Zidorio Ana Paula Caio, Dutra Eliane Said, Leão Dryelle Oliveira Dias, Costa Izelda Maria Carvalho
Universidade de Brasília, Brasília, DF, Brazil.
An Bras Dermatol. 2015 Mar-Apr;90(2):217-23. doi: 10.1590/abd1806-4841.20153206.
Epidermolysis Bullosa is a genetic disorder that affects mainly the skin, however, all others systems are influenced. The nutritional care of children and adolescents with Epidermolysis Bullosa is a key treatment strategy, since the energy needs are increased due to the disease's metabolism, burdening the immune system and cicatrization process, symptoms caused by the disease hinder the intake and adequate absorption of nutrients, which may result in inadequate growth and development. Because this is a rare disease, there are few professionals who know the characteristics of both the clinical evolution and nutritional and dietary treatments. This literature review discusses the latest knowledge on energy and specific nutrient requirements to the dietary treatment and monitoring of children and adolescents with Epidermolysis Bullosa.
大疱性表皮松解症是一种主要影响皮肤的遗传性疾病,然而,其他所有系统也会受到影响。大疱性表皮松解症患儿及青少年的营养护理是一项关键的治疗策略,因为疾病代谢会增加能量需求,给免疫系统和愈合过程带来负担,疾病引发的症状会妨碍营养物质的摄入和充分吸收,这可能导致生长发育不足。由于这是一种罕见疾病,了解临床进展以及营养和饮食治疗特点的专业人员很少。这篇文献综述讨论了大疱性表皮松解症患儿及青少年在饮食治疗和监测方面能量及特定营养素需求的最新知识。