A nutrition-based approach to epidermolysis bullosa: Causes, assessments, requirements and management.

Simona Salera, Gianluca Tadini, Donata Rossetti, Francesca Sofia Grassi, Paola Marchisio, Carlo Agostoni, Claudia Giavoli, Giulia Rodari, Sophie Guez

Journal: Clinical nutrition (Edinburgh, Scotland) 2021;39(2):343-352

PMID: 30857908

Full Text on Elsevier Science View on PubMed

Abstract

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.

Address: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Pediatria Alta Intensità di Cura, 20122, Milano, Italy. Electronic address: [email protected].; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Pediatria Alta Intensità di Cura, 20122, Milano, Italy.; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Pediatria Alta Intensità di Cura, 20122, Milano, Italy; Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università di Milano, 20122, Milano, Italy.; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Pediatria Media Intensità di Cura, 20122, Milano, Italy; Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, 20122, Milano, Italy.; Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, 20122, Milano, Italy; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Endocrinologia, 20122, Milano, Italy.

DOI: 10.1016/j.clnu.2019.02.023

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MeSH Terms: Epidermolysis Bullosa, Humans, Malnutrition, Nutritional Status, Nutritional Support