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遗传性血管性水肿:最新技术综述,VII:2007年加拿大匈牙利遗传性血管性水肿诊断、治疗和管理国际共识算法

Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.

作者信息

Bowen Tom, Cicardi Marco, Bork Konrad, Zuraw Bruce, Frank Mike, Ritchie Bruce, Farkas Henriette, Varga Lilian, Zingale Lorenza C, Binkley Karen, Wagner Eric, Adomaitis Peggy, Brosz Kristylea, Burnham Jeanne, Warrington Richard, Kalicinsky Chrystyna, Mace Sean, McCusker Christine, Schellenberg Robert, Celeste Lucia, Hebert Jacques, Valentine Karen, Poon Man-Chiu, Serushago Bazir, Neurath Doris, Yang William, Lacuesta Gina, Issekutz Andrew, Hamed Azza, Kamra Palinder, Dean John, Kanani Amin, Stark Donald, Rivard Georges-Etienne, Leith Eric, Tsai Ellie, Waserman Susan, Keith Paul K, Page David, Marchesin Silvia, Longhurst Hilary J, Kreuz Wolfhart, Rusicke Eva, Martinez-Saguer Inmaculada, Aygören-Pürsün Emel, Harmat George, Füst George, Li Henry, Bouillet Laurence, Caballero Teresa, Moldovan Dumitru, Späth Peter J, Smith-Foltz Sara, Nagy Istvan, Nielsen Erik W, Bucher Christoph, Nordenfelt Patrik, Xiang Zhi Yu

机构信息

Department of Medicine and Paediatrics, University of Calgary, Alberta, Canada.

出版信息

Ann Allergy Asthma Immunol. 2008 Jan;100(1 Suppl 2):S30-40. doi: 10.1016/s1081-1206(10)60584-4.

DOI:10.1016/s1081-1206(10)60584-4
PMID:18220150
Abstract

BACKGROUND

We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) in 2004.

OBJECTIVE

To ensure that this consensus remains current.

METHODS

In collaboration with the Canadian Network of Rare Blood Disorder Organizations, we held the second Canadian Consensus discussion with our international colleagues in Toronto, Ontario, on February 3, 2006, and reviewed its content at the Fifth C1 Inhibitor Deficiency Workshop in Budapest on June 2, 2007. Papers were presented by international investigators, and this consensus algorithm approach resulted.

RESULTS

This consensus algorithm outlines the approach recommended for the diagnosis, therapy, and management of HAE, which was agreed on by the authors of this report. This document is only a consensus algorithm approach and continues to require validation. As such, participants agreed to make this a living 2007 algorithm, a work in progress, and to review its content at future international HAE meetings.

CONCLUSIONS

There is a paucity of double-blind, placebo-controlled trials on the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Controlled trials currently under way will provide further insight into the management of HAE. As with our Canadian 2003 Consensus, this 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of HAE was formed through the meeting and agreement of patient care professionals along with patient group representatives and individual patients.

摘要

背景

我们于2004年发表了《加拿大2003年遗传性血管性水肿(HAE;C1抑制剂[C1-INH]缺乏症)诊断、治疗及管理国际共识算法》。

目的

确保该共识与时俱进。

方法

2006年2月3日,我们与加拿大罕见血液疾病组织网络合作,在安大略省多伦多市与国际同行举行了第二次加拿大共识讨论会,并于2007年6月2日在布达佩斯举行的第五届C1抑制剂缺乏症研讨会上对其内容进行了审议。国际研究人员提交了论文,并由此产生了这种共识算法方法。

结果

该共识算法概述了推荐用于HAE诊断、治疗及管理的方法,本报告的作者对此达成了一致。本文件仅是一种共识算法方法,仍需验证。因此,与会者一致同意将其作为2007年现行算法,一项正在进行的工作,并在未来的国际HAE会议上审议其内容。

结论

关于HAE治疗的双盲、安慰剂对照试验较少,支持该算法的证据水平不尽人意。目前正在进行的对照试验将为HAE的管理提供进一步的见解。与我们的加拿大2003年共识一样,这份2007年HAE诊断、治疗及管理国际共识算法是通过患者护理专业人员以及患者群体代表和个体患者的会议与共识形成的。

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