Document Type : Original article

Authors

• Pegah Rahbarinejad ^{1, 2}
• Fatemeh Sadat Hashemi Javaheri ^{1, 2}
• Mostafa Shahraki Jazinaki ^{1, 2}
• Hamid Reza Kianifar ^{3, 4}
• Saeedeh Talebi ^{3, 4}

¹ Department of Nutrition, school of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

² Student Research Committee, Mashhad University of Medical Sciences, Mashhad, Iran

³ Department of Pediatric, Faculty of Medicine, Mashhad University of Medical sciences, Mashhad, Iran

⁴ Department of Nutrition, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Abstract

Introduction: Epidermolysis bullosa (EB) represents a diverse set of disorders that affect the skin and mucous membranes. Ensuring proper nutrition for children and adolescents with Epidermolysis Bullosa is a vital aspect of their treatment plan. The objective of this study is to demonstrate how nutritional intervention in a specialized nutrition clinic can enhance their well-being.
Methods and material: This longitudinal study was conducted over a 3-year period at Akbar Children Hospital, a tertiary facility affiliated with Mashhad University of Medical Sciences in Iran. The study included all patients diagnosed with EB based on clinical symptoms and genetic studies.
Results: In the present study, the median (25-75 IQR) age of the participants was 81.0 (36.0-156.0) months, and 19% of the participants were girls. The median (25-75 IQR) weight was 17.5 (10.8-24.5) kg, and the mean ± SD of height was 109.9 ± 31.1 cm. Among all types of malnutrition, there was only a significant association between gastrointestinal complications and BMI-for-age z-score (OR: 0.08, P-value=0.039) in the crude model. After adjustment, there was no significant association between gastrointestinal complications and malnutrition. The mean values of weight at the baseline, the first, and the second appointment of the study were 21.3, 21.2, and 24.8 kg, respectively. Moreover, the mean height at the baseline, the first, and the second appointment of the study were 109.4, 121.0, and 123.4 cm, respectively.
Conclusion: Regular clinic visits and tailored nutritional interventions positively impact EB patients, emphasizing the importance of managing anemia and deficiencies for their well-being.

Keywords

• Keywords: Epidermolysis bullosa (EB)
• Nutritional Support
• Children
• Adolescents