Nutritional support for children with epidermolysis bullosa

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Abstract:

The term epidermolysis bullosa (EB) describes a number of rare, genetically determined blistering disorders characterized by excessive fragility of the skin and mucous membranes. Complications in the mouth, oesophagus and anus, combined with chronic skin infections and blood loss can severely compromise nutritional intake, leading to prolonged malnutrition and extreme growth failure. However, with early nutritional assessment and intervention, it is possible to achieve substantial improvements in nutritional status and growth, benefiting a variety of aspects of quality of life and reducing morbidity and mortality. In severe cases, attempts to enhance oral intake alone fail to promote satisfactory growth, and placement of a button gastrostomy device is the treatment of choice. In a multisystem condition such as EB, the success of dietary manipulations invariably depends on satisfactory management of a number of related factors-notably chronic constipation, gastro-oesophageal reflux and skin care. To promote appropriate strategies of intervention, it is highly advantageous for the dietitian to understand the aetiology and prognosis of the different types of EB, and to operate as an integral and pro-active member of the large multidisciplinary team required to manage these children.

Keywords: children; constipation; epidermolysis bullosa; growth; nutrition

Document Type: Research Article

Affiliations: Room 125, Institute of Child Health, 30 Guilford Street, London WC1N 1EH,UK.

Publication date: April 1, 1998

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