RESUMO
Epidermolysis bullosa (EB) is a genetic disorder with con-tinuous formation of blisters and erosions in the skin and mu-cous membranes as well as multi-systemic involvement.Patients are at high-risk of malnutrition due to decreased foodintake and increased nutrient demand. This cross-sectionalretrospective study evaluated the daily caloric intake and nu-tritional status of pediatric patients with EB at a specializedclinic through anthropometric measurements and estimationof the daily intake by 24-hour dietary recall. We used the Waterlow and World Health Organization (WHO) malnutritionclassification schemes. Descriptive statistics were used. Weincluded 17 patients with a mean age of 8.4 years (SD 4.6),82.3% had malnutrition. Those with more severe subtypes,junctional and recessive dystrophic EB, had acute superim-posed on chronic malnutrition (100% and 63.4% respec-tively), wasting (100% and 72.6%), and stunting (0% and54.4%) more frequently. Most patients required supplemen-tation (caloric 76.4% and vitamin/mineral 100%). We concluded that there is a high frequency of malnutritionin our EB patients. Although their energy requirements is cal-culated to be increased in 100-150% of the estimate, our pa-tients only reach 73.1% of that, thus requiring supplementa-tion. Patients with more severe subtypes of EB had chronicmalnutrition more frequently. Even though malnutrition isclosely linked to wound healing and adequate growth and de-velopment of patients, there are few studies about nutritionin EB worldwide. We believe evaluating the nutritional status of these patients is the first step to identifying deficiencies, of-fering adequate comprehensive medical care and establishingnutritional interventions in a timely manner.(AU)
