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先天性鱼鳞病的管理:护理指南:第二部分:2024年更新

Management of congenital ichthyoses: guidelines of care: Part two: 2024 update.

作者信息

Mazereeuw-Hautier Juliette, Paller Amy S, O'Toole Edel, Dreyfus Isabelle, Bodemer Christine, Akiyama Masashi, Diociaiuti Andrea, El Hachem Maya, Fischer Judith, Gonzalez-Sarmiento Rogelio, Gutiérrez-Cerrajero Carlos, Ott Hagen, Has Cristina, Jonca Nathalie, Granier Tournier Céline, Martinez Ana, Traupe Heiko, Maria Salavastru Carmen, Schmuth Matthias, Sprecher Eli, Giehl Kathrin, Aldwin Mandy, Anton Morales Ruth, Santos Saturnino, Morren Marie-Anne, Audouze Anne, Malhotra Raman, Veldman Karin, Narbutt Joanna, Süßmuth Kira, Gostynski Antoni, Hernandez-Martin Angela

机构信息

CHU Toulouse, Dermatology, Centre de Référence des Maladies Rares de la Peau, Toulouse, Occitanie, France.

出版信息

Br J Dermatol. 2025 Jun 20;193(1):28-43. doi: 10.1093/bjd/ljaf077.

Abstract

In 2019, a group of experts published the first European guidelines for the management of congenital ichthyoses after a multidisciplinary expert meeting held in 2016. An update of these guidelines and literature search was planned every 5 years, given the clinical, molecular and therapeutic advances, including the use of biologic therapies. We present here updated guidelines that have been developed by a reorganized multidisciplinary group of international experts after a systematic review of recent literature, discussions and consensus reached at an expert conference held in June 2023. The guidelines provide summarized evidence and expert-based recommendations that aim to guide clinicians in the management of these rare and often complex diseases. These guidelines consist of two sections. Part one is reported elsewhere. Here, Part two covers the management of complications (eye, ear-nose-throat, pruritus, pain, cutaneous infections, vaccinations, growth failure and nutritional deficiency, hair and nail anomalies, reaction to hot and cold climates, physical limitations, comorbidities) and the particularities of the neonatal period and Netherton syndrome.

摘要

2019年,一组专家在2016年召开的多学科专家会议后发布了首份欧洲先天性鱼鳞病管理指南。鉴于临床、分子和治疗方面的进展,包括生物疗法的应用,计划每5年对这些指南进行更新并开展文献检索。在此,我们展示的是由重组后的国际专家多学科小组制定的更新指南,该小组在对近期文献进行系统综述、于2023年6月召开的专家会议上进行讨论并达成共识之后完成了这些指南的制定。这些指南提供了总结性证据和基于专家意见的建议,旨在指导临床医生管理这些罕见且通常复杂的疾病。这些指南分为两个部分。第一部分已在其他地方报道。在此,第二部分涵盖并发症(眼睛、耳鼻喉、瘙痒、疼痛、皮肤感染、疫苗接种、生长发育迟缓与营养缺乏、毛发和指甲异常、对炎热和寒冷气候的反应、身体限制、合并症)以及新生儿期和Netherton综合征的特殊性。

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