{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T03:52:07Z","timestamp":1788580327193,"version":"build-2803163510"},"reference-count":39,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2019,6,3]],"date-time":"2019-06-03T00:00:00Z","timestamp":1559520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior - Brasil (CAPES)","award":["001"],"award-info":[{"award-number":["001"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>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\u201333 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.<\/jats:p>","DOI":"10.3390\/nu11061257","type":"journal-article","created":{"date-parts":[[2019,6,3]],"date-time":"2019-06-03T11:31:01Z","timestamp":1559561461000},"page":"1257","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Resting Energy Expenditure and Protein Balance in People with Epidermolysis Bullosa"],"prefix":"10.3390","volume":"11","author":[{"given":"Ana Paula","family":"Zidorio","sequence":"first","affiliation":[{"name":"Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasilia, Campus Universit\u00e1rio Darcy Ribeiro; Bras\u00edlia 70910-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille","family":"Togo","sequence":"additional","affiliation":[{"name":"Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasilia, Campus Universit\u00e1rio Darcy Ribeiro; Bras\u00edlia 70910-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosie","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Dietetics, Birmingham Children\u2019s Hospital, Birmingham B4 6NH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliane","family":"Dutra","sequence":"additional","affiliation":[{"name":"Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasilia, Campus Universit\u00e1rio Darcy Ribeiro; Bras\u00edlia 70910-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2799-1038","authenticated-orcid":false,"given":"Kenia","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasilia, Campus Universit\u00e1rio Darcy Ribeiro; Bras\u00edlia 70910-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,6,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.clindermatol.2011.03.012","article-title":"Inherited epidermolysis bullosa: New diagnostic criteria and classification","volume":"30","author":"Intong","year":"2012","journal-title":"Clin. Dermatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1097\/MPG.0b013e31812f5667","article-title":"Gastrointestinal complications of inherited epidermolysis bullosa: Cumulative experience of the National Epidermolysis Bullosa Registry","volume":"46","author":"Fine","year":"2008","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1016\/j.jaad.2014.01.903","article-title":"Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification","volume":"70","author":"Fine","year":"2014","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.det.2010.01.010","article-title":"Nutrition for Children with Epidermolysis Bullosa","volume":"28","author":"Haynes","year":"2010","journal-title":"Dermatol. Clin."},{"key":"ref_5","first-page":"265","article-title":"Nutritional outcomes in children with epidermolysis bullosa: Long-term follow-up","volume":"35","author":"Zidorio","year":"2018","journal-title":"Nutr. Hosp."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/j.jaad.2009.03.052","article-title":"Extracutaneous manifestations and complications of inherited epidermolysis bullosa: Part I. Epithelial associated tissues","volume":"61","author":"Fine","year":"2009","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1111\/j.1525-1470.1988.tb00879.x","article-title":"Nutritional and Metabolic Profile of Children with Epidermolysis Bullosa","volume":"5","author":"Honig","year":"1988","journal-title":"Pediatr. Dermatol."},{"key":"ref_8","unstructured":"Lechner-Gruskay, D. (1986). Nutritional Assessment of the Child with Junctional and Recessive Distrofic Epidermolysis Bullosa. [Master\u2019s Thesis, Drextel University]."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1001\/archderm.1988.01670050100031","article-title":"Nutritional problems of the acute and chronic burn patient. Relevance to epidermolysis bullosa","volume":"124","author":"Gamelli","year":"1988","journal-title":"Arch. Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"R90","DOI":"10.1186\/cc6102","article-title":"Burn size determines the inflammatory and hypermetabolic response","volume":"11","author":"Jeschke","year":"2007","journal-title":"Crit. Care"},{"key":"ref_11","first-page":"E576","article-title":"Total energy expenditure in burned children using the doubly labeled water technique","volume":"259","author":"Goran","year":"1990","journal-title":"Am. J. Physiol. Metab."},{"key":"ref_12","first-page":"16","article-title":"Comparison of estimated energy requirements in severely burned patients with measurements by using indirect calorimetry","volume":"18","author":"Tancheva","year":"2005","journal-title":"Ann. Burns Fire Disasters"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/0002-9343(84)90248-1","article-title":"Goals of nutritional support in acute infections","volume":"76","author":"Tayek","year":"1984","journal-title":"Am. J. Med."},{"key":"ref_14","unstructured":"Haynes, L. (2018, August 13). Clinical Practice Guidelines for Nutrition Support in Children with Epidermolysis Bullosa Including Tool to Help Identify Nutritional Compromise (THINC) in EB. Available online: http:\/\/www.debra.org.uk\/uploads\/resources\/EB_Guidelines_Final_for_web.pdf:."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1590\/abd1806-4841.20153206","article-title":"Nutritional aspects of children and adolescents with epidermolysis bullosa: Literature review","volume":"90","author":"Zidorio","year":"2015","journal-title":"An. Bras. Dermatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/S0002-8223(95)00157-3","article-title":"Nutrition management of patients with epidermolysis bullosa","volume":"95","author":"Birge","year":"1995","journal-title":"J. Am. Diet. Assoc."},{"key":"ref_17","first-page":"58","article-title":"Estado nutricional y gasto energ\u00e9tico en paciente con epidermi\u00f3lisis ampollosa hereditaria distr\u00f3fica","volume":"19","author":"Figueredo","year":"2004","journal-title":"Nutr. Hosp."},{"key":"ref_18","unstructured":"WHO Multicentre Growth Reference Study Group (2006). WHO Child Growth Standards: Length\/Height-for-Age, Weight-for-Age, Weight-for-Length, Weight-for-Height and Body Mass Index-for-Age: Methods and Developments, World Health Organization."},{"key":"ref_19","unstructured":"World Health Organization (1995). Physical status: The use and interpretation of anthropometry. Report of a WHO Expert Committee. WHO Technical Report Series, WHO. No. 854."},{"key":"ref_20","unstructured":"Minist\u00e9rio da Sa\u00fade (2018, August 19). Secretaria de Aten\u00e7\u00e3o \u00e0 Sa\u00fade. Departamento de Aten\u00e7\u00e3o B\u00e1sica. Orienta\u00e7\u00f5es Para A Coleta e An\u00e1lise de Dados Antropom\u00e9tricos Em Servi\u00e7os de Sa\u00fade: Norma T\u00e9cnica do SISTEMA de Vigil\u00e2ncia Alimentar e Nutricional\u2014Sisvan. Bras\u00edlia. Available online: http:\/\/200.17.213.49\/lib\/exe\/fetch.php\/projetos:obesidade:sisvannormatecnica.pdf."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1016\/j.clnu.2016.06.010","article-title":"Indirect calorimetry in nutritional therapy. A position paper by the ICALIC study group","volume":"36","author":"Oshima","year":"2017","journal-title":"Clin. Nutr."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1177\/0115426507022004377","article-title":"Indirect calorimetry: A practical guide for clinicians","volume":"22","author":"Haugen","year":"2007","journal-title":"Nutr. Clin. Pract."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.burns.2005.10.023","article-title":"Resting energy expenditure in severely burned children: Analysis of agreement between indirect calorimetry and prediction equations using the Bland\u2013Altman method","volume":"32","author":"Suman","year":"2006","journal-title":"Burns"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1079\/PHN2005801","article-title":"Basal metabolic rate studies in humans: Measurement and development of new equations","volume":"8","author":"Henry","year":"2005","journal-title":"Public Health Nutr."},{"key":"ref_25","first-page":"5","article-title":"Predicting basal metabolic rate, new standards and review of previous work","volume":"39","author":"Schofield","year":"1985","journal-title":"Hum. Nutr. Clin. Nutr."},{"key":"ref_26","unstructured":"Costa, T.H.M. (2018, August 31). CalcNut: Plataforma Para C\u00e1lculo de Dieta. Departamento de Nutri\u00e7\u00e3o, Faculdade de Ci\u00eancias da Sa\u00fade, Universidade de Bras\u00edlia. Available online: https:\/\/fs.unb.br\/nutricao\/calcnut\/?page_id=47."},{"key":"ref_27","unstructured":"Universidade Estadual de Campinas\u2014UNICAMP (2011). Tabela Brasileira de Composi\u00e7\u00e3o de Alimentos\u2014TACO. 4. ed. rev. e ampl, UNICAMP\/NEPA. Available online: http:\/\/www.unicamp.br\/nepa\/taco\/tabela.php?ativo=tabela."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1136\/jcp.21.2.218","article-title":"Assessment of automated nitrogen analysis of biological fluids with reference to the Kjeldahl method","volume":"21","author":"Jacobs","year":"1968","journal-title":"J. Clin. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/S0140-6736(86)90837-8","article-title":"Statistical methods for assessing agreement between two methods of clinical measurement","volume":"327","author":"Bland","year":"1986","journal-title":"Lancet"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1073\/pnas.4.12.370","article-title":"A biometric study of human basal metabolism","volume":"4","author":"Harris","year":"1918","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_31","unstructured":"Institute of Medicine, and National Research Council (2005). Dietary Reference Intakes for Energy, Carbohydrate, Fiber, Fat, Fatty Acids, Cholesterol, Protein and Amino Acids (Macronutrients), National Academy Press. Available online: http:\/\/www.nap.edu\/catalog\/10490."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1016\/j.clindermatol.2010.03.036","article-title":"Nutrition and bullous skin diseases","volume":"28","author":"Fedeles","year":"2010","journal-title":"Clin. Dermatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/S0738-081X(03)00050-6","article-title":"Management of epidermolysis bullosa in infants and children","volume":"21","author":"Bello","year":"2003","journal-title":"Clin. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1002\/jpen.1146","article-title":"Estimation of Resting Energy Expenditure Using Predictive Equations in Critically Ill Children: Results of a Systematic Review","volume":"42","author":"Moullet","year":"2018","journal-title":"J. Parenter. Enter. Nutr."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1177\/014860710302700121","article-title":"Clinical use of the respiratory quotient obtained from indirect calorimetry","volume":"27","author":"Lowen","year":"2003","journal-title":"J. Parenter. Enter. Nutr."},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"De Cosmi, V., Milani, G.P., Mazzocchi, A., D\u2019Oria, V., Silano, M., Calderini, E., Agostoni, C., and D\u2019Oria, V. (2017). The metabolic response to stress and infection in critically ill children: The opportunity of an individualized approach. Nutrients, 9.","DOI":"10.3390\/nu9091032"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1177\/001857870504001210","article-title":"Using nitrogen balance in clinical practice","volume":"40","author":"Dickerson","year":"2005","journal-title":"Hosp. Pharm."},{"key":"ref_38","unstructured":"World Health Organization (2002). Protein and amino acid requirements in human nutrition. WHO Technical Report Series, World Health Organization."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1111\/j.1365-2133.2011.10592.x","article-title":"Nutritional outcome in children with severe generalized recessive dystrophic epidermolysis bullosa: A short-and long-term evaluation of gastrostomy and enteral feeding","volume":"166","author":"Colomb","year":"2012","journal-title":"Br. J. Dermatol."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/11\/6\/1257\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T12:55:43Z","timestamp":1760187343000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/11\/6\/1257"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6,3]]},"references-count":39,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2019,6]]}},"alternative-id":["nu11061257"],"URL":"https:\/\/doi.org\/10.3390\/nu11061257","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,6,3]]}}}