Epidermólise bolhosa : uma abordagem clínico-nutricional

Tese (doutorado)—Universidade de Brasília, Faculdade de Ciências da Saúde, Programa de Pós Graduação em Nutrição Humana, 2019.Introdução: A epidermólise bolhosa (EB) é uma doença rara caracterizada pela fragilidade da pele e das mucosas que causa o desenvolvimento de bolhas. Dentre os tipos que acometem o estado nutricional, a EB distrófica recessiva (EBDR) é um dos mais graves. A condição clínico-metabólica de pessoas com EB tem sido comparada à de pessoas com queimaduras, devido a presença de lesão aberta de pele, risco de infecção, perda de calor, maior turnover de proteínas e elevado dispêndio energético. Objetivo: Estudar os aspectos clínico-nutricionais que afetam pessoas com EB. Métodos: Inicialmente foi realizada revisão sistemática da literatura sobre a efetividade da gastrostomia (GTT) na qualidade de vida (QV) e no estado nutricional das pessoas com EB, que envolveu a investigação da evolução dos dados antropométricos e os relatos da qualidade de vida antes e após a inserção da GTT. Em estudo original, foram estudados o gasto energético de repouso (GER), estimado tanto por calorimetria indireta (CI) quanto por fórmulas preditivas e a presença de catabolismo proteico por meio da realização de balanço nitrogenado em pessoas com EBDR. Resultados: Revisão sistemática: Grande parte dos estudos incluídos na revisão identificou que, após a inserção da GTT, houve melhora na evolução dos dados antropométricos e também resultados positivos na QV. As complicações com a GTT relatadas nesta revisão sistemática não diferem das observadas em pacientes com outras doenças. Artigo original: Foram avaliadas 10 pessoas com EBDR, 7 delas do sexo feminino, com idades que variaram entre 4 e 33 anos. Os valores preditos do GER por equação foram menores do que os valores aferidos pela CI, exceto para as duas participantes adolescentes. A magnitude das diferenças entre os valores aferidos e os valores preditos, entretanto, foi bastante variável. Todos os participantes apresentaram consumo dietético hiperproteico, sendo que para 5 deles o consumo foi de 4 a 5 gramas de proteína por quilograma de peso corporal. Ainda assim, o catabolismo proteico foi observado em aproximadamente 70% dos indivíduos, especialmente naqueles com maior superfície corporal com lesão (SCL) infectada. Todos os participantes com superfície corporal infeccionada estavam em catabolismo proteico, sendo que aqueles que manifestaram níveis mais elevados de catabolismo proteico apresentaram, comparativamente, os maiores valores percentuais de SCL. A maioria dos participantes manifestaram alto consumo energético, contudo apenas os menores de 17 anos que apresentaram consumo energético em torno de 130 quilocalorias por quilograma de peso corporal estavam em anabolismo. Conclusões: A GTT parece ser uma estratégia segura e eficaz para melhorar o estado nutricional e a QV das pessoas com EB. O GER é subestimado pela equação preditiva, e a presença de catabolismo proteico é consistente com maior SCL, o que sugere que pessoas com EBDR têm GER aumentado e balanço nitrogenado negativo. Estes resultados são relevantes ao fornecer dados mais concretos sobre o comprometimento nutricional das pessoas com EBDR.Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES).Background: Epidermolysis bullosa (EB) is a rare disease characterized by fragility of the skin and mucous membranes which causes repeatedly the development of blisters. The recessive dystrophic sub-type of EB (RDEB) strongly impacts on nutritional status, and its clinical-metabolic condition has been compared with the ones of burned people, due to the presence of open skin lesion, risk of infection, loss of heat, increased turnover of proteins and high energy expenditure. Objective: To study the clinical-nutritional aspects that affect people with EB. Methods: Initially, a systematic review about the effectiveness of gastrostomy (GTT) on the quality of life (QOL) and on the nutritional status of people with EB was performed, which involved anthropometric data evolution and reports of quality of life before and after the insertion of the GTT. It was studied the resting energy expenditure (REE), estimated by indirect calorimetry (IC) as well as by predictive formulas, and the presence of protein catabolism through nitrogen balance in people with RDEB. Results: Systematic review: Most of the studies included in the review identified that, after insertion of GTT, there was an improvement in the evolution of anthropometric data and positive results in QOL. Complications with GTT reported in this systematic review do not differ from those observed in patients with other diseases. Original article: Ten individuals with EBDR were evaluated, 7 of them were female, with ages range from 4 to 33 years. The GER values predicted by equation were lower than the IC values, except for the two adolescent participants. The magnitude of the differences between the values measured and the values predicted, however, was quite variable. All participants had hyperproteic dietary intake, and for 5 of them the intake was between 4 and 5 grams of protein per kilogram of body weight. Even so, protein catabolism was observed in approximately 70% of the subjects, especially those with greater body surface area (BSA) with infected skin. Most of the participants presented high energy intake. However, only those who presented energy consumption around 130 kilocalories per kilogram of body weight were in anabolism. Conclusions: GTT appears to be a safe and effective strategy to improve nutritional status and QOL of people with EB. REE was generally underestimated by the predictive equation, and the presence of protein catabolism is consistent with higher BSA, which suggests that people with RDEB present increased REE and negative nitrogen balance. These results are relevant in providing more concrete data on the nutritional commitment of people with EBDR

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

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

Does probiotic consumption enhance wound healing? a systematic review

The use of probiotics is one of the emerging lines of treatment for wound healing. This systematic review aimed to summarize currently available evidence on the effect of oral or enteral probiotic therapy on skin or oral mucosal wound healing in humans. To verify the developments in this field and the level of available scientific evidence, we applied a broad search strategy with no restrictions on wound type, target population, probiotic strain, or intervention protocol used. This review included seven studies involving 348 individuals. Four studies reported positive outcomes for healing improvement after probiotic therapy, and none of the studies reported adverse effects or a marked increase in wound healing time. The positive or neutral results observed do not generate strong evidence regarding the effectiveness of probiotics for wound healing. However, they suggest a promising field for future clinical research where the probiotic strains used, type of wounds, and target population are controlled for

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