Manifestaciones bucales de la enfermedad celíaca y alergia al trigo: Reporte de tres casos y revisión literatura / Oral Manifestations of Celiac Disease and Wheat Allergy: Report of Three Cases and Review of Literature

Resumen: La Enfermedad Celíaca (EC) es una enfermedad sistémica inmunomediada, provocada por el gluten y prolaminas relacionadas, en individuos genéticamente susceptibles. Se caracteriza por manifestaciones clínicas dependientes del gluten, anticuerpos específicos de EC, haplotipos HLA DQ2 o DQ8 y enteropatía. La mayoría de los pacientes son asintomáticos o con manifestaciones leves. Afecta principalmente el sistema gastrointestinal causando síntomas y signos como diarrea, dolor abdominal recurrente y distensión abdominal; además manifestaciones extradigestivas. Los pacientes con EC pueden tener manifestaciones bucales que incluyen estomatitis aftosa recurrente (EAR), hipoplasia del esmalte, glositis atrófica, entre otras. El diagnóstico de EC se basa en la historia clínica, serología, endoscopía e histología. Existen otras entidades que deben diferenciarse de la EC, como la sensibilidad al gluten no celíaca y la alergia al trigo. El objetivo de este trabajo es presentar tres pacientes con antecedentes de EAR y otras manifestaciones clínicas asociadas al diagnóstico precoz de EC y alergia alimentaria.

Abastract: Celiac Disease (CD) is an immune-mediated systemic disease, caused by gluten and related prolamins, in genetically susceptible individuals. It is characterized by gluten-dependent clinical manifestations, CD-specific antibodies, HLA DQ2 or DQ8 haplotypes, and enteropathy. Most patients are asymptomatic or with mild manifestations. It mainly affects the gastrointestinal system causing symptoms and signs such as diarrhea, recurrent abdominal pain and abdominal distension; also extradigestive manifestations. Patients with CD may have oral manifestations that include recurrent aphthous stomatitis (RAS), enamel hypoplasia, atrophic glossitis, among others. The diagnosis of CD is based on clinical history, serology, endoscopy and histology. There are other entities that should be differentiated from CD, such as non-celiac gluten sensitivity and wheat allergy. The aim of this study is to present three patients with a history of RAS and other clinical manifestations associated with the early diagnosis of CD and food allergy.

Aporte de energía, macronutrientes y fibra de alimentos farináceos libres de gluten elaborados a partir de premezclas comerciales / Energy, macronutrients and fiber contribution of gluten free farinaceous products produced from commercial premix

Resumen La dieta libre de gluten (DLG) es el único tratamiento frente a la enfermedad celíaca, la alergia al trigo y la sensibilidad al gluten no celíaca. Se reporta a la DLG como una dieta desbalanceada, escasa en fibra y alta en grasas y carbohidratos. El objetivo del estudio fue evaluar el aporte de energía, de macronutrientes y de fibra de alimentos libres de gluten (bizcochuelos, panes estilo inglés, galletitas dulces y pizzas) elaborados a partir de diferentes premezclas comerciales, disponibles en el mercado local. Además, los resultados fueron comparados con un homólogo comercial elaborado con harina de trigo. Para la determinación de la composición centesimal se aplicó la metodología oficial AOAC 2016. El valor energético se calculó utilizando los factores de Atwater. Los resultados obtenidos para los bizcochuelos fueron: 6,0-8,3 g% de proteína; 9,2-11,3 g% de grasas; 1,5-2,3 g% de fibra dietaria; 40,0-53,1 g% de carbohidratos y 285-339 Kcal% de valor energético. Para los panes: 3,0-6,6 g% de proteína; 3,1-12,7 g% de grasas; 2,7-4,0 g% de fibra dietaria; 36,8-50,1 g% de carbohidratos y 217-290 Kcal% de valor energético. Para las galletitas: 3,5-8,5 g% de proteína; 25,9-31,0 g% de grasas; 0,9-5,9 g% de fibra dietaria; 53,9-60,1 g% de carbohidratos y 483-527 Kcal% de valor energético. Para las pizzas: 7,4-9,3 g% de proteína; 7,3-11,2 g% de grasas; 2,8-8,0 g% de fibra dietaria; 19,1-29,6 g% de carbohidratos y 172-240 Kcal% de valor energético. Los alimentos elaborados con premezclas formuladas únicamente con harina de arroz y féculas contribuyen poco a cubrir el valor diario recomendado de fibra. Los que contienen otras fuentes farináceas (harina de sorgo, trigo sarraceno y teff) reflejan un mayor contenido de fibra. La presencia de harina de arveja, soja y teff refleja un aumento del contenido proteico. Los alimentos estudiados frente a su homólogo comercial con harina de trigo presentan, en general, igual o menor aporte proteico, similar aporte energético y de carbohidratos y un mayor aporte de grasas y fibra dietaria.

Abstract The gluten free diet (GFD) is the only treatment for celiac disease, wheat allergy and non-celiac gluten/wheat sensitivity. GFD is reported as an unbalanced diet, low in fibre and high in fat and carbohydrates. The aim of this study was to evaluate energy, macronutrients and fiber contribution of gluten-free cereal products (pound cake, tin loaf, cookies and pizza) coming from different commercial premixes, available at the local market. Also, the results were compared with a commercial equivalent made with wheat flour. For centesimal composition determination, AOAC Official Method 2016 was applied. The energy was calculated using Atwater factors. The results obtained for pound cake were: 6.0-8.3g% protein; 9.2-11.3g% fat; 1.5-2.3g% dietary fibre; 40.0-53.1g% carbohydrate and 285-339Kcal% energy. For tin loaf: 3.0-6.6g% protein; 3.1-12.7% fat; 2.7-4.0g% dietary fibre; 36.8-50.1g% carbohydrate and 217-290Kcal% energy. For cookies: 3.5-8.5g% protein; 25.9-31.0g% fat; 0.9-5.9g% dietary fibre; 53.9-60.1g% carbohydrate and 483-527Kcal% energy. For pizza: 7.4-9.3g% protein; 7.3-11.2g% fat; 2.8-8.0g% dietary fibre; 19.1-29.6g% carbohydrate and 172-240Kcal% of energy. Products made with premix that only contains rice flour and starches contribute little to meeting recommended daily intake. The ones that contain other farinaceous sources (sorghum flour, buckwheat and teff) show higher dietary fibre content. The presence of pea flour, soybean and teff show an increase of protein content. The analysed products present same or lower protein intake, similar energy and carbohydrate intake and higher fat and dietary fibre intake, compared to their commercial equivalent made with wheat flour.

Correlação entre o autodiagnóstico de alergia alimentar e a presença de IgE específica / Correlation between self-diagnosis of food allergy and presence of specific IgE

Introdução: Nos últimos anos a prevalência de alergia alimentar tem aumentado, contudo, o número de autodiagnósticos errados, também. Essa superestimação dos diagnósticos, frequentemente, culmina em dietas restritivas desnecessárias que podem, muitas vezes, ocasionar mais danos do que benefícios para a saúde. Objetivo: Avaliar a relação entre o autodiagnóstico de alergia alimentar e a presença de IgE específica para o referido alimento. Método: Trata-se de um estudo transversal e observacional que avaliou 100 pacientes que aceitaram participar da pesquisa, com idade entre 18 e 75 anos, por meio de um questionário próprio para investigação do autodiagnóstico de doenças alérgicas e um teste cutâneo de leitura imediata ou prick test para detecção de IgE específica para alimentos que podem induzir a uma reação alérgica. Resultados: Foram aplicados e analisados 100 questionários em voluntários na faixa etária de 18 a 75 anos. Destes, 35 pacientes afirmaram ter alergia alimentar durante a aplicação do questionário. Apenas 10 tiveram resultado positivo ao prick test. A maioria dos resultados positivos no prick test estavam associados ao camarão, ao amendoim e ao caranguejo. Conclusão: O presente estudo evidenciou uma importante superestimação do número de autodiagnósticos de alergias alimentares, sendo o número de pacientes que se autodeclararam alérgicos a alimentos consideravelmente maior do que os resultados positivos no prick test. Constata-se que é necessário mais estudos que possuam em sua metodologia informações pré e pós-testes diagnósticos de alergia alimentar, para uma correta avaliação da proporção de casos.

Introduction: In recent years, the prevalence of food allergy has increased, as well as the number of incorrect self-diagnoses. This overestimation of diagnoses often culminates in unnecessary restrictive diets that may cause more harm than benefits to health. Objective: To evaluate the relationship between self-diagnosis of food allergy and the presence of specific IgE for that food. Method: This is a cross-sectional and observational study that evaluated 100 patients who agreed to participate in the research, aged between 18 and 75 years. A questionnaire was used to investigate the self-diagnosis of allergic diseases, and a skin prick test was used to detect food-specific IgE that may induce an allergic reaction. Results: One hundred questionnaires were administered and analyzed in volunteers aged from 18 to 75 years. Of these, 35 patients claimed to have food allergy during the administration of the questionnaire. Only 10 tested positive on the prick test. Most positive prick test results were associated with shrimp, peanut, and crab. Conclusion: The present study showed an important overestimation of the number of self-diagnosed food allergies, as the number of patients who self-reported being allergic to food was considerably greater than that of positive prick test results. More studies that have in their methods pre- and post-diagnostic information from food allergy tests are needed for a correct assessment of the proportion of cases.

Receitas para teste de provocação oral duplo-cego, controlado por placebo / Recipes for double-blind, placebo-controlled oral food challenge

Introdução: O teste de provocação oral (TPO) é o método mais confiável para verificar a relação entre o consumo de um alimento e o desencadeamento de reações adversas. Dentre as dificuldades na realização do TPO, destaca-se o mascaramento dos alimentos em TPO duplo-cego, controlado por placebo (TPODCCP). Objetivo: O objetivo deste trabalho foi elaborar receitas para uso em TPO-DCCP com leite de vaca, soja, ovo e trigo. Métodos: A elaboração das receitas considerou a necessidade de mascaramento do alimento a ser testado, de modo que a receita real e o placebo fossem indistinguíveis. Foram considerados também a quantidade de alimento a ser testado e o volume final das preparações, bem como a hipoalergenicidade dos demais ingredientes utilizados. Resultados: Foram desenvolvidas cinco receitas para TPO-DCCP, sendo duas para testes com leite de vaca, e as outras para testes com soja, ovo e trigo. As receitas placebo e real ficaram semelhantes em relação às cores, texturas, consistências, sabores e aromas. Conclusão: As receitas aqui apresentadas são de preparo fácil e rápido e atendem à maioria dos critérios exigidos para uso em TPO com alimentos. Há, porém, a necessidade de testá-las em estudos de validação para verificarse a possibilidade de serem usadas em protocolos científicos.

Introduction: Oral food challenge (OFC) is the most reliable method to assess the relationship between food consumption and onset of adverse reactions. Among the difficulties in performing OFC there is the masking of food in double-blind, placebocontrolled OFC (DBPC-OFC). Objective: The objective of this study was to prepare recipes to be used in DBPC-OFC with cow's milk, soy, egg and wheat. Methods: Recipe preparation focused on the need of masking the food to be tested, so that actual and placebo recipes were indistinguishable. Also, the amount of food to be tested and the final volume of preparations were considered, and the hypoallergenicity of other ingredients, as well. Results: Five recipes were developed for DBPC-OFC, two for cow's milk tests and the others for soy, egg and wheat tests. Placebo and actual recipes were similar in color, texture, consistency, taste and flavor. Conclusion: The present recipes are quick and easy to prepare and meet most of the criteria required for use in OFC. However, there is the need to test them in validation studies to assess the possibility of use in scientific protocols.

Enfermedad celíaca y otras patologías relacionadas con el gluten / Celiac disease and other pathologies related to gluten

Se intentan aclarar los conceptos diferenciales de enfermedad celíaca, sensibilidad al gluten no celíaca y alergia al trigo. Se abordan las manifestaciones clínicas junto al Score clínico desarrollado en el Servicio para calcular matemáticamente la presencia de enfermedad celíaca. Finalmente, se aborda el tartamiento, seguimiento, y las nuevas patologías relacionadas a la enfermedad

Enfermedad celíaca, sensibilidad no celíaca al gluten y alergia al trigo: comparación de patologías diferentes gatilladas por un mismo alimento / Celiac disease, non celiac gluten sensitivity and wheat allergy: comparison of 3 different diseases triggered by the same food

El gluten y otras proteínas relacionadas del trigo, centeno y cebada, tienen propiedades antigénicas que pueden desencadenar reacciones adversas en individuos susceptibles. La enfermedad celíaca fue la primera patología en que se estableció relación causal con estas proteínas alimentarias. Recientemente se han descrito la alergia al trigo y la sensibilidad no celíaca al gluten. Si bien sus formas de presentación clínica y su relación con la ingesta pueden ser similares, sus mecanismos patogénicos, forma de diagnóstico y tratamiento difieren. Dado que su prevalencia en conjunto es relativamente alta, resulta necesario que los médicos de atención primaria y pediatras se familiaricen con estas patologías, sepan cómo diferenciarlas y enfrentarlas. El objetivo de esta revisión es comparar los principales aspectos de epidemiología, fisiopatología, diagnóstico y tratamiento de estas 3 condiciones.

Gluten and other related proteins of the wheat, rye and barley, have antigenic properties that may trigger adverse reactions in susceptible individuals. Celiac disease was the first pathology with clear causal association related to the intake of these proteins. Recently, wheat allergy and non celiac gluten sensitivity have been described. Although, clinical presentation and its relation with protein ingestion may be similar and elicit confusion, their pathogenic mechanism, diagnosis and treatment are quite different. Since the prevalence of these diseases is relatively high as a whole, it is essential that these become familiar to primary care doctors and general pediatricians, thus they will know how to differentiate and face them. The aim of this review is to compare the main aspects of epidemiology, pathofisiology, diagnosis and treatment of these 3 conditions.

Nutritional status variation and intestinal and extra intestinal symptomatology in patients with celiac disease and non-celiac gluten sensitivity given specialized dietary advice / Evolução do estado nutricional e sintomatologia intestinal e extra intestinal em pacientes com doença celíaca e sensibilidade ao glúten não celíaca submetidos à orientação dietética especializada

ABSTRACT Objective: To investigate the nutritional status variation and symptomatology of patients with celiac disease and non-celiac gluten sensitivity after specialized dietary advice Methods: This prospective study included 80 patients with celiac disease and non-celiac gluten sensitivity. Clinical, metabolic, and nutritional variables were collected from medical records, and the symptomatology was investigated by the Metabolic Screening Questionnaire. The variables were assessed on two occasions (T1 - before dietary advice and T2 - after dietary advice) with an interval of three months between T1 and T2 Results: The median age was 42 years. The prevalences of celiac disease and non-celiac gluten sensitivity were 66.2% and 33.8%, respectively. Normal weight prevailed at T1 (58.8%) and T2 (56.3%), but 30.0% of the patients at T1 and 34.9% of the patients at T2 had excess weight. The two conditions had similar symptomatology. The most frequent signs and symptoms on both occasions involved the gastrointestinal tract, followed by energy/activity and emotions. All symptoms decreased significantly after the introduction of a proper diet Conclusion: The patients were normal weight on both study occasions (T1 and T2), and the symptoms improved after dietary advice. Thus, we reinforce the importance of proper dietary management in both clinical conditions to make dietary adjustments that improve these individuals' symptomatology.

RESUMO Objetivo: Investigar a evolução do estado nutricional e a sintomatologia de pacientes com doença celíaca e sensibilidade ao glúten não celíaca após orientação dietética especializada Métodos: O estudo prospectivo foi realizado com 80 pacientes portadores da doença celíaca e de sensibilidade ao glúten não celíaca. A coleta das variáveis clínicas, metabólicas e nutricionais foi feita por análise dos prontuários, enquanto a sintomatologia foi investigada por meio de Questionário de Rastreamento Metabólico. As variáveis foram avaliadas em dois momentos (T1 - antes da orientação dietética e T2 - após orientação dietética), com intervalo de 3 meses entre T1 e T2 Resultados: A mediana de idade foi de 42 anos. A prevalência de doença celíaca e de sensibilidade ao glúten não celíaca foi de 66,2% e 33,8%, respectivamente. A eutrofia prevaleceu como estado nutricional de T1 (58,8%) e T2 (56,3%), porém 30,0% dos pacientes em T1 e 34,9% em T2 apresentaram excesso de peso. A sintomatologia foi similar entre as duas enfermidades. Os sinais e sintomas mais frequentes nos dois tempos foram os do trato digestório, seguido pelos relacionados à energia/atividade e às emoções. Todos os sintomas diminuíram significativamente após a introdução da dieta Conclusão: Os pacientes apresentaram estado nutricional de eutrofia nos dois momentos avaliados (T1 e T2) e houve redução dos sintomas após as orientações nutricionais. Dessa forma, reitera-se a importância do manejo dietético adequado para ambas as enfermidades clínicas a fim de adequar a dieta que favoreça a melhora da sintomatologia apresentada por esses indivíduos.

La dieta sin gluten y los alimentos libres de gluten / Gluten-free diet and gluten-free foods

The gluten-free foods global market has significantly grown during the last decade. Gluten-free diet represents the treatment for celiac disease, non-celiac gluten sensitivity and wheat allergy Another group of persons that follows gluten-free diet has emerged, perceiving this diet as healthier feeding habit. It is especially this latter group that has increased to such an extent that they modified the market of gluten-free foods, improving availability of these products. However, because their demands and requirements are different, gluten content control of many of the currently existing local gluten-free foods is unsatisfactory We present the information available on these issues, emphasizing the few local data available, which refer to school-age snacks and the "Basic Food Basket", with and without gluten. Local studies show that gluten-free snacks for school-age children and the gluten-free Basic Food Basket are less available, their cost is higher and less than 8% meet FAO/WHO nutritional recommendations.

El mercado global de los productos sin gluten ha tenido un aumento sin precedente en la última década. La dieta sin gluten constituye el tratamiento de la enfermedad celíaca, la sensibilidad no-celíaca al gluten y de la alergia al trigo. Recientemente ha aparecido otro grupo de personas que elimina el gluten por considerarlo una opción de alimentación más saludable. Es especialmente este último grupo que ha logrado mejorar el mercado de alimentos sin gluten, pero sus requerimientos son distintos, por lo que parte del aumento del mercado ha sido en base de productos sin control adecuado de su contenido de gluten. Analizamos la información existente, poniendo énfasis en los datos nacionales recientemente obtenidos acerca de las colaciones escolares y la Canasta Básica Familiar sin gluten. En ambos casos la disponibilidad de productos equivalentes sin gluten es menor, el costo tres o más veces mayor y menos del 8% de ellos cumplen las recomendaciones nutricionales de la FAO/OMS.

Sensibilidad no celíaca al gluten: Una patología más que responde al gluten / Non-celiac gluten sensitivity: Another condition that responds to gluten

Remission of gastrointestinal and general symptoms after gluten withdrawal has been described in some non-celiac individuals for nearly 30 years. Only recently, efforts have been made to define this entity, now referred to as "non- celiac gluten sensitivity". It includes patients that clinically respond to gluten free diet without exhibiting allergic or autoimmune features to explain such response. Wheat allergy, celiac disease, irritable bowel syndrome and symptoms induced by high FODMAPs (Fermentable, Oligo-, Di-, Mono-saccharides And Polyols) consumption are the main differential diagnoses. The relationship with neuropsychiatric disorders such as schizophrenia and autism has not been demonstrated, but currently it gives ground to great hope in families with affected children. Epidemiology of non-celiac gluten sensitivity is not clear. It is described as more common among women and less common in children. Genetic and immune factors, changes in intestinal microbiota and non-gluten components present in wheat grains are main factors postulated in the pathogenesis of this condition. To date, there are no specific biomarkers for non-celiac gluten sensitivity and diagnosis is reached by excluding other causes of disease. A trial with gluten-free diet and subsequent gluten challenge is the methodology most frequently used to confirm diagnosis.

Sensibilidad al gluten: presentación de tres casos / Gluten sensitivity: presentation of three cases

En los últimos años, ha cobrado mayor interés la existencia de un cuadro clínico muy similar al de la enfermedad celíaca, que no se ajusta a los cánones tradicionales de diagnóstico. Se trata de pacientes con una alta sospecha diagnóstica de enfermedad celíaca, que presentan serología y biopsia de intestino delgado normal. La literatura relata, desde la década del 80, la existencia de un síndrome que relaciona el gluten de la dieta con un efecto tóxico generador de síntomas gastrointestinales en presencia de una mucosa normal. A esta entidad se la denominó síndrome de Cooper-Cook. En los últimos años, ha habido numerosas publicaciones que hacen referencia a esta entidad, pero ahora bajo la denominación de sensibilidad al gluten. En el siguiente artículo, se presentan tres casos clínicos que hacen referencia a esta enfermedad.

In the last few years, the existence of a clinical profile similar to celiac disease has become important; this disease does not adapt to the traditional diagnosis canons. It is related to a number of patients who are diagnosed as having the celiac disease but present normal serology and small bowel's biopsy. Since the 80's, medical literature reports the existence of a syndrome that connects gluten diet with a toxic effect that produces gastrointestinal symptoms even though the mucosa remains normal. This disease is called the Cooper-Cook syndrome. Over the last few years, there have been lots of publications about this disease under the name "gluten sensitivity". In the following article, three clinical cases that refer to this condition are presented.

Bases estructurales en la enfermedad celíaca / Structural bases in the disease celíaca

La fisiopatogenia de la enfermedad celíaca (EC) ha sido y es el objetivo de numerosas investigaciones; sin embargo, existen aún muchas incógnitas sin resolver en esta enfermedad. En la última década se establecieron nuevos conceptos relacionados con la edad de aparición de los síntomas, las formas de presentación y la incidencia de la enfermedad. El factor causante de la EC es atribuido generalmente a la toxicidad de las prolaminas que contiene el trigo. En la actualidad también se discute si la avena es tóxica o no para el celíaco, y este es un tema que diferentes grupos de investigación están intentando dilucidar. En esta Tesis de Doctorado se estudió la participación de la avena en la patogénesis de la enfermedad celíaca, comparándola con los efectos producidos por el trigo. También se examinó el rol de los aceites vegetales y de las proteínas de la leche de vaca en la inducción de los cambios histopatológicos del intestino que se presentan en la EC. Con este objetivo se desarrolló un modelo experimental en animales de laboratorio en el que se reprodujeron las típicas lesiones intestinales mediante la sensibilización a las proteínas causantes de la EC y su comportamiento ante los desafíos de antígenos específicos y la exclusión de los componentes tóxicos de la dieta.La sensibilización inmunogénica provocada fue similar tanto para la avenina como la gliadina, demostrándose además una reactividad cruzada entre gliadina y avenina en los animales desafiados con avena y gluten.La modificación del tipo y calidad de grasa de la dieta también influyeron en el grado de intensidad de las lesiones intestinales. El uso de aceite de oliva de alta calidad exhibe un efecto protector sobre la mucosa intestinal al disminuir el edema y la infiltración inflamatoria.La exclusión de leche vacuna de la dieta también contribuyó significativamente en la prevención de las alteraciones histológicas