A nutrition-based approach to epidermolysis bullosa: Causes, assessments, requirements and management. Issue 2 (February 2020)

• Record Type: Journal Article
• Authors: Salera, Simona; Tadini, Gianluca; Rossetti, Donata; Grassi, Francesca Sofia; Marchisio, Paola; Agostoni, Carlo; Giavoli, Claudia; Rodari, Giulia; Guez, Sophie
• Is Part Of: Clinical nutrition. Volume 39:Issue 2(2020)
• Page Start: 343
• Page End: 352
• Publication Date: 2020-02
• Subjects: Epidermolysis bullosa -- Undernutrition -- Hypercatabolism -- Growth retardation -- Nutritional status -- Enteral nutrition; Critically ill -- Nutrition -- Periodicals; Diet therapy -- Periodicals; Parenteral feeding -- Periodicals; Enteral feeding -- Periodicals; Enteral Nutrition -- Periodicals; Parenteral Nutrition -- Periodicals; Metabolism -- Periodicals; Diétothérapie -- Périodiques; Alimentation parentérale -- Périodiques; Alimentation entérale -- Périodiques; Nutrition -- Périodiques; Diet therapy; Enteral feeding; Nutrition; Parenteral feeding; Electronic journals; Periodicals; Electronic journals; 615.854
• DOI: 10.1016/j.clnu.2019.02.023
• Languages: English
• ISSNs: 0261-5614

Abstract

Summary: Inherited epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of rare diseases characterized by skin and mucous membrane fragility. EB primarily involves the skin and, in specific subtypes, the mucous membrane, resulting in complications which can strongly affect nutritional status (e.g. gastrointestinal complications, hand deformities, pain). The aims of nutritional support mainly include improving nutritional status, alleviating the stress of oral feeding and minimizing nutritional deficiencies, thus consequently improving growth, pubertal development, bowel function, immune status and wound healing. The aim of this review is to discuss knowledge of different aspects of the disease related to nutrition and growth.