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Abstract(s)
Objetivos: Estudos recentes sugerem que o paradigma de apresentação da Doença Celíaca tem sofrido alterações, com uma maior prevalência de sintomas atípicos, razão pela qual as mais recentes Guidelines da ESPGHAN sugerem a passagem de uma classificação em doença clássica ou atípica para uma baseada em sintomas gastrointestinais e/ou extra-intestinais. O presente estudo pretende determinar a incidência de Doença Celíaca e avaliar a existência de diferenças na sua apresentação clínica nas populações pediátrica e de adultos de um Hospital terciário em Portugal.
Métodos: com base na identificação dos doentes incluidos numa base de dados pediátrica pré-existente e através dos resultados de anticorpos positivos para doença celiaca realizados neste hospital, foram analisados os processos electrónicos e em papel dos pacientes diagnosticados entre Janeiro de 1986 e Julho de 2017. Foram recolhidos dados respeitantes à idade de diagnóstico e ao tipo de sintomas à apresentação (gastro-intestinais e/ou extra-intestinais) e atribuida uma classificação em clássica, atípica, latente ou silenciosa. As variáveis tipo de sintomas e classificação atribuida foram comparadas consoante a idade de diagnóstico, utilizando o teste do Qui Quadrado.
Resultados: Das 145 crianças e 37 adultos diagnosticados, o sexo feminino representou 61,4% e 80,5% das amostras, respetivamente. No que respeita ao tipo de sintomas, 76.4% dos sujeitos pediátricos reportaram uma combinação de sintomas gastro-intestinais e extra-intestinais, com apenas 38.9% dos adultos a referiram ambos os tipos de sintomas. Cumpriram os critérios para Doença Celiaca clássica, 62,1% dos doentes da amostra pediátrica e 62,9% da amostra de adultos. A classificação da Doença Celíaca atribuida parece ser independente da idade em que o diagnóstico ocorreu (p= 0.172) mas o tipo de sintomas apresentados parecem ser dependentes dessa idade (p<0,001). A incidência de Doença Celíaca foi calculada em 1:255 em indíviduos com idade < a 18 anos e 1:3774 em indíviduos com = 18 anos.
Conclusões: Comparando a incidência obtida nesta população pediátrica com a prevalência encontrada por Antunes et al. (1:134), podemos concluir que por cada criança diagnosticada existe ainda outra por diagnosticar. A classificação da Doença Celíaca em clássica e atipica não parece ser a mais adequada, uma vez que pode conduzir a uma perda crítica de informação possivelmente importante para o refinamento do diagnóstico diferencial perante manifestações extra-intestinais.
Objectives and study: The clinical presentation of Coeliac Disease (CD) has showed greater frequency of ‘atypical’ symptoms, especially in adults. Recent ESPGHAN Guidelines recommend a change in the way we categorize patients, from a classification based on the classical/atypical pattern to one based on the type of symptoms presented - gastrointestinal/extra intestinal. The aims of this study were to determinate the incidence of CD in the paediatric and adults’ populations and to evaluate the existence of differences in the clinical presentation based on the age of diagnosis in a tertiary hospital in Portugal. Methods: Were analysed the electronic and paper medical records of patients diagnosed with CD in the Gastroenterology Department and Paediatrics’ Gastroenterology Unit in a tertiary hospital between January 1986 and July 2017, using a previous existing data base and positive antibodies results. The data – symptoms at presentation (extra-intestinal versus gastrointestinal) and CD classification (classical, atypical, silent or latent) were separated according to the age of diagnosis (before the age of 18 and with 18 years old or more) and analysed. The Montreal Chi-Square Test was used to compare the results. Results: Of the 145 paediatric and 37 adults’ patients diagnosed, 61.4% and 80.5%, respectively, were females. Regarding the type of symptoms at presentation, 76.4% of the paediatrics patients reported both gastrointestinal and extra-intestinal symptoms, with only 38.9% of the adults’ patients referring both types of symptoms. Fulfilled the criteria for classical CD 62.1% of the paediatric subjects and 62.9% of the adults. The analyse of the independence of the variables in study showed that the CD classification was independent from the age of diagnosis (p= 0.172) but the type of symptoms at presentation was not (p<0.001), reinforcing the need to address the differences between patients based on the symptoms presented and not on one category assigned. The CD incidence calculated was 1:255 in the patients with < 18 years old and 1:3774 in patients with = 18 years. Conclusions: Comparing the incidence found in the paediatrics population with the previous prevalence obtained by Antunes et al. (1:134), we can conclude that for every child diagnosed with CD there is another without proper diagnosis. The CD classification as classical or atypical doesn’t seem to be the most accurate to stablish clinical patterns, as it appears to conduct to a loss of critical information that may present as important for future refinement of differential diagnosis.
Objectives and study: The clinical presentation of Coeliac Disease (CD) has showed greater frequency of ‘atypical’ symptoms, especially in adults. Recent ESPGHAN Guidelines recommend a change in the way we categorize patients, from a classification based on the classical/atypical pattern to one based on the type of symptoms presented - gastrointestinal/extra intestinal. The aims of this study were to determinate the incidence of CD in the paediatric and adults’ populations and to evaluate the existence of differences in the clinical presentation based on the age of diagnosis in a tertiary hospital in Portugal. Methods: Were analysed the electronic and paper medical records of patients diagnosed with CD in the Gastroenterology Department and Paediatrics’ Gastroenterology Unit in a tertiary hospital between January 1986 and July 2017, using a previous existing data base and positive antibodies results. The data – symptoms at presentation (extra-intestinal versus gastrointestinal) and CD classification (classical, atypical, silent or latent) were separated according to the age of diagnosis (before the age of 18 and with 18 years old or more) and analysed. The Montreal Chi-Square Test was used to compare the results. Results: Of the 145 paediatric and 37 adults’ patients diagnosed, 61.4% and 80.5%, respectively, were females. Regarding the type of symptoms at presentation, 76.4% of the paediatrics patients reported both gastrointestinal and extra-intestinal symptoms, with only 38.9% of the adults’ patients referring both types of symptoms. Fulfilled the criteria for classical CD 62.1% of the paediatric subjects and 62.9% of the adults. The analyse of the independence of the variables in study showed that the CD classification was independent from the age of diagnosis (p= 0.172) but the type of symptoms at presentation was not (p<0.001), reinforcing the need to address the differences between patients based on the symptoms presented and not on one category assigned. The CD incidence calculated was 1:255 in the patients with < 18 years old and 1:3774 in patients with = 18 years. Conclusions: Comparing the incidence found in the paediatrics population with the previous prevalence obtained by Antunes et al. (1:134), we can conclude that for every child diagnosed with CD there is another without proper diagnosis. The CD classification as classical or atypical doesn’t seem to be the most accurate to stablish clinical patterns, as it appears to conduct to a loss of critical information that may present as important for future refinement of differential diagnosis.
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Keywords
Atípica Clássica Incidência de Doença Celíaca Sintomas Extra-Intestinais Sintomas Gastro-Intestinais