Consumo alimentar habitual e evolução antropométrica de crianças e adolescentes portadores de epidermólise bolhosa : estudo de uma série de casos

Abstract

Introdução: Epidermólise Bolhosa engloba grupo de desordens hereditárias, caracterizadas pela ocorrência de bolhas na pele e membranas mucosas como resultado de mínimo trauma mecânico. Comprometimento nutricional do portador está relacionado com a gravidade das complicações, sendo as necessidades nutricionais do portador um reflexo da idade, extensão das bolhas, presença de infecção, necessidade de crescimento e manifestações clínicas. Objetivo: Investigar a adequação do consumo alimentar habitual de energia, da contribuição percentual de macronutrientes na dieta, da quantidade de proteínas, de ferro, zinco e fibra alimentar, bem como, a evolução antropométrica e as principais manifestações clinicas que afetam o consumo alimentar de dez portadores de Epidermólise Bolhosa, menores de 19 anos de idade, acompanhados nos ambulatórios de nutrição pediátrica e de dermatologia do Hospital Universitário de Brasília. Método: Trata-se de um estudo transversal analítico, do tipo estudo de uma série de casos, de uma coorte clínica de portadores de dois tipos diferenciados de epidermólise bolhosa a simples e a distrófica recessiva. E um recorte longitudinal de dados antropométricos desde a primeira consulta até maio de 2013. A primeira etapa do estudo refere-se à revisão de literatura acerca dos principais conceitos e aspectos do tratamento nutricional na EB. Foram coletados dados antropométricos, de consumo alimentar e informações complementares quanto às condições do nascimento, da alimentação no primeiro ano de vida e de manifestações clinicas que afetam o consumo alimentar, bem como identificação do percentual da superfície corporal atingida por bolhas. Elaboradas curvas de crescimento de acordo com os padrões da Organização Mundial da Saúde. O método de avaliação do consumo probabilidade de consumo inadequado para 90% dos casos. Consumo habitual de ferro e zinco se mostrou adequado para 90% dos casos. Adequação do consumo habitual de fibras apresentou-se inconclusiva para 90% dos casos. Conclusão: Dados do primeiro ano de vida irão contribuir para delineamento do perfil nutricional do portador. Monitoramento do peso e da altura deve ser feito em curto intervalo a fim de intervir em tempo oportuno. Estabelecimento das necessidades energéticas parece ser o maior desafio, devido à interação entre aumento das necessidades nutricionais pela atividade da doença, ao surgimento de manifestações clínicas que afetam o consumo alimentar e à necessidade de crescimento. ______________________________________________________________________________________ ABSTRACT

Introduction: Epidermolysis bullosa is composed of a group of inherited disorders characterized by the occurrence of blisters on the skin and mucous membranes as a result of mechanical trauma. Nutritional impairment carrier is associated with the severity of complications, and the nutritional requirements of patients reflects the age, extent of blistering, presence of infection, catch up growth and clinical manifestations. Purpose: Analyze habitual food intake, growth curves and main clinical manifestations that affect food consumption of ten patients with epidermolysis bullosa less than nineteen years followed at Hospital Universitário de Brasília between 2004 and 2013. Methods: This is an analytical cross-sectional study, comprises a series of cases study of a clinical cohort of patients with two different types of epidermolysis bullosa: simple and recessive dystrophic. In addition, there is a longitudinal cut of anthropometric data from the first visit until May 2013. The first stage of the study refers to the literature review of key concepts and aspects of nutrition therapy in EB. In the second stage, anthropometric data, food consumption and additional information regarding the conditions of birth, feeding in the first year of life and clinical manifestations that affect food consumption, as well as identifying the percentage of body surface affected by bubbles were collected. Then, growth curves prepared in accordance with the standards of the World Health Organization. The measurement of food intake was the dietary record of seven consecutive days. It was applied the Dietary Reference Intakes in assessing the adequacy of habitual protein intake in grams per day of iron, zinc and dietary fiber. Specific equation was used for EB patient and percentage of average need for estimating energy requirement. It was also used the acceptable macronutrient distribution ranges for evaluating the adequacy of the percentage contribution of macronutrients in total dietary energy. Results: Production of a review article entitled " Nutritional aspects of children and adolescents with epidermolysis bullosa: a literature review ". In a series of cases, All the patients were born at term, 90% with adequate birth weight. Age at diagnosis ranged from birthday until after the first year of life. Most patients (90%) received breastfeeding. Complications in the introduction of complementary foods were found in over half the cases of patients with dystrophic epidermolysis of the recessive type. Clinical manifestations were present in all patients. Microstomy reached 70% of the cases and blistering in the oral cavity 60% of cases. Dysphagia, chewing problems, ankyloglossia, odynophagia and gastroesophageal reflux also were identified. All patients were fed orally and the consistency of supply ranged from normal until liquid. As for the growth curves, a carrier of epidermolysis simplex type grew at or above the 85th percentile. All carriers of recessive dystrophic epidermolysis had growth in weight and height for age with values below the 3rd percentile, large on the bow curve. Adequacy of energy consumption in specific equation to estimate the energy requirements of patients showed deficient for more than half of patients with recessive dystrophic epidermolysis. Habitual protein intake showed a low probability of inadequate intake to 90% of cases. Habitual consumption of iron and zinc was adequate for 90% of cases. Adequacy of usual dietary fiber intake was inconclusive in 90% of cases. Conclusion: Data from the first year of life will help to design the nutritional profile of the carrier in the first year of life. Monitoring of weight and height should be done in a short period in order to intervene in a timely manner. Establishment of energy needs seems to be the biggest challenge, due to the interaction between increased nutritional needs for disease activity, the appearance of clinical symptoms that affect food consumption and the need for growth.