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very early onset IBD

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https://www.readbyqxmd.com/read/29927059/rapid-rise-in-the-incidence-and-clinical-characteristics-of-pediatric-inflammatory-bowel-disease-in-a-southeast-asian-cohort-in-singapore-1994-2015
#1
Christina Ong, Marion M Aw, Maria Janelle Liwanag, Quak Seng Hock, Phua Kong Boo
BACKGROUND AND AIMS: Epidemiological studies on pediatric-onset inflammatory bowel disease (PIBD) are scarce in Southeast Asia (SEA). Phenotypes of PIBD in Asian were reportedly more complicated than Caucasian in the same western centres. This study aims to evaluate the incidence trend and clinical characteristics of PIBD in a SEA cohort in Singapore over a 22-year period (1994-2015). METHODS: Case records of PIBD ≤ 18years from the only two tertiary pediatric hospitals in Singapore were reviewed for relevant data...
June 21, 2018: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/29892152/neonatal-presentation-of-unremitting-inflammatory-bowel-disease
#2
Sara Ebrahimi, Gholamreza Khademi, Seyed Ali Jafari, Nona Zaboli Nejad, Abdolreza Norouzy, Bahareh Imani
Very-early-onset inflammatory bowel disease (VEO-IBD) has a distinct phenotype and should be considered a specific entity. VEO-IBD presents with very severe clinical pictures and is frequently known by an indeterminate colitis whose clinical remission is unmanageable. This study examines the case of a neonate with VEO-IBD, not responding to medical and surgical treatment. A 7-day-old Iranian female neonate presented with severe bloody diarrhea, poor feeding, abdominal distention, and dehydration suggesting severe proctocolitis due to an allergy to the protein in cow's milk...
May 2018: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29788474/umbilical-cord-blood-transplantation-corrects-very-early-onset-inflammatory-bowel-disease-in-chinese-patients-with-il10ra-associated-immune-deficiency
#3
Kaiyue Peng, Xiaowen Qian, Zhiheng Huang, Junping Lu, Yuhuan Wang, Ying Zhou, Huijun Wang, Bingbing Wu, Ying Wang, Lingli Chen, Xiaowen Zhai, Ying Huang
Background: Hematopoietic stem cell transplantation is considered the only curative therapy for very early-onset inflammatory bowel disease with specific immune defects, such as interleukin-10 receptor deficiency. We performed reduced-intensity conditioning before umbilical cord blood transplantation in patients with interleukin-10 receptor-A deficiency. Methods: We enrolled 9 very early-onset inflammatory bowel disease patients with typical manifestations. We diagnosed the patients with interleukin-10 receptor-A deficiency by whole-exome sequencing...
May 18, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29788237/diagnostic-yield-of-next-generation-sequencing-in-very-early-onset-inflammatory-bowel-diseases-a-multicenter-study
#4
Fabienne Charbit-Henrion, Marianna Parlato, Sylvain Hanein, Rémi Duclaux-Loras, Jan Nowak, Bernadette Begue, Sabine Rakotobe, Julie Bruneau, Cécile Fourrage, Olivier Alibeu, Frédéric Rieux-Laucat, Eva Lévy, Marie-Claude Stolzenberg, Fabienne Mazerolles, Sylvain Latour, Christelle Lenoir, Alain Fischer, Capucine Picard, Marina Aloi, Jorge Amil Dias, Mongi Ben Hariz, Anne Bourrier, Christian Breuer, Anne Breton, Jiri Bronski, Stephan Buderus, Mara Cananzi, Stéphanie Coopman, Clara Crémilleux, Alain Dabadie, Clémentine Dumant-Forest, Odul Egritas Gurkan, Alexandre Fabre, Aude Fischer, Marta German Diaz, Yago Gonzalez-Lama, Olivier Goulet, Graziella Guariso, Neslihan Gurcan, Matjaz Homan, Jean-Pierre Hugot, Eric Jeziorski, Evi Karanika, Alain Lachaux, Peter Lewindon, Rosa Lima, Fernando Magro, Janos Major, Georgia Malamut, Emmanuel Mas, Istvan Mattyus, Luisa M Mearin, Jan Melek, Victor Manuel Navas-Lopez, Anders Paerregaard, Cecile Pelatan, Bénédicte Pigneur, Isabel Pinto Pais, Julie Rebeuh, Claudio Romano, Nadia Siala, Caterina Strisciuglio, Michela Tempia-Caliera, Patrick Tounian, Dan Turner, Vaidotas Urbonas, Stéphanie Willot, Frank M Ruemmele, Nadine Cerf-Bensussan
Background and Aims: An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases (VEO-IBD). The present study aimed at defining how next-generation sequencing (NGS) methods can be used to improve identification of known molecular diagnosis and adapt treatment. Methods: 207 children were recruited in 45 Paediatric centres through an international collaborative network (ESPGHAN GENIUS working group) with a clinical presentation of severe VEO-IBD (n=185) or an anamnesis suggestive of a monogenic disorder (n=22)...
May 18, 2018: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/29764567/-features-and-management-of-very-early-onset-inflammatory-bowel-disease
#5
Jie-Yu You
Inflammatory bowel disease (IBD) is a chronic nonspecific intestinal inflammatory disease of unknown etiology. This disease includes three main types: Crohn′s disease (CD), ulcerative colitis (UC), and IBD-unclassified (IBD-U). IBD is frequently presented in adults, but in recent years, there is a rising incidence in pediatric populations. Very early onset IBD (VEO-IBD) is a fraction of pediatric IBD, but they have exclusive phenotypic and genetic characteristics such that they are accompanied by severe disease course and resistance to conventional therapy...
May 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29722812/antibiotics-in-ibd-still-a-role-in-the-biological-era
#6
Oren Ledder, Dan Turner
Despite compelling evidence pointing to a critical role of gut microflora in inflammatory bowel disease (IBD) pathogenesis, the role of antibiotics in clinical practice remains limited, largely due to heterogeneous trials with often conflicting evidence. In this review, we revisit previous randomized controlled trials and high-quality uncontrolled studies in an effort to better elucidate the role of antibiotics in contemporary treatment algorithms. The most established role of antibiotics is in perianal Crohn's disease (CD), utilizing ciprofloxacin with or without metronidazole often as an adjunct to biological therapy...
May 2, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29697845/novel-nod2-mutation-in-early-onset-inflammatory-bowel-phenotype
#7
Martina Girardelli, Claudia Loganes, Alessia Pin, Elisabetta Stacul, Eva Decleva, Diego Vozzi, Gabriele Baj, Costantino De Giacomo, Alberto Tommasini, Anna Monica Bianco
Background: Nucleotide-binding oligomerization domain 2 (NOD2) is a key intracellular protein of the innate immune system. NOD2 variants are associated with inflammatory bowel disease (IBD) and other inflammatory phenotypes. We described the case of a baby with a very early-onset IBD who is characterized by a rare homozygous variant in NOD2, found through whole-exome sequencing, Its pathogenic effect was investigated through bioinformatics and functional studies. Methods: The microbicide activity of the patient's phagocytes was analyzed using Escherichia coli...
May 18, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29534824/long-term-outcomes-for-children-with-very-early-onset-colitis-implications-for-surgical-management
#8
Kristy L Rialon, Eileen Crowley, Natashia M Seemann, Aodhnait S Fahy, Aleixo Muise, Jacob C Langer
PURPOSE: The timing of J-pouch surgery following colectomy for children with very early-onset colitis is controversial, with some advocating early reconstruction and others delaying reconstruction because of fear that the colitis may be owing to Crohn's disease (CD). We sought to determine the long-term incidence of CD in this population and whether there may be clinical features that predict the risk of CD. METHODS: Children with noninfectious colitis diagnosed prior to age 10, who underwent subtotal colectomy and ileostomy from 2000 to 2015, were reviewed...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29531467/phenotypic-and-genotypic-characterization-of-inflammatory-bowel-disease-in-children-under-six-years-of-age-in-china
#9
You-Hong Fang, You-You Luo, Jin-Dan Yu, Jin-Gan Lou, Jie Chen
AIM: To analyze clinical differences between monogenic and nonmonogenic very-early-onset inflammatory bowel disease (VEO-IBD) and to characterize monogenic IBD phenotypically and genotypically via genetic testing. METHODS: A retrospective analysis of children aged 0 to 6 years diagnosed with VEO-IBD in a tertiary hospital in southern China from 2005 to 2017 was performed. Clinical data for VEO-IBD patients were collected, and genetic characteristics were analyzed using whole exome sequencing or target gene panel sequencing...
March 7, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29383303/higher-morbidity-of-monogenic-inflammatory-bowel-disease-compared-to-the-adolescent-onset-inflammatory-bowel-disease
#10
Kwang Yeon Kim, Eun Joo Lee, Ju Whi Kim, Jin Soo Moon, Ju Young Jang, Hye Ran Yang, Jae Sung Ko
Purpose: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. Methods: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder...
January 2018: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29321166/fetal-and-early-life-antibiotics-exposure-and-very-early-onset-inflammatory-bowel-disease-a-population-based-study
#11
Anne K Örtqvist, Cecilia Lundholm, Jonas Halfvarson, Jonas F Ludvigsson, Catarina Almqvist
OBJECTIVE: Earlier studies on antibiotics exposure and development of IBD (Crohn's disease (CD) and ulcerative colitis (UC)) may have been biased by familial factors and gastroenteritis. We aimed to estimate the association between antibiotics during pregnancy or infantile age and very early onset (VEO) IBD. DESIGN: In this cohort study of 827 239 children born in Sweden between 2006 and 2013, we examined the link between exposure to systemic antibiotics and VEO-IBD (diagnosis <6 years of age), using Cox proportional hazard regression models...
January 10, 2018: Gut
https://www.readbyqxmd.com/read/29248579/genetic-profile-of-patients-with-early-onset-inflammatory-bowel-disease
#12
Martina Girardelli, Federica Basaldella, Sara Della Paolera, Josef Vuch, Alberto Tommasini, Stefano Martelossi, Sergio Crovella, Anna Monica Bianco
Inflammatory Bowel disease (IBD) is a widespread pathological condition with clinical heterogeneity and with different levels of severity. Although IBD usually occurs in young adults, onset in childhood and infancy are described in patients within the 10th and second year of age. By genome-wide association studies and meta-analysis, several genetic loci have been identified associated with an increased risk of developing IBD in Western populations with variants that may alter the normal mucosal immunity in the gastrointestinal tract...
March 1, 2018: Gene
https://www.readbyqxmd.com/read/29174094/missense-mutation-of-ttc7a-mimicking-tricho-hepato-enteric-sd-the-syndrome-in-a-patient-with-very-early-onset-inflammatory-bowel-disease
#13
João Farela Neves, Isabel Afonso, Luis Borrego, Catarina Martins, Ana Isabel Cordeiro, Conceição Neves, Caroline Lacoste, Catherine Badens, Alexandre Fabre
Tricho-hepato-enteric syndrome (SD/THE) and Multiple intestinal atresia with combined immune deficiency (MIA-CID) are autosomal recessive disorders that present immunological and gastrointestinal features. There are two different phenotypes of patients with TTC7A mutations: the severe form, caused by null mutations and leading to the classical MIA-CID; and the mild form, caused by missense mutations and leading to predominant features of VEO-IBD, less severe immunological involvement and hair abnormalities...
April 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29126503/very-early-onset-inflammatory-bowel-disease
#14
Christopher J Moran
Very early onset inflammatory bowel disease (VEO-IBD) represents a unique and growing subset of patients with inflammatory bowel disease (IBD). Some VEO-IBD patients present with immunodeficiency and possess loss of function genetic mutations involving immune pathways that cause their IBD. A search for Mendelian causes of IBD is likely most beneficial when the presentation involves extra-intestinal autoimmunity or involves intestinal histopathology that is atypical for IBD. While a subset of these young patients will have highly aggressive courses (and likely present with immunodeficiency), the majority of patients with VEO-IBD appear to have disease courses similar to that of their older counterparts...
December 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29091079/nox1-loss-of-function-genetic-variants-in-patients-with-inflammatory-bowel-disease
#15
T Schwerd, R V Bryant, S Pandey, M Capitani, L Meran, J-B Cazier, J Jung, K Mondal, M Parkes, C G Mathew, K Fiedler, D J McCarthy, P B Sullivan, A Rodrigues, S P L Travis, C Moore, J Sambrook, W H Ouwehand, D J Roberts, J Danesh, R K Russell, D C Wilson, J R Kelsen, R Cornall, L A Denson, S Kugathasan, U G Knaus, E G Serra, C A Anderson, R H Duerr, D Pb McGovern, J Cho, F Powrie, V Sw Li, A M Muise, H H Uhlig
Genetic defects that affect intestinal epithelial barrier function can present with very early-onset inflammatory bowel disease (VEOIBD). Using whole-genome sequencing, a novel hemizygous defect in NOX1 encoding NAPDH oxidase 1 was identified in a patient with ulcerative colitis-like VEOIBD. Exome screening of 1,878 pediatric patients identified further seven male inflammatory bowel disease (IBD) patients with rare NOX1 mutations. Loss-of-function was validated in p.N122H and p.T497A, and to a lesser degree in p...
March 2018: Mucosal Immunology
https://www.readbyqxmd.com/read/29027576/neonatal-ulcerative-colitis-associated-with-familial-mediterranean-fever-a-case-report
#16
Elif Sag, Ferhat Demir, Mustafa Emre Ercin, Mukaddes Kalyoncu, Murat Cakir
Neonatal inflammatory bowel disease (IBD) is a subclass of very early onset IBD that includes children younger than 1 month. It is characterized by more colonic involvement and monogenetic etiology, resistance to classical anti-inflammatory/immunomodulatory treatments and associated with colitis in first-degree family members. Herein we report a 3 month-old girl who was admitted with bloody diarrhea since 10 days of age. Her symptoms persist despite diet elimination. She was diagnosed with neonatal ulcerative colitis (UC) based on clinical, laboratory and histopathological examination...
January 2018: Rheumatology International
https://www.readbyqxmd.com/read/28936210/novel-mutations-of-the-tetratricopeptide-repeat-domain-7a-gene-and-phenotype-genotype-comparison
#17
Reyin Lien, Yung-Feng Lin, Min-Wei Lai, Hui-Ying Weng, Ren-Chin Wu, Tang-Her Jaing, Jing-Long Huang, Shih-Feng Tsai, Wen-I Lee
The gastrointestinal tract contains the largest lymphoid organ to react with pathogenic microorganisms and suppress excess inflammation. Patients with primary immunodeficiency diseases (PIDs) can suffer from refractory diarrhea. In this study, we present two siblings who began to suffer from refractory diarrhea with a poor response to aggressive antibiotic and immunosuppressive treatment after surgical release of neonatal intestinal obstruction. Their lymphocyte proliferation was low, but superoxide production and IL-10 signaling were normal...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28864178/very-early-onset-inflammatory-bowel-disease-investigation-of-the-il-10-signaling-pathway-in-iranian-children
#18
Shahram Nemati, Shahram Teimourian, Mina Tabrizi, Mehri Najafi, Naghi Dara, Farid Imanzadeh, Mitra Ahmadi, Maryam Kazemi Aghdam, Mohmoud Tavassoli, Pejman Rohani, Seyyed Ramin Madani, Martin de Boer, T W Kuijpers, Dirk Roos
BACKGROUND & AIM: Comparing to adult inflammatory bowel disease (IBD), those with early onset manifestations have different features in terms of the underlying molecular pathology, the course of disease and the response to therapy. We investigated the IL-10 signaling pathway previously reported as an important cause of infantile (Very Early Onset) IBD to find any possible variants. METHOD: With the next generation sequencing technique we screened IL-10, IL-10RA and IL10RB genes of 15 children affected by very early onset-GI (gastrointestinal) disorders...
December 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28728256/-single-center-retrospective-study-of-184-children-with-inflammatory-bowel-disease-seen-from-2000-2014
#19
D X Guan, F H Yu, G L Wang, J Zhou, D Y Wang, X L Nie, X W Xu
Objective: To investigate the clinical data of children with inflammatory bowel disease (IBD) retrospectively, including Crohn's disease (CD) and ulcerative colitis (UC) and identify the clinical characteristics and trends of change. Method: Clinical data of hospitalized patients diagnosed as IBD in Beijing Children's Hospital from January 2000 to December 2014 were collected and retrospectively analyzed. Patients were divided into six groups based on type of disease and year of admission: Group A1(CD, 2000-2004) included 12 patients, Group B1(CD, 2005-2009) included 11, Group C1(CD, 2010-2014) included 51; Group A2(UC, 2000-2004) included 17, Group B2(UC, 2005-2009) included 25, Group C2(UC, 2010-2014) included 68...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28718031/newcomers-in-paediatric-gi-pathology-childhood-enteropathies-including-very-early-onset-monogenic-ibd
#20
REVIEW
Arzu Ensari, Judith Kelsen, Pierre Russo
Childhood enteropathies are a group of diseases causing severe chronic (>2-3 weeks) diarrhoea often starting in the first week of life with the potential for fatal complications for the affected infant. Early identification and accurate classification of childhood enteropathies are, therefore, crucial for making treatment decisions to prevent life-threatening complications. Childhood enteropathies are classified into four groups based on the underlying pathology: (i) conditions related to defective digestion, absorption and transport of nutrients and electrolytes; (ii) disorders related to enterocyte differentiation and polarization; (iii) defects of enteroendocrine cell differentiation; and (iv) disorders associated with defective modulation of intestinal immune response...
January 2018: Virchows Archiv: An International Journal of Pathology
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