C1 inhibitor deficiency: 2014 United Kingdom consensus document

被引：28

作者：

Longhurst, H. J. ^{[1
]}

Tarzi, M. D. ^{[2
]}

Ashworth, F. ^{[3
]}

Bethune, C. ^{[4
]}

Cale, C. ^{[5
]}

Dempster, J. ^{[1
]}

Gompels, M. ^{[6
]}

Jolles, S. ^{[7
]}

Seneviratne, S. ^{[8
]}

Symons, C. ^{[4
]}

Price, A. ^{[9
]}

Edgar, D. ^{[10
]}

机构：

[1] Barts Hlth NHS Trust & Med Adviser HAE, Dept Immunol, London, England

[2] Brighton & Sussex Med Sch, Dept Med, Brighton, E Sussex, England

[3] Sheffield Teaching Hosp NHS Trust, Dept Immunol, Sheffield, S Yorkshire, England

[4] Plymouth Hosp NHS Trust, Dept Immunol, Plymouth, Devon, England

[5] Great Ormond St Hosp Sick Children, Dept Immunol, London WC1N 3JH, England

[6] North Bristol NHS Trust, Dept Immunol, Bristol, Avon, England

[7] Univ Wales Hosp, Dept Immunol, Cardiff CF4 4XW, S Glam, Wales

[8] Royal Free London NHS Trust, Dept Immunol, London, England

[9] HAE UK, Herditary Angioedema, Bristol, Avon, England

[10] UK Primary Immunodeficiency Network UK PIN, Newcastle Upon Tyne, Tyne & Wear, England

来源：

CLINICAL AND EXPERIMENTAL IMMUNOLOGY | 2015年 / 180卷 / 03期

关键词：

C1 inhibitor deficiency; guidelines; HAE; hereditary angioedema; HEREDITARY ANGIOEDEMA ATTACKS; ACQUIRED C1-INHIBITOR DEFICIENCY; QUALITY-OF-LIFE; INTERNATIONAL CONSENSUS; ECALLANTIDE TREATMENT; RECEPTOR ANTAGONIST; DOUBLE-BLIND; MANAGEMENT; ICATIBANT; THERAPY;

D O I：

10.1111/cei.12584

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organization.

引用

页码：475 / 483

页数：9

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