ,Adolescent ,Gastrointestinal Diseases ,Rome ,Gastroenterology ,Mediterranean area ,Irritable Bowel Syndrome ,Cross-Sectional Studies ,Functional gastrointestinal disorder ,Child, Preschool ,Surveys and Questionnaires ,Pediatrics, Perinatology and Child Health ,Prevalence ,Humans ,Child ,Constipation - Abstract
Objectives:The aim of this study was to assess the prevalence of functional gastrointestinal disorders (FGIDs) in children of Mediterranean area using Rome IV criteria and to compare the prevalence of FGIDs using Rome IV and Rome III criteria.Methods:This was a cross-sectional study enrolling children and adolescents living in Croatia, Greece, Israel, Italy, Macedonia, and Serbia. Subjects were examined in relation to the presence of FGIDs, using the Rome IV criteria. Data were compared with the results of a previous study using Rome III data.Results:We analyzed 1972 children ages 4 to 9 years (group A), and 2450 adolescents 10 to 18 years old (group B). The overall prevalence of FGIDs was 16% in group A and 26% in group B, with statistical differences among countries in both groups (P
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- 2021
3. Scoring Endoscopy in Pediatric Inflammatory Bowel Disease: A Way to Improve Quality
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M.T. Illiceto, Claudio Romano, Monica Paci, Lorenzo Norsa, Giovanni Di Nardo, Alberto Ferrari, Erasmo Miele, Erminia Romeo, Matteo Bramuzzo, Marco Deganello Saccomani, Marco Daperno, Salvatore Oliva, Serena Arrigo, Norsa, Lorenzo, Ferrari, Alberto, Arrigo, Serena, Bramuzzo, Matteo, Deganello Saccomani, Marco, Di Nardo, Giovanni, Illiceto, Maria Teresa, Miele, Erasmo, Paci, Monica, Romano, Claudio, Romeo, Erminia, Daperno, Marco, and Oliva, Salvatore
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Adult ,medicine.medical_specialty ,Intraclass correlation ,Concordance ,Colonoscopy ,Fleiss' kappa ,Severity of Illness Index ,Inflammatory bowel disease ,03 medical and health sciences ,0302 clinical medicine ,Crohn Disease ,crohn disease ,endoscopic score ,ulcerative colitis ,030225 pediatrics ,Internal medicine ,Severity of illness ,medicine ,Humans ,Child ,medicine.diagnostic_test ,business.industry ,Gastroenterology ,Reproducibility of Results ,Inflammatory Bowel Diseases ,medicine.disease ,Ulcerative colitis ,Confidence interval ,Pediatrics, Perinatology and Child Health ,Colitis, Ulcerative ,030211 gastroenterology & hepatology ,business - Abstract
Objectives and study There is a large interobserver variability in evaluating mucosal lesions of inflammatory bowel disease (IBD), especially in pediatric patients. This multicenter prospective observational study aims to evaluate interobserver agreement (IOA) among pediatric endoscopists in assigning validated IBD endoscopic scores in children. Methods Fifteen videos of follow-up ileocolonoscopies in children with IBD were recorded and selected as cases. Eleven pediatric endoscopists from different centers blindly evaluated all videos and calculated scores: either Ulcerative Colitis Endoscopic Index of Severity (UCEIS) or Simple Endoscopic Score for Crohn Disease (SES-CD). Scores from all reviewers were compared in order to calculate IOA for general videos and specific sections. Scores from an expert adult reader were used to calculate possible reviewer's characteristics affecting scores' reliability. Results Intraclass correlation was 0.298 (95% confidence interval [CI]: 0.13-0.55) for ulcerative colitis (UC) and 0.266 (0.11-0.52) for Crohn disease (CD). When a disease activity categorization was adopted (remission, mild, moderate, severe activity) Fleiss kappa coefficient was 0.408 (0.29-0.53) for UC and 0.552 (0.43-0.73) for CD. When stratified by item, vascular pattern of UC was the most reliable item IC: 0.624 (0.321-0.854). In multivariable analysis, none of the reviewer's characteristics affected the readers' errors. Conclusions This multicenter study shows low agreement among pediatric endoscopists in evaluating endoscopic scores in children with IBD. By using disease activity categorization, agreement slightly increased, mostly for CD. All readers showed a low-grade concordance with the expert adult gastroenterologist's evaluations. Future-specific training programs should be considered to increase IOA in using IBD endoscopic activity scores.
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- 2021
4. Drugs in Focus: The Use of Racecadotril in Paediatric Gastrointestinal Disease
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Emmanuel Mas, Ilse Broekaert, Erasmo Miele, Christos Tzivinikos, Jernej Dolinsek, Carmen Ribes-Koninckx, Marc A. Benninga, Corina Pienar, Rut Anne Thomassen, Nikhil Thapar, Rok Orel, Mike Thomson, Victor Babeş University of Medicine and Pharmacy (UMFT), Emma Children’s Hospital Academic Medical Centre, University Hospital of Cologne, University medical centre Maribor (UKC Maribor), Institut de Recherche en Santé Digestive (IRSD ), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Università degli studi di Napoli Federico II, University Medical Centre Ljubljana [Ljubljana, Slovenia] (UMCL), Hospital Universitario y Politécnico La Fe, Oslo University Hospital [Oslo], NHS Foundation Trust, Al Jalila Children's Specialty Hospital, UCL Great Ormond Street Institute of Child Health [London, UK], Great Ormond Street Hospital, Partenaires INRAE, Queensland Children's Hospital, Pienar, Corina, Benninga, Marc A, Broekaert, Ilse J, Dolinsek, Jernej, Mas, Emmanuel, Miele, Erasmo, Orel, Rok, Ribes-Koninckx, Carmen, Thomassen, Rut-Anne, Thomson, Mike, Tzivinikos, Christo, Thapar, Nikhil, Paediatric Gastroenterology, AGEM - Digestive immunity, AGEM - Re-generation and cancer of the digestive system, Amsterdam Reproduction & Development (AR&D), ProdInra, Migration, University of Naples Federico II = Università degli studi di Napoli Federico II, and Hospital Universitari i Politècnic La Fe = University and Polytechnic Hospital La Fe
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Diarrhea ,Drug ,Thiorphan ,[SDV.BIO]Life Sciences [q-bio]/Biotechnology ,Gastrointestinal Diseases ,medicine.drug_class ,media_common.quotation_subject ,medicine.medical_treatment ,Pharmacology ,Racecadotril ,03 medical and health sciences ,0302 clinical medicine ,adjuvant ,030225 pediatrics ,Antidiarrhoeal ,medicine ,Humans ,Antidiarrheals ,Child ,Adverse effect ,Neprilysin ,media_common ,racecadotril ,business.industry ,digestive, oral, and skin physiology ,Gastroenterology ,medicine.disease ,Small intestine ,[SDV.BIO] Life Sciences [q-bio]/Biotechnology ,3. Good health ,[SDV.AEN] Life Sciences [q-bio]/Food and Nutrition ,medicine.anatomical_structure ,Pharmaceutical Preparations ,Gastrointestinal disease ,Pediatrics, Perinatology and Child Health ,030211 gastroenterology & hepatology ,business ,gastroenteritis ,[SDV.AEN]Life Sciences [q-bio]/Food and Nutrition ,Adjuvant ,medicine.drug - Abstract
International audience; Acute diarrhoea is a leading cause of morbidity and mortality in the paediatric population. Racecadotril is an antisecretory drug recommended as an adjuvant anti-diarrhoeal treatment.In the small bowel, the enzyme neutral endopeptidase (NEP) inhibits the action of enkephalins, which prevent water and electrolyte hypersecretion. By inhibiting NEP, racecadotril allows enkephalins to exhibit their antisecretory effects. Consequently, racecadotril reduces the secretion of water and electrolytes in the small intestine, without having an effect on intestinal motility. No serious adverse events related to racecadotril have been reported.Racecadotril has proven its efficacy as an adjuvant anti-diarrhoeal drug with a good safety profile. Its addition to oral rehydration solution (ORS) appears clinically beneficial and potentially leads to health care savings.
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- 2020
5. Pediatric Eosinophilic Esophagitis: Results of the European Retrospective Pediatric Eosinophilic Esophagitis Registry (RetroPEER)
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Michal Kori, Noam Zevit, Sonny K. F. Chong, Jorge Amil Dias, Carolina G Junquera, Alexandra Papadopoulou, Claudio Romano, Víctor Vila Miravet, Andrzej Załęski, Gloria Domínguez-Ortega, Luba Marderfeld, Salvatore Oliva, Vaidotas Urbonas, Frédéric Gottrand, Marcus Auth, Sebastian Otte, Johanna H. Oudshoorn, Assaf Hoofien, Monica Malamisura, Maria Jm Gomez, Francesca Rea, Rok Orel, Roger Garcia-Puig, Mark Furman, Saskia Vande Velde, Amir Ben Tov, Nicolas Kalach, Erasmo Miele, Eleftheria Roma, Danielle Nijenhuis-Hendriks, Hoofien, Assaf, Dias, Jorge A, Malamisura, Monica, Rea, MARTINA FRANCESCA, Chong, Sonny, Oudshoorn, Johanna, Nijenhuis-Hendriks, Danielle, Otte, Sebastian, Papadopoulou, Alexandra, Romano, Claudio, Gottrand, Frederic, Miravet, Victor V, Orel, Rok, Oliva, Salvatore, Junquera, Carolina G, Załęski, Andrzej, Urbonas, Vaidota, Garcia-Puig, Roger, Gomez, Maria J M, Dominguez-Ortega, Gloria, Auth, Marcus K-H, Kori, Michal, Ben Tov, Amir, Kalach, Nicola, Velde, Saskia V, Furman, Mark, Miele, Erasmo, Marderfeld, Luba, Roma, Eleftheria, and Zevit, Noam
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Male ,medicine.medical_specialty ,Adolescent ,proton pump inhibitor ,medicine.drug_class ,MEDLINE ,Proton-pump inhibitor ,eosinophilic esophagitis ,03 medical and health sciences ,0302 clinical medicine ,children ,Food allergy ,030225 pediatrics ,medicine ,Humans ,Registries ,Child ,Eosinophilic esophagitis ,Retrospective Studies ,allergen ,Eosinophilic Oesophagitis ,Children ,business.industry ,Gastroenterology ,Retrospective cohort study ,respiratory system ,medicine.disease ,Dermatology ,Europe ,Clinical Practice ,Multicenter study ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Female ,030211 gastroenterology & hepatology ,business - Abstract
OBJECTIVES: Recommendations for diagnosing and treating eosinophilic esophagitis (EoE) are evolving; however, information on real world clinical practice is lacking. To assess the practices of pediatric gastroenterologists diagnosing and treating EoE and to identify the triggering allergens in European children. METHODS: Retrospective anonymized data were collected from 26 European pediatric gastroenterology centers in 13 countries. Inclusion criteria were: Patients diagnosis with EoE, completed investigations prescribed by the treating physician, and were on stable medical or dietary interventions. RESULTS: In total, 410 patients diagnosed between December 1999 and June 2016 were analyzed, 76.3% boys. The time from symptoms to diagnosis was 12 ± 33.5 months and age at diagnosis was 8.9 ± 4.75 years. The most frequent indications for endoscopy were: dysphagia (38%), gastroesophageal reflux (31.2%), bolus impaction (24.4%), and failure to thrive (10.5%). Approximately 70.3% had failed proton pump inhibitor treatment. The foods found to be causative of EoE by elimination and rechallenge were milk (42%), egg (21.5%), wheat/gluten (10.9%), and peanut (9.9%). Elimination diets were used exclusively in 154 of 410 (37.5%), topical steroids without elimination diets in 52 of 410 (12.6%), both diet and steroids in 183 of 410 (44.6%), systemic steroids in 22 of 410 (5.3%), and esophageal dilation in 7 of 410 (1.7%). Patient refusal, shortage of endoscopy time, and reluctance to perform multiple endoscopies per patient were noted as factors justifying deviation from guidelines. CONCLUSIONS: In this "real world" pediatric European cohort, milk and egg were the most common allergens triggering EoE. Although high-dose proton pump inhibitor trials have increased, attempted PPI treatment is not universal.
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- 2019
6. Impact of Environmental and Familial Factors in a Cohort of Pediatric Patients With Inflammatory Bowel Disease
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F.P. Giugliano, Annamaria Staiano, Caterina Strisciuglio, Luigi Greco, Erasmo Miele, S. Cenni, Massimo Martinelli, Strisciuglio, Caterina, Giugliano, Francesca Paola, Martinelli, Massimo, Cenni, Sabrina, Greco, Luigi, Staiano, Annamaria, Miele, Erasmo, and Giugliano, FRANCESCA PAOLA
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Male ,medicine.medical_specialty ,Adolescent ,Mediterranean diet ,Environment ,Diet, Mediterranean ,digestive system ,Inflammatory bowel disease ,03 medical and health sciences ,0302 clinical medicine ,Crohn Disease ,Risk Factors ,Surveys and Questionnaires ,Internal medicine ,medicine ,Humans ,Colitis ,Child ,Retrospective Studies ,business.industry ,Crohn disease ,Developed Countries ,Gastroenterology ,Case-control study ,Infant ,Retrospective cohort study ,Protective Factors ,medicine.disease ,digestive system diseases ,Logistic Models ,Italy ,Case-Control Studies ,Child, Preschool ,030220 oncology & carcinogenesis ,Multivariate Analysis ,Pediatrics, Perinatology and Child Health ,Immunology ,Cohort ,Colitis, Ulcerative ,Female ,030211 gastroenterology & hepatology ,business - Abstract
The primary role of environment on inflammatory bowel disease (IBD) onset has been recently stressed. We aimed to investigate the effect of environmental factors in an IBD pediatric cohort.A total of 467 subjects (264 IBD and 203 controls) were enrolled. All patients underwent a questionnaire including 5 different groups of environmental risk factors: family history of IBD and autoimmune diseases, perinatal period, home amenities and domestic hygiene, childhood diseases and vaccinations, and diet.In a multivariate model, mother's degree (odds ratio [OR]: 5.5; 2.5-11.6), duration of breast feeding3rd month (OR: 4.3; 1.6-10.5), father's employment (OR: 3.7; 1.2-8.7), gluten introduction6th month (OR: 2.8; 1.5-5), number of siblings2 (OR: 2.8; 1.5-5.3), and family history of autoimmune diseases (OR: 2.7; 1.4-5.3) were significant risk factors for Crohn disease. Low adherence to Mediterranean diet (OR: 2.3; 1.2-4.5), gluten introduction6th month (OR: 2.8; 1.6-4.9), and number of siblings2 (OR: 2; 1.1-3.6) were significant risk factors for ulcerative colitis. Owning pets (OR: 0.3; 0.1-0.7) and bed sharing (OR: 0.2; 0.1-0.6) were protective factors for Crohn disease, whereas owning pets (OR: 0.4; 0.2-0.8) and family parasitosis (OR: 0.07; 0.01-0.4) were protective factors for ulcerative colitis.Our study confirms that environmental factors are closely linked to IBD onset and may partly explain IBD rise in developed countries.
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- 2017
7. Effect of Magnesium Alginate Plus Simethicone on Gastroesophageal Reflux in Infants
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Erasmo Miele, Felice Crocetto, Elisa Sciorio, Annamaria Staiano, Massimo Martinelli, Elena Scarpato, Dario Ummarino, Dario, Ummarino, Miele, Erasmo, Martinelli, Massimo, Elena, Scarpato, Crocetto, Felice, Elisa, Sciorio, and Staiano, Annamaria
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Male ,medicine.medical_specialty ,Alginates ,Simethicone ,Antifoaming Agents ,Severity of Illness Index ,Gastroenterology ,law.invention ,Pharmacotherapy ,Glucuronic Acid ,Randomized controlled trial ,law ,Surveys and Questionnaires ,Internal medicine ,Severity of illness ,medicine ,Humans ,Prospective Studies ,Prospective cohort study ,Life Style ,business.industry ,Hexuronic Acids ,Reflux ,Infant ,food and beverages ,Infant Formula ,Clinical trial ,Pediatrics, Perinatology and Child Health ,Gastroesophageal Reflux ,Drug Therapy, Combination ,Female ,business ,Magnesium alginate ,medicine.drug - Abstract
OBJECTIVES: Gastroesophageal reflux (GER) is a frequently occurring condition in infants capable of causing distressing symptoms. The aim of our study is to evaluate the efficacy of Mg alginate plus simethicone (Gastrotuss Baby, DMG Italia SRL, Pomezia, Italy), compared with rice-starch-thickened formula or with reassurance alone, in the treatment of GER in infants. METHODS: The present randomized controlled trial was conducted in full-term infants affected by symptoms suggestive of GER, evaluated through a validated questionnaire (Infant Gastroesophageal Reflux Questionnaire Revised). The patients were randomized into 3 groups according to treatment (group A: Mg alginate plus simethicone; group B: thickened formula; group C: reassurance with lifestyle changes). Evaluation of symptom scores was performed after 1 month (T1) and 2 months (T2). RESULTS: A total of 64 (85.3%) of 75 enrolled infants (median age 5 months; range 1-10) concluded the study. After 1 month of treatment (T1), infants treated with Mg alginate plus simethicone showed a statistically significant improvement in symptoms compared with the thickened formula and reassurance (P
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- 2015
8. Impact of Hiatal Hernia on Pediatric Dyspeptic Symptoms
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Massimo Martinelli, Elena Scarpato, Severo Campione, Annamaria Staiano, Maria D'Armiento, A. Alessandrella, Valeria Mancusi, Erasmo Miele, Scarpato, Elena, D'Armiento, Maria, Martinelli, Massimo, Mancusi, V, Campione, Severo, Alessandrella, A, Staiano, Annamaria, and Miele, Erasmo
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Male ,medicine.medical_specialty ,Adolescent ,Gastrointestinal Diseases ,gastroesophageal reflux disease ,Gastroenterology ,Hiatal hernia ,Heartburn ,Internal medicine ,medicine ,Esophagitis ,Humans ,Hernia ,Endoscopy, Digestive System ,Prospective Studies ,Dyspepsia ,Child ,Prospective cohort study ,medicine.diagnostic_test ,business.industry ,Esophagogastroduodenoscopy ,medicine.disease ,Confidence interval ,Abdominal Pain ,Hernia, Hiatal ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Gastroesophageal Reflux ,Population study ,Female ,medicine.symptom ,business ,hiatal hernia - Abstract
Hiatal hernia (HH) affects from 10% to 50% of adult population. The correlation between HH, gastroesophageal reflux disease, dyspeptic symptoms, and esophagitis has long been known in adults. The primary objective of our prospective observational study was to estimate the prevalence of HH in children undergoing esophagogastroduodenoscopy (EGD), irrespective of their symptoms. METHODS: We prospectively enrolled 111 consecutive children (48 boys and 63 girls; mean age 94.9 ± 52.3 months) referred for EGD. In all of the patients a asymptomatic score assessment based on the Rome III criteria was used to measure frequency, severity, and duration of gastrointestinal symptoms. HH presence was endoscopically defined; esophagitis presence was evaluated either endoscopically and histologically. Children were divided in 2 age-range groups: 48 months (group 2). RESULTS: Twenty-three patients of 111 (20.7%) had evidence of a sliding HH at EGD. In children from group 2, we found a statistically significant association of HH with heartburn (P = 0.03, 95% confidence interval 1-9.3, r = 0.1) and regurgitation (P = 0.003, 95% confidence interval 1.7-20.4, r = 0.3). Regarding esophagitis presence, no association was found at any age either with defined esophagitis or with dilated intercellular spaces. CONCLUSIONS: Prevalence of HH in our study population was 20.7%. According to our data, HH correlates with the presence of heartburn and regurgitation in children, but not in toddlers. No association was found with esophagitis at any age.
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- 2014
9. Serum and Intestinal Celiac Disease–associated Antibodies in Children With Celiac Disease Younger Than 2 Years of Age
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Mariantonia Maglio, Viviana Indolfi, Riccardo Troncone, Erasmo Miele, Renata Auricchio, Antonella Tosco, Francesco Paparo, Viviana Granata, and B. Colicchio
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medicine.medical_specialty ,Adolescent ,Tissue transglutaminase ,Disease ,Gastroenterology ,Coeliac disease ,Serology ,GTP-Binding Proteins ,Immunopathology ,Internal medicine ,medicine ,Humans ,Protein Glutamine gamma Glutamyltransferase 2 ,Intestinal Mucosa ,Child ,Autoantibodies ,Transglutaminases ,biology ,business.industry ,Age Factors ,Infant ,medicine.disease ,Immunoglobulin A ,Celiac Disease ,El Niño ,Connective Tissue ,Child, Preschool ,Immunoglobulin G ,Pediatrics, Perinatology and Child Health ,Immunology ,Anti-gliadin antibodies ,biology.protein ,Histopathology ,business - Abstract
In children younger than 2 years of age, a diagnosis of celiac disease (CD) is difficult to make because anti-endomysium (anti-EMA)/anti-tissue transglutaminase 2 (anti-TG2) antibodies are less sensitive than in older children. The aim of our study was to evaluate how many children younger than 2 years of age and diagnosed with CD, were negative for serum anti-TG2 antibodies and to test the hypothesis that in these patients, TG2-specific IgA deposits could instead be present at mucosal level.A total of 104 children younger than 2 years of age and 179 children older than 2 years, all of whom had been diagnosed with CD, were investigated for serum CD-associated antibodies (anti-gliadin [AGA] IgA and IgG, EMA-IgA, anti-TG2-IgA). The presence of intestinal anti-TG2 extracellular IgA deposits was searched by using double immunofluorescence in 56 of the patients younger than 2 years of age and in 40 of those who were older than 2 years.In children with CD who were younger than 2 years of age, high levels of AGA-IgA were found in 93/104 (89%) and 98/104 (94%) were found of have high levels of AGA-IgG. In children older than the age of 2 years with CD, 120/179 (67%) had high levels of AGA-IgA and 151/179 (84%) had high levels of AGA-IgG. Serum EMA were present in 92/104 (88%) in the younger group and in 176/179 (98%) of the older group. Ninety-one of 104 children (87%) younger and 172/179 (96%) older than 2 years showed high serum levels of anti-TG2. Finally, 41/56 (73%) children younger than 2 years and all of the 40 children (100%) older than 2 years of age showed mucosal anti-TG2-IgA deposits.EMA and anti-TG2-antibody measurements show higher sensitivity for the diagnosis of CD in children older than 2 years compared with younger children. The search for mucosal deposits of anti-TG2-IgA does not improve the diagnostic performance.
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- 2010
10. Chronic Diarrhea in Mucopolysaccharidosis IIIB
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Gianfranco Pontarelli, Giancarlo Parenti, Michelina Sibilio, Annamaria Staiano, Rossella Turco, Generoso Andria, Raffaella Vecchione, Luca Astarita, Erasmo Miele, Carla Ungaro, and Paola Di Natale
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Diarrhea ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Pathology ,Constipation ,Mucopolysaccharidosis ,Disease ,Scintigraphy ,Gastroenterology ,Central nervous system disease ,Mucopolysaccharidosis III ,Internal medicine ,Humans ,Medicine ,Child ,Sanfilippo syndrome ,medicine.diagnostic_test ,business.industry ,medicine.disease ,Pathophysiology ,Chronic Disease ,Pediatrics, Perinatology and Child Health ,medicine.symptom ,business - Abstract
Mucopolysaccharidosis (MPS) IIIB (Sanfilippo syndrome, OMIM 252920) is a lysosomal storage disorder caused by the deficiency of α-N-acetylglucosaminidase (NAGLU), a lysosomal hydrolase involved in the degradation of heparan sulphate (HS) (1). MPS IIIB is characterized by multisystem involvement and a complex phenotype. The most debilitating manifestations of the disease are those related to central nervous system disease, with severe and progressive mental retardation, hyperactivity, and behavioral problems. Skeletal and visceral manifestations are less prominent, as compared with other MPS. Gastrointestinal manifestations, including diarrhea and constipation, have occasionally been described in patients with MPS IIIB, but they have been poorly characterized and their pathophysiology is not known. Although gastrointestinal symptoms are often overshadowed by the severe neurological phenotype, they may affect the quality of life of patients and of their families. We describe a case of MPS IIIB, referred to our hospital because of chronic diarrhea, in which abnormalities of intestinal endoscopy, histology, and scintigraphy with Tc-99m-labeled human serum albumin were found, partially overlapping with the features of intestinal lymphangiectasia. In this patient, a low-fat diet and supplementation of medium-chain triglycerides were started, leading to persisting improvement of diarrhea. These results add information on the pathophysiology of intestinal manifestations in MPS IIIB patients and possibly in other MPS patients with a history of chronic diarrhea.
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- 2009
11. Gastrointestinal Stromal Tumor of the Small Bowel in a 12-year-old Girl
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Annamaria Staiano, G. Boccia, Erasmo Miele, Guido Pettinato, Simona Avilia, and Roberta Migliorati
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medicine.medical_specialty ,Pathology ,Gastrointestinal Stromal Tumors ,business.industry ,media_common.quotation_subject ,Gastroenterology ,Cancer ,medicine.disease ,Small intestine ,Treatment Outcome ,medicine.anatomical_structure ,Internal medicine ,Intestinal Neoplasms ,Pediatrics, Perinatology and Child Health ,medicine ,Humans ,Female ,Girl ,Stromal tumor ,Child ,Tomography, X-Ray Computed ,business ,Ultrasonography ,media_common - Published
- 2008
12. PP-8 ESOPHAGEAL HIGH RESOLUTION MANOMETRY IN NEUROLOGICALLLY IMPAIRED CHILDREN AND GASTRO-OESOPHAGEAL REFLUX DISEASE
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Del Giudice E, Gaetano Terrone, Dario Ummarino, Annamaria Staiano, Rossella Turco, Erasmo Miele, Turco, Rossella, Ummarino, Dario, Miele, Erasmo, Terrone, Gaetano, DEL GIUDICE, Ennio, and Staiano, Annamaria
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medicine.medical_specialty ,business.industry ,Gastro ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Gastroenterology ,medicine ,Reflux ,Disease ,business ,High resolution manometry - Abstract
Mechanism underlying the occurrence of Gastroesophageal reflux disease (GERD) in neurologically impaired children (NIC) is poorly understood. We sought to characterize, by Esophageal High Resolution Manometry (EHRM), alterations of esophageal motility associated with GERD in NIC and to compare with a group with a suspicion of GERD and normal psychomotor development (NDP).
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- 2015
13. HYPERGANGLIONOSIS AND HIRSCHSPRUNG'S DISEASE
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Annamaria Staiano, Erasmo Miele, C. Toraldo, Arturo Tozzi, and A. Tramontano
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Pediatrics ,medicine.medical_specialty ,business.industry ,Pediatrics, Perinatology and Child Health ,Gastroenterology ,Medicine ,business ,medicine.disease ,Hirschsprung's disease ,Hyperganglionosis - Published
- 1999
14. DEVELOPMENT OF SUBMUCOSAL AND MYENTERIC PLEXUSES IN RELATION TO GESTATIONAL AGE
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Arturo Tozzi, Francesco Paolo D'Armiento, G. Mossetti, C. Toraldo, Erasmo Miele, and Annamaria Staiano
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medicine.medical_specialty ,Obstetrics ,business.industry ,Pediatrics, Perinatology and Child Health ,Gastroenterology ,medicine ,Gestational age ,business - Published
- 1997
15. Human Antichimeric Antibody in Children and Young Adults with Inflammatory Bowel Disease Receiving Infliximab.
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Erasmo Miele
- Published
- 2004
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