Orphanet journal of rare diseases · 2025
The health-related quality of life of Brazilians with epidermolysis bullosa.
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Togo Camille Cristine Gomes, Zidório Ana Paula Caio, Gonçalves Vivian Siqueira Santos, Dutra Eliane Said
Аннотация
BACKGROUND: Epidermolysis bullosa (EB) presents various complications that can affect an individual's health-related quality of life (HRQoL) and nutritional status. This cross-sectional study evaluates HRQoL and its association with the socio-demographic, nutritional, and clinical profiles of Brazilians with EB.
RESULTS: The sample consisted of 129 individuals (median age: 15 years), approximately 57% female, mostly composed of the EB simplex clinical type, followed by the dystrophic EB type (DEB). The mean score on the Children's Dermatology Life Quality Index questionnaire was 11.01 [standard deviation (SD) = 7.31], with a moderate impact on HRQoL. The median and interquartile range (IQR) of the score obtained in the Quality of Life Evaluation in Epidermolysis Bullosa- Brazilian Portuguese questionnaire was 13 (IQR: 8-19), which also indicated a moderate impact on HRQoL. In the subgroup aged ≥ 17 years, schooling positively influenced HRQoL, and decreased HRQoL was directly associated with underweight nutritional status. Family income promoted HRQoL in both subgroups.
CONCLUSIONS: HRQoL assessment showed that the DEB type was associated with the most severe effect. Additionally, the large impact, in < 17 years, was associated with family income, but did not present an association with nutritional status. While the severe impact on HRQoL, in ≥ 17 years, presented an association with nutritional status, family income, and education. Future research is important, to understand this population's needs, provide adequate care, and promote the best HRQoL.
RESULTS: The sample consisted of 129 individuals (median age: 15 years), approximately 57% female, mostly composed of the EB simplex clinical type, followed by the dystrophic EB type (DEB). The mean score on the Children's Dermatology Life Quality Index questionnaire was 11.01 [standard deviation (SD) = 7.31], with a moderate impact on HRQoL. The median and interquartile range (IQR) of the score obtained in the Quality of Life Evaluation in Epidermolysis Bullosa- Brazilian Portuguese questionnaire was 13 (IQR: 8-19), which also indicated a moderate impact on HRQoL. In the subgroup aged ≥ 17 years, schooling positively influenced HRQoL, and decreased HRQoL was directly associated with underweight nutritional status. Family income promoted HRQoL in both subgroups.
CONCLUSIONS: HRQoL assessment showed that the DEB type was associated with the most severe effect. Additionally, the large impact, in < 17 years, was associated with family income, but did not present an association with nutritional status. While the severe impact on HRQoL, in ≥ 17 years, presented an association with nutritional status, family income, and education. Future research is important, to understand this population's needs, provide adequate care, and promote the best HRQoL.