Nutritional aspects of children and adolescents with epidermolysis bullosa: literature review

Abstract

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.

Epidermólisis bullosa en el Perú: estudio clínico y epidemiológico de pacientes atendidos en un hospital pediátrico de referencia nacional, 1993-2015

“…Más del 60% de pacientes con EB presentaron anemia, cifra que podría ser mayor ya que el 12,9% de los casos no tenía estudios de laboratorio; además, 45 pacientes procedían de provincia, lo que dificultó su seguimiento clínico y de laboratorio. Por todo ello, son importantes el consejo y soporte nutricionales personalizados para promover su crecimiento y desarrollo, optimizar el cierre de las heridas y mejorar su calidad de vida (23) .…”

Neonate with Severe Complications of Epidermolysis Bullosa and Bilateral Clubfoot: An Unusual Case Presentation and Treatment

“…Although new knowledge about the muta ons of genes that encode structural proteins responsible for adhesion between cutaneous structures allows precise classifi ca on of pa ents in subcategories of EB 12,13 , in our case, we could not classify the type of EB and explain the possible connec on with clubfoot due to limited resources.…”

“…Medical surveillance for involvements of other systems should be a part of the rou ne evalua on. In children, complica ons such as: cutaneous losses, infec ons, anemia, respiratory failure, dehydra on or diges ve problems lead to impaired nutri on and growth and is some cases even death 4,12,16 . Despite the progress on the knowledge about the cause of EB, currently, there is no sa sfactory treatment available and the long-term prognosis of the disease is poor.…”

“…Gene, protein replacement and cell-based therapies are being inves gated, but these approaches are at the early stages of inves ga on, and there are a number of uncertain es surrounding them 13,17 . Treatment is primarily suppor ve and preven ve consis ng of: wound healing, infec on control, pain management, nutri onal support, adequate dressing and other therapies depending on the complica ons that arise 4,12,17 .…”

Dystrophic epidermolysis bullosa associated with congenital contractures of the upper and lower limbs: literature review

“…Наличие язвенных элементов в ротовой полости, микростомии, сте-ноза пищевода, нарушение переваривания и вса-сывания, с одной стороны, и постоянное уве-личение потребности в энергии и питательных веществах -с другой, приводят к замедлению репарации кожных покровов и возникновению воспалительных и инфекционных процессов. Поэтому в комплексном лечении БЭ важную роль играет восполнение белково-энергетической не-достаточности, коррекция водно-электролитных нарушений у детей раннего возраста, остеоиндук-тивная терапия (витамин D 3 , препараты кальция), а также хирургическое лечение вторичных стрик-тур желудочно-кишечного тракта [18].…”