Estudo da prevalência da doença celíaca em crianças e adolescentes com diabetes melito tipo 1: resultado de 10 anos de acompanhamento / Study of prevalence of celiac disease in children with type 1 diabetes mellitus: result of 10 years of follow-up

OBJETIVO: Avaliar a prevalência da doença celíaca (DC) em crianças e adolescentes com diabetes melito tipo 1 (DM1) atendidos no Serviço de Endocrinologia Pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais. SUJEITOS E MÉTODOS: Incluídos no estudo crianças e adolescentes com diagnóstico prévio de DM1 acompanhadas no serviço no período de março de 1999 a abril de 2009, com idades entre zero e 18 anos. Todos foram rastreados para DC na primeira consulta e anualmente. A investigação foi realizada por meio da dosagem dos anticorpos da classe IgA (AGAA) e IgG (AGAG) antigliadina. Os pacientes com AGAA e/ou AGAG acima de duas vezes o valor de referência foram submetidos à biópsia intestinal. RESULTADOS: Foram excluídos 21 pacientes do total inicial de 384. Destes, 50 tiveram a sorologia positiva e 29 foram submetidos à biópsia intestinal. A prevalência encontrada foi de 3,1%. CONCLUSÃO: O rastreamento periódico da DC nos pacientes diabéticos deve ser encorajado, dada sua alta prevalência.

OBJECTIVE: To estimate the prevalence of celiac disease (CD) in children and adolescents with type 1 diabetes mellitus (T1DM) treated in the Children's Division of Endocrinology, at the Universidade Federal de Minas Gerais Hospital das Clínicas. SUBJECTS AND METHODS: Children and adolescents diagnosed with T1DM, aged 0 to 18 year, were included in this study performed from March 1999 to April 2009. All patients were screened for CD at their first visit and, again, annually. The investigation was performed through the measurement of IgA (AGAA) and IgG (AGAG) antigliadin antibodies. Patients with values of AGAA and/or AGAG above two times the cutoff mark undertook intestinal biopsy. RESULTS: A group of 21 patients were excluded from the initial total of 384 patients. Out of the remaining, 50 patients had positive serology and 29 underwent intestinal biopsy. The prevalence index was 3.1%. CONCLUSION: The periodic screening of CD in diabetic patients should be encouraged, due to its high prevalence.

Comparison of Specific IgE Antibodies to Wheat Component Allergens in Two Phenotypes of Wheat Allergy

Specific IgE to gliadin was proposed as a marker for wheat dependent exercise induced anaphylaxis, while Tri a 14 was found to induce IgE response in baker's asthma. We evaluated whether these components could be used for discriminating phenotypes of wheat allergy. Twenty-nine patients who were wheat-induced anaphylaxis and/or urticaria (n=21, group I) and baker's asthma (n=8, group II) were enrolled. The prevalence of serum specific IgE to Tri a 14 was higher in group II (25%) than in group I (4.8%), while the serum specific IgE to gliadin was significantly higher in group I (70%) than in group II (12.5%). The cutoff value for predicting the baker's asthma using the ratio of serum specific IgE to Tri a 14 to gliadin was 742.8 optical densityx1,000/(kU/L) with high sensitivity and specificity. These findings suggest that Tri a 14/gliadin may be a potential marker for predicting baker's asthma.

Enfermedad celíaca silente en epilepsia criptogénica del adulto / Silent celiac disease among 21 patients with cryptogenic epilepsy

Background: Celiac disease (CD) is predominant in women and young people. Atypical, non-enteric symptoms are more common among adults. There is also an association between CD and neurological disorders, especially with cerebellar ataxia, polyneuropathy and epilepsy. Aim: To study the frequency of CD in a group of adults with cryptogenic epilepsy. Material and Methods: Twenty one patients with cryptogenic epilepsy, aged 20 to 65years (14 women) were studied, measuring IgA-anti transglutaminase antibodies and deamidated gliadin peptide (DGP) IgG and IgA antibodies. Results: One patient had elevated titers of both types of antibodies. Small bowel biopsy showed villous atrophy and lymphocytic infiltration compatible with CD. Conclusions: One of 21 adult patients with cryptogenic epilepsy had a silent CD.

Prevalence of serological markers for celiac disease (IgA and IgG class antigliadin antibodies and IgA class antiendomysium antibodies) in patients with autoimmune rheumatologic diseases in Belo Horizonte, MG, Brazil / Pesquisa de anticorpos antigliadina (classes IgA e IgG) e anticorpos antiendomísio classe IgA, em pacientes com doenças reumatológicas autoimunes em Belo Horizonte, Brasil

CONTEXT: Patients with autoimmune rheumatologic conditions and celiac disease tend to have a variety of autoantibodies, many of which have no clear pathogenic role. The literature contains frequent reports of celiac disease being more prevalent in patients with rheumatologic diseases, although this remains controversial. OBJECTIVES: To investigate the prevalence of positive serum tests for celiac disease, particularly IgA and IgG antigliadin (AGA) antibodies and IgA antiendomysium antibodies (EmA) in patients with autoimmune rheumatologic diseases. A second aim was to correlate positive serum tests with prednisone and immunosuppressant medication. METHODS: A total of 190 adults and pediatric patients with a variety of autoimmune rheumatologic diseases (systemic lupus erythematosus, rheumatoid arthritis, juvenile rheumatoid arthritis and spondyloarthrophathies) were evaluated and tested for IgA and IgG antigliadin-antibodies and IgA antiendomysium antibodies. Patients with positive serum tests underwent endoscopic duodenal biopsies for pathology studies. RESULTS: There were four positive sera (2.1 percent) for AGA IgA, all of which tested negative for AGA IgG and EmA. Three sera (1.6 percent) tested positive for AGA IgG; all were negative for AGA IgA and EmA. The EmA test at a 1:2.5 serum dilution tested positive in 94 patients (49.5 percent); at a 1:5 serum dilution it was positive in 41 patients (21.6 percent). Eleven subjects tested positive for EmA at 1:40 dilution; and all of these tested negative for IgA tissue antitransglutaminase (tTG) antibodies. Nine of the 11 EmA-positive patients and all 7 patients with positive antigliadin antibodies tests underwent duodenal endoscopic biopsies, and no significant changes were demonstrated in their duodenal mucosa. A positive EmA was associated with elevated optical density AGA IgA readings; however, there was no relationship between positive EmA and AGA IgG optical density readings. Prednisone and immunosuppressant use were unrelated to AGA IgA optical density readings or AGA IgG readings. These drugs were associated with fewer positive EmA tests. CONCLUSIONS: Positive AGAA, AGAG or EmA results are probably nonspecific for the presence of celiac disease among autoimmune rheumatologic disease patients. The intake of prednisone and immunosuprressant drugs seems to reduce the prevalence of IgA EmA, but it does not interfere with antigliadin antibodies tests.Further studies are required to estimate more accurately the prevalence of this disease in rheumatologic patients.

CONTEXTO: Tanto os pacientes com doenças reumatológicas autoimunes quanto os com doença celíaca costumam apresentar vários tipos de autoanticorpos, muitos deles ainda sem papel definido na etiopatogênese dessas afecções. Apesar de tratar-se de assunto controverso, é bastante citada na literatura a maior prevalência da doença celíaca em diversos grupos de pacientes reumatológicos. OBJETIVO: Investigar a prevalência de marcadores sorológicos positivos para doença celíaca: anticorpos antigliadina (AGA) classes IgA e IgG (AGAA e AGAG) e anticorpos antiendomísio classe IgA (EmA), em pacientes com doenças reumatológicas autoimunes. Procurou-se também avaliar a correlação entre a positividade dos testes sorológicos com o uso de prednisona e de medicamentos imunossupressores. MÉTODOS: Foram avaliados 190 pacientes adultos e pediátricos com doenças reumatólogicas variadas (lúpus eritematoso sistêmico, artrite reumatóide, artrite reumatóide juvenil e espondiloartropatias. Em todos foram realizadas pesquisas de AGAA e AGAG e de EmA, encaminhando-se os casos positivos para biopsias endoscópica duodenal e estudos histológicos. RESULTADOS: Houve quatro soros positivos (2,1 por cento) para AGAA, todos com resultados negativos para AGAG e EmA. Três soros (1,6 por cento) tiveram resultados positivos para AGAG, todos com resultados negativos para AGAA e EmA. Na pesquisa de EmA, a diluição do soro em 1:2,5 mostrou resultados positivos em 94 pacientes (49,5 por cento) e na diluição de 1:5, em 41 (21,6 por cento). Em 11 indivíduos obteve-se resultado positivo para EmA na diluição 1:40 e todos eles tiveram resultado negativo para a pesquisa de anticorpos antitransglutaminase tecidual IgA (tTg). Nove dos 11 pacientes positivos para EmA e todos os 7 pacientes com anticorpos antigliadina positivos foram submetidos a biopsia duodenal endoscópica, não se constatando alterações significativas da mucosa duodenal em nenhum deles. Todos os soros positivos para EmA apresentaram resultados negativos para a pesquisa de anticorpos antitransglutaminase tecidual classe IgA (tTG). A positividade para EmA associou-se a leituras de densidade óptica mais altas para AGAA. O mesmo não foi observado para AGAG. O uso de prednisona e de imunossupressores não se relacionou às leituras de densidade óptica dos AGAA, tampouco dos AGAG. O uso dessas medicações se relacionou, contudo, a menor positividade para EmA. CONCLUSÃO: Resultados positivos para AGAA, AGAG ou EmA demonstraram-se inespecíficos para a presença de doença celíaca em pacientes com doenças reumatológicas autoimune. O uso de prednisona e drogas imunossupressoras parece diminuir a prevalência de anticorpos antiendomísio IgA, mas não de anticorpos antigliadina. Mais estudos são necessários para se avaliar com maior precisão a prevalência da doença celíaca em pacientes reumatológicos.

Discordância de apresentação da doença celíaca em gêmeos monozigóticos

CONTEXTO: A doença celíaca é uma enteropatia autoimune causada pela sensibilidade ao glúten em indivíduos geneticamente predispostos. Apesar da característica genética da doença, estudos demonstram discordância de 30 por cento na sua apresentação em gêmeos monozigóticos. OBJETIVO: Apresentar dois pares de gêmeos monozigóticos, comprovados por estudos genéticos, discordantes para apresentação da doença celíaca. MÉTODO: Os pacientes foram acompanhados no Serviço de Gastroenterologia Pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais desde 1990, sendo submetidos a exames clínicos periódicos, biopsias intestinais e sorologia para anticorpos IgG e IgA antigliadina, determinados pela técnica de ELISA (ensaio imunoenzimático), e anticorpos classe IgA antiendomísio, determinados pela técnica de imunofluorescência indireta. Estudos genéticos foram realizados através da técnica de amplificação por PCR e posterior tipagem de loci de microssatélites do tipo STR (short tandem repeats). RESULTADOS: Em cada par de gêmeos, apenas um apresentou doença celíaca até o momento, mostrando que, apesar do genótipo idêntico, este não foi o único determinante para a expressão da doença. CONCLUSÃO: Outros fatores, ambientais e genéticos, parecem contribuir para determinação da doença.

CONTEXT: The celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. Despite the genetic characteristic of the disease, studies show discrepancy of 30 percent in its presentation in monozygotic twins. OBJECTIVE: To present two pairs of monozygotic confirmed by genetic study and discordant for presentation of celiac disease. METHODS: The patients were followed up at the Pediatric Gastroenterology Service - Hospital das Clínicas da Universidade Federal de Minas Gerais, MG, Brazil, since 1990, and were submitted to periodical clinical examinations, intestinal biopsies and serology for IgA and IgG antigliadin antibodies, determined by the ELISA technique, and IgA antiendomysial, determined by indirect immunofluorescence. Genetic study was conducted by the technique of amplification by PCR and later typing loci of microsatellites type of STR (short tandem repeats). RESULTS: In each pair of twins only one has presented celiac disease so far, demonstrating that despite the identical genotype, it was not the single determinant to express the condition. CONCLUSION: Other environmental and genetic factors might contribute to determining the disease.

Serological screening for celiac disease in symptomatic 12 to 36 month-old children

CONTEXT: The correct diagnosis of celiac disease in environmentally deprived children is frequently hindered by the common presence of other causes for the classical celiac disease symptoms: malnutrition, failure to thrive and frequent diarrheas. OBJECTIVES: To determine the prevalence of celiac disease in a group of 12 to 36 month-old children using immunoglobulin antibodies against gliadin (IgG and IgA-AGA), against endomysium (IgA-EMA), and against human tissue transglutaminase (IgA-tTG) as screening method. METHODS: A total of 214 children (114 boys), aged 12 to 36 months, on gluten-containing diet, were admitted to the study. IgG and IgA-AGA, IgA-tTG and IgA-EMA tests were performed in all sera. Biopsy was obtained from all children showing positive result in one or more of the serologic tests, excluding those in which IgG-AGA had been the only positive result. In those cases, polymerase chain reaction (PCR) HLA genotyping for the identification of celiac disease predisposing alleles was applied. HLA genotyping was also performed to confirm the diagnosis in children identified as celiac by means of positive serologic testing and compatible biopsy results. RESULTS: Normal results were obtained in 131 children. Ten children out of 68 identified as positive exclusively on the IgG-AGA test disclosed the presence of celiac disease predisposing alleles on PCR and underwent jejunal biopsy with normal results. All serologic tests were positive in four children. A fifth child showed positive IgG and IgA-AGA and IgA-tTG results but disclosed a negative IgA-EMA test. Jejunal biopsy of these five children revealed characteristic lesions of celiac disease. CONCLUSION: A prevalence of 2.3 percent was found among symptomatic 12- to 36-month-old children that had not been previously diagnosed as celiac.

CONTEXTO: O diagnóstico correto da doença celíaca em crianças ambientalmente carentes é frequentemente dificultado pela presença usual de causas outras para os clássicos sintomas da doença celíaca. OBJETIVO: Determinar a prevalência de doença celíaca em um grupo de crianças com idades compreendidas entre 12 e 36 meses, utilizando a pesquisa de anticorpos antigliadina (IgG e IgA-AGA), antiendomísio (IgA-EMA) e antitransglutaminase recombinante humana (IgA-tTG) como método de rastreio. MÉTODOS: Foram incluídas no estudo 214 crianças (114 meninos), com 12 a 36 meses de idade, todas em uso de dieta contendo glúten. Em todos os soros foi pesquisada a presença de anticorpos anti-IgG e IgA-AGA, anti-IgA-EMA e anti-IgA-tTG humana. Biopsia jejunal foi sugerida e efetuada em todas as crianças com resultados positivos em um ou mais testes sorológicos, excetuando-se as crianças em que o IgG-AGA tinha sido o único teste positivo. Nesta última situação, efetuou-se genotipagem para identificação de possíveis alelos HLA predisponentes por meio do método de PCR. Para confirmação do diagnóstico, a genotipagem dos alelos HLA também foi efetuada nas crianças identificadas como celíacas com base a testes sorológicos positivos e resultado da biopsia jejunal compatível. RESULTADOS: Em 131 crianças os resultados dos testes sorológicos foram normais. Em 68 delas, foi detectada apenas a presença de anticorpos anti-IgG-AGA. Em 10 destas, por terem apresentado presença de alelos HLA predisponentes, foi realizada biopsia jejunal, que revelou mucosa sem alterações. Todos os testes sorológicos foram positivos em quatro crianças. Os testes igG e IgA-AGA e IgA-tTG foram positivos numa quinta criança que, no entanto, apresentou teste IgA-EMA negativo. A biopsia jejunal dessas cinco crianças revelou lesões de mucosa típicas e compatíveis com o diagnóstico de doença celíaca. CONCLUSÃO: Prevalência de 2,3 por cento foi encontrada entre crianças de 12 a 36 meses de idade, ...

Enfermedad celíaca y su patogenia / An update on the pathogenesis of celiac disease

Celiac disease (CD), with a 1 percent worldwide prevalence, is an enteropathy caused by an autoimmune reaction to gluten in genetically susceptible individuals, which codify for histocompatibility molecules HLA DQ-2/DQ-8. From the anatomical point of view, CD is characterized by intestinal villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis (IELs) and leukocyte infiltration of the lamina propriety. Patients achieve a complete clinical and endoscopic remission with a gluten free diet. However, symptoms and anatomical alterations recur when this protein is reintroduced in the diet. The pathogenic mechanisms in this disease are not yet well understood, but it is clear that genetic, environmental and immunological factors play a role. The latter are the focus of this review, since this is the only autoimmune disease whose precipitating factor for immunological tissue damage is known.

Estado actual del diagnóstico y presentaciones clínicas de enfermedad celíaca: Estudio multicéntrico / Diagnosis and clinical presentations of celiac disease: A multicenter study

Background: Ample use of serological markers of high sensitivity and specificity led to relevant changes in the epidemiology of celiac disease. The impact of these changes in our country is poorly known. Aim: To assess the diagnostic procedures, clinical presentations and follow up of celiac disease as conducted in current pediatric practice. Material and methods: A multicentric retrospective study of patients diagnosed between 2000 and 2005 in five pediatric hospitals in Santiago, Chile. Data was obtained from clinical records, recorded in electronic spreadsheets and analyzed by descriptive statistics. Results: Seventy four of 83 identified patients fulfilled the inclusion criteria and were analyzed. Mean time to reach the diagnosis was 2.1 years. Cases younger than 10 years presented digestive manifestations such as chronic diarrhea and abdominal distension. Twenty one percent of older patients had atypical presentations (mainly short stature, refractory anaemia). Ten percent of cases were screened because a first degree relative had celiac disease. All patients had significant duodenal/jejunal lesion. IgA-antiendomysial antibodies (n =65) and IgA-antigliadin antibodies (n =23) were the most commonly used screening tests used but often, they were not available for follow up. A second biopsy was planned in all patients but only 26 had it due to repeated dietary transgressions, often due to unnoticed consumption of gluten in poorly labeled products. Conclusions: Digestive manifestations were the main presentation form for celiac disease among patients under 10 years of age. Atypical symptoms become relevant in patients older than 10 years. Antiendomysial and antitransglutaminase antibody measurement should be incorporated for routine screening and follow up of celiac disease in public hospitals. To improve food labeling about their gluten content is needed.

[Frequency of antigliadin and anti-transglutaminase in patients with irritable bowel syndrome]

On one hand the prevalence of Irritable Bowel Syndrome [IBS] ranges from 4 to 12 percent in the community; on the other hand, Celiac disease is prevalent in 4% of the IBS patients. Regarding the fact that cereals containing gluten are routinely used in Iran, diagnosing celiac disease in IBSpatients is important for conducting the treatments. The objective of this study was to assess the frequency of Celiac in IBS patients. This study was designed as a case control prospective study. Eighty six subjects were enrolled in this study according to Rome II Criteria. The control group included healthy subjects that were checked up on a routine base. The evaluation of Celiac was done through serology exams such as Anti Gliadin and Anti Transglutaminase [IgG and IgA]. Thirty eight male and 48 female patients suffering from IBS with a mean age of 34 +/- 11.7 years were enrolled in this study. The case and control groups were not significantly different in terms of sex and age. Flatulence, excessive gas passing and mucus passage were the most frequent symptoms in IBS patients. Night symptoms and weight loss [alarm signs] were observed in 37% and 32%, respectively. Elevated Antigliadin Titer was evident in 22 subjects [25%] which suggests a significantly higher rate for the case group as compared to the control group [t=4.67,P=0.0001]. Elevated IgG Anti Transglutaminase Titer was observed in 2 cases [2.3%] while IgA Anti Transglutaminase was not elevated in any of the IBS subjects, neither in the control group nor in the case group. Biopsy of small intestine can be suggested to rule out the elevated Antigliadin Titer Celiac Disease in IBS patients

Estudio de enfermedad celíaca en pacientes con psoriasis / Frequency of celiac disease among patients with psoriasis

Background: A possible relationship has been reported between psoriasis and celiac disease, with common pathogenic mechanisms that may need further investigation. Aim: To investigate the presence of clinical and serological markers for celiac disease in a group of Chilean psoriatic patients. Material and methods: We included 80 psoriatic patients (42 males) aged 16 to 79 years, whose serum was tested for antitransglutaminase antibodies (ATGA) and antiendomysial antibodies (AEMA). Patients with weakly positive AEMA tests were also tested for antigliadin antibodies (AGA). Results: In six patients (7.5 percent), AEMA and AGA were positive and one patient was positive for ATGA. An upper gastrointestinal endoscopy and duodenal biopsy was offered to these six patients and five accepted the procedure. Only one had a pathological diagnosis of celiac disease. Conclusions: Only one of 80 patients with psoriasis had celiac disease (1.2 percent). Other four patients with positive serologic markers had a normal duodenal biopsy. This group of patients may have latent celiac disease and they should be followed up.

Celiac disease in India.

This review of the current scenario of celiac disease (CD) in India covers both pediatric and adult CD. CD is primarily reported from northern India with isolated case reports from the rest of the country. CD cases among Indian children are associated with multiple DR3-DQ2 haplotypes. Delay in diagnosis is contributed by multiple factors including atypical presentations. Use of serological tests, IgA EMA and anti-tTG antibodies, along with modified ESPGHAN criteria provides a definitive diagnosis of CD. Dietary management is often difficult due to non-availability of labeled and marketed gluten-free foods. A majority of children with CD show normalization of nutrition, substantial improvement in growth parameters and attainment of healthy percentile curves on gluten-free diet. Small bowel histology remarkably improves but does not normalize even after 2-3 years on gluten-free diet. The true burden of the disease should be addressed by large epidemiological studies.

Celiac disease serology in dermatitis herpetiformis: which is the best option for detecting gluten sensitivity? / Serología de enfermedad celíaca en dermatitis herpetiforme. ¿Cuál es la mejor opción para detectar sensibilidad al gluten?

Background: Dermatitis herpetiformis (DH), a wellestablished gluten-sensitive skin disorder presenting variable degrees of enteropathy, constitutes a very useful model in order to assess the utility of the celiac disease (CD)-related serology in patients with mild intestinal damage. Objective: Our aim was to explore comparatively the performance of a panel of CD-related serologic tests in patients with DH. Methods: We assessed a series of 18 consecutive patients with skin biopsy proven DH presenting the overall spectrum of intestinal damage ranging from normal mucosa (n=6) to total villous atrophy (TVA) (n=6) through partial villous atrophy (PVA) (n=6). Sera were obtained from all patients while consuming a gluten containing diet. Serologic tests were antiendomysial, anti-tissue transglutaminase and antigliadin antibodies, and newly developed tests detecting both antibody isotypes (IgA and IgG) against deamidated synthetic gliadin-derivedpeptides (a-GDP). Results: Serologic tests had a variable behaviour depending on the degree of enteropathy. While the majority of tests detected patients with TVA, only 50% of those with normal histology had positive assays. Patients with PVA had discordant results. Classical CD-specific tests were positive in only some patients with mild damage while all of them were identified by a single assay detecting both isotypes of a-GDP. Conclusion: The detection of a-GDP antibodies was the most reliable tool in order to identify gluten sensitivity in DH patients presenting a wide range of intestinal damage. Further studies should explore if these findings can be extrapolated to patients with CDhaving mild enteropathy.

Introducción: la dermatitis herpetiformis (DH), una lesión dermatológica consecuencia de sensibilidad al gluten y asociada a grados variables de enteropatía, constituye un modelo muy útil con el objeto de evaluar la eficacia de la serología de la enfermedad celíaca(EC) en pacientes con daño intestinal leve. Objetivo: explorar comparativamente la utilidad de una serie de anticuerpos empleados en EC en pacientes con DH. Métodos: analizamos una serie de 18 pacientes consecutivos con diagnóstico de DH por biopsia de piel que presentaban el más amplio espectro de daño intestinal variando desde una mucosa normal (n=6) a la atrofia vellosa total (AVT) (n=6) y pasando por atrofia vellosaparcial (AVP) (n=6). Se obtuvo plasma de todos los pacientes mientras consumían gluten. Las pruebas serológicas empleadas fueron anticuerpos antiendomisio, anti-transglutaminasa y atigliadina, y unas pruebas recientemente desarrolladas que detectan anticuerpos IgA e IgG dirigidos contra péptidos sintéticos deamidados derivados de la gliadina (a-GDP). Resultados: las diferentes pruebas tuvieron un comportamiento variable dependiendo del grado de lesión intestinal. Mientras que la mayoría de las pruebas detectaron a todos los pacientes con AVT, sólo el 50% de aquellos con histologíanormal tuvieron resultados positivos. Los pacientes con AVP tuvieron resultados discordantes. Así las pruebas clásicas fueron positivas en sólo algunos pacientescon daño leve, mientras que todos ellos fueron positivos a una prueba para detectar ambos isotipos del a-GDP. Conclusión: la determinación de anticuerpos a-GDP fue la herramienta más confiable con el objeto de identificar serológicamente la sensibilidad al gluten en pacientes con DH que presentan variables grados de daño intestinal. Otros estudios deberían explorar si estos hallazgos podrían ser extrapolados a pacientes conEC con enteropatía de grado leve.

Soroprevalência da doença celíaca em crianças e adolescentes com diabetes melito tipo 1 / Serum prevalence of celiac disease in children and adolescents with type 1 diabetes mellitus

OBJETIVO: A associação de doença celíaca e diabetes melito já é conhecida há várias décadas. Pode ser encontrada em uma grande proporção de pacientes diabéticos, que geralmente são assintomáticos. O objetivo do estudo foi avaliar a soroprevalência da doença celíaca em crianças e adolescentes com diabetes melito tipo 1. MÉTODOS: Através de um estudo transversal, realizou-se triagem sorológica com anticorpo IgA antitransglutaminase humana em 354 crianças e adolescentes diabéticos, atendidos em ambulatórios de endocrinologia pediátrica de Recife, Pernambuco, no período de janeiro a junho de 2004. RESULTADOS: O antitransglutaminase humana foi positivo em 37/354 pacientes, resultando em soroprevalência de 10,5 por cento (IC95 por cento 7,6-14,2 por cento). Dentre os pacientes soropositivos, houve predomínio do sexo masculino (56,8 por cento) em relação ao feminino (43,2 por cento), porém sem significância estatística. O anticorpo antiendomísio foi realizado nos pacientes com antitransglutaminase humana positivo, sendo negativo em 14/37 (37,8 por cento) e positivo em 22/37 (59,5 por cento). CONCLUSÕES: A soroprevalência da doença celíaca em crianças e adolescentes diabéticos encontrada em Pernambuco é elevada, sendo comparável à observada em estudos da América do Norte e Europa e menor do que na Africa, sugerindo que a triagem sorológica para doença celíaca seja realizada em todas as crianças e adolescentes com diabetes melito tipo 1.

Coeliac disease in Sudanese children with clinical features suggestive of the disease

Our prospective hospital- based study examined frequency, clinical presentation and serological indicators of coeliac disease that correlated with intestinal biopsy among high- risk Sudanese children. From July 2001 to July 2002, 80 children aged 15 months- 18 years presented with poor appetite, weight loss, pallor and proximal muscle wasting. We diagnosed coeliac disease in 18 [22.5%]. Antigliadin antibodies [AGA- IgG, AGA- IgA or both] were high in 44; endomysial antibody retest was high in 30. Guardians of 12 children refused consent for biopsy. The other 18 were biopsied: 5 had total villous atrophy, 8 subtotal and 5 partial. All improved with gluten- free diet. Degree of villous atrophy did not correlate with diarrhoea duration or severity, anaemia severity or serological titres

Association between anti-endomysial antibody and total intestinal villous atrophy in children with coeliac disease.

BACKGROUND: There is growing evidence to suggest that detection of anti-gliadin antibody (AGA) and anti-endomysial antibody (EmA) can serve as sensitive markers of the degree of histological abnormalities in patients with coeliac disease. AIM: To evaluate the association between the presence of AGA and EmA and villous atrophy in intestinal biopsies of children with suspected coeliac disease. SETTINGS AND DESIGN: Intestinal samples of 46 children with failure to thrive, chronic diarrhoea, malabsorption and short stature with either AGA and/or EmA positivity were evaluated, retrospectively. The diagnosis of coeliac disease was based on ESPGHAN criteria. METHODS AND MATERIAL: Patients with total villous atrophy who fulfilled the ESPGHAN criteria for the diagnosis of coeliac disease were diagnosed to have coeliac disease. Nine patients without villous atrophy were taken as negative controls for this study. AGA-IgA was measured both by immunoflourescence (IF) and ELISA and EmA-IgA by IF while patients were on normal diet. Relationship between autoantibody positivity and intestinal total villous atrophy was evaluated. RESULTS: Overall positivity for AGA IgA was 85% (39/46) by IF+ELISA and EmA positivity was 85% (39/46) by IF within the study group. Histological examination revealed total villous atrophy with lymphocyte infiltration and crypt hyperplasia in 37 (80%) patients. AGA IgA was positive in 14 (38%) and 31 (84%) of these children by ELISA and IF, respectively. EmA positivity was detected in 35/37 (95%) cases with atrophy and 4/9 (44%) without atrophy (p=0.002). Thirty out of 37 (81%) patients with villous atrophy had both AGA IgA (IF) and EmA positivity (p=0.186). All of the sixteen patients that had both positive AGA IgA (ELISA+IF) and EmA had total villous atrophy (p=0.037). CONCLUSION: A significant association between total villous atrophy and EmA positivity has been documented in this study.

Comparaçäo dos anticorpos anti reticulina e antiendomísio classe IgA para diagnóstico e controle da dieta na doença celíaca / Comparison of IgA class reticulin an endomysium antibodies for diagnosis and control of the diet in celiac disease

Sensibilidade ao glúten é um estado de elevada resposta imunológica (celular e humoral) à ingestão de proteínas do glúten do trigo, centeio, cevada e aveia, em indivíduos geneticamente predispostos. A doença celíaca é sua expressão mais freqüente, variando as formas de apresentação. Tem como tratamento a exclusão de alimentos contendo as gliadinas tóxicas. Embora a biopsia do intestino delgado proximal seja necessária, tem-se ressaltado a importância de testes sorológicos no rastreamento, diagnóstico e monitorização da dieta isenta de glúten em pacientes com doença celíaca. O objetivo do presente estudo foi investigar a presença dos anticorpos antiendomísio (EmA-IgA) e anti-reticulina (ARA-IgA) em 56 pacientes celíacos (17 recém-diagnosticados; 24 aderentes à dieta; 15 com trasngressão à dieta). Os anticorpos foram detectados por imunofluorescência indireta, utilizando como substrato cordão umbilical humano para os EmA-IgA, fígado e rim de rato para os ARA-IgA. Nos pacientes recém diagnosticados e no grupo com transgressão à dieta houve positividade total de 100 por cento para os EmA-IgA e 59,4 por cento para ARA-IgA. Nos pacientes aderentes à dieta nenhum dos anticorpos foi detectado. Dentre os 32 pacientes positivos, a concordância foi de 59,4 por cento (19), sendo que 40,6 por cento (13/32) eram ARA-IgA negativo e EmA-IgA positivo. Nenhum paciente mostrou-se positivo para os ARA-IgA e negativo para os EmA-IgA. Portanto, a sensibilidade para os EmA-IgA foi de 100 por cento e de 59,4 por cento para os ARA-IgA. A associação dos dois testes não aumentou os índices de positividade total nas amostras. Conclui-se que, atualmente, a pesquisa dos EmA-IgA pode constituir teste sorológico de escolha, seja para diagnóstico, seja para seguimento dos pacientes celíacos, pelo alto valor preditivo, alta sensibilidade e especificidade e relativo baixo custo quando se utiliza cordão umbilical humano como substrato.

Anticorpos séricos na doença celíaca / Celiac disease serum antibodies

O diagnóstico acurado da doença celíaca é muito importante porque os pacientes devem aderir a uma dieta sem glúten por toda a vida e diante do maior risco de complicações, como as neoplasias intestinais, que poderá advir do não cumprimento rigoroso da dieta. Nesta revisão são apresentados os novos conceitos referentes às formas de apresentação da doença (ativa, silenciosa, latente e potencial) e sua associação com outras enfermidades e são focalizados principalmente o valor e a eficácia da determinação dos anticorpos séricos antigliadina e dos autoanticorpos anti-reticulina, antiendomísio e antitransglutaminase tecidual, no auxílio ao diagnóstico e seguimento da doença celíaca.

Serological diagnosis of celiac disease.

In developing countries like India, unlike in the West, diagnosis of celiac disease is a dilemma because the histological changes consistent with celiac disease may not be pathognomonic of the disease. We need clarity on how to use one or more of the serological antibodies, namely IgG and IgA anti-gliadin and IgA anti-endomysial antibodies as independent disease markers or as adjuncts to clinico-histological diagnosis. In this review, various possible algorithms have been discussed. The serological antibodies can be used as reliable screening tests to decide whom to biopsy and to interpret histological changes in our settings where malnutrition and intercurrent enteric infection are common.

Estudio de los anticuerpos antigliadina y antiendomisio en familiares directos de enfermos celíacos / Study of antigliadin and antiendomysium antibodies on close relatives of coeliac patients

La Enfermedad Celíaca (EC) tiene mayor frecuencia entre familiares de primer grado de enfermos celíacos. Su diagnóstico precoz y adecuado tratamiento evitan posibles complicaciones de la enfermedad. Por su alta sensibilidad y especificidad los Anticuerpos Antigliadina (Ac AGA) y Antiendomisio (Ac EMA) se utilizan como método de screening. Objetivo: detección de la enfermdad en los familiares directos mediante la determinación de Ac AGA como método de screening inicial y de Ac EMA como método confirmatorio. Material y método: se estudiaron 82 familiares de primer grado de 32 pacientes celíacos con determinación de Ac AGA de tipo Ig G por inmunofluorescencia indirecta. A los positivos se les realiza Ac EMA de tipo Ig A mediante igual técnica. A los resultados Ac EMA negativos se les dosificó la Ig A. A los Ac EMA positivos y Ac EMA negativos con déficit de Ig A se le realiza biopsia de intestino delgado. Resultados: 13 Ac AGA positivos (16 por ciento), 6 dudosos. 5/13 Ac AGA positivos y 0/6 Ac AGA dudosos son Ac EMA positivos. De los 8 Ac EMA positivos (100 por ciento) presentaron atrofia. La biopsia del paciente con déficit de Ig A no la presenta. En suma: se diagnosticó 6 por ciento de EC entre los familiares, 3 hijos y 2 hermanos. Conclusiones: el screening de la EC entre los familiares de EC con anticuerpos, es factible en nuestro medio y de relevancia dado el porcentaje de enfermos celíacos que se diagnostican con este método