Health-related quality of life and clinical severity in people with epidermolysis bullosa - A proposal for assessing nutritional compromise by body mass index (Birmingham Epidermolysis Bullosa Severity Score)

Health-related quality of life and clinical severity in people with epidermolysis bullosa - A proposal for assessing nutritional compromise by body mass index (Birmingham Epidermolysis Bullosa Severity Score)

Introduction and objectives: epidermolysis bullosa (EB) is a rare genetic disease characterised by skin fragility with blisters and erosions on the skin and/or mucous membranes. People with EB often experience several extracutaneous manifestations, including clinical and health-related quality of li...

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Published in:Nutrición hospitalaria : organo oficial de la Sociedad Española de Nutrición Parenteral y Enteral Vol. 41; no. 2; pp. 400 - 408
Main Authors: Togo, Camille Cristine Gomes, Zidório, Ana Paula Caio, Sá, Natan Monsores de, Dutra, Eliane Said
Format: Journal Article
Language:English
Published: Spain 26-04-2024
Subjects:
Adolescent
Adult
Body Mass Index
Brazil
Child
Child, Preschool
Epidermolysis Bullosa - psychology
Female
Humans
Male
Nutritional Status
Quality of Life
Severity of Illness Index
Young Adult
Brazil
Calidad de vida. Epidermólisis bullosa. Índice de masa corporal
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Summary:Introduction and objectives: epidermolysis bullosa (EB) is a rare genetic disease characterised by skin fragility with blisters and erosions on the skin and/or mucous membranes. People with EB often experience several extracutaneous manifestations, including clinical and health-related quality of life (HRQoL) complications. Herein, we evaluate their HRQoL and clinical severity and propose an objective criterion for estimating nutritional compromise using the Birmingham Epidermolysis Bullosa Severity Score (BEBS) tool. Methods: this series of cases included people with EB, monitored by a multi-professional team. Clinical severity was assessed with the BEBS, using body mass index ranges by age, as an objective proposal, to estimate the degree of nutritional compromise. To assess HRQoL, the Children's Dermatology Life Quality Index (individuals aged 4-16 years) and the Quality of Life Evaluation in Epidermolysis Bullosa - Brazilian Portuguese (individuals 17 years and over) were used. Results: of the nine individuals with recessive dystrophic EB (88.9 % female and 12.91 (SD = 11.71) years), the mean total BEBS score was 24.47 (SD = 12.80) points on a scale of 0 to 100 points. Six participants had significant nutritional compromise according to the proposed criteria. Five of the six participants evaluated for HRQoL reported experiencing some impact, with individuals aged 17 and over being more affected and with greater clinical severity. Conclusions: individuals with greater clinical severity of EB experience a more significant impact on their HRQoL. The proposed quantitative criteria for assessing nutritional compromise may help standardise assessments by professionals monitoring the nutritional status of individuals with EB. Keywords: Quality of life. Epidermolysis bullosa. Body mass index.
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ISSN:0212-1611
1699-5198
DOI:10.20960/nh.04827