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Open AccessArticle

Resting Energy Expenditure and Protein Balance in People with Epidermolysis Bullosa

1
Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasilia, Campus Universitário Darcy Ribeiro; Brasília 70910-900, Brazil
2
Department of Dietetics, Birmingham Children’s Hospital, Birmingham B4 6NH, UK
*
Author to whom correspondence should be addressed.
Nutrients 2019, 11(6), 1257; https://doi.org/10.3390/nu11061257
Received: 23 April 2019 / Revised: 22 May 2019 / Accepted: 29 May 2019 / Published: 3 June 2019
(This article belongs to the Special Issue Nutrition Intake and Skin Health: Vitamin D and beyond)
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. View Full-Text
Keywords: epidermolysis bullosa; nutrition; energy expenditure; nitrogen balance; catabolism epidermolysis bullosa; nutrition; energy expenditure; nitrogen balance; catabolism
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MDPI and ACS Style

Zidorio, A.P.; Togo, C.; Jones, R.; Dutra, E.; de Carvalho, K. Resting Energy Expenditure and Protein Balance in People with Epidermolysis Bullosa. Nutrients 2019, 11, 1257. https://doi.org/10.3390/nu11061257

AMA Style

Zidorio AP, Togo C, Jones R, Dutra E, de Carvalho K. Resting Energy Expenditure and Protein Balance in People with Epidermolysis Bullosa. Nutrients. 2019; 11(6):1257. https://doi.org/10.3390/nu11061257

Chicago/Turabian Style

Zidorio, Ana P.; Togo, Camille; Jones, Rosie; Dutra, Eliane; de Carvalho, Kenia. 2019. "Resting Energy Expenditure and Protein Balance in People with Epidermolysis Bullosa" Nutrients 11, no. 6: 1257. https://doi.org/10.3390/nu11061257

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