促进大疱性表皮松解症儿童伤口愈合的营养概况和主题管理:证据有哪些?系统回顾

Maria Lúcia dos Santos, Ana Cristina Monteiro, Andréa Nascimento, Sívia Barbosa, Artur F Delgado, Patrícia Zamberlan
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引用次数: 0

摘要

大疱性表皮松解症(Epidermolysis Bullosa,EB)是一种罕见的遗传性疾病,其特点是皮肤脆弱,容易起泡和撕裂,导致严重的发病率和死亡率。根据特定的基因突变和所涉及的蛋白质,EB 可分为几种亚型,其分子复杂性因突变类型(错义、无义、插入、缺失)、突变在基因中的位置以及对蛋白质功能的影响而变得复杂。本系统综述旨在识别和综合有关伤口愈合干预措施和确诊 EB 儿童营养状况的现有证据。通过全面检索,共获得 28 篇文章,包括 21 项临床试验和 7 项观察性研究,涉及 994 名不同亚型的 EB 患者。大多数研究描述了EB亚型,如单纯性EB(EBS)、交界性EB(JEB)、营养不良性EB(DEB)和EB Kindler。促进伤口愈合的主要干预措施包括使用胶原蛋白、生物纤维素和各种外用药膏进行包扎。营养评估非常有限,只有六项研究对营养状况进行了检查,主要是通过人体测量和饮食摄入分析。分组分析表明,与 JEB 相比,DEB 患者的营养不良率更高。综述强调了在 EB 管理中应对伤口愈合和营养挑战的重要性。关键词营养;儿科;伤口愈合;表皮松解症
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Nutritional Profile and Topic Management for Wound Healing in Children with Epidermolysis Bullosa: What Is the Evidence? A Systematic Review
Epidermolysis Bullosa (EB) is a rare genetic disorder characterized by fragile skin that blisters and tears easily, leading to significant morbidity and mortality. Depending on the specific genetic mutations and the proteins involved, EB can be classified into several subtypes whose molecular complexity is compounded by the variability in mutation types (missense, nonsense, insertions, deletions), their locations within the genes, and the resultant effects on protein function. This systematic review aimed to identify and synthesize available evidence on wound healing interventions and the nutritional profile of children diagnosed with EB. A comprehensive search yielded 28 articles, including 21 clinical trials and seven observational studies, encompassing 994 patients with various EB subtypes. The majority of studies described subtypes such as Simplex EB (EBS), Junctional EB (JEB), Dystrophic EB (DEB), and EB Kindler. The primary interventions for wound healing included dressings with collagen, biocellulose, and various topical creams. Nutritional assessment was limited, with only six studies examining nutritional status, predominantly through anthropometry and dietary intake analysis. Subgroup analyses indicated higher malnutrition rates among patients with DEB compared to JEB. The review underscores the importance of addressing wound healing and nutritional challenges in EB management. Further research is needed to explore effective interventions and optimize care for this vulnerable population.KeywordsNutrition; pediatrics; wound healing; epidermolysis bullosa
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