Zidório Ana Paula Caio, Carvalho Kenia Mara Baiocchi de, Dutra Eliane Said
Clinical Nutrition Unit. Hospital Universitário de Brasília. Faculdade de Ciências de Saúde. Universidade de Brasília.
Graduate Program in Human Nutrition. Faculdade de Ciências de Saúde. Universidade de Brasília.
Nutr Hosp. 2023 Apr 20;40(2):286-294. doi: 10.20960/nh.04330.
Background and aims: epidermolysis bullosa (EB) is a rare disease presenting with skin fragility and formation of blisters. Constant presence of skin lesions, loss of blood, wastage of heat through the open skin, increased turnover of proteins and infections, result in increased energy, protein, and micronutrient needs. This study investigated the intake adequacy of energy, protein, zinc, iron, and dietary fiber in children with epidermolysis bullosa. Methods: this cross-sectional, analytical study was conducted with children and adolescents of up to 18 years of age with recessive dystrophic EB, severe subtype. Demographic data and clinical manifestations affecting food consumption were collected. Nutritional assessment was performed through anthropometric data. We used a formula to estimate specific energy needs for EB, and ratio of skin lesions was also evaluated to assist in estimating energy needs. A protein adequacy of 115 % to 200 % of the Recommended Dietary Allowance was considered for the adequacy of protein intake. And the Dietary Reference Intake tables according to sex and age were used as a reference for micronutrients. Intake assessment was performed using seven consecutive daily food records. Sip feed consumption was considered for intake assessment. Results: all patients showed undernutrition and presented at least three clinical symptoms that affect food consumption: pseudosyndactyly, microstomy, and blisters in the oral cavity. Sip feed constituted between 20 % and 50 % of the patients' energy intake. Intake of iron and zinc was adequate for most patients (confidence of adequacy ≥ 0.85), while fiber intake was below the reference value. Conclusions: this study underscores the importance of nutritional monitoring for EB patients, which is often interpreted as a skin disease but has enormous nutritional repercussions.
大疱性表皮松解症（EB）是一种罕见疾病，表现为皮肤脆弱和水疱形成。皮肤损伤持续存在、失血、通过开放性皮肤散热、蛋白质周转增加以及感染，导致能量、蛋白质和微量营养素需求增加。本研究调查了大疱性表皮松解症患儿能量、蛋白质、锌、铁和膳食纤维的摄入充足情况。方法：本横断面分析研究针对18岁及以下患有隐性营养不良型EB严重亚型的儿童和青少年进行。收集了影响食物摄入的人口统计学数据和临床表现。通过人体测量数据进行营养评估。我们使用一个公式来估算EB患者的特定能量需求，还评估了皮肤损伤比例以辅助估算能量需求。蛋白质摄入量充足性以推荐膳食摄入量的115%至200%来衡量。微量营养素以按性别和年龄划分的膳食参考摄入量表作为参考。使用连续7天的每日食物记录进行摄入量评估。经口喂养摄入量纳入摄入量评估。结果：所有患者均表现为营养不良，且至少有三种影响食物摄入的临床症状：并指畸形、小口畸形和口腔水疱。经口喂养占患者能量摄入的20%至50%。大多数患者铁和锌的摄入量充足（充足置信度≥0.85），而纤维摄入量低于参考值。结论：本研究强调了对EB患者进行营养监测的重要性，该病常被视为一种皮肤病，但却具有巨大的营养影响。
