Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH

被引：14

作者：

Minford, Adrian ^{[1
]}

Brandao, Leonardo R. ^{[2
,3
]}

Othman, Maha ^{[4
,5
]}

Male, Christoph ^{[6
]}

Abdul-Kadir, Rezan ^{[7
,8
,9
]}

Monagle, Paul ^{[10
,11
]}

Mumford, Andrew D. ^{[12
]}

Adcock, Dorothy ^{[13
]}

Dahlback, Bjorn ^{[14
]}

Miljic, Predrag ^{[15
]}

DeSancho, Maria T. ^{[16
]}

Teruya, Jun ^{[17
,18
,19
,20
]}

机构：

[1] Bradford Royal Infirm, Dept Paediat, Bradford, W Yorkshire, England

[2] Univ Toronto, Hosp Sick Children, Toronto, ON, Canada

[3] Univ Toronto, Dalla Lana Sch Publ Hlth, Toronto, ON, Canada

[4] Queens Univ, Sch Med, Kingston, ON, Canada

[5] St Lawrence Coll, Sch Baccalaureate Nursing, Kingston, ON, Canada

[6] Med Univ Vienna, Dept Paediat, Vienna, Austria

[7] Royal Free NHS Fdn Hosp, Katharine Dormandy Haemophilia & Thrombosis Ctr, London, England

[8] Royal Free NHS Fdn Hosp, Dept Obstet & Gynaecol, London, England

[9] UCL, Inst Womens Hlth, London, England

[10] Royal Childrens Hosp Melbourne, Kids Canc Ctr, Dept Haematol, Murdoch Childrens Res Inst,Sydney Childrens Hosp, Melbourne, Vic, Australia

[11] Univ Melbourne, Melbourne, Vic, Australia

[12] Univ Bristol, Sch Cellular & Mol Med, Bristol, Avon, England

[13] Lab Corp Amer, Burlington, NC USA

[14] Lund Univ, Dept Translat Med, Malmo, Sweden

[15] Univ Belgrade, Fac Med, Clin Ctr Serbia, Clin Hematol, Belgrade, Serbia

[16] Weill Cornell Med, New York Presbyterian Hosp, Dept Med, Div Hematol Oncol, New York, NY USA

[17] Texas Childrens Hosp, Dept Pathol & Immunol, Houston, TX 77030 USA

[18] Texas Childrens Hosp, Dept Pediat, Houston, TX 77030 USA

[19] Texas Childrens Hosp, Dept Med, Houston, TX 77030 USA

[20] Baylor Coll Med, Houston, TX 77030 USA

来源：

JOURNAL OF THROMBOSIS AND HAEMOSTASIS | 2022年 / 20卷 / 07期

关键词：

children; disseminated intravascular coagulation; neonates; purpura fulminans; thrombosis; REPLACEMENT THERAPY; CONCENTRATE; INHIBITORS; THROMBOSIS; MUTATION; BLOOD;

D O I：

10.1111/jth.15732

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women's Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard.

引用

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页码：1735 / 1743

页数：9

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