Year-end Sale % OFF 
Abstract
Epidermolysis bullosa (EB) is a group of conditions characterized by severe fragility of the skin that causes recurring blistering. The recessive dystrophic subtype of EB (RDEB) has a strong impact on the nutritional status. We evaluated the resting energy expenditure (REE) and presence of protein catabolism in patients with RDEB. REE was assessed in 10 subjects (7 females; age range 4–33 years) by indirect calorimetry and using a predictive equation. Nitrogen balance was calculated by protein intake and 24 h urinary urea excretion estimations. An assessment of body surface area (BSA) with infected and non-infected skin lesions was applied to the nitrogen balance burn equation that was adapted to EB. The REE values predicted by the equation were consistently lower than the ones measured, except for two subjects. All subjects recorded high protein and energy intake, with protein intake being higher than 4 g protein/kg/day for five subjects. Even so, protein catabolism was observed in six subjects, three of whom had infected wounds. This study raises the hypothesis that the clinical and nutritional risks of people with RDEB are associated with an increased REE and negative nitrogen balance, which reinforces the importance of nutritional support.
Highlights
Epidermolysis bullosa (EB) is a rare group of diseases characterized by skin fragility due to mutations in several structural proteins, which causes the repeated development of blisters [1,2,3].EB subtypes are classified into four major groups, including EB simplex (EBS), Junctional EB (JEB), Dystrophic EB (DEB) with dominant (DDEB) or recessive (RDEB) types, and Kindler syndrome (1).recessive dystrophic subtype of EB (RDEB) is one of the most severe types of EB that affect the nutritional status [4,5]
All subjects presented with malnutrition, which was characterized by a body mass index (BMI) below the recommended values
Serious nutritional compromise was observed as well as increased energy demand and protein catabolism, which were consistent with the severity of the disease in terms of body surface area (BSA) with skin lesions
Summary
Epidermolysis bullosa (EB) is a rare group of diseases characterized by skin fragility due to mutations in several structural proteins, which causes the repeated development of blisters [1,2,3]. Any damage to the skin removes the external protective barrier, which permits the dissipation of heat and water This increases the energy requirements for maintaining normal body temperature. EB, REE appears to the supply of dietary energy and protein must be high in order to reduce catabolism and promote increase proportionally to the body surface area with blister involvement [7], and the supply of dietary growthand [4,7,14,15]. No and RDEB [8], and, in another study [17], REE was measured by indirect calorimetry in an adult This This studystudy testedtested the hypothesis that people with with experience protein catabolism.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
