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      The Spectrum of Differences between Childhood and Adulthood Celiac Disease

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          Abstract

          An old saying states that ‘’children are not little adults” and this certainly holds true for celiac disease, as there are many peculiar aspects regarding its epidemiology, diagnosis, clinical presentations, associated diseases, and response to treatment in pediatric compared to adult populations, to such an extent that it merits a description of its own. In fact, contrary to the past when it was thought that celiac disease was a disorder predominantly affecting childhood and characterized by a malabsorption syndrome, nowadays it is well recognized that it affects also adult and elderly people with an impressive variability of clinical presentation. In general, the clinical guidelines for diagnosis recommend starting with specific serologic testing in all suspected subjects, including those suffering from extraintestinal related conditions, and performing upper endoscopy with appropriate biopsy sampling of duodenal mucosa in case of positivity. The latter may be omitted in young patients showing high titers of anti-transglutaminase antibodies. The subsequent management of a celiac patient differs substantially depending on the age at diagnosis and should be based on the important consideration that this is a lifelong condition.

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          Most cited references165

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          Coeliac disease.

          Coeliac disease is a chronic inflammatory disorder of the small bowel induced in genetically susceptible people by the irritant gluten and possibly other environmental cofactors. The disorder is characterised by a diverse clinical heterogeneity that ranges from asymptomatic to severely symptomatic, and it manifests with frank malabsorption, an increased morbidity attributable to the frequent association with autoimmune disorders and increased mortality resulting from the emergence of T-cell clonal proliferations that predispose the patient to enteropathy-type T-cell lymphoma. Our understanding of the molecular basis for this disorder has improved and enabled the identification of targets for new therapies, although a strict gluten-free diet remains the mainstay of safe and effective treatment. In this Seminar we critically reassess the clinical and diagnostic aspects of this disease and new perspectives in its pathogenesis and treatment.
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            Integration of genetic and immunological insights into a model of celiac disease pathogenesis.

            Celiac disease (CD) is a gluten-sensitive enteropathy that develops in genetically susceptible individuals by exposure to cereal gluten proteins. This review integrates insights from immunological studies with results of recent genetic genome-wide association studies into a disease model. Genetic data, among others, suggest that viral infections are implicated and that natural killer effector pathways are important in the pathogenesis of CD, but most prominently these data converge with existing immunological findings that CD is primarily a T cell-mediated immune disorder in which CD4(+) T cells that recognize gluten peptides in the context of major histocompatibility class II molecules play a central role. Comparison of genetic pathways as well as genetic susceptibility loci between CD and other autoimmune and inflammatory disorders reveals that CD bears stronger resemblance to T cell-mediated organ-specific autoimmune than to inflammatory diseases. Finally, we present evidence suggesting that the high prevalence of CD in modern societies may be the by-product of past selection for increased immune responses to combat infections in populations in which agriculture and cereals were introduced early on in the post-Neolithic period.
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              Prevalence of Celiac disease among children in Finland.

              Wheat, rye, and barley proteins induce celiac disease, an autoimmune type of gastrointestinal disorder, in genetically susceptible persons. Because the disease may be underdiagnosed, we estimated the prevalence of the disease and tested the hypothesis that assays for serum autoantibodies can be used to detect untreated celiac disease and that positive findings correlate with specific HLA haplotypes. Serum samples were collected from 3654 students (age range, 7 to 16 years) in 1994 and screened in 2001 for endomysial and tissue transglutaminase antibodies. HLA typing was also performed on stored blood samples. All antibody-positive subjects were asked to undergo small-bowel biopsy in 2001. Of the 3654 subjects, 56 (1.5 percent) had positive antibody tests, as determined in 2001. Results of the two antibody tests were highly concordant. As of 1994, none of the subjects had received a clinical diagnosis of celiac disease, but 10 who had positive tests for both antibodies in serum obtained in 1994 received the diagnosis between 1994 and 2001. Of the 36 other subjects with positive antibody assays who agreed to undergo biopsy in 2001, 27 had evidence of celiac disease on biopsy. Thus, the estimated biopsy-proved prevalence was 1 case in 99 children. All but two of the antibody-positive subjects had either the HLA-DQ2 or the HLA-DQ8 haplotype. The prevalence of the combination of antibody positivity and an HLA haplotype associated with celiac disease was 1 in 67. The presence of serum tissue transglutaminase and endomysial autoantibodies is predictive of small-bowel abnormalities indicative of celiac disease. There is a good correlation between autoantibody positivity and specific HLA haplotypes. We estimate that the prevalence of celiac disease among Finnish schoolchildren is at least 1 case in 99 children. Copyright 2003 Massachusetts Medical Society
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                Author and article information

                Journal
                Nutrients
                Nutrients
                nutrients
                Nutrients
                MDPI
                2072-6643
                22 October 2015
                October 2015
                : 7
                : 10
                : 8733-8751
                Affiliations
                [1 ]Rachele Ciccocioppo, Center for the Study and Cure of Celiac Disease, Clinica Medica I, Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, 19–27100 Pavia, Italy; cangemi.giusy@ 123456gmail.com (G.C.C.); elena.betti19@ 123456gmail.com (E.B.)
                [2 ]International Clinical Research Center, St. Anne’s University Hospital and Masaryk University, 65691 Brno, Czech Republic; peter.kruzliak@ 123456savba.sk
                [3 ]Faculty of Military Health Sciences, University of Defence, Trebešská 1575-500 01 Hradec Kralove, Czech Republic; miroslav.pohanka@ 123456gmail.com
                [4 ]Department of Geology and Pedology, Faculty of Forestry and Wood Technology, Mendel University in Brno, 61300 Brno, Czech Republic
                [5 ]Gastroenterology Unit, Hospital Universitario Central de Asturias, 33000 Oviedo, Spain; meugelb@ 123456hotmail.com (E.L.); lrodrigosaez@ 123456gmail.com (L.R.)
                Author notes
                [* ]Correspondence: rachele.ciccocioppo@ 123456unipv.it ; Tel.: +39-382-502-786; Fax: +39-382-502-618
                Article
                nutrients-07-05426
                10.3390/nu7105426
                4632446
                26506381
                bd77511d-953c-465e-b43f-efd6cda7a06d
                © 2015 by the authors; licensee MDPI, Basel, Switzerland.

                This article is an open access article distributed under the terms and conditions of the Creative Commons by Attribution (CC-BY) license ( http://creativecommons.org/licenses/by/4.0/).

                History
                : 01 August 2015
                : 12 October 2015
                Categories
                Review

                Nutrition & Dietetics
                adulthood,associated diseases,childhood,complications
                Nutrition & Dietetics
                adulthood, associated diseases, childhood, complications

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