Haynes Lesley
Great Ormond Street Hospital for Children NHS Trust, London.
Br J Nurs. 2006;15(20):1097-101. doi: 10.12968/bjon.2006.15.20.22292.
Epidermolysis bullosa (EB) comprises a rare group of genetically determined skin blistering disorders characterized by extreme fragility of the skin and mucous membranes, with recurrent blister formation. The cornerstones of management are control of infection, wound management, pain relief, promotion of optimal nutritional status and mobility, surgical intervention and provision of the best possible quality of life. There is currently no cure for EB and, throughout life, those with the more severe types are at risk of significant nutritional compromise which impacts negatively on health and overall quality of life. Nutritional support is an important facet of holistic care and the dietetic challenges can be considerable. This paper describes some of the issues involved in optimizing the nutritional status of children with this disorder.
大疱性表皮松解症（EB）是一组罕见的遗传性皮肤水疱性疾病，其特征是皮肤和黏膜极度脆弱，反复形成水疱。治疗的基石包括控制感染、伤口处理、缓解疼痛、促进最佳营养状况和活动能力、手术干预以及提供尽可能好的生活质量。目前尚无治愈EB的方法，在整个生命过程中，病情较重的患者有显著营养受损的风险，这会对健康和整体生活质量产生负面影响。营养支持是整体护理的一个重要方面，饮食方面面临的挑战可能相当大。本文描述了优化患有这种疾病儿童营养状况所涉及的一些问题。
