TY  - JOUR
AU  - dos Santos, Maria Lúcia
AU  - Monteiro, Ana Cristina
AU  - Nascimento, Andréa
AU  - Barbosa, Sívia
AU  - Delgado, Artur
AU  - Zamberlan, Patrícia
PY  - 2024
DA  - 2024/07/17
TI  - Nutritional Profile and Topic Management for Wound Healing in Children with Epidermolysis Bullosa: What Is the Evidence? A Systematic Review
JO  - Recent Progress in Nutrition
SP  - 010
VL  - 04
IS  - 03
AB  - 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
SN  - 2771-9871
UR  - https://doi.org/10.21926/rpn.2403010
DO  - 10.21926/rpn.2403010
ID  - dos Santos2024
ER  -