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Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa

BACKGROUND: Anemia is a common complication of severe forms of epidermolysis bullosa (EB). To date, there are no guidelines outlining best clinical practices to manage anemia in the EB population. The objective of this manuscript is to present the first consensus guidelines for the diagnosis and man...

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Detalles Bibliográficos
Autores principales: Liy-Wong, Carmen, Tarango, Cristina, Pope, Elena, Coates, Thomas, Bruckner, Anna L., Feinstein, James A., Schwieger-Briel, Agnes, Hubbard, Lynne D., Jane, Clapham, Torres-Pradilla, Mauricio, Zmazek, Matija, Lara-Corrales, Irene
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9948325/
https://www.ncbi.nlm.nih.gov/pubmed/36823529
http://dx.doi.org/10.1186/s13023-022-02448-w
Descripción
Sumario:BACKGROUND: Anemia is a common complication of severe forms of epidermolysis bullosa (EB). To date, there are no guidelines outlining best clinical practices to manage anemia in the EB population. The objective of this manuscript is to present the first consensus guidelines for the diagnosis and management of anemia in EB. RESULTS: Due to the lack of high-quality evidence, a consensus methodology was followed. An initial survey exploring patient preferences, concerns and symptoms related to anemia was sent to EB patients and their family members. A second survey was distributed to EB experts and focused on screening, diagnosis, monitoring and management of anemia in the different types of EB. Information from these surveys was collated and used by the panel to generate 26 consensus statements. Consensus statements were sent to healthcare providers that care for EB patients through EB-Clinet. Statements that received more than 70% approval (completely agree/agree) were adopted. CONCLUSIONS: The end result was a series of 6 recommendations which include 20 statements that will help guide management of anemia in EB patients. In patients with moderate to severe forms of EB, the minimum desirable level of Hb is 100 g/L. Treatment should be individualized. Dietary measures should be offered as part of management of anemia in all EB patients, oral iron supplementation should be used for mild anemia; while iron infusion is reserved for moderate to severe anemia, if Hb levels of > 80–100 g/L (8–10 g/dL) and symptomatic; and transfusion should be administered if Hb is < 80 g/L (8 g/dL) in adults and < 60 g/L (6 g/dL) in children. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-022-02448-w.