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A Belgian consensus strategy to identify familial hypercholesterolaemia in the coronary care unit and its subsequent cascade screening and treatment: BEL-FaHST (The BELgium Familial Hypercholesterolaemia STrategy)
Atherosclerosis ( IF 4.9 ) Pub Date : 2018-10-01 , DOI: 10.1016/j.atherosclerosis.2018.05.037
Olivier S Descamps 1 , Olivier Van Caenegem 2 , Michel P Hermans 3 , Jean-Luc Balligand 4 , Christophe Beauloye 2 , Antoine Bondue 5 , Stéphane Carlier 6 , Emilie Castermans 7 , Fabien Chenot 8 , Marc Claeys 9 , Christophe De Block 10 , Anne de Leener 11 , Antoine De Meester 12 , Fabian Demeure 13 , Herbert De Raedt 14 , Walter Desmet 15 , Ivan Elegeert 16 , Michel Guillaume 17 , Etienne Hoffer 18 , Raymond Kacenelenbogen 19 , Patrizio Lancellotti 20 , Michel Langlois 21 , Attilio Leone 22 , Ann Mertens 23 , Nicolas Paquot 24 , Olivier Vanakker 25 , Jean-Louis Vanoverschelde 2 , Ann Verhaegen 26 , Pieter Vermeersch 27 , Caroline Wallemacq 28 , Ernst Rietzschel 29 ,
Affiliation  

BACKGROUND AND AIMS Familial hypercholesterolaemia (FH) is an autosomal dominant lipoprotein disorder characterized by significant elevation of low-density lipoprotein cholesterol (LDL-C) and markedly increased risk of premature cardiovascular disease (CVD). Because of the very high coronary artery disease risk associated with this condition, the prevalence of FH among patients admitted for CVD outmatches many times the prevalence in the general population. Awareness of this disease is crucial for recognizing FH in the aftermath of a hospitalization of a patient with CVD, and also represents a unique opportunity to identify relatives of the index patient, who are unaware they have FH. This article aims to describe a feasible strategy to facilitate the detection and management of FH among patients hospitalized for CVD. METHODS A multidisciplinary national panel of lipidologists, cardiologists, endocrinologists and cardio-geneticists developed a three-step diagnostic algorithm, each step including three key aspects of diagnosis, treatment and family care. RESULTS A sequence of tasks was generated, starting with the process of suspecting FH amongst affected patients admitted for CVD, treating them to LDL-C target, finally culminating in extensive cascade-screening for FH in their family. Conceptually, the pathway is broken down into 3 phases to provide the treating physicians with a time-efficient chain of priorities. CONCLUSIONS We emphasize the need for optimal collaboration between the various actors, starting with a "vigilant doctor" who actively develops the capability or framework to recognize potential FH patients, continuing with an "FH specialist", and finally involving the patient himself as "FH ambassador" to approach his/her family and facilitate cascade screening and subsequent treatment of relatives.

中文翻译:

在冠状动脉监护病房中识别家族性高胆固醇血症及其后续级联筛查和治疗的比利时共识策略:BEL-FaHST(比利时家族性高胆固醇血症策略)

背景和目的 家族性高胆固醇血症 (FH) 是一种常染色体显性脂蛋白疾病,其特征是低密度脂蛋白胆固醇 (LDL-C) 显着升高和早发性心血管疾病 (CVD) 风险显着增加。由于与这种情况相关的冠状动脉疾病风险非常高,因此因 CVD 入院的患者中 FH 的患病率比一般人群的患病率高出许多倍。对这种疾病的认识对于在 CVD 患者住院后识别 FH 至关重要,并且也代表了识别不知道自己患有 FH 的指示患者亲属的独特机会。本文旨在描述一种可行的策略,以促进因 CVD 住院的患者中 FH 的检测和管理。方法 一个由脂质学家、心脏病学家、内分泌学家和心脏遗传学家组成的多学科国家小组开发了一种三步诊断算法,每一步都包括诊断、治疗和家庭护理的三个关键方面。结果 产生了一系列任务,从在因 CVD 入院的受影响患者中怀疑 FH 的过程开始,将他们治疗到 LDL-C 目标,最终在其家庭中对 FH 进行广泛的级联筛查。从概念上讲,该途径分为 3 个阶段,以便为治疗医生提供一个具有时间效率的优先级链。结论 我们强调各参与者之间最佳合作的必要性,首先是“警惕的医生”,他积极开发识别潜在 FH 患者的能力或框架,然后继续“
更新日期:2018-10-01
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