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Autoimmune enteropathy

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https://www.readbyqxmd.com/read/28197149/treatment-of-infantile-inflammatory-bowel-disease-and-autoimmunity-by-allogeneic-stem-cell-transplantation-in-lps-responsive-beige-like-anchor-deficiency
#1
Shahrzad Bakhtiar, Laura Gámez-Díaz, Andrea Jarisch, Jan Soerensen, Bodo Grimbacher, Bernd Belohradsky, Klaus-Michael Keller, Christoph Rietschel, Thomas Klingebiel, Sibylle Koletzko, Michael H Albert, Peter Bader
Inflammatory bowel disease (IBD) in young children can be a clinical manifestation of various primary immunodeficiency syndromes. Poor clinical outcome is associated with poor quality of life and high morbidity from the complications of prolonged immunosuppressive treatment and malabsorption. In 2012, mutations in the lipopolysaccharide-responsive beige-like anchor (LRBA) gene were identified as the cause of an autoimmunity and immunodeficiency syndrome. Since then, several LRBA-deficient patients have been reported with a broad spectrum of clinical manifestations without reliable predictive prognostic markers...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28184376/seronegative-adult-autoimmune-enteropathy-in-a-male-traveler
#2
Patrick McCabe, Latifat Alli-Akintade, Jesse Stondell
Autoimmune enteropathy (AIE) is rare but damaging. The lack of consistent objective findings makes diagnosis a challenge. A 45-year-old male developed noninfectious diarrhea with significant weight loss and electrolyte abnormalities. Computed tomography delineated enteritis. Colonoscopy and esophagogastroduodenoscopy showed villous atrophy, chronic inflammation, and ulceration of the terminal ileum and cecum. Pathology showed cryptitis with apoptosis and abscesses throughout the small and large bowel and absent goblet cells...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28154909/beyond-moulage-sign-and-ttg-levels-the-role-of-cross-sectional-imaging-in-celiac-sprue
#3
Shannon P Sheedy, John M Barlow, Joel G Fletcher, Thomas C Smyrk, Francis J Scholz, Don C Codipilly, Badr F Al Bawardy, Jeff L Fidler
Celiac disease is an autoimmune disorder that causes inflammation and destruction in the small intestine of genetically susceptible individuals following ingestion of gluten. Awareness of the disease has increased; however, it remains a challenge to diagnose. This review summarizes the intestinal and extraintestinal cross-sectional imaging findings of celiac disease. Small intestine fold abnormalities are the most specific imaging findings for celiac disease, whereas most other imaging findings reflect a more generalized pattern seen with malabsorptive processes...
February 2, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28126998/gastrointestinal-diseases-and-their-oro-dental-manifestations-part-3-coeliac-disease
#4
T van Gils, H S Brand, N K H de Boer, C J J Mulder, G Bouma
Coeliac disease is a chronic autoimmune-mediated enteropathy, caused by exposure to dietary gluten in genetically predisposed individuals that affects approximately 0.5-1% of the western population. Despite increased awareness of the disease, the majority of patients still remain undiagnosed. Disease frequently manifests in early childhood, but a significant proportion of patients are nowadays diagnosed above the age of 50. Timely diagnosis is important in order to start a gluten-free diet and prevent complications...
January 27, 2017: British Dental Journal
https://www.readbyqxmd.com/read/28125074/outcome-of-referrals-for-non-responsive-celiac-disease-in-a-tertiary-center-low-incidence-of-refractory-celiac-disease-in-the-netherlands
#5
R L J van Wanrooij, G Bouma, H J Bontkes, A Neefjes-Borst, N C van Grieken, B M E von Blomberg, C J J Mulder
OBJECTIVES: Refractory celiac disease (RCD) is a severe cause of non-responsive celiac disease (CD) due to its association with the enteropathy associated T-cell lymphoma (EATL). Conflicting data exist on the prevalence and the clinical manifestations of RCD type I (RCD I) and type II (RCD II). The aim of the current study was to provide insight in the incidence of RCD and in the distinction with other causes of non-responsive CD. METHODS: A total of 106 CD patients were referred to our tertiary referral center between January 2006 and December 2011 for evaluation of non-responsive CD...
January 26, 2017: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/28123330/the-plethora-clinical-manifestations-and-treatment-options-of-autoimmunity-in-patients-with-primary-immunodeficiency
#6
Hatice Ezgi Barış, Ayça Kıykım, Ercan Nain, Ahmet Oğuzhan Özen, Elif Karakoç-Aydıner, Safa Barış
AIM: Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28097735/autoimmune-enteropathy-and-hepatitis-in-pediatric-heart-transplant-recipient
#7
Kimberly Lewis, Ryan Butts, J Antonio Quiros, Michelle Hudspeth, Katherine Twombley, Andrew Savage, Sally Self, Ali Burnette, Shaoli Sun
AIE is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune-mediated damage to intestinal mucosa. AIE is often associated with various syndromes of immunodeficiency including IPEX syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X-linked). Dysfunctional T regulatory cells are the source of pathology in both IPEX syndrome and AIE as they are essential in maintaining tolerance to self-antigens and eliminating autoreactive B cells. This case report describes a 10-year-old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with AIE and extraintestinal manifestations of cyclical hepatitis...
January 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28092152/issues-in-nutrition-dietary-considerations-in-select-chronic-conditions
#8
Margaret E Thompson, Mary Barth Noel
Chronic diseases that affect the gastrointestinal tract also tend to affect nutrition. The incidence of chronic liver disease is increasing. As the prevalence of obesity rises, so do the incidences of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis. Patients with chronic liver disease usually have some degree of malnutrition. In the absence of encephalopathy, patients with chronic liver disease should consume more protein than that in the average diet. There is some controversy about whether diet plays a role in the development of inflammatory bowel disease...
January 2017: FP Essentials
https://www.readbyqxmd.com/read/28011187/mucosal-associated-invariant-t-cells-are-depleted-and-functionally-altered-in-patients-with-common-variable-immunodeficiency
#9
Serena Arduini, Jean Dunne, Niall Conlon, Conleth Feighery, Derek G Doherty
Common variable immunodeficiency (CVID) is a primary immunoglobulin deficiency characterized by recurrent infections and complications, including autoimmunity, enteropathy, polyclonal lymphocytic infiltration or lymphoid malignancy. Innate T cells can support B cell maturation and antibody production. We investigated the numbers, phenotypes and functions of circulating B cell, γδ T cell, invariant natural killer T (iNKT) cell and mucosal-associated invariant T (MAIT) cell subsets in 23 CVID patients and 27 healthy controls...
December 21, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28000302/pd-1-inhibitor-gastroenterocolitis-case-series-and-appraisal-of-immunomodulatory-gastroenterocolitis
#10
Raul S Gonzalez, Safia N Salaria, Caitlin D Bohannon, Aaron R Huber, Michael M Feely, Chanjuan Shi
AIMS: PD-1 inhibitors facilitate immune response against certain tumour types, including melanoma. These drugs have led to prolonged survival but can also result in autoimmune-type side effects, including gastrointestinal inflammation. The histopathological effects of this medication class have not been well studied. METHODS AND RESULTS: We identified 37 gastrointestinal tract biopsies from 20 patients taking a PD-1 or PD-L1 inhibitor and evaluated clinicopathological findings...
March 2017: Histopathology
https://www.readbyqxmd.com/read/27990287/coeliac-disease-a-unique-model-for-investigating-broken-tolerance-in-autoimmunity
#11
REVIEW
Melinda Y Hardy, Jason A Tye-Din
Coeliac disease, a prevalent immune-mediated enteropathy driven by dietary gluten, provides an exceptional human model to dissect the genetic, environmental and immunologic factors operating in autoimmunity. Despite the causative antigen being an exogenous food protein, coeliac disease has many features in common with autoimmune disease including a strong HLA class II association and the presence of pathogenic CD4(+) T cells and autoantibodies. CD8(+) intraepithelial lymphocytes specifically target and destroy intestinal epithelium in response to stress signals and not a specific antigen...
November 2016: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/27936497/proinflammatory-cytokine-interferon-%C3%AE-and-microbiome-derived-metabolites-dictate-epigenetic-switch-between-forkhead-box-protein-3-isoforms-in-coeliac-disease
#12
G Serena, S Yan, S Camhi, S Patel, R S Lima, A Sapone, M M Leonard, R Mukherjee, B J Nath, K M Lammers, A Fasano
Coeliac disease (CD) is an autoimmune enteropathy triggered by gluten and characterized by a strong T helper type 1 (Th1)/Th17 immune response in the small intestine. Regulatory T cells (Treg ) are CD4(+) CD25(++) forkhead box protein 3 (FoxP3(+) ) cells that regulate the immune response. Conversely to its counterpart, FoxP3 full length (FL), the alternatively spliced isoform FoxP3 Δ2, cannot properly down-regulate the Th17-driven immune response. As the active state of CD has been associated with impairments in Treg cell function, we aimed at determining whether imbalances between FoxP3 isoforms may be associated with the disease...
March 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27935231/prolonged-tacrolimus-use-for-pediatric-gastrointestinal-disorder-a-double-edged-sword
#13
Kenji Hosoi, Katsuhiro Arai, Kentaro Matsuoka, Hirotaka Shimizu, Koichi Kamei, Atsuko Nakazawa, Toshiaki Shimizu, Julian Tang, Shuichi Ito
BACKGROUND: Although tacrolimus (TAC) appeared useful in inducing remission in children with refractory inflammatory bowel disease (IBD) or autoimmune gastroenteropathy (AGE), its use in maintenance therapy remains controversial. The aim of this study was to reveal the potential nephrotoxic nature of prolonged TAC use. METHODS: This retrospective study reviews children with gastrointestinal disorder who underwent kidney biopsy for the evaluation of renal damage during TAC therapy of over one year...
December 9, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27918167/-coeliac-disease-as-a-possible-cause-of-some-gynecological-and-obstetric-abnormalities
#14
K Fiolková, K Biringer, M Hrtánková, R Fiolka, J Danko
OBJECTIVE: To bring a review of available literature sources on the prevalence of coeliac disease and its possible impact on gynecological and obstetric disorders. DESIGN: Review article. SETTING: Gynecology and Obstetrics Clinic, Jessenius Faculty of Medicine, Comenius University in Bratislava, Martin, Slovakia. METHODS: Analysis of literary sources. CONCLUSION: Coeliac disease is an autoimmune enteropathy caused by abnormal immune system response to gluten...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27902982/limitation-of-simultaneous-analysis-of-t-cell-receptor-and-%C3%AE%C2%BA-deleting-recombination-excision-circles-based-on-multiplex-real-time-polymerase-chain-reaction-in-common-variable-immunodeficiency-patients
#15
Faranaz Atschekzei, Fareed Ahmad, Torsten Witte, Roland Jacobs, Reinhold E Schmidt
AIM OF STUDY: We used a triplex real-time polymerase chain reaction (PCR) to classify our common variable immunodeficiency (CVID) patients into distinct groups according to the amount of their T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs). MATERIALS AND METHODS: TREC and KREC analysis was performed using a multiplex real-time PCR assay. The T- and B-lymphocyte subsets were measured by flow cytometry. RESULTS: The copy number of TRECs and KRECs was significantly reduced in CVID patients compared to healthy controls...
2016: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/27852543/screening-of-patients-with-juvenile-idiopathic-arthritis-and-those-with-rheumatoid-arthritis-for-celiac-disease-in-southwestern-iran
#16
Mozhgan Moghtaderi, Shirin Farjadian, Elham Aflaki, Naser Honar, Soheila Alyasin, Maryam Babaei
BACKGROUND/AIMS: Celiac disease (CD) is a common enteropathy triggered by the ingestion of gluten in genetically susceptible individuals. It is frequently found in conjunction with other autoimmune diseases. The purpose of this study was to investigate the prevalence of CD in patients with juvenile idiopathic arthritis (JIA) and those with rheumatoid arthritis (RA) in southwestern Iran. MATERIALS AND METHODS: A total of 53 children with JIA and 55 adults with RA were enrolled...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27825568/osteoarticular-manifestations-of-celiac-disease-and-non-celiac-gluten-hypersensitivity
#17
Stéphanie Dos Santos, Frédéric Lioté
Celiac disease is a chronic inflammatory autoimmune enteropathy based disorder that is triggered by the ingestion of gluten in genetically susceptible individuals. The global prevalence of 1% to 2% represents only the tip of the iceberg. The diagnosis is confirmed by positive specific antibody, anti-transglutaminase or anti-endomysium, specific lesions of the small intestine and a response to strict gluten-free diet. The diagnosis is difficult and often delayed because the clinical variability is very large, ranging from digestive clinical presentation "classic" to "atypical" symptoms, often extra-intestinal, that are sometimes attributed to a concomitant disease or a complication...
November 4, 2016: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/27803128/stat-mutations-as-program-switchers-turning-primary-immunodeficiencies-into-autoimmune-diseases
#18
REVIEW
Tiziana Lorenzini, Laura Dotta, Mauro Giacomelli, Donatella Vairo, Raffaele Badolato
STAT proteins are a family of transcription factors that mediate cellular response to cytokines and growth factors. Study of patients with familial susceptibility to pathogens and/or autoimmune diseases has led to the identification of 7 inherited disorders that are caused by mutations of 4 STAT family genes. Homozygous or compound heterozygous mutations of STAT1 lead to complete or partial forms of STAT1 deficiency that are associated with susceptibility to intracellular pathogens and herpetic infections. Patients with heterozygous STAT1 gain-of-function (GOF) mutations usually present with chronic mucocutaneous candidiasis (CMC) but may also experience bacterial and viral infections, autoimmune manifestations, lymphopenia, cerebral aneurysms, and increased risk to develop tumors...
January 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/27801603/inborn-errors-of-t-cell-immunity-underlying-autoimmune-diseases
#19
László Maródi
No abstract text is available yet for this article.
February 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27760369/autoimmunity-to-heat-shock-proteins-and-vitamin-d-status-in-patients-with-celiac-disease-without-associated-dermatitis-herpetiformis
#20
Stefan Tukaj, Anna Görög, Konrad Kleszczyński, Detlef Zillikens, Sarolta Kárpáti, Michael Kasperkiewicz
Inflammation-induced heat shock proteins (HSPs) and hypovitaminosis D have been reported to impact immune responses and to be associated with autoimmune diseases including celiac disease (CD), a gluten-sensitive enteropathy mediated by autoantibodies against tissue transglutaminase (TG2). Recently, we provided evidence for a role of autoantibodies to HSPs in patients with dermatitis herpetiformis (DH), an inflammatory skin disease induced by underlying latent CD. In this study, we aimed at investigating the humoral autoimmune response to HSPs and vitamin D status in CD patients (n=15) presenting without the cutaneous disease manifestation...
October 16, 2016: Journal of Steroid Biochemistry and Molecular Biology
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