The Experts below are selected from a list of 1773 Experts worldwide ranked by ideXlab platform
Jan Ejderhamn - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune Enteropathy in Swedish children, 1985–2002: A call for strict diagnostic criteria
Scandinavian journal of gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
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Autoimmune Enteropathy in swedish children 1985 2002 a call for strict diagnostic criteria
Scandinavian Journal of Gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
Yukio Sakiyama - One of the best experts on this subject based on the ideXlab platform.
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identification of an Autoimmune Enteropathy related 75 kilodalton antigen
Gastroenterology, 1999Co-Authors: Ichiro Kobayashi, Keisaku Imamura, Mitsuru Kubota, Susumu Ishikawa, Masafumi Yamada, Hidefumi Tonoki, Motohiko Okano, Wulf B. Storch, Tetsuya Moriuchi, Yukio SakiyamaAbstract:Abstract Background & Aims: We have previously reported a 75-kilodalton autoantigen specific to X-linked Autoimmune Enteropathy (AIE) associated with tubulonephropathy. The aim of this study was to identify the autoantigen. Methods: Complementary DNA (cDNA) clones were isolated by immunoscreening a human duodenal cDNA-expression library with serum from a patient with AIE. Results: cDNA encoding the 75-kilodalton antigen (AIE-75) was identified. The composite nucleotide sequence of the cDNA for AIE-75 was 2214 base pairs long and encoded 552 amino acids. The genomic sequence of AIE-75 was found in Sequence DataBank, which consisted of 21 exons and was located on the chromosome 11p14.3. Recombinant AIE-75 specifically reacted with sera from 3 of 4 unrelated patients with AIE but not with 58 control sera. AIE-75 was predominantly distributed in the epithelial cells of the luminal surface and the upper half of the crypts of the intestine and in the proximal renal tubulus. Similarity searches revealed that the AIE-75 cDNA sequence was an authentic form of several colon cancer–related cDNAs of unknown function. The deduced amino acid sequence contained 3 conserved PSD-95/Dlg/ZO-1 (PDZ) domains. Conclusions: AIE-75 is a PDZ domain–containing protein expressed in the differentiated epithelial cells of the intestine and kidney and may be involved in protein-protein interaction. The identification of the autoantigen may prove useful in the approach to the pathogenesis of this poorly understood disease. GASTROENTEROLOGY 1999;117:823-830
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Identification of an Autoimmune Enteropathy–related 75-kilodalton antigen
Gastroenterology, 1999Co-Authors: Ichiro Kobayashi, Keisaku Imamura, Mitsuru Kubota, Susumu Ishikawa, Masafumi Yamada, Hidefumi Tonoki, Motohiko Okano, Wulf B. Storch, Tetsuya Moriuchi, Yukio SakiyamaAbstract:Abstract Background & Aims: We have previously reported a 75-kilodalton autoantigen specific to X-linked Autoimmune Enteropathy (AIE) associated with tubulonephropathy. The aim of this study was to identify the autoantigen. Methods: Complementary DNA (cDNA) clones were isolated by immunoscreening a human duodenal cDNA-expression library with serum from a patient with AIE. Results: cDNA encoding the 75-kilodalton antigen (AIE-75) was identified. The composite nucleotide sequence of the cDNA for AIE-75 was 2214 base pairs long and encoded 552 amino acids. The genomic sequence of AIE-75 was found in Sequence DataBank, which consisted of 21 exons and was located on the chromosome 11p14.3. Recombinant AIE-75 specifically reacted with sera from 3 of 4 unrelated patients with AIE but not with 58 control sera. AIE-75 was predominantly distributed in the epithelial cells of the luminal surface and the upper half of the crypts of the intestine and in the proximal renal tubulus. Similarity searches revealed that the AIE-75 cDNA sequence was an authentic form of several colon cancer–related cDNAs of unknown function. The deduced amino acid sequence contained 3 conserved PSD-95/Dlg/ZO-1 (PDZ) domains. Conclusions: AIE-75 is a PDZ domain–containing protein expressed in the differentiated epithelial cells of the intestine and kidney and may be involved in protein-protein interaction. The identification of the autoantigen may prove useful in the approach to the pathogenesis of this poorly understood disease. GASTROENTEROLOGY 1999;117:823-830
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Extremely high serum level of IgE during immunosuppressive therapy: paradoxical effect of cyclosporine A and tacrolimus.
International archives of allergy and immunology, 1997Co-Authors: Nobuaki Kawamura, Ichiro Kobayashi, Motohiko Okano, Hirofumi Furuta, Atsushi Tame, Tadashi Ariga, Yukio SakiyamaAbstract:A case of X-linked Autoimmune Enteropathy was successfully treated with cyclosporine A (CsA) or tacrolimus (FK506) and developed extremely high serum levels of IgE during the immunosuppressive therapy
Lars M. Marthinsen - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune Enteropathy in Swedish children, 1985–2002: A call for strict diagnostic criteria
Scandinavian journal of gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
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Autoimmune Enteropathy in swedish children 1985 2002 a call for strict diagnostic criteria
Scandinavian Journal of Gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
Helge Scott - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune Enteropathy in Swedish children, 1985–2002: A call for strict diagnostic criteria
Scandinavian journal of gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
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Autoimmune Enteropathy in swedish children 1985 2002 a call for strict diagnostic criteria
Scandinavian Journal of Gastroenterology, 2008Co-Authors: Lars M. Marthinsen, Helge Scott, Jan EjderhamnAbstract:Objective. To investigate the incidence, prevalence and the long-term outcome of Autoimmune Enteropathy in Sweden. Material and methods. In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985–2002. Results. The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of Autoimmune Enteropathy. The incidence was 0.06 to 0.12×10−5 and the prevalence was 0.05 to 0.10×10−5 for children aged 0–16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in Autoimmune Enteropathy. Conclusions. Autoimmune Enteropathy in its severe forms is a rare disease in Sweden. None...
Gino Roberto Corazza - One of the best experts on this subject based on the ideXlab platform.
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New insights into immune mechanisms underlying Autoimmune diseases of the gastrointestinal tract.
Autoimmunity reviews, 2015Co-Authors: Antonio Di Sabatino, Alessandro Vanoli, Marco Vincenzo Lenti, Paolo Giuffrida, Gino Roberto CorazzaAbstract:Recent progresses in the immune mechanisms implicated in chronic inflammatory disorders have led to a more in-depth knowledge of the pathogenesis of Autoimmune diseases of the gastrointestinal tract, including Autoimmune atrophic gastritis, celiac disease, Autoimmune Enteropathy and ulcerative colitis. While the pathogenic role of specific circulating autoantibodies, i.e., respectively anti-parietal cell, anti-tissue transglutaminase, anti-enterocyte and anti-neutrophil cytoplasmic, is still controversial, some common T-cell mediated mechanisms for inflammation - increase in T helper cell type 1/type 17 pro-inflammatory cytokines- or losing self-tolerance-abnormal regulatory T cell function - are recognized as crucial mediators of the tissue damage causing atrophy of the stomach mucosa in Autoimmune atrophic gastritis, villous flattening of the small bowel in celiac disease and Autoimmune Enteropathy, and mucosal ulceration of the colon in ulcerative colitis. This review deals with novel advances in the immunological bases of the aforementioned Autoimmune gastrointestinal disorders, and it also highlights immune mechanisms of progression from chronic inflammation to cancer and implications for new therapeutic targets.
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Autoimmune Enteropathy in a 13-year-old celiac girl successfully treated with infliximab.
Journal of clinical gastroenterology, 2014Co-Authors: Francesco Valitutti, Gino Roberto Corazza, Maria Barbato, Marina Aloi, Adriana Marcheggiano, Giovanni Di Nardo, Stefania Leoni, Donatella Iorfida, Salvatore CucchiaraAbstract:Autoimmune Enteropathy (AIE) is a rare cause of small bowel villous atrophy, characterized by malabsorption, unresponsiveness to dietary restriction, circulating autoantibodies to enterocytes, and an overall predisposition to autoimmunity. Albeit mainly regarded as a disease of early childhood, several adult-onset AIE cases have been identified. This report describes for the first time the life-threatening clinical presentation and the management of overlapping AIE in a compliant-to-diet young celiac girl. A 13-year-old celiac girl was admitted because of vomiting, weight loss, diarrhea, hypoproteinemia, and neurological disturbances such as head tremors, vertical nystagmus, and lower limb hyperesthesia. Before this, she had always been compliant on a strict gluten-free diet and her medical history was unremarkable. The diagnosis of AIE was established on histologic findings and on the presence of antienterocyte antibodies. She was initially treated with high-dose Methylprednisolone and Azathioprine. However, only Infliximab proved itself as a highly effective tool for achieving clinical remission and restoring small bowel villous architecture.
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Mechanisms of villous atrophy in Autoimmune Enteropathy and coeliac disease.
Clinical and experimental immunology, 2002Co-Authors: Rachele Ciccocioppo, Simona D'alo', A. Di Sabatino, Raffaella Parroni, Mario Rossi, Claudio Doglioni, Maria Grazia Cifone, Gino Roberto CorazzaAbstract:Since in coeliac disease mucosal flattening has been suggested to result from an increased enterocyte apoptosis triggered by Fas/Fas ligand system and perforin cytolytic granules, we looked for a similar mechanism in Autoimmune Enteropathy. Moreover, we tried to assess whether enterocyte autoantibodies, which are the hallmark of Autoimmune Enteropathy, may be involved in triggering enterocyte apoptosis in this condition. Immunohistochemical staining with anti-Fas, -FasL and -perforin MoAb, and TUNEL technique were applied on endoscopic duodenal biopsies of two Autoimmune Enteropathy patients, two untreated coeliac patients and two biopsied controls. Cytotoxicity assays were carried out by incubating peripheral blood mononuclear cells from a healthy subject (effectors) with enterocytes primed with patient or control sera (targets). In Autoimmune Enteropathy a large number of enterocytes were apoptotic, as in coeliac disease, whereas neither Fas/Fas ligand or perforin expressions were up-regulated. On the other hand, antibody-dependent cellular cytotoxicity assay revealed the ability of sera from patients with Autoimmune Enteropathy to mediate enterocyte death through apoptosis. These results point to enterocyte autoantibody-dependent cellular cytotoxicity as the prevalent mechanism of increased enterocyte apoptosis in Autoimmune Enteropathy but not in coeliac disease.
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Autoimmune Enteropathy and villous atrophy in adults
Lancet (London England), 1997Co-Authors: Gino Roberto Corazza, Umberto Volta, Federico Biagi, Lucia De Franceschi, Giovanni GasbarriniAbstract:Summary Background Autoimmune Enteropathy is a condition described in children and characterised by villous atrophy, which is unresponsive to any dietary restrictions, and by the presence of enterocyte autoantibodies. We report two adult patients who fulfilled all the criteria for the diagnosis of this disorder. Methods Over the past 5 years we have seen four adult patients (all women, median age 51·5 [range 38–64] years) with subtotal villous atrophy, which was unresponsive to a gluten-free diet. The patients were HLA-DQ2 positive. IgA antigliadin and antiendomysial antibodies were not found in any of the patients. We did an indirect immunofluorescence search for enterocyte autoantibodies on monkey jejunum and for other autoantibodies for all four patients. Findings Of the four patients, two were positive for enterocyte autoantibodies and one of these two patients was positive for antiactin, antiparietal cell, and antithyroid microsomal autoantibodies. Interpretation To the best of our knowledge the two patients affected by severe Enteropathy, who had never responded to any exclusion diet, and who were positive for enterocyte autoantibodies are the first cases of Autoimmune Enteropathy described in adults. We propose that adult patients whose disorders are unresponsive to a gluten-free diet should be tested for enterocyte autoantibodies.
