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Celiac Disease in Children

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Celiac Disease in Children Case report Celiac disease in children Alejandra Wilches Luna, MD,1 Carolina Gómez López de Mesa, MD.2 1 Garrahan Hospital Pediatrician., Buenos Aires, Abstract Argentina Garrahan Hospital Gastroenterologist, Buenos Aires, Argentina Celiac disease (CD) is an autoimmune enteropathy triggered by ingestion of the gluten fraction of wheat Docent in Pediatric gastroenterology, University of proteins and similar alcohol-soluble proteins of barley and rye in genetically susceptible subjects. For many Antioquia, Medellin, Colombia. years, celiac disease has been under diagnosed. However, because of today’s greater knowledge of its Pediatric gastroenterologist Hospital Universitario San Vicente de Paul, Medellín, Colombia. presentations and the availability of new more accurate serologic tests, it is now known that CD is relatively 2 Pontificia Bolivariana University Doctor common. Although CD can occur at any age, with a broad clinical spectrum affecting any organ, typical cases Resident of Pediatrics. Universidad de Antioquia, often manifest in infancy. This review aims to bring together the most significant current knowledge about this Medellin, Colombia. disease. ........................................ Received: 19-07-09 Key words Accepted: 26-05-10 Celiac disease, gluten, autoimmunity, tissue transglutaminase, intraepithelial lymphocytes. IntroductIon patients when they replaced bread with food that did not contain grain. Patients suffered relapses after cereals were Celiac disease (CD), also known as celiac sprue and gluten returned to their diets (4). sensitive enteropathy, is an autoimmune disease triggered A series of studies carried out after the Second World by the ingestion of gliadin fractions present in gluten and War by Dicke, Wingers and van de Kamer administered similar proteins of rye and barley by genetically predispo- different diets to children suffering from CD. By measuring sed individuals (1). Gluten is the major protein compo- variations in the weight of the feces and degree of steatorr- nent of wheat, rye and barley. In CD, it triggers an immune hea they were able to measured the degree of malabsorption reaction that leads to bowel inflammation mediated by T clearly showing that wheat, barley and rye aggravated the lymphocytes. Hyperplastic crypts, intraepithelial lym- disease, whereas exclusion of these grains from the diet led phocytes (IELs) and atrophy of the villi develop causing to improvement of the patients (5). These studies allowed chronic enteropathy with a broad range of manifestations. identification of gliadin (wheat gluten fraction soluble in The result is a systemic disease of varying severity (2). alcohol) as the toxic agent in this disease (6). Adherence to a gluten-free diet by these patients leads to clinical and histological improvement, with long-term EpIdEmIology normalization of intestinal architecture. Symptoms recur if gluten is reintroduced into the diet (3). The epidemiology of CD has changed dramatically in the There have been descriptions of CD since the first cen- last decade. In the past it was considered a rare disorder tury BC, but its association with the ingestion of gluten with a prevalence estimated at 1:500 to 1:8,000 people in was only found in 1940. During the World War II pediatri- the general population (7). But large increases in the use cians Dicke and Dutch observed clinical improvement in of techniques that look for endomysial antibodies (EMA) 194 © 2010 Asociaciones Colombianas de Gastroenterología, Endoscopia digestiva, Coloproctología y Hepatología and anti-tissue transglutaminase (tTG) in circulation have class II, HLA-DQ2 and HLA-DQ8 which are located on shown that this disease occurs much more frequently than chromosome 6p21. About 95% of CD patients express was previously estimated. In addition it is now know that HLA-DQ2 while the remaining patients are usually there are incomplete or atypical clinical presentations. HLA-DQ8 positive (12). Although having antigens to Recent studies show that the CD has been under-diagno- DQ2 or DQ8 is necessary for development of CD, it is sed (8), and it is in fact one of the most common disorders not sufficient. The risk for development of CD among worldwide. It affects 0.5-1% of the general population of the patients with these haplotypes is 36% to 53%. The pre- United States and other developed countries. Previously, sence of the HLA-DQ2 allele is common in the general CD geographical distribution was restricted to Europe population, and it is present in approximately 30% of and other developed countries such as the USA, Canada Caucasian individuals (9). and Australia. New epidemiological studies show that this Different studies have found several loci associated with disorder is also common in other parts of the world inclu- this disease. They show significant heterogeneity across ding the Asian continent (9). different populations and have smaller impact than HLA In Latin America little epidemiological data exists. In DQ2 and DQ8. Among the predisposing loci for CD are Argentina a prevalence of 1 in 167 adults has been reported CELIAC1 on chromosome 6 (HLA-DQ2 and HLA-DQ8), (10), but no statistics exist for the pediatric population. In CELIAC2 on chromosome 5q31-33, CELIAC3 on chro- Colombia there are no data about CD. The under-diagnosis mosome 2q33 (includes T-lymphocyte regulatory genes can be explained by the celiac iceberg model (Figure 1) CD28, CTLA4, and ICOS), and CELIAC4 (the myosin which is related to the frequency of predisposing haploty- IXB gene, MYO9XB) on chromosome 19p13.1.ls. pes in each population and of gluten consumption habits. The MYO9B gene encodes an unconventional myosin The tip of the iceberg is the symptomatic patients, but the molecule involved in actin remodeling of epithelial entero- submerged part of the iceberg contains the majority of the cytes. It is believed that this genetic variant may lead to an cases who have not been diagnosed. They may have atypi- alteration in the intestinal barrier which allows the passage cal symptoms or may not even have symptoms. For every of immunogenic gluten peptides (13). Also this genetic case diagnosed, it is estimated that there are 5 to 10 undiag- variation has been found in patients with systemic lupus nosed cases (11). erythematosus and rheumatoid arthritis suggesting that the gene MYO9B is an autoimmune risk factor (14). In general, genetic predisposition to CD depends on genes with large effects on adaptive immune response to gluten peptides (HLA-DQ2/DQ8). This is influenced by Classic Symptoms/ Mucus Lesion many other genes involved in different aspects of adaptive Non-classic symptoms Manifestations or innate immune reactions, intestinal permeability and general autoimmune predispositions (9). Abnormal Serology Silent CD EtIopAthogEnEsIs CD is the only autoimmune disease in which there is a clearly Normal Latent CD mucosa identified environmental trigger. 95% of CD patients have autoantibodies against tTG enzyme (1). CD is characterized by three key components. CD is triggered by gluten proteins. Antibodies against tTG are present in CD patients, and the Genetic Susceptibility: DQ2 and/or DQ8 expression of certain HLA phenotypes is required (8). Multiple pathways are involved in the pathogene- Figure 1. Iceberg model of Celiac Disease. Modified from de: Fasano sis of CD which finally lead to the destruction of the A. Celiac disease in children. Best Practice & Research Clinical enterocyte and subsequent atrophy of intestinal villi. Gastroenterology 2005; 19:467-478. Gluten, the non soluble water protein fraction of wheat flour, plays the leading role. Proteins in wheat flour can be gEnEtIcs separated into glutens which are insoluble in ethanol, and gliadins which are soluble in ethanol. Both have high glu- Progress in genetic studies has shown that genetic predis- tamine and proline contents. Gliadin is a prolamin. Two position plays a key role in CD. It is well known that CD is other similar prolamins, secalins, and hordein have been strongly associated with human leukocyte antigen (HLA) isolated in rye and barley. Celiac disease in children 195 Innate and adaptive immune responses are involved in Increased permeability allows passage of large molecules. CD (15). Tissue transglutaminase (tTG), an intestinal The disaccharidases, peptidases, alkaline phosphatases, enzyme, deaminates gliadin peptides, increasing immune adenosine triphosphatases and esterases are reduced. The power. In the adaptive immune response CD4+ T cells amount of intraepithelial lymphocytes (IELs)/mm increa- recognize deaminated gliadin peptides in the lamina propia ses, although with no real increase in absolute numbers through HLA class II molecules DQ2 or DQ8 on antigen- because the absorptive surface is greatly reduced (19). The presenting cells. This promotes the release of proinflam- proximal intestine most affected, with severity decreasing matory cytokines and metalloproteinases with consequent towards the distal small intestine. Exclusive compromise of intestinal damage. Regarding innate immunity, the inges- the distal segment is not a feature of celiac disease. tion of gliadin causes direct toxicity to enterocytes by ove- The IELs occupy a particular place in the immune sys- rexpression of IL-15 in the intestine (16, 17). Subsequent tem and play an important role in intestinal homeostasis. positive regulation of stress molecules MIC-A on the sur- The normal ration of IELs to epithelial cells in the small face of the enterocyte causes activation of intraepithelial
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