Introduction: Youth with special health care needs (YSHCN) are particularly vulnerable during the transition from pediatric to adult healthcare. Although nutritional status is a potentially modifiable determinant of health, its relationship with transition readiness and health-related quality of life (HRQoL) during this period remains poorly understood. Objective: To investigate the association of nutritional status and socioeconomic factors with transition readiness and HRQoL in YSHCN undergoing healthcare transition. Methods: This cross-sectional study included 218 consecutive YSHCN followed at a tertiary university hospital in Brazil. Transition readiness was assessed using the Transition Readiness Assessment Questionnaire (TRAQ), and HRQoL was evaluated using the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0). Nutritional status was assessed using body mass index-for-age (zBMI/A) and height-for-age (zH/A) z-scores according to World Health Organization standards. Multivariable linear regression models were used to identify factors independently associated with TRAQ and PedsQL scores. Logistic regression analyses were performed to identify predictors of low transition readiness (TRAQ <3.5). Results: Nutritional status assessed by BMI-for-age (zBMI/A) was not associated with transition readiness or HRQoL. In multivariable analyses, female sex ( β = 0.18, p = 0.002) and older age ( β = 0.12, p = 0.001) were independently associated with higher TRAQ scores. Higher height-for-age (zH/A) ( β = 1.86, p = 0.031) and higher family income ( β = 2.87, p = 0.025) were independently associated with better HRQoL, whereas female sex was associated with lower HRQoL ( β = −7.25, p = 0.001). In logistic regression analyses, younger age (OR = 0.82, 95% CI 0.70–0.96, p = 0.023) and male sex (OR = 0.48, 95% CI 0.25–0.92, p = 0.023) were significant predictors of low transition readiness, while nutritional risk was not associated with the outcome. The logistic model demonstrated modest discrimination (AUC = 0.689). Conclusion: In YSHCN undergoing healthcare transition, BMI-for-age was not associated with transition readiness or HRQoL. In contrast, linear growth and family income were independently associated with better HRQoL, highlighting the importance of developmental and socioeconomic factors in patient-reported outcomes. These findings reinforce the multifactorial nature of transition readiness and HRQoL and support the incorporation of nutritional, developmental, and psychosocial assessment into multidisciplinary healthcare transition programs.
Epidermolysis Bullosa (EB) is a rare genetic disorder characterized by fragile skin that blisters and tears easily, leading to significant morbidity and mortality. Depending on the specific genetic mutations and the proteins involved, EB can be classified into several subtypes whose molecular complexity is compounded by the variability in mutation types (missense, nonsense, insertions, deletions), their locations within the genes, and the resultant effects on protein function. This systematic review aimed to identify and synthesize available evidence on wound healing interventions and the nutritional profile of children diagnosed with EB. A comprehensive search yielded 28 articles, including 21 clinical trials and seven observational studies, encompassing 994 patients with various EB subtypes. The majority of studies described subtypes such as Simplex EB (EBS), Junctional EB (JEB), Dystrophic EB (DEB), and EB Kindler. The primary interventions for wound healing included dressings with collagen, biocellulose, and various topical creams. Nutritional assessment was limited, with only six studies examining nutritional status, predominantly through anthropometry and dietary intake analysis. Subgroup analyses indicated higher malnutrition rates among patients with DEB compared to JEB. The review underscores the importance of addressing wound healing and nutritional challenges in EB management. Further research is needed to explore effective interventions and optimize care for this vulnerable population.