Academic Journal
Gluten-sensitive enteropathy in recessive dystrophic epidermolysis bullosa
| العنوان: | Gluten-sensitive enteropathy in recessive dystrophic epidermolysis bullosa |
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| المؤلفون: | Sacedón, Rosa, de Arriba, M Carmen, Martínez-Santamaría, Lucía, Maseda, Rocío, Herráiz-Gil, Sara, Jiménez, Eva, Rosales, Isabel, Quintana, Lucía, Illera, Nuria, García, Marta, Butta, Nora, Fernández-Bello, Ihosvany, Lwin, Su M, Fernández-Arquero, Miguel, León, Carlos, McGrath, John A, Vicente, M Ángeles, del Río, Marcela, de Lucas, Raúl, Sánchez-Ramón, Silvia, Escámez, María José |
| المساهمون: | Institute of Health Carlos III, European Union, patient advocacy groups DEBRA-Spain and Berritxuak, Spanish Ministry of Science and Innovation and European Regional Development Fund, DEBRA Austria, DEBRA, EB-LOPPET, EB Research Network, UC3M-PhD research training scholarship |
| المصدر: | British Journal of Dermatology ; volume 189, issue 6, page 774-776 ; ISSN 0007-0963 1365-2133 |
| بيانات النشر: | Oxford University Press (OUP) |
| سنة النشر: | 2023 |
| الوصف: | Recessive dystrophic epidermolysis bullosa (RDEB) is a blistering genodermatosis due to biallelic loss-of-function variants in the type VII collagen (C7) gene (COL7A1). We report the impact of inflammation/autoimmunity on the gut (and other organs) in the nine children with RDEB recruited to an early-phase clinical trial of systemic cell therapy (NCT04153630). This pilot study provides evidence that autoimmunity may play an important role in sustaining chronic inflammation and the coexistence of coeliac disease, which, in turn, could exacerbate anaemia/malnutrition and progression in RDEB. Testing this hypothesis in a larger cohort including children and adults with RDEB and other epidermolysis bullosa (EB) subtypes is warranted so that targeted interventions may improve outcomes. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1093/bjd/ljad313 |
| DOI: | 10.1093/bjd/ljad313/51841774/ljad313.pdf |
| الاتاحة: | https://doi.org/10.1093/bjd/ljad313 https://academic.oup.com/bjd/advance-article-pdf/doi/10.1093/bjd/ljad313/51841774/ljad313.pdf https://academic.oup.com/bjd/article-pdf/189/6/774/53474839/ljad313.pdf |
| Rights: | https://academic.oup.com/pages/standard-publication-reuse-rights |
| رقم الانضمام: | edsbas.BE563CF7 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/bjd/ljad313 |
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