Maria Lúcia dos Santos, Ana Cristina Monteiro, Andréa Nascimento, Sívia Barbosa, Artur F Delgado, Patrícia Zamberlan
{"title":"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","authors":"Maria Lúcia dos Santos, Ana Cristina Monteiro, Andréa Nascimento, Sívia Barbosa, Artur F Delgado, Patrícia Zamberlan","doi":"10.21926/rpn.2403010","DOIUrl":null,"url":null,"abstract":"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","PeriodicalId":74647,"journal":{"name":"Recent progress in nutrition","volume":" 7","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Nutritional Profile and Topic Management for Wound Healing in Children with Epidermolysis Bullosa: What Is the Evidence? A Systematic Review\",\"authors\":\"Maria Lúcia dos Santos, Ana Cristina Monteiro, Andréa Nascimento, Sívia Barbosa, Artur F Delgado, Patrícia Zamberlan\",\"doi\":\"10.21926/rpn.2403010\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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\",\"PeriodicalId\":74647,\"journal\":{\"name\":\"Recent progress in nutrition\",\"volume\":\" 7\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-17\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Recent progress in nutrition\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.21926/rpn.2403010\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Recent progress in nutrition","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.21926/rpn.2403010","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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