Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa.
Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa.
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DOI：
10.1186/s13023-022-02448-w
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发表时间：
2023-02-23
影响因子：
3.7
通讯作者：
中科院分区：
文献类型：
--
作者：
贫血是严重大疱性表皮松解症 (EB) 的常见并发症。迄今为止，还没有概述治疗 EB 人群贫血的最佳临床实践的指南。本手稿的目的是提出 EB 贫血诊断和管理的第一个共识指南。由于缺乏高质量证据，采用了共识方法。向 EB 患者及其家人发送了一项初步调查，探讨患者与贫血相关的偏好、担忧和症状。第二项调查分发给 EB 专家，重点关注不同类型 EB 贫血的筛查、诊断、监测和管理。专家组对这些调查的信息进行了整理和使用，形成了 26 份共识声明。共识声明已通过 EB-Clinet 发送给护理 EB 患者的医疗保健提供者。获得 70% 以上批准（完全同意/同意）的声明被采纳。最终结果是一系列 6 项建议，其中包括 20 条声明，将有助于指导 EB 患者贫血的管理。对于中度至重度 EB 患者，所需的最低 Hb 水平为 100 g/L。治疗应个体化。所有 EB 患者应将饮食措施作为贫血管理的一部分，轻度贫血应使用口服铁补充剂；而铁剂输注则用于中度至重度贫血，如果血红蛋白水平 > 80–100 g/L (8–10 g/dL)并且有症状；如果成人 Hb ≤ 80 g/L (8 g/dL)，儿童 Hb ≤ 60 g/L (6 g/dL)，则应输血。在线版本包含可在 10.1186/s13023-022-02448-w 获取的补充材料。
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. 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. 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. The online version contains supplementary material available at 10.1186/s13023-022-02448-w.
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影响因子：
2.7
作者：
Fedeles, Flavia;Murphy, Michael;Grant-Kels, Jane M.
通讯作者：
Grant-Kels, Jane M.
影响因子：
--
作者：
Hwang SJE;Daniel BS;Fergie B;Davey J;Murrell DF
通讯作者：
Murrell DF
影响因子：
2.4
作者：
Haynes, Lesley
通讯作者：
Haynes, Lesley
DOI：
10.3390/ph11040097
发表时间：
2018-10-04
期刊：
Pharmaceuticals (Basel, Switzerland)
影响因子：
--
作者：
Gómez-Ramírez S;Brilli E;Tarantino G;Muñoz M
通讯作者：
Muñoz M
影响因子：
14.6
作者：
Brouwers, Melissa C.;Kho, Michelle E.;Zitzelsberger, Louise
通讯作者：
Zitzelsberger, Louise