南非大疱性表皮松解症患者跨学科护理的共识声明：第1部分。
Consensus statements for the transdisciplinary care of patients with epidermolysis bullosa in South Africa: Part 1.
作者信息
Chateau Antoinette V, Hlela Carol, Dlova Ncoza, Isaacs Thuraya, Naicker Thirona, Nupen Tracey, Ambler Julia, Maruma Frans, Pillay Lushen, Mankahla Avumile, Moosa Fatima, Makhubele Jabulile, Lehloenya Rannakoe, Visser Willem I, Velazquez Caridad, Cameron-Mackintosh Sinead, Todd Gail, Blackbeard David, Aldous Colleen
机构信息
Department of Dermatology, Faculty of Health Sciences, Greys Hospital, Pietermaritzburg, South Africa.
Department of Dermatology, Faculty of Health Sciences, University of KwaZulu-Natal, Durban, South Africa.
出版信息
Health SA. 2025 Aug 6;30:2963. doi: 10.4102/hsag.v30i0.2963. eCollection 2025.
BACKGROUND
Epidermolysis bullosa (EB) is a rare, painful and blistering genodermatosis with no cure. Treatment aims to prevent new lesions and manage complications. Previously, there were no management guidelines or consensus recommendations for patients with EB in Africa.
AIM
The aim of this study is to produce a comprehensive, transdisciplinary and practical care guide that is contextually appropriate to the cultural setting and resource limitations in South Africa.
SETTING
Multicentre, multiprovincial study involving healthcare practitioners from five South African provinces - KwaZulu-Natal, Gauteng (Johannesburg, Pretoria), Western Cape (Cape Town, Stellenbosch), Free State (Bloemfontein) and the Eastern Cape (Umtata).
METHODS
Consensus recommendations for the care of patients with EB were developed by a transdisciplinary team of specialists in consultation with EB patients. The modified Delphi technique was used to reach a robust consensus with a threshold of 80% for each action point to ensure the validity and reliability of the recommendations.
RESULTS
In all, 16 consensus statements were developed, and the main themes included the clinical clues to the diagnosis, complications as per the subtype of EB, diagnostics in a resource-limited environment, management of EB, pruritus and pain, palliative care and genetic counselling.
CONCLUSION
A transdisciplinary approach is essential for the holistic care of patients and their families with EB in the context of their resource limitations and cultural diversity providing much-needed guidance for clinicians in South Africa and similar settings.
CONTRIBUTION
This is the first consensus recommendation of care for patients with EB in Africa.
背景
大疱性表皮松解症（EB）是一种罕见、疼痛且会出现水疱的遗传性皮肤病，无法治愈。治疗旨在预防新的皮损并处理并发症。此前，非洲地区没有针对EB患者的管理指南或共识性建议。
目的
本研究的目的是制定一份全面、跨学科且实用的护理指南，使其在文化背景和资源限制方面与南非的实际情况相适应。
地点
一项多中心、多省份研究，涉及来自南非五个省份的医疗从业者——夸祖鲁 - 纳塔尔省、豪登省（约翰内斯堡、比勒陀利亚）、西开普省（开普敦、斯泰伦博斯）、自由邦省（布隆方丹）和东开普省（乌姆塔塔）。
方法
一个跨学科专家团队与EB患者协商，制定了针对EB患者护理的共识性建议。采用改良德尔菲技术达成强有力的共识，每个行动点的阈值为80%，以确保建议的有效性和可靠性。
结果
共制定了16条共识声明，主要主题包括诊断的临床线索、根据EB亚型的并发症、资源有限环境下的诊断、EB的管理、瘙痒和疼痛、姑息治疗以及遗传咨询。
结论
在资源有限和文化多样的背景下，跨学科方法对于全面护理EB患者及其家庭至关重要，为南非及类似环境中的临床医生提供了急需的指导。
贡献
这是非洲地区首个关于EB患者护理的共识性建议。
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